0
Get Alert
Please Wait... Processing your request... Please Wait.
You must sign in to sign-up for alerts.

Please confirm that your email address is correct, so you can successfully receive this alert.

1
Special Article   |    
Neuropsychiatric Correlates and Treatment of Lenticulostriatal DiseasesA Review of the Literature and Overview of Research Opportunities in Huntington's, Wilson's, and Fahr's Diseases
A Report of the ANPA Committee on Research; Edward C. Lauterbach, M.D.;; Jeffrey L. Cummings, M.D.;; James Duffy, M.B., Ch.B.;; C. Edward Coffey, M.D.;; Daniel Kaufer, M.D.;; Mark Lovell, Ph.D.;; Paul Malloy, Ph.D.;; Alison Reeve, M.D.;; Donald R. Royall, M.D.;; Teresa A. Rummans, M.D.;; Stephen P. Salloway, M.D.
The Journal of Neuropsychiatry and Clinical Neurosciences 1998;10:249-266.
View Author and Article Information

Huntington's DiseaseWilson's DiseaseFahr's Disease

Accepted April 3, 1998. From the American Neuropsychiatric Association (ANPA) Committee on Research. Address correspondence to Dr. Lauterbach, Division of Adult and Geriatric Psychiatry, Department of Psychiatry and Behavioral Sciences, Mercer University School of Medicine, 1550 College Street, Macon, GA 31207.

Abstract

This report reviews clinical neuropsychiatric findings and opportunities for research in Huntington's, Wilson's, and Fahr's diseases. Consistent, systematic methodology is lacking among neuropsychiatric studies in these lenticulostriatal diseases. Systematic cross-sectional and longitudinal assessments are needed to ascertain the prevalence of psychiatric disorders as a function of disease course. Preliminary synthesis of existing data suggests the following heuristic relationships in these diseases: depression with parkinsonian states; personality changes with caudate or putamen disease; psychosis, impulsivity, and sexual disorders with caudate disease; dementia and mania with caudate and pallidal diseases; and compulsions with pallidal disease. Correlation of neuropsychiatric findings with disease stage, clinical signs, and radiologic, metabolic, physiologic, and pathologic markers of disease will add to our understanding of these conditions.

Abstract Teaser
Figures in this Article

The basal ganglia lenticulostriatal system is integral to processing cortical and limbic information. This system is composed of circuits communicating between the lenticular nucleus (putamen and globus pallidus) and the striatum (caudate nucleus and putamen). The lenticulostriatal system receives information from multiple limbic structures including the amygdala, cingulate gyrus, and nucleus accumbens.1 Projections to the thalamus and frontal lobe make the basal ganglia lenticulostriatal system of great importance in understanding behavior.2

The lenticulostriatal system is connected to the frontal cortex through five functionally segregated circuits implicated in motor, cognitive, and psychiatric manifestations of basal ganglia diseases.3,4 Three of these circuits—the dorsal prefrontal, orbitofrontal, and anterior cingulate—are implicated in primary psychiatric illnesses including depression, obsessive-compulsive disorder, and schizophrenia. Moreover, neurologic diseases involving these circuits carry an unusual proclivity for neuropsychiatric5 and behavioral6 disorders. Consequently, the basal ganglia lenticulostriatal system is one of the most important brain systems in neuropsychiatry.

This report reviews neuropsychiatric findings in major basal ganglia diseases with principal lenticulostriatal pathology. We focus on three representative diseases that primarily produce principal structural pathologic changes in the striatum and lenticular nucleus: Huntington's disease, Wilson's disease, and Fahr's disease. Our goal is to provide an overview of relevant neuropsychiatric findings of clinical interest in these illnesses and highlight directions for future research. Other basal ganglia diseases lacking principal pathology in these structures (Parkinson's disease), predominantly affecting other structures (dentatorubropallidoluysian atrophy), or with etiologies coincidentally affecting the lenticulostriatal complex (basal ganglia infarcts, carbon monoxide poisoning) are not considered here. Instead, we have limited our consideration to Huntington's disease (affecting principally the caudate), Wilson's disease (affecting principally the putamen), and Fahr's disease (affecting principally the globus pallidus).

Huntington's disease (HD) is a dominantly inherited CAG trinucleotide repeat disease affecting the IT15 gene at 4p16.3.7,8 HD prevalence approximates 4.1—7.5 per 100,000 but varies in different parts of the world.9 An expanded IT15 CAG repeat (37 or more repeats) is 100% specific and 98.8% sensitive for HD.10,11 Longer repeat length is correlated with earlier ages of onset and death.10,12 Age at onset is variable, but HD typically presents between ages 35 and 50.13 Genetic anticipation is associated with paternal transmission of the gene. Average life expectancy after HD onset is 15 years, although some patients survive up to 40 years.13

Expanded CAG sequences are translated into expanded polyglutamine repeats in the protein huntingtin, the gene product.14 Although the function of huntingtin is not known, its effects may be amplified by this polyglutamine expansion,15 possibly through interactions with other proteins.1618 Pathogenic mechanisms in HD may involve glutamatergic excitotoxicity, free-radical generation, oxidative metabolism defects, and apoptosis.19,20 Caspase 3 (apopain), important in apoptosis, cleaves huntingtin in a CAG length—dependent manner.21

The pathology of HD is well known. It principally involves the loss of striatal medium spiny GABAergic neurons, especially in the caudate nucleus, although neuronal loss also can be observed in the globus pallidus, reticular portion of the substantia nigra, ventral anterior nucleus of the thalamus, cerebral cortex, cerebellar dentate nucleus, brainstem, and spinal cord.22 Striatal atrophy becomes apparent on computed tomography (CT) and magnetic resonance imaging (MRI) during HD progression.23 Reduced striatal D1 and D2 dopamine receptor binding occurs in HD and is more severe in rigid HD.24 Frontal white matter atrophy occurs later in the illness.25

Clinical features vary, but they generally include movement disorders (usually chorea, sometimes myoclonus, dystonia, or parkinsonism), dementia, and psychiatric disorders (principally mood disorders). Childhood-onset HD usually presents with rigidity rather than chorea, cerebellar signs, and rapid cognitive decline. As the disease progresses, patients become demented, mute, achoreic, and often parkinsonian. Whether CAG repeat length influences disease progression26,27 or neuropsychiatric presentation remains controversial.

Presymptomatic genetic testing for HD by means of a blood sample for determining an expanded CAG repeat sequence is available; there are attendant ethical considerations. Most subjects receiving test results seem to cope well over the short term when the findings are disclosed in the context of education and counseling,28,29 although significant exceptions have occurred.30 Clinical guidelines for presymptomatic testing have been developed,31 and the psychological consequences of this testing have been reviewed.32 Pre- and post-test counseling is an important element of presymptomatic testing.

+

Neurological Features

Involuntary movement disorders in HD can be followed by use of the Quantified Neurologic Examination33 and the Unified Huntington's Disease Rating Scale.34 Loss of lateral striatopallidal projections correlates with chorea, whereas loss of medial striatopallidal projections correlates with akinetic-rigid presentations.35 Medial pallidal γ-aminobutyric acid (GABA) concentrations increase as the chorea progresses.36 Disturbances of voluntary eye movements also occur (e.g., abnormal saccadic and pursuit eye movements). In contrast to the waning of chorea late in the illness, these disturbances progress throughout the illness13,37 and correlate with functional disability.38

+

Neuropsychiatric Features

HD is dominated by abnormal involuntary movements, dementia, and psychiatric disorders. Psychiatric features are the presenting manifestation of HD in 24% to 79% of cases and occur at some point during the illness in 35% to 79%,39 although these estimates were determined by using a wide variety of methods. Irritability, apathy, and mood disorders are the most common psychiatric disorders.9 Ranen has recently reviewed clinical aspects of psychiatric disorders in HD.40

+

Dementia:

Initial manifestations of dementia include impairments in cognitive speed, mental flexibility, concentration, executive function, new verbal learning, and the reverse serial sevens task of the Mini-Mental State Examination.4143 Memory retrieval is affected more than storage, and patients may respond to cueing.44,45 The dementia progresses to become more global, impairing visuospatial ability and judgment.46 Dementia has been related to caudate GABA and glutamate depletions.47 Attempts to relate other psychiatric manifestations to cognitive changes have usually shown little correlation.48 Aphasia and agnosia are absent in HD dementia.49

+

Personality Changes:

Personality changes may present prodromally and may occur to some extent in all patients as the disease progresses.48 Personality changes were found in 72% of 110 patients with HD in one recent prevalence survey.50 Apathy and aggression are greater in HD than in Alzheimer's disease.51 Aggression, irritability, and apathy did not correlate with each other in one study.51 Personality changes may relate to reduced ventrobasal striatal glucose metabolism and orbitofrontal dysregulation.4,52

Apathy occurs in nearly half of patients,51 and in a small sample of patients it became more prominent over the course of HD.53

Among the more troubling conditions are irritability and disinhibited aggression. Irritability occurs in about half of patients,51,54 is severe in about one-third,9 may be directed against specific individuals, and is associated with premorbid irritable traits.51 Subjects at risk for HD have higher ratings of anger and hostility than control subjects on measures of present mood.55

Elevated aggression scale scores were found in 59% of patients with HD.51 Although aggression most commonly occurs early in the course of HD,56 one-third of patients with advanced HD in nursing homes exhibited aggression that correlated with the severity of their functional impairment.57 Violent behavior in patients with HD may at times assume the form of assault, arson, and homicide.58,59

+

Psychoses:

Symptomatic schizophrenia has been observed in up to 9% of patients with HD.9,50 Psychotic symptoms can occur in up to 25% of patients with HD and can include multiple forms of hallucinations and delusions.13,39,60 Rates of psychosis vary by setting and sample selection factors.60 Psychotic symptoms are more common in early-onset HD.9 Psychosis in HD has been correlated with medial caudate pathology61 and reduced anterior hemispheric metabolism.52

+

Mood Disorders:

Mood disorders occur in about 40% of patients with HD.9,50 Bipolar disorders occur in approximately 10%.9 Depression often precedes the onset of the movement disorder.9 The pattern of depressive symptoms in HD resembles idiopathic major depression, and psychotic features tend to be mood congruent.62 Depression did not correlate with apathy or irritability but did correlate with reduced orbitofrontal glucose metabolism in a fluorodeoxyglucose positron emission tomography (PET) study of HD.63 Depression may relate to dorsomedial caudate pathology.13,64

Suicide rates in HD are as high as 12.7%,65 which is more common than in stroke or Parkinson's disease with depression.48 Suicide risk is especially of concern in older patients with HD.65 In a retrospective case-controlled study, risk for suicide in patients with HD was chiefly determined by childlessness.66 Family history of suicide, single marital status, living alone, depression, and contact with others afflicted with HD contributed weakly to suicide risk.

+

Anxiety Disorders:

An early study reported that anxiety occurred in nearly 12% of 102 patients with HD and was the most common prodromal symptom.56 Anxiety occurs early in the course of HD and was associated with longer survival in one study.67 Although panic disorder, generalized anxiety, and obsessive-compulsive disorder (OCD) have been observed in patients with HD, the prevalences of these anxiety disorders have not yet been determined. Obsessions often involve cleanliness and checking as in primary OCD, and certain families with HD may carry a predisposition toward OCD.68 Mixed anxiety and depression has been associated with akinetic-rigid HD.56

+

Impulse Control and Sexual Disorders:

Intermittent explosive disorder was found in 31% of 186 patients with HD.13 Sexual disinhibition, hypersexuality, and paraphilias have been described,54,58,69 although most patients eventually develop sexual apathy or impotence.13 Hypersexuality occurred in 12% of men and 7% of women in one study.58 By DSM-III-R criteria, 82% of 39 patients with HD had sexual disorders, with the most frequent being hypoactive sexual disorder.70

+

Treatment

Several strategies aimed at reducing the underlying pathological effects of excitotoxic amino acids have been suggested.71 Blinded controlled trials of baclofen72 and idebenone73 did not evidence any slowing of HD progression. d-alpha-tocopherol 3,000 IU per day improved neurological symptoms early in the course of HD.74 Treatment with coenzyme Q10 reduced elevated cortical lactate concentrations in patients with HD, although clinical correlates were not studied.75 Ciliary neurotrophic factor demonstrated a protective effect against quinolinic acid—induced striatal degeneration in a monkey model of HD.76

Movement disorder treatment attempts have yielded mixed results. Optimal reduction of chorea with haloperidol was obtained at doses of 1.5—10 mg/day (2—5 ng/ml), whereas higher doses rarely produced additional benefit.77 Under double-blind placebo-controlled conditions, apomorphine actually improved chorea and motor impersistence,78 clozapine variably improved chorea but worsened functional capacity,79 and piracetam worsened chorea.80 Cannabidiol failed to alter chorea in a randomized double-blind placebo-controlled crossover study.81 Milacemide, a glycine prodrug, affected neither chorea nor cognition under double-blind placebo-controlled conditions.82

Neuropsychiatric treatment data are limited by a paucity of controlled trials.62 There are no specific treatments for the cognitive disorder of HD. Fluoxetine failed to improve cognition, functional capacity, or neurological status in a randomized double-blind placebo-controlled study in nondepressed patients with HD, although there was a slight improvement in agitation and in need for routine.83 Ketamine, an N-methyl-d-aspartate (NMDA) receptor antagonist, worsened memory and exacerbated psychiatric symptoms in a double-blind placebo-controlled study involving patients with HD.84 Sertraline was effective in 2 consecutive cases against irritability and violent behavior.85 Propranolol is reported to relieve aggression associated with frustration and impatience in HD.86 Anecdotal evidence also supports the utility of buspirone in aggression.87,88

Psychotic features may be either responsive or refractory to neuroleptic treatment.53 There is some evidence that clozapine may be particularly efficacious in psychosis89,90 and that delusions may be more responsive to neuroleptics than hallucinations,62 but more data are needed. Although mood stabilizers in HD require study, lithium rarely has been considered to be effective in treating bipolar disorders associated with HD.62

Major depression in HD is thought to be undertreated,91,92 presumably due to underrecognition. Tricyclic antidepressants and selective serotonin reuptake inhibitors (SSRIs) are regarded as first-line treatments for depression,53,62,93 and patients with HD seem to respond to antidepressant treatment in a manner similar to patients with primary depression, although appropriate studies are lacking.53 Monoamine oxidase inhibitors may be useful for patients not responding to first-line agents.94 Augmentation of fluoxetine with deprenyl led to beneficial mood, motor, and behavioral effects in 1 patient.95 Delusional depression predicted the most favorable response to ECT among 5 depressed patients with HD.96

There is some evidence that medroxyprogesterone and leuprolide may be useful in reducing sexual disinhibition in HD.97 Although there are several descriptions of family therapy in HD, controlled outcome data are lacking.98

+

Opportunities for Further HD Research

  • Effects of CAG repeat length. The effect of HD gene CAG repeat length on disease progression and neuropsychiatric symptoms requires clarification.

  • Heritability of neuropsychiatric phenomena. Definition of the heritability of neuropsychiatric phenomena (such as OCD) in kindreds with HD may distinguish those at risk and improve our understanding of these phenomena.

  • Prevalence of psychiatric disorders. Determination of the prevalence of anxiety disorders by use of standardized diagnostic criteria is needed. Replication of prevalences of psychiatric disorders across different clinical settings is needed to determine the generalizability of earlier studies, as well as the effects of ascertainment and sample biases in previous studies.

  • Correlates of psychiatric disorders over disease course. Elucidation of neuropathological correlates of psychiatric disorders and their evolutionary time courses in relation to HD progression would facilitate our understanding of the basal ganglia circuitry mediating these conditions.

  • Functional imaging correlates of psychiatric disorders. Additional correlation of functional imaging data with particular psychiatric sequelae would be useful, as would further characterization of the differences among subjects with HD and depression, HD without depression, and primary depression without HD.

  • Neuroprotective agents. Controlled treatment trials of the effects of neuroprotective agents against HD progression, employing larger sample sizes, are indicated. Such trials may allow detection of subtle protective effects not evident in previous studies.

  • Pharmacologic interventions. Controlled treatment trials of neuropharmacologic and psychopharmacologic interventions in HD are needed. This is especially true of the cognitive disorder, which currently lacks any specific treatment. Appropriate controls would include patients with progressive dementing illnesses, medically ill patients with depression or anxiety, and patients with primary psychiatric disorders.

  • Treatment-refractory conditions. Novel approaches to treating neuropsychiatric conditions refractory to conventional treatments are needed.

  • Psychotherapy and genetic testing. Issues relating to psychotherapy and the psychological effects of genetic testing require further detailed study.

  • Family therapy. The efficacy of family therapy interventions requires further assessment. Caregiver burden should be a particular focus of concern.

Wilson's disease (WD) is an inherited disorder of copper metabolism leading to abnormal copper deposition in the liver, brain, and other organs. Mutations of the gene ATP7B on chromosome 13q14 band 14.1—21.199 disrupt coding for a copper-containing P-type adenosine triphosphatase that alters apoceruloplasmin function, a copper-transporting protein. The absence of biliary ceruloplasmin in WD apparently prevents biliary excretion of copper.100 Subsequent copper accumulation with its pro-oxidant effects may lead to cellular demise, including mitochondrial DNA mutations.101

More than 40 mutations of the ATP7B gene are known.102 Some mutations are associated with ethnicity, WD severity, age at onset, or presentation.103 Mutations leading to severe gene dysfunction produce an aggressive disease course (early-onset WD with severe liver manifestations), whereas mutations leading to milder gene dysfunction produce a less aggressive disease course (late-onset neurological and subclinical liver manifestations).104

The worldwide incidence of WD approximates 12—30 per million,103,105 with higher prevalences in Japan linked to consanguinity.106 Mean age at onset ranges from 5 to 35 years of age (mean age 17), but onset and initial manifestations sometimes vary between families. Generally, a rule of thirds applies to the manifestations, with approximately one-third each presenting with predominantly hepatic, neurological, or psychiatric symptomatology. Although neurological and psychiatric features constitute the most common manifestations, WD can affect a wide range of organs throughout the body. An important extrahepatic manifestation is copper deposition at the corneal limbus, the Kayser-Fleischer ring (KF ring), which correlates with WD severity.107 Treatment-related reductions in KF rings correlate with improvements in MRI scan quantitative scores in WD.108 Treatment may prolong survival beyond 20 years. In the absence of treatment, patients may die within 5 years after developing WD manifestations, and patients with neurologic features may die within 6 months.

On MRI, increased signal on T2-weighted images due to copper deposition is usually present in the putamen or other structures.109,110 T1 signal is generally reduced, but signal can be increased in the globus pallidus in patients with portal-systemic encephalopathy due to liver necrosis in WD.111,112 Several recent studies have examined the distribution of MRI findings in WD.112114 The putamen and pons were most frequently affected (80%—90% of patients) in two studies.112,113 In one study, there was a predilection for abnormal signal in the anterior rim of the putamen and the ventral nuclear mass of the thalamus, although the sample size (N=25) was too small to indicate any correlation with clinical findings.113 The dorsal and central regions were the most commonly involved pontine areas.112 In another study, parkinsonian signs correlated with striatal and pontocerebellar abnormalities, whereas cerebellar signs correlated with dentatothalamic tract abnormalities.114 Cortical and subcortical abnormalities tended to occur predominantly within the frontal lobe.112 The predilection for copper deposition in particular brain regions is poorly understood. On CT, the most common findings are ventricular dilatation, basal ganglia hyperdensities, and atrophy of cerebral cortex, brainstem, or cerebellum.115 Basal ganglia MRI T2 hypointensity and CT hypodensity are consistent with cavitations found in more aggressive WD.

Reduced glucose metabolism in cerebellum, striatum, cerebral cortex, and thalamus may be seen on PET in WD.116 Reduced striatal glucose metabolism was correlated with neurological sign severity, and striatal glucose metabolism and D2 receptor binding improved with clinical improvement in response to chelation therapy.117 On MR spectroscopy, pallidal N-acetylaspartate was reduced in WD patients compared with control subjects, and WD patients with portosystemic shunting had lower pallidal myo-inositol than WD patients without shunting.118 In patients with neurologic WD, reduced D2 binding on single-photon emission computed tomography (SPECT) correlated with the overall severity of neurological deficits but not with specific neurological signs.119

Laboratory diagnosis of WD is made by identifying abnormal ceruloplasmin and high urinary copper levels and is confirmed by hepatic biopsy disclosing copper. Ceruloplasmin is reduced in 95% of WD cases, but it can also be normal or elevated. Serum copper levels are not of diagnostic value. Neuroimaging is supportive but not diagnostic, and the clinical neurological exam is more diagnostically sensitive than CT.120 Western blot analysis of ceruloplasmin can distinguish WD homozygosity, heterozygosity, and other non-WD hypoceruloplasmic conditions.100 Presymptomatic molecular genetic testing using DNA linkage analysis (restriction fragment length polymorphisms and polymerase chain reaction—based analysis) is useful in studying siblings.121

+

Neurological Features

A wide range of oculomotor, cerebellar, speech, movement, pyramidal, autonomic, and seizure disorders have been described in WD. Prevalences of some neurological signs have been established in small samples. Dysdiadochokinesia and dysarthria occur most commonly.122 Initial presenting neurological features include mild tremor, speech problems, and micrographia.123 Features at 10-year follow-up have also been determined.124 Dystonia and chorea seem to occur most commonly in childhood-onset and in endstage WD.103 Whereas 6% of patients with WD manifest seizures, more than 60% remit with WD treatment.125 Visual and sensory systems are curiously spared despite high copper concentrations in sensory cortex.103 The prevalence of KF rings in neurologic WD approximates 50%, in contrast to less than 10% in hepatic WD.122

+

Neuropsychiatric Features

Clinical suspicion is critical in diagnosing WD. The diagnosis was missed in two-thirds of neurologic WD initial presentations, producing a 13-month delay in diagnosis.126 Across studies, up to one-fifth of patients with WD present initially with psychiatric features in isolation, one-third present with predominating psychiatric features, and two-thirds of patients with WD eventually develop psychiatric features. The exact lifetime prevalence of psychiatric disorders is not known but is estimated to range between 30% and 100%.127129 Half of patients may undergo psychiatric hospitalization before WD is recognized. Psychiatric manifestations may precede neurological signs in the early stages of WD.127 Incongruous behavior, irritability, depression, and cognitive impairment were the most common psychiatric symptoms among 129 patients with WD.130 It is thought that psychiatric disturbances secondary to liver dysfunction are rare in isolated hepatic WD, but systematic study is needed. Selection factors, sample sizes, and diagnostic approaches vary across studies, and other variables are not universally taken into account. As an example of selection factors that can vary, one study involving 24 patients with neurological manifestations disclosed a high rate of personality changes (71%).123 Findings in patients lacking neurological manifestations may be different.

+

Cognitive Disorders:

Cognitive impairment is generally mild,131 occurring in less than 25% of patients.122,128,132 Cognitive impairment worsens with increasing disease duration133 and neurological manifestations.124 Some apparent cognitive deficits may be due to reduced motor speed rather than reduced information processing speed.134 Hence, exact rates of true cognitive impairment in neurological and other symptomatic presentations of WD remain unclear.

+

Personality Changes:

The life-prevalence of persistent personality change ranges between 46% and 71%,123,132 typically manifesting as irritability or aggression.122,127,129,132,135,136 Personality syndromes have been correlated with dyskinesia, dysarthria, and lesions of the putamen and pallidum.137 Sociopathic features correlate with dysarthria.135 Irritability and incongruous behavior are associated with brainstem signs and dystonia but not with tremor.128

+

Psychoses:

Schizophreniform disorders, catatonia, and hallucinations are no more common in WD than in the general populction,122,128 but psychosis and catatonia occurred somewhat more commonly (8% each) in neurological WD.123

+

Mood Disorders:

Essentially nothing is known about mania or its prevalence in WD. Major depression occurred in 27% in a prospective study of 45 subjects with WD.122 Mild depression has been correlated with cognitive impairment, parkinsonian rigidity, bradykinesia, and third-ventricle dilatation137 as well as unspecified gait disorders.135 Whether depression is a psychological reaction to WD or is due to biologic compromise is unresolved. Application of diagnostic criteria will be needed in future studies to distinguish adjustment disorders from mood disorders in WD. Suicidal behavior occurs in 4% to 16% of patients with WD across studies.122,127

+

Other Psychiatric Disorders:

As with mania, essentially nothing is known about anxiety disorders, substance abuse, or other psychiatric disorders in WD.

+

Treatment

Clinical improvement in WD with treatment is generally limited to the first 5 years of symptoms and the first 2 years of WD treatment.127,130 Early recognition and treatment are therefore essential. Treatments include cupriuretic copper chelators (penicillamine and trientine) and copper-depleting agents (zinc and thiomolybdate). Specific indications for these various agents remain controversial. There are some outcome data regarding these drugs, but these data usually refer to neurological outcome, whereas psychiatric outcome is more anecdotally described.

Of 137 patients with neurologic WD undergoing chelation, 42% became asymptomatic, 26% improved neurologically, 17% were disabled, and 15% died; of the entire sample, 22% suffered neurological exacerbations.138 Glucose metabolism and dopamine receptor binding on PET correlated with improved motor function after 1 year of treatment in a single patient with WD.139 Auditory and somatosensory evoked potentials have detected neurological improvement with treatment140142 but did not correlate well with psychiatric improvement.124 Psychiatric symptoms resolved more often in patients without dysarthria, incongruous behavior, and hepatic symptoms.130 Incongruous behavior and cognitive impairment improved more than irritability and depression during WD treatment.130 Memory, Performance IQ, Full Scale IQ, and psychosis have each responded to treatment.127,130,143145 Other manifestations resolving with WD treatment have included amnestic disorder, dementia, hypersexuality, aggression, hyperactivity, and disinhibition.

d-penicillamine treatment has been correlated with improvements in T2 abnormalities on MRI, dopamine receptor binding on SPECT146 and PET,139,146 and glucose metabolism.139 Neurological exacerbations can occur with penicillamine therapy and may lead to further neurological disability, seizures, movement disorders, and neuropsychiatric disturbances including psychosis.147 These exacerbations may be irreversible and can occur even in previously asymptomatic patients.148

Tetrathiomolybdate has been suggested as the initial treatment for neurologic WD, although more data are needed.149 Good to excellent recoveries have been obtained in most patients reported.150 In psychiatric WD treated with this agent, symptomatic exacerbations did not occur and most patients had made a good recovery at 1-year follow-up.149

Zinc has been considered to act too slowly to be used as the initial treatment for neurological WD.123 Although neurologic exacerbations can occur,151 dramatic improvements in the neurological exam and nearly complete resolution of increased T2 signal in the putamen, thalamus, and brainstem on MRI were demonstrated in a patient undergoing zinc treatment.152 Simultaneous administration of chelators and zinc does not increase the effectiveness of treatment.153

Both transient154 and irreversible155 neurological exacerbations have been observed with trientine. Dimercaprol, a chelator, was the earliest treatment for WD, but it requires frequent, painful injections and is rarely used. Potassium disulfide, ascorbic acid, and vitamin E are, as yet, of unproven value. Whereas deionized or distilled water may be useful in communities with high copper levels in drinking water,123 a low-copper diet is ineffective.156 Pregnant patients with WD should be treated with zinc, which actually protects against some forms of birth defects157 as well as WD-related fetal demise. Penicillamine is teratogenic and can produce hydrocephalus, cerebral palsy, craniofacial defects, and other stigmata in the developing fetus, although normal pregnancies and births have been reported.

Secondary manifestations of hepatic WD can reverse with WD treatment, and liver transplantation is not always necessary in severe hepatic disease.158,159 Liver transplantation indications and outcome have been reviewed elsewhere.160162 Neurological signs may improve as early as 4 weeks after transplantation.162

Controlled trials of psychotropic treatment, psychotherapy, and family therapy have not been carried out in WD.163 It is anecdotally reported that patients with WD are predisposed to extrapyramidal side effects from antipsychotics.164 A case of neuroleptic malignant syndrome in WD that responded to bromocriptine and dantrolene has been reported.144 Whether this predisposition to extrapyramidal syndromes also applies to treatment with SSRIs is not known. ECT for delusional depression in a patient with WD led to a 3-minute motor seizure followed by a switch to mania.165 Further ECT treatment was interrupted by orthopedic surgery after a fall related to manic behavior. The authors pondered whether this case suggests greater sensitivity to ECT due to intracerebral copper deposition. Beyond these rare anecdotes, little is known about treating psychiatric conditions in WD.

+

Opportunities for Further WD Research

  • Role of copper deposition. Basic research may help establish the pathogenic role of copper and the basis for localized copper deposition in specific brain structures, clarifying how certain structures (e.g., sensory cortex) endure substantial copper deposition yet still maintain functional integrity.

  • Early neuropsychiatric signs. Confirmation of the earliest neuropsychiatric manifestations is needed because early detection is critical to successful treatment, and early WD is frequently missed. This is especially important in patients lacking neurological signs, since these patients have been less well studied.

  • Assessment instruments for WD. The relative sensitivities of the neurologic exam, mental status exam, CT, MRI, and physiologic tests for diagnosing and following WD require further evaluation.

  • Prevalences of neuropsychiatric disorders. The prevalences of neuropsychiatric manifestations need replication across a variety of settings to reduce sample biases. Prevalences should be determined while controlling for severity and clinical features. In particular, the prevalence of neuropsychiatric manifestations in patients with hepatic WD should be carefully and systematically evaluated, since data are lacking.

  • Pattern and course of psychiatric disorders. The pattern and course of certain psychiatric disorders should be consistently characterized by using uniform diagnostic criteria and sensitive quantitative rating scales. Specific questions that might be addressed follow:

  • What is the nature of cognitive impairment in WD? Specifically, how much is due to motor slowing? What are its clinical, anatomical, and physiological correlates? Which features respond to treatment?

  • What are the prevalence rates of various depressive diagnoses in WD? What are the characteristic features of depressive illness in WD? Do these features differ from those of depression in other illnesses? What are the most reliable correlates of depression (since a number of correlates have been linked to depression)?

  • Do mania, anxiety disorders, or substance abuse occur? If so, what are their prevalences, symptomatologies, courses, correlates, and responses to treatment?

  • Are certain neuropsychiatric features correlated with certain mutations? Do neuropsychiatric disorders cluster in certain kindreds with WD?

  • Correlates of neuropsychiatric disorders over disease course. The progression of neuropsychiatric manifestations and their clinical, radiologic, neuropathologic, metabolic, and neurotransmitter correlates over the course of WD require further characterization. Moreover, the impact of individual manifestations on overall outcome and family function should be studied.

  • Copper chelating and depleting agents. Treatment studies are needed to define the effectiveness of specific copper chelating and depleting agents on individual neuropsychiatric manifestations, including the relative rates of neuropsychiatric exacerbations with each drug.

  • Pharmacologic interventions. The efficacy and risks of neuropharmacologic and psychopharmacologic interventions for WD and correlates of good and poor outcome with these interventions remain to be determined.

  • Psychotherapy and family therapy. Psychotherapy and family therapy issues need definition in WD.

Fahr's disease (FD) refers to idiopathic calcification of the basal ganglia. This condition has been known since the middle 1800s. The more general term Fahr's syndrome has been applied variably throughout the literature to indicate either FD or cases of secondary basal ganglia calcification. For the purpose of clarity, we will use the term basal ganglia calcification or BGC when referring to BGC in general (including idiopathic FD and secondary calcification), and FD when referring specifically to idiopathic BGC.

Lowenthal set forth pathological criteria defining FD, including characteristic pallidal calcification evident on CT and on macroscopic pathological exam.166 Although clinical manifestations vary, clinical definitions have been devised. One definition requires bilateral calcifications with neuropsychiatric and extrapyramidal disorders attended by normal calcium and phosphorus metabolism.167 Another stipulates seizures, rigidity, and dementia with characteristic calcification of the basal ganglia.168

Clinical findings are important because radiologists may view BGC as an incidental finding.169 Incidental discovery of BGC before age 50 merits diagnostic investigation.170 The course of FD is progressive.171 In adult-onset FD, calcium deposition generally begins in the third decade of life, with neurological deterioration two decades later,172 but BGC can also occur in pediatric populations.

The frequency of BGC apparent on CT in radiologic studies approximates 0.93% of 29,484 scans,173179 but the prevalence of neurological findings in BGC can vary from 0%175 to 20%.178 The radiologic prevalence of calcification is higher in children (15%) than in adults,180 indicating that BGC is a frequent nonspecific response in a variety of pediatric neurological disorders.

Although calcifications can involve other structures as well, the globus pallidus is most commonly involved.181,182 The lateral pallidum tended to be more affected than the medial pallidum in one study.183 Defective iron transport and free radical production may damage tissue, initiating calcification.168 The mineral and biochemical content as well as the histopathological correlates of calcifications have been defined.166,184186 Mineral composition varies by anatomic site and proximity to vasculature.187

Despite variable calcification distributions and severities, clinical findings in FD are remarkably constant, including seizures of all types, EEG abnormalities, and movement, cognitive, and psychiatric disorders.166 BGC is associated with a wide variety of conditions. Besides idiopathic FD, endocrinologic disorders are leading etiologies of BGC. The frequencies of calcifications have been determined in various endocrinologic disorders,188,189 and the relationship of endocrine dysfunction to BGC has been assessed.174176,178,181,184,190 These associations are detailed elsewhere.173

CT has greater diagnostic specificity for BGC, whereas MRI correlates better with functional impairment.172,191,192 Reduced blood flow to calcified regions correlates with clinical signs.193 Calcification in primary hypoparathyroidism is more diffuse than in other etiologies of calcification,194 whereas post-thyroidectomy hypoparathyroidism calcifications are more focal.179 The EEG displays various abnormalities in FD and BGC, showing no characteristic pattern.195

Familial BGC is associated with a diversity of modes of inheritance, chromosomal aberrations, and clinical manifestations.166,172,196215 Cases of BGC associated with Down's syndrome, trisomy 5, mitochondrial encephalopathies, and other conditions may have pediatric presentations.

Other neuropsychiatric conditions have been found among the many causes of BGC, including CNS lupus,216,217 tuberous sclerosis,218 early-onset Alzheimer's disease,198 motor neuron disease,219 myotonic muscular dystrophy,220 and others. Mitochondrial encephalopathies are yet another cause.221228 Of 11 cases of mitochondrial encephalopathies, 5 had BGC.226 Neurobrucellosis is linked to BGC, occurring in 13.8% of brucellosis patients with neuropsychiatric symptoms.229 BGC in AIDS may occur in patients with abnormal calcium metabolism.230 Anticonvulsants have also been associated with BGC.178 Microencephaly, pigmentary macular degeneration, progeria, and abnormal calcium metabolism are variably associated with infantile and juvenile BGC.

+

Neurological Features

About half of patients with BGC have neurological features.178,179,182 Headache, vertigo, movement disorders, paresis, strokelike events (including episodes resembling transient ischemic attacks), seizures, and syncope represent the most common manifestations of BGC.179 The frequency of strokelike episodes in clinical populations may indicate that unrecognized mitochondrial encephalopathies are a leading cause of BGC, but research is needed to establish this observation. Other specific neurological features include paresis, spasticity, gait disorder, speech impairment, coma, dementia, parkinsonism, chorea, tremor, dystonia, myoclonus, and orthostatic hypotension.171,179,231234 Seizures occurred in 22% and extrapyramidal movement disorders in 56% in a literature review of 213 cases.179 Konig analyzed the frequency of neurological features in patients initially presenting with BGC, as well as on follow-up.179 Again, movement disorders were the most common feature, and these and seizures became more common over time. These figures need replication across larger samples. Seizures correlated best with isolated pallidal calcifications in one series.182 In a Bavarian door-to-door study with CT follow-up, 1 of 17 subjects with parkinsonism (5.9%) had BGC.235 Infantile BGC is rare and heterogeneous in etiology but can be associated with infantile spasms, a burst-suppression pattern on EEG, microcephaly, blindness, early death, hyperammonemia, reduced hepatic ornithine transcarbamylase activity, and consanguinity.236 Aicardi-Goutieres syndrome, an autosomal recessive disorder with BGC, is characterized by increased CSF alpha interferon and lymphocytosis, with encephalopathy manifesting shortly after birth and causing developmental arrest.237

+

Neuropsychiatric Features

A problem with the literature is that most studies involve patients with BGC of various etiologies, including FD. Consequently the data are limited for pure FD, although neuropsychiatric manifestations do not appear to vary by BGC etiology.179 About 40% of patients with BGC present initially with psychiatric features.179 Cognitive, psychotic, and mood disorders are common.

Symptomatic features may change over time.234,238 More extensive calcification and subarachnoid space dilatation correlate with the presence of psychiatric manifestations,178,179,181 but calcific distribution and etiology do not.179 Konig analyzed the frequency of psychiatric disorders in patients with BGC on both initial presentation and follow-up.179 Mood disorders were the most common, and these, intellectual impairment, and compulsions became more common over time. These figures need replication across larger samples. Dementia, chronic cognitive disorders, paranoia, hallucinations, substance abuse, and personality changes were found as manifestations.179

+

Cognitive Disorders:

FD may present as a progressive subcortical dementia in the sixth decade of life.239 Delirium, apathy,240 and amnesia234 also occur. Earlier presentation as a psychosis may progress to dementia with cortical features.238 Follow-up on patients within 2 years after CT identification of BGC revealed impairments in motor speed, executive function, visuospatial skills, and selected memory functions when compared with matched control subjects.182 The neuropathology of FD dementia may include frontotemporal atrophy, cortical neurofibrillary tangles, and neuronal loss in the nucleus basalis of Meynert, but senile plaques are lacking.241 It is not clear whether this neuropathologic description applies to FD in general or only to the Japanese BGC "diffuse neurofibrillary tangles with calcification."242 Other neuropathological features include calcareous deposits, white matter demyelination, and fibrous gliosis. Nearly all of 35 subjects with BGC showed intellectual impairment on long-term follow-up, and nearly one-third of patients had dementia or chronic cognitive disorders.179

+

Psychoses:

Paranoid and psychotic features often present between the ages of 20 and 40 in FD.239,243 Symptoms include auditory hallucinations (sometimes musical), complex visual hallucinations, perceptual distortions, paranoid delusions or nondelusional trends, and fugue states.206,207,234,238,244 Ideas of reference or influence and catatonia also have been observed. A statistical association between psychosis and BGC is disputed,169 with some finding239,245 and others contesting179,246,247 an association. Nevertheless, schizophreniform psychosis was associated with BGC extending over four generations in one family.206,207 Moreover, review of 23 cases in the literature indicates two patterns of psychotic presentation in FD, including early onset (mean age 30.7 years) with minimal movement disorder and late onset (mean age 49.4 years) attended by dementia and movement disorder.243

+

Mood Disorders:

Mood disorders include mania and depression and are the most common behavioral changes in BGC, initially present in one-fifth, eventually occurring in about two-thirds, and occurring more commonly than in neuropsychiatric control subjects.179,247 Of 58 patients, mania eventually occurred in up to 31%.167,179,182 Perhaps up to one-half of patients develop depression at some point over the illness course. One study found DSM-III depressive disorders in 37%,179 and another found DSM-III-R minor depressive symptomatology in 16.7%.182

+

Anxiety and Other Disorders:

Up to one-third of patients with BGC meet DSM-III-R criteria for obsessive-compulsive disorder.179,182 Exact rates of other anxiety disorders in FD remain to be determined. Correlates of anxiety in FD require definition. Substance abuse and personality alterations occur in 8%,179 similar to rates in the general population. Other disorders have not been reported.

+

Treatment

Although treatment of underlying etiologies (such as hypoparathyroidism248,249 or mitochondrial encephalopathy250) has led to neuropsychiatric improvement, there are no specific treatments to limit calcification progression to our knowledge, except for a theoretically appealing yet unconfirmed report of improvement using chelators (xydifon, penicillamine, deferoxamine) with antioxidants and calcium antagonists.251

There are no systematically conducted controlled psychotropic treatment studies in FD. Patients with secondary BGC may also have heart, renal, or other system diseases that influence the use of psychotropics. Patients may or may not respond to conventional treatments. BGC-related parkinsonism often responds to levodopa,252254 but some patients fail to respond to either levodopa or treatment of associated hypoparathyroidism.255

Psychosis responds variably to treatment and is sometimes unresponsive.206,243 Four of 7 patients were found to be particularly susceptible to extrapyramidal side effects.206 Consequently, patients may be predisposed to neuroleptic malignant syndrome, which, in 1 case, was refractory to bromocriptine but responsive to dantroline.238 Some cases of psychosis have proven refractory to haloperidol but have responded instead to lithium.256 In a series of 5 cases of FD with mood disorders, 3 of 4 patients with depression responded to antidepressant treatments including imipramine and ECT; chronic mania in the fifth patient did not respond to lithium, haloperidol, or carbamazepine.167 ECT is probably best avoided, given BGC seizure risk and the variable presence of increased intracranial pressure in some patients.

+

Opportunities For Further FD Research

  • Calcium deposition. Basic research may help establish the basis for localized deposition of calcium in the brain.

  • Mitochondrial disease. The frequency of occult mitochondrial encephalopathy with lactacidosis and strokelike episodes presenting as FD requires investigation.

  • Relationships of FD and BGC. Clarification of the relationships, if any, between FD and BGC is needed.

  • Heritability of neuropsychiatric disorders in familial FD. Definition of the heritability of neuropsychiatric phenomena in kindreds with familial FD may be instructive. Are certain neuropsychiatric features correlated with certain mutations? Do neuropsychiatric disorders cluster in certain kindreds with FD?

  • Prevalences of psychiatric disorders. Further determination and replication of the prevalences of psychiatric disorders in FD, using standardized diagnostic criteria and controlling for FD severity and clinical features, are needed.

  • Imaging correlates of neuropsychiatric disorders. Structural and functional correlates of neuropsychiatric disorders in FD would assist our understanding of the basal ganglia circuitry involved in mediating these conditions.

  • Longitudinal assessment of neuropsychiatric disorders. Longitudinal assessment is needed of the evolution and progression of individual neuropsychiatric manifestations over the course of FD, and their clinical, radiologic, metabolic, and neurotransmitter correlates over the disease course require characterization.

  • Neuroprotective agents. Controlled treatment trials of the effects of neuroprotective agents on FD progression and neuropsychiatric manifestations are needed.

  • Pharmacologic interventions. The efficacy and risks of neuropharmacologic and psychopharmacologic interventions in FD and correlates of good and poor outcome with these interventions remain to be defined.

  • Treatment-refractory conditions. Novel approaches are needed in treating conditions such as psychosis that are refractory to conventional treatments.

  • Psychotherapy. Psychotherapy issues in FD remain to be elucidated.

HD, WD, and FD are very different disorders, although each is dominated by mood and movement disorders. Preliminary observations identify phenomenologically different profiles of behavioral disturbances, suggesting that the form of neuropsychiatric features elicited may depend heavily on which component of the lenticulostriatal system is damaged. Although further work is needed, similarities and differences between HD, WD, and FD suggest the importance of caudatopallidal circuits in cognition and mania (in HD and FD), perhaps related to thalamic disinhibition.234 Personality changes have been documented principally in striatal diseases (HD and WD), whereas depression is equally prominent in HD, WD, and FD.

Regrettably, the current state of the literature makes it difficult to compare neuropsychiatric sequelae in different lenticulostriatal diseases (LSDs). Studies have employed varying diagnostic criteria and different assessment instruments in different stages of these illnesses. It seems necessary to determine neuropsychiatric prevalences at individual stages of these progressive illnesses, denoting the extent and neuroanatomic distribution of the pathology, before definite comparative conclusions about the differential roles of the caudate, putamen, and internal and external pallidal segments can be drawn across LSDs. Correlation of neuropsychiatric features with radiologic, metabolic, and pharmacologic measures has the potential to increase our understanding of the function of these structures in neuropsychiatric illnesses.

+

Opportunities For Further Research in Lenticulostriatal Diseases

The following tentative conclusions drawn from the extant data provide testable heuristic hypotheses, provided that appropriate methodology is applied:

  • HD is particularly associated with chorea, dementia, personality changes, psychosis, mania, depression, suicidality, impulse dyscontrol, and sexual disorders.

  • WD is especially associated with dysdiadochokinesia, dysarthria, bradykinesia, tremor, cognitive impairment, personality changes, and depression.

  • In FD, extrapyramidal disorders, seizures, dementia, mania, depression, and compulsions are particularly common.

  • Across LSDs, parkinsonian features may correlate with medial striatal and lateral pallidal disease, whereas hyperkinesias and mania may correlate with lateral striatal and medial pallidal disease.

  • Dementia and mania in LSDs may be associated with caudate and pallidal disease rather than putaminal pathology.

  • Personality changes other than irritability in LSDs may be associated with striatal disease.

  • Irritability may be prominent in LSDs and may correlate with striatal pathology, especially in HD and WD.

  • Psychosis, suicidality, impulse dyscontrol, and sexual disorders may be associated with caudate disease, especially in HD.

  • Depressive disorders in LSDs may correlate with parkinsonian features, especially in HD and WD.

  • Mania and compulsions may be especially associated with pallidal disease, as in FD.

If these tentative conclusions are confirmed, further attention to the questions of which specific regions of lenticulostriatal structures are pathologically involved, what additional pathology is present, and the clinical and physiologic correlates of these syndromes may clarify the functional significance of these structures. Following neuropsychiatric features over the longitudinal course of these illnesses also can add to this understanding, as well as lead to better prediction of outcome in these diseases. Treatments aimed at limiting disease progression and studies of efficacy and risks of both neuropharmacologic and psychopharmacologic treatments in LSDs are needed. Methodological difficulties involved in carrying out such research include small sample sizes, variable symptomatic presentations, and neurological confounds of psychiatric rating scale items, among others. Adequate and standardized observations such as these will be best carried out through collaborative ventures.

+

Collaborative Research Opportunities

The Committee on Research of the American Neuropsychiatric Association (ANPA) has a dedicated interest in fostering collaborative research endeavors in neuropsychiatric disorders. If you are interested in collaborative research in these disorders, contact the Committee on Research through this journal or through ANPA, Suite 550, 700 Ackerman Road, Columbus, OH 43202.

Nauta WJ: Limbic innervation of the striatum. Adv Neurol  1982; 35:41—47
[PubMed]
 
Haber SN, Groenewegen HJ, Grove EA, et al: Efferent connections of the ventral pallidum: evidence of a dual striato pallidofugal pathway. J Comp Neurol  1985; 235:322—335
[CrossRef] | [PubMed]
 
Alexander GE, DeLong MR, Strick PL: Parallel organization of functionally segregated circuits linking basal ganglia and cortex. Annu Rev Neurosci  1986; 9:357—381
[CrossRef] | [PubMed]
 
Cummings JL: Frontal subcortical circuits and human behavior. Arch Neurol  1993; 50:873—880
[PubMed]
 
Robinson RG: Psychiatric management in stroke, in Psychiatric Management in Neurological Diseases—Major Basal Ganglia—Cortical Disorders, edited by Lauterbach EC. Washington, DC, American Psychiatric Press (in press)
 
Caplan LR, Schmahmann JD, Kase CS, et al: Caudate infarcts. Arch Neurol  1990; 47:133—143
[PubMed]
 
Huntington's Disease Collaborative Research Group: A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell  1993; 72:917—983
 
MacDonald ME, Gusella JF: Huntington's disease: translating a CAG repeat into a pathogenic mechanism. Curr Opin Neurobiol  1996; 6:638—643
[CrossRef] | [PubMed]
 
Folstein SE, Chase GA, Wahl WE, et al: Huntington disease in Maryland: clinical aspects of racial variation. Am J Hum Genet  1987; 41:168—179
[PubMed]
 
Duyao M, Ambrose C, Myers R, et al: Trinucleotide repeat length instability and age of onset in Huntington's disease. Nat Genet  1993; 4:387—392
[CrossRef] | [PubMed]
 
Kremer B, Goldberg P, Andrew SE, et al: A worldwide study of the Huntington's disease mutation: the sensitivity and specificity of measuring CAG repeats. N Engl J Med  1994; 330:1401—1406
[CrossRef] | [PubMed]
 
Sieradzan K, Mann DM, Dodge A: Clinical presentation and patterns of regional cerebral atrophy related to the length of trinucleotide repeat expansion in patients with adult onset Huntington's disease. Neurosci Lett  1997; 28:45—48
 
Folstein SE: Huntington's Disease: A Disorder of Families. Baltimore, The Johns Hopkins University Press, 1989
 
Jones AL, Wood JD, Harper PS: Huntington disease: advances in molecular and cell biology. J Inherit Metab Dis  1997; 20:125—138
[CrossRef] | [PubMed]
 
Gusella JF, MacDonald ME: Huntington's disease: CAG genetics expands neurobiology. Curr Opin Neurobiol  1995; 5:656—662
[CrossRef] | [PubMed]
 
Li X-J, Li S-H, Sharp AH, et al: A Huntington-associated protein enriched in brain with implications for pathology. Nature  1995; 378:398—402
[CrossRef] | [PubMed]
 
Ross CA, Becher MW, Colomer V, et al: Huntington's disease and dentatorubral-pallidoluysian atrophy: proteins, pathogenesis and pathology. Brain Pathol  1997; 7:1003—1016
[CrossRef] | [PubMed]
 
Nasir J, Goldberg YP, Hayden MR: Huntington disease: new insights into the relationship between CAG expansion and disease. Hum Mol Genet 1996; 5(special number):1431—1435
 
Beal MF, Ferrante RJ, Swartz KJ, et al: Chronic quinolinic acid lesions in rats closely resemble Huntington's disease. J Neurosci  1991; 11:1649—1659
[PubMed]
 
Brouillet E, Hantraye P, Ferrante RJ, et al: Chronic mitochondrial energy impairment produces selective striatal degeneration and abnormal choreiform movements in primates. Proc Natl Acad Sci USA  1995; 92:7105—7109
[CrossRef] | [PubMed]
 
Wellington CL, Brinkman RR, O'Kusky JR, et al: Toward understanding the molecular pathology of Huntington's disease. Brain Pathol  1997; 7:979—1002
[CrossRef] | [PubMed]
 
Roos RAC: Neuropathology of Huntington's chorea, in Handbook of Clinical Neurology, vol 5(49): Extrapyramidal Disorders, edited by Vinken PJ, Bruyn GW, Klawans HL. Amsterdam, Elsevier Science, 1986, pp 315—326
 
Nagel JS, Ichise M, Holman BL: The scintigraphic evaluation of Huntington's disease and other movement disorders using single photon emission computed tomography perfusion brain scans. Semin Nucl Med  1991; 21:11—23
[CrossRef] | [PubMed]
 
Turjanski N, Weeks R, Dolan R, et al: Striatal D1 and D2 receptor binding in patients with Huntington's disease and other choreas: a PET study. Brain  1995; 118:689—696
[CrossRef] | [PubMed]
 
Aylward EH, Anderson NB, Bylsma FW, et al: Frontal lobe volume in patients with Huntington's disease. Neurology  1998; 50:252—258
[PubMed]
 
Brandt J, Bylsma FW, Gross R, et al: Trinucleotide repeat length and clinical progression in Huntington's disease. Neurology  1996; 46:527—531
[PubMed]
 
Kieburtz K, MacDonald M, Shih C, et al: Trinucleotide repeat length and progression of illness in Huntington's disease. J Med Genet  1994; 31:872—874
[CrossRef] | [PubMed]
 
Brandt J, Quaid KA, Folstein SE, et al: Presymptomatic diagnosis of delayed onset disease with linked DNA markers: the experience in Huntington's disease. JAMA  1989; 261:3108—3114
[CrossRef] | [PubMed]
 
Wiggins S, Whyte P, Huggins M, et al: The psychological consequences of predictive testing for Huntington's disease. N Engl J Med  1992; 327:1401—1405
[CrossRef] | [PubMed]
 
Wexler NS: The Tiresias complex: Huntington's disease as a paradigm for testing for late-onset disorders. FASEB J  1992; 6:2820—2825
[PubMed]
 
International Huntington Association and World Federation of Neurology Research Group on Huntington's Chorea: Guidelines for the molecular genetics predictive test in Huntington's disease. Neurology  1992; 44:1533—1536
 
Hayden MR, Bloch M, Wiggins S: Psychological effects of predictive testing for Huntington's disease. Adv Neurol  1995; 65:201—210
[PubMed]
 
Folstein SE, Jensen B, Leigh RJ, et al: The measurement of abnormal movement: methods developed for Huntington's disease. Neurobehav Toxicol Teratol  1983; 5:605—609
[PubMed]
 
Huntington Study Group: The Unified Huntington Disease Rating Scale: reliability and consistency. Mov Disord  1996; 4:136—142
 
Albin RL, Reiner A, Anderson KD, et al: Striatal and nigral neuron subpopulations in rigid Huntington's disease: implications for the functional anatomy of chorea and rigidity-akinesia. Ann Neurol  1990; 27:357—365
[CrossRef] | [PubMed]
 
Pearson SJ, Heathfield KW, Reynolds GP: Pallidal GABA and chorea in Huntington's disease. J Neural Transm Gen Sect  1990; 81:241—246
[CrossRef] | [PubMed]
 
Penney JB Jr, Young AB, Shoulson I, et al: Huntington's disease in Venezuela: 7 years of follow-up on symptomatic and asymptomatic individuals. Mov Disord  1990; 5:93—99
[CrossRef] | [PubMed]
 
Brandt J, Strauss ME, Larus J, et al: Clinical correlates of dementia and disability in Huntington's disease. J Clin Neuropsychol  1984; 6:401—412
[CrossRef] | [PubMed]
 
Morris M: Psychiatric aspects of Huntington's disease, in Huntington's Disease, edited by Harper PS. Philadelphia, WB Saunders, 1991, pp 81—126
 
Ranen NG: Psychiatric management in Huntington's disease, in Psychiatric Management in Neurological Diseases—Major Basal Ganglia—Cortical Disorders, edited by Lauterbach EC. Washington, DC, American Psychiatric Press (in press)
 
Mayeux R, Stern Y, Herman A, et al: Correlates of early disability in Huntington's disease. Ann Neurol  1986; 20:727—731
[CrossRef] | [PubMed]
 
Brandt J: Cognitive investigations in Huntington's disease, in Neuropsychological Explorations of Memory and Cognition: Essays in Honor of Nelson Butters, edited by Cermak LS. New York, Plenum, 1994, pp 4135—4136
 
Brandt J, Folstein SE, Folstein MF: Differential cognitive impairment in Alzheimer's disease and Huntington's disease. Ann Neurol  1988; 23:555—561
[CrossRef] | [PubMed]
 
Butters N, Wolfe J, Granholm E, et al: An assessment of verbal recall, recognition and fluency abilities in patients with Huntington's disease. Cortex  1986; 22:11—32
[PubMed]
 
Brandt J, Corwin J, Krafft L: Is verbal recognition memory really different in Huntington's and Alzheimer's disease? J Clin Exp Neuropsychol  1992; 14:773—784
 
Morris M: Dementia and cognitive changes in Huntington's disease. Adv Neurol  1995; 65:187—200
[PubMed]
 
Reynolds GP, Pearson SJ: Neurochemical-clinical correlates in Huntington's disease: applications of brain banking techniques. J Neural Transm Suppl  1993; 39:207—214
[PubMed]
 
Cummings JL: Behavioral and psychiatric symptoms associated with Huntington's disease. Adv Neurol  1995; 65:179—186
[PubMed]
 
McHugh PR, Folstein MR: Psychiatric syndromes of Huntington's chorea: a clinical and phenomenological study, in Psychiatric Aspects of Neurologic Disease, edited by Benson DF, Blumer D. New York, Grune and Stratton, 1975, pp 267—286
 
Shiwach R: Psychopathology in Huntington's disease patients. Acta Psychiatr Scand  1994; 90:241—246
[CrossRef] | [PubMed]
 
Burns A, Folstein SE, Brandt J, et al: Clinical assessment of irritability, aggression, and apathy in Huntington and Alzheimer disease. J Nerv Ment Dis  1990; 178:20—26
[CrossRef] | [PubMed]
 
Kuwert T, Lange HW, Langen K-J, et al: Cerebral glucose consumption measured by PET in patients with and without psychiatric symptoms of Huntington's disease. Psychiatry Res  1989; 29:361—362
[CrossRef] | [PubMed]
 
Caine ED, Shoulson I: Psychiatric syndromes in Huntington's disease. Am J Psychiatry  1983; 140:728—733
[PubMed]
 
Bolt JMW: Huntington's chorea in the west of Scotland. Br J Psychiatry  1970; 116:259—270
[CrossRef] | [PubMed]
 
Baxter LR, Mazziotta JC, Pahl JJ, et al: Psychiatric, genetic, and positron emission tomographic evaluation of persons at risk for Huntington's disease. Arch Gen Psychiatry  1992; 49:148—154
[PubMed]
 
Dewhurst K, Oliver J, Trick KLK, et al: Neuro-psychiatric aspects of Huntington's disease. Confin Neurol  1969; 31:258—268
[CrossRef] | [PubMed]
 
Shiwach RS, Patel V: Aggressive behaviour in Huntington's disease: a cross-sectional study in a nursing home population. Behav Neurol  1993; 6:43—47
 
Dewhurst K, Oliver JE, McKnight AL: Socio-psychiatric consequences of Huntington's disease. Br J Psychiatry  1970; 116:255—258
[CrossRef] | [PubMed]
 
Rosenbaum D: Psychosis with Huntington's chorea. Psychiatr Q  1941; 15:93—99
[CrossRef]
 
Beckson M, Cummings JL: Psychosis in basal ganglia disorders. Neuropsychiatry Neuropsychol Behav Neurol  1992; 5:126—131
 
Vonsattel J-P, Myers RH, Stevens TJ, et al: Neuropathological classification of Huntington's disease. J Neuropathol Exp Neurology  1985; 44:559—577
[CrossRef]
 
Ranen NG, Peyser CE, Folstein SE: A Physician's Guide to the Management of Huntington's Disease: Pharmacologic and Non-Pharmacologic Interventions, New York, HDSA, 1993
 
Mayberg HS, Starkstein SE, Peyser CE, et al: Paralimbic frontal lobe hypometabolism in depression associated with Huntington's disease. Neurology  1992; 42:1791—1797
[PubMed]
 
Peyser CE, Folstein SE: Huntington's disease as a model for mood disorders: clues from neuropathology and neurochemistry. Mol Chem Neuropathol  1990; 12:99—119
[CrossRef] | [PubMed]
 
Schoenfeld M, Myers RH, Cupples LA, et al: Increased rate of suicide among patients with Huntington's disease. J Neurol Neurosurg Psychiatry  1984; 47:1283—1287
[CrossRef] | [PubMed]
 
Lipe H, Schultz A, Bird TD: Risk factors for suicide in Huntington's disease: a retrospective case controlled study. Am J Med Genet  1993; 48:231—233
[CrossRef] | [PubMed]
 
Pflanz S, Besson JA, Ebmeier KP, et al: The clinical manifestation of mental disorder in Huntington's disease: a retrospective case record study of disease progression. Acta Psychiatr Scand  1991; 83:53—60
[CrossRef] | [PubMed]
 
Cummings JL, Cunningham K: Obsessive-compulsive disorder in Huntington's disease. Biol Psychiatry  1992; 31:263—270
[CrossRef] | [PubMed]
 
Watt DC, Seller A: A clinico-genetic study of psychiatric disorder in Huntington's chorea. Psychol Med Suppl  1993; 23:1—46
[CrossRef]
 
Fedoroff JP, Peyser C, Franz ML, et al: Sexual disorders in Huntington's disease. J Neuropsychiatry Clin Neurosci  1994; 6:147—153
[PubMed]
 
Young AB: Role of excitotoxins in heredito-degenerative neurologic disease. Res Publ Assoc Res Nerv Ment Dis  1993; 71:175—189
[PubMed]
 
Shoulson I, Odoroff C, Oakes D, et al: A controlled trial of baclofen as protective therapy in early Huntington's disease. Ann Neurol  1989; 25:252—259
[CrossRef] | [PubMed]
 
Ranen NG, Peyser CE, Coyle JT, et al: A controlled trial of idebenone in Huntington's disease. Mov Disord 1996;11:549—554
 
Peyser CE, Folstein M, Chase GA, et al: Trial of d-α-tocopherol in Huntington's disease. Am J Psychiatry  1995; 152:1771—1775
[PubMed]
 
Koroshetz WJ, Jenkins BG, Rosen BR, et al: Energy metabolism defects in Huntington's disease and effects of coenzyme Q10. Ann Neurol  1997; 41:160—165
[CrossRef] | [PubMed]
 
Emerich DF, Winn SR, Hantraye PM, et al: Protective effect of encapsulated cells producing neurotrophic factor CNTF in a monkey model of Huntington's disease. Nature  1997; 386:395—399
[CrossRef] | [PubMed]
 
Barr AN, Fischer JH, Koller WC, et al: Serum haloperidol concentration and choreiform movements in Huntington's disease. Neurology  1988; 38:84—88
[PubMed]
 
Albanese A, Cassetta E, Carretta D, et al: Acute challenge with apomorphine in Huntington's disease: a double-blind study. Clin Neuropharmacol  1995; 18:427—434
[CrossRef] | [PubMed]
 
van Vugt JP, Siesling S, Vergeer M, et al: Clozapine versus placebo in Huntington's disease: a double blind randomised comparative study. J Neurol Neurosurg Psychiatry  1997; 63:35—39
[CrossRef] | [PubMed]
 
Mateo D, Gimenez-Roldan S: [The effect of piracetam on involuntary movements in Huntington's disease: a double-blind, placebo-controlled study.] Neurologia  1996; 11:16—19
[PubMed]
 
Consroe P, Laguna J, Allender J, et al: Controlled clinical trial of cannabidiol in Huntington's disease. Pharmacol Biochem Behav  1991; 40:701—708
[CrossRef] | [PubMed]
 
Giuffra ME, Mouradian MM, Chase TN: Glutamatergic therapy of Huntington's chorea. Clin Neuropharmacol  1992; 15:148—151
[CrossRef] | [PubMed]
 
Como PG, Rubin AJ, O'Brien CF, et al: A controlled trial of fluoxetine in nondepressed patients with Huntington's disease. Mov Disord  1997; 12:397—401
[CrossRef] | [PubMed]
 
Murman DL, Giordani B, Mellow AM, et al: Cognitive, behavioral, and motor effects of the NMDA antagonist ketamine in Huntington's disease. Neurology  1997; 49:153—161
[PubMed]
 
Ranen NG, Lipsey JR, Treisman G, et al: Sertraline in the treatment of severe aggressiveness in Huntington's disease. J Neuropsychiatry Clin Neurosci  1996; 8:338—340
[PubMed]
 
Stewart JT: Huntington's disease and propranolol. Am J Psychiatry  1993; 150:166—167
[PubMed]
 
Byrne A, Martin W, Hnatko G: Beneficial effects of buspirone therapy in Huntington's disease (letter). Am J Psychiatry  1994; 151:1097
 
Findling RL: Treatment of aggression in juvenile-onset Huntington's disease with buspirone (letter). Psychosomatics  1993; 34:460—461
[PubMed]
 
Sajatovic M, Verbanac P, Ramirez LF, et al: Clozapine treatment of psychiatric symptoms resistant to neuroleptic treatment in patients with Huntington's chorea. Neurology  1990; 41:156
 
Bonucelli U, Ceravolo R, Maremmani C, et al: Clozapine in Huntington's chorea. Neurology  1994; 44:821—823
[PubMed]
 
Korenyi C, Whittier JR: Drug treatment in 117 cases of Huntington's disease with special reference to fluphenazine (Prolixin). Psychiatr Q  1967; 41:203—210
[CrossRef] | [PubMed]
 
Ringel SP, Guthrie M, Klawans HL: Current treatment of Huntington's chorea. Adv Neurol  1973; 1:797—801
 
Whittier J, Haydu G, Crawford J: Effect of imipramine (Tofranil) on depression and hyperkinesia in Huntington's disease. Am J Psychiatry  1961; 118:79
[PubMed]
 
Ford MF: Treatment of depression in Huntington's disease with monoamine oxidase inhibitors. Br J Psychiatry  1986; 149:654—656
[CrossRef] | [PubMed]
 
Patel SV, Tariot PN, Asnis J: l-Deprenyl augmentation of fluoxetine in a patient with Huntington's disease. Ann Clin Psychiatry  1996; 8:23—26
[CrossRef] | [PubMed]
 
Ranen NG, Peyser CE, Folstein SE: ECT as a treatment for depression in Huntington's disease. J Neuropsychiatry Clin Neurosci  1994; 6:154—159
[PubMed]
 
Rich SS, Ovsiew F: Leuprolide acetate for exhibitionism in Huntington's disease. Mov Disord  1994; 9:353—357
[CrossRef] | [PubMed]
 
Lauterbach EC: Family management in neurological disorders, in Psychiatric Management in Neurological Diseases—Major Basal Ganglia—Cortical Disorders, edited by Lauterbach EC. Washington, DC, American Psychiatric Press (in press)
 
Frydman M: Genetic aspects of Wilson's disease. J Gastroenterol Hepatol  1990; 5:483—490
[CrossRef] | [PubMed]
 
Chowrimootoo GF, Andoh J, Seymour CA: Western blot analysis in patients with hypocaeruloplasminaemia. Q J Med  1997; 90:197—202
 
Mansouri A, Gaou I, Fromenty B, et al: Premature oxidative aging of hepatic mitochondrial DNA in Wilson's disease. Gastroenterology  1997; 113:599—605
[CrossRef] | [PubMed]
 
Nanji MS, Nguyen VT, Kawasoe JH, et al: Haplotype and mutation analysis in Japanese patients with Wilson disease. Am J Hum Genet  1997; 60:1423—1429
[CrossRef] | [PubMed]
 
Walshe JM: Copper: not too little, not too much, but just right. Based on the triennial Pewterers Lecture delivered at the National Hospital for Neurology, London, 23 March 1995. J R Coll Physicians Lond  1995; 19:280—288
 
Houwen RH, Juyn J, Hoogenraad TU, et al: H714Q mutation in Wilson disease is associated with late, neurological presentation. J Med Genet  1995; 32:480—482
[CrossRef] | [PubMed]
 
Hoogenraad TU, Houwen RHJ: Prevalence and genetics, in Wilson's Disease, edited by Hoogenraad TU. London, WB Saunders, 1996, pp 14—24
 
Chu NS, Hung TP: Geographic variations in Wilson's disease. J Neurol Sci  1993; 117:1—7
[CrossRef] | [PubMed]
 
Rodman R, Burnstine M, Esmaeli B, et al: Wilson's disease: presymptomatic patients and Kayser-Fleischer rings. Ophthalmic Genet  1997; 18:79—85
[CrossRef] | [PubMed]
 
Esmaeli B, Burnstine MA, Martonyi CL, et al: Regression of Kayser-Fleischer rings during oral zinc therapy: correlation with systemic manifestations of Wilson's disease. Cornea  1996; 15:582—588
[PubMed]
 
Magalhaes AC, Caramelli P, Menezes JR, et al: Wilson's disease: MRI with clinical correlation. Neuroradiology  1994; 36:97—100
[CrossRef] | [PubMed]
 
Starosta-Rubinstein S, Young AB, Kluin K, et al: Clinical assessment of 31 patients with Wilson's disease: correlations with structural changes on magnetic resonance imaging. Arch Neurol  1987; 44:365—370
[PubMed]
 
Mochizuki H, Kamakura K, Masaki T, et al: Atypical MRI features of Wilson's disease: high signal in globus pallidus on T1-weighted images. Neuroradiology  1997; 39:171—174
[CrossRef] | [PubMed]
 
Saatci I, Topcu M, Baltaoglu FF, et al: Cranial MR findings in Wilson's disease. Acta Radiol  1997; 38:250—258
[PubMed]
 
King AD, Walshe JM, Kendall BE, et al: Cranial MR imaging in Wilson's disease. Am J Roentgenol  1996; 167:1579—1584
 
van Wassenaer-van Hall HN, van den Heuvel AG, Algra A, et al: Wilson disease: findings at MR imaging and CT of the brain with clinical correlation. Radiology  1996; 198:531—536
[PubMed]
 
Jardim LB, Carneiro A, Hansel S, et al: CT hypodensity on cerebral white matter in Wilson's disease. Arq Neuropsiquiatr  1991; 49:211—214
[CrossRef] | [PubMed]
 
Kuwert T, Hefter H, Scholz D, et al: Regional cerebral glucose consumption measured by positron emission tomography in patients with Wilson's disease. Eur J Nucl Med  1992; 19:96—101
[PubMed]
 
Schlaug G, Hefter H, Engelbrecht V, et al: Neurological impairment and recovery in Wilson's disease: evidence from PET and MRI. J Neurol Sci  1996; 136:129—139
[CrossRef] | [PubMed]
 
van Den Heuvel AG, Van der Grond J, Van Rooij LG, et al: Differentiation between portal-systemic encephalopathy and neurodegenerative disorders in patients with Wilson disease: H-1 MR spectroscopy. Radiology  1997; 203:539—543
[PubMed]
 
Oder W, Brucke T, Kollegger H, et al: Dopamine D2 receptor binding is reduced in Wilson's disease: correlation of neurological deficits with striatal 123I-iodobenzamide binding. J Neural Transm  1996; 103:1093—1103
[CrossRef] | [PubMed]
 
Roach ES, Ford CS, Spudis EV, et al: Wilson's disease: evoked potentials and computed tomography. J Neurol  1985; 232:20—23
[CrossRef] | [PubMed]
 
Maier-Dobersberger T, Mannhalter C, Rack S, et al: Diagnosis of Wilson's disease in an asymptomatic sibling by DNA linkage analysis. Gastroenterology  1995; 109:2015—2018
[CrossRef] | [PubMed]
 
Oder W, Grimm G, Kollegger H, et al: Neurological and neuropsychiatric spectrum of Wilson's disease: a prospective study of 45 cases. J Neurol  1991; 238:281—287
[PubMed]
 
Brewer GJ, Yuzbasiyan-Gurkan V: Wilson disease. Medicine (Baltimore)  1992; 71:139—164
[PubMed]
 
Arendt G, Hefter H, Stremmel W, et al: The diagnostic value of multi-modality evoked potentials in Wilson's disease. Electromyogr Clin Neurophysiol  1994; 34:137—148
[PubMed]
 
Dening TR, Berrios GE, Walshe JM: Wilson's disease and epilepsy. Brain  1988; 111:1139—1155
[CrossRef] | [PubMed]
 
Walshe JM, Yealland M: Wilson's disease: the problem of delayed diagnosis. J Neurol Neurosurg Psychiatry  1992; 55:692—696
[CrossRef] | [PubMed]
 
Akil M, Brewer GJ: Psychiatric and behavioral abnormalities in Wilson's disease. Adv Neurol  1995; 65:171—178
[PubMed]
 
Dening TR, Berrios GE: Wilson's disease. Psychiatric symptoms in 195 cases. Arch Gen Psychiatry  1989; 46:1126—1134
[PubMed]
 
Scheinberg IH, Sternlieb I: Wilson's disease, in Major Problems in Internal Medicine, vol 23, edited by Smith LH Jr. Philadelphia, WB Saunders, 1984
 
Dening TR, Berrios GE: Wilson's disease: a longitudinal study of psychiatric symptoms. Biol Psychiatry  1990; 28:255—265
[CrossRef] | [PubMed]
 
Rathbun JK: Neuropsychological aspects of Wilson's disease. Int J Neurosci  1996; 85:221—229
[CrossRef] | [PubMed]
 
Akil M, Schwartz JA, Dutchak D, et al: The psychiatric presentations of Wilson's disease. J Neuropsychiatry Clin Neurosci  1991; 3:377—382
[PubMed]
 
Knehr CA, Bearn AG: Psychological impairment in Wilson's disease. J Nerv Ment Dis  1956; 124:251—255
[CrossRef] | [PubMed]
 
Littman E, Medalia A, Senior G, et al: Rate of information processing in patients with Wilson's disease. J Neuropsychiatry Clin Neurosci  1995; 7:68—71
[PubMed]
 
Dening TR, Berrios GE: Wilson's disease: a prospective study of psychopathology in 31 cases. Br J Psychiatry  1989; 155:206—213
[CrossRef] | [PubMed]
 
Dening TR: Psychiatric aspects of Wilson's disease. Br J Psychiatry  1985; 147:677—682
[CrossRef] | [PubMed]
 
Oder W, Prayer L, Grimm G, et al: Wilson's disease: evidence of subgroups derived from clinical findings and brain lesions. Neurology  1993; 43:120—124
[PubMed]
 
Walshe JM, Yealland M: Chelation treatment of neurological Wilson's disease. Q J Med  1993; 86:197—204
[PubMed]
 
Schlaug G, Hefter H, Nebeling B, et al: Dopamine D2 receptor binding and cerebral glucose metabolism recover after d-penicillamine-therapy in Wilson's disease. J Neurol  1994; 241:577—584
[CrossRef] | [PubMed]
 
Selwa LM, Vanderzant CW, Brunberg JA, et al: Correlation of evoked potential and MRI findings in Wilson's disease. Neurology  1993; 43:2059—2064
[PubMed]
 
Grimm G, Oder W, Prayer L, et al: Evoked potentials in assessment and follow-up of patients with Wilson's disease. Lancet  1990; 336:963—964
[CrossRef] | [PubMed]
 
Nazer H, Brismar J, al-Kawi MZ, et al: Magnetic resonance imaging of the brain in Wilson's disease. Neuroradiology  1993; 35:130—133
[CrossRef] | [PubMed]
 
Goldstein NP, Ewert MA, Randall RV, et al: Psychiatric aspects of Wilson's disease (hepatolenticular degeneration): results of psychometric tests during long-term therapy. Am J Psychiatry  1968; 124:1155—1561
 
Kontaxakis V, Stefanis C, Markidis M, et al: Neuroleptic malignant syndrome in a patient with Wilson's disease (letter). J Neurol Neurosurg Psychiatry  1988; 51:1001—1002
[CrossRef] | [PubMed]
 
Rosselli M, Lorenzana P, Rosselli A, et al: Wilson's disease, a reversible dementia: case report. J Clin Exp Neuropsychol  1987; 9:399—406
[CrossRef] | [PubMed]
 
Schwarz J, Antonini A, Kraft E, et al: Treatment with d-penicillamine improves dopamine D2-receptor binding and T2-signal intensity in de novo Wilson's disease. Neurology  1994; 44:1079—1082
[PubMed]
 
McDonald LV, Lake CR: Psychosis in an adolescent patient with Wilson's disease: effects of chelation therapy. Psychosom Med  1995; 57:202—204
[PubMed]
 
Glass JD, Reich SG, DeLong MR: Wilson's disease: development of neurological disease after beginning penicillamine therapy. Arch Neurol  1990; 47:595—596
[PubMed]
 
Brewer GJ: Interactions of zinc and molybdenum with copper in therapy of Wilson's disease. Nutrition 1995; 11(suppl 1):114—116
 
Brewer GJ, Johnson V, Dick RD, et al: Treatment of Wilson disease with ammonium tetrathiomolybdate, II: initial therapy in 33 neurologically affected patients and follow-up with zinc therapy. Arch Neurol  1996; 53:1017—1025
[PubMed]
 
Lang CJ, Rabas-Kolominsky P, Engelhardt A, et al: Fatal deterioration of Wilson's disease after institution of oral zinc therapy. Arch Neurol  1993; 50:1007—1008
 
Heckmann JM, Eastman RW, De Villiers JC, et al: Wilson's disease: neurological and magnetic resonance imaging improvement on zinc treatment (letter). J Neurol Neurosurg Psychiatry  1994; 57:1273—1274
[CrossRef] | [PubMed]
 
Brewer GJ, Yuzbasiyan-Gurkan V, Johnson V, et al: Treatment of Wilson's disease with zinc, XI: interaction with other anticopper agents. J Am Coll Nutr  1993; 12:26—30
[PubMed]
 
Saito H, Watanabe K, Sahara M, et al: Triethylene-tetramine (trien) therapy for Wilson's disease. Tohoku J Exp Med  1991; 164:29—35
[CrossRef] | [PubMed]
 
Dahlman T, Hartvig P, Lofholm M, et al: Long-term treatment of Wilson's disease with triethylene tetramine dihydrochloride (trientine). Q J Med  1995; 88:609—616
 
Scheinberg IH, Sternlieb I: Wilson disease and idiopathic copper toxicosis. Am J Clin Nutr  1996; 63:842S—845S
 
Nunns D, Hawthorne B, Goulding P, et al: Wilson's disease in pregnancy. Eur J Obstet Gynecol Reprod Biol  1995; 62:141—143
[CrossRef] | [PubMed]
 
Schilsky ML, Scheinberg IH, Sternlieb I: Prognosis of Wilsonian chronic active hepatitis. Gastroenterology  1991; 100:762—767
[PubMed]
 
Santos Silva EE, Sarles J, Buts JP, et al: Successful medical treatment of severely decompensated Wilson disease. J Pediatr  1996; 128:285—287
[CrossRef] | [PubMed]
 
Rothfus WE, Hirsch WL, Malatack JJ, et al: Improvement of cerebral CT abnormalities following liver transplantation in a patient with Wilson disease. J Comput Assist Tomogr  1988; 12:138—140
[CrossRef] | [PubMed]
 
Bellary S, Hassanein T, Van Thiel DH: Liver transplantation for Wilson's disease. J Hepatol  1995; 23:373—381
[CrossRef] | [PubMed]
 
Schumacher G, Platz KP, Mueller AR, et al: Liver transplantation: treatment of choice for hepatic and neurological manifestation of Wilson's disease. Clin Transplant  1997; 11:217—224
[PubMed]
 
Lauterbach EC: Psychiatric management in Wilson's disease (progressive hepatolenticular degeneration), in Psychiatric Management in Neurological Diseases—Major Basal Ganglia—Cortical Disorders, edited by Lauterbach EC. Washington, DC, American Psychiatric Press (in press)
 
Hoogenraad TU: Wilson's Disease. London, WB Saunders, 1996
 
Negro PJ Jr, Louzã Neto MR: Results of ECT for a case of depression in Wilson's disease (letter). J Neuropsychiatry Clin Neurosci  1995; 7:384
[PubMed]
 
Lowenthal A: Striopallidodentate calcifications, in Handbook of Clinical Neurology, vol 5 (49): Extrapyramidal Disorders, edited by Vinken PJ, Bruyn GW, and Klawans HL. Amsterdam, Elsevier Science, 1986, pp 417—436
 
Trautner RJ, Cummings JL, Read SL, et al: Idiopathic basal ganglia calcification and organic mood disorder. Am J Psychiatry  1988; 145:350—353
[PubMed]
 
Beall SS, Patten BM, Mallette L, et al: Abnormal systemic metabolism of iron, porphyrin, and calcium in Fahr's syndrome. Ann Neurol  1989; 26:569—575
[CrossRef] | [PubMed]
 
Flint J, Goldstein LH: Familial calcification of the basal ganglia: a case report and review of the literature. Psychol Med  1992; 22:581—595
[CrossRef] | [PubMed]
 
Rasmussen MJ, Pilo L, Nielsen HR: [Basal ganglia calcifications demonstrated by CT. Is further investigation necessary when this is found incidentally?] Ugeskr Laeger  1991; 153:2051—2053
 
Nishiyama K, Honda E, Mizuno T, et al: [A case of idiopathic, symmetrical non-arteriosclerotic, intracerebral calcification (Fahr's disease) associated with M-proteinemia, followed by multiple myeloma.] Rinsho Shinkeigaku  1991; 31:781—784
[PubMed]
 
Manyam BV, Bhatt MH, Moore WD, et al: Bilateral striopallidodentate calcinosis: cerebrospinal fluid, imaging, and electrophysiological studies. Ann Neurol  1992; 31:379—384
[CrossRef] | [PubMed]
 
Lauterbach EC: Psychiatric management in Fahr's syndrome, in Psychiatric Management in Neurological Diseases—Major Basal Ganglia—Cortical Disorders, edited by Lauterbach EC. Washington, DC, American Psychiatric Press (in press)
 
Murphy MJ: Clinical correlations of CT scan-detected calcifications of the basal ganglia. Ann Neurol  1979; 6:507—511
[CrossRef] | [PubMed]
 
Vles JS, Lodder J, van der Lugt PJ: Clinical significance of basal ganglia calcifications detected by CT (a retrospective study of 33 cases). Clin Neurol Neurosurg  1981; 83:253—256
[CrossRef] | [PubMed]
 
Harrington MG, MacPherson P, McIntosh WB, et al: The significance of the incidental finding of basal ganglia calcification on computed tomography. J Neurol Neurosurg Psychiatry  1981; 44:1168—1170
[CrossRef] | [PubMed]
 
Stellamor K, Stellamor V: [Roentgen diagnosis of Fahr's disease.] Roentgenblatter  1983; 36:194—196
 
Kazis AD: Contribution of CT scan to the diagnosis of Fahr's syndrome. Acta Neurol Scand  1985; 71:206—211
[CrossRef] | [PubMed]
 
Konig P: Psychopathological alterations in cases of symmetrical basal ganglia sclerosis. Biol Psychiatry  1989; 25:459—468
[CrossRef] | [PubMed]
 
Kendall B, Cavanagh N: Intracranial calcification in pediatric computed tomography. Neuroradiology  1986; 28:324—330
[CrossRef] | [PubMed]
 
Taxer F, Haller R, Konig P: [Clinical early symptoms and CT findings in Fahr syndrome.] Nervenarzt  1986; 57:583—588
[PubMed]
 
López-Villegas D, Kulisevsky J, Deus J, et al: Neuropsychological alterations in patients with computed tomography—detected basal ganglia calcification. Arch Neurol  1996; 53:251—256
[PubMed]
 
Adachi M, Hosoya T, Yamaguchi K: [Non-calcified line in calcification of the globus pallidus.] Nippon Igaku Hoshasen Gakkai Zasshi  1994; 54:1347—1351
[PubMed]
 
Bruyn GW, Bots GT, Staal A: Familial bilateral vascular calcification in the central nervous system. Psychiatry Neurol Neurochir  1964; 67:342—376
 
Kobayashi S, Yamadori I, Miki H, et al: Idiopathic nonarteriosclerotic cerebral calcification (Fahr's disease): an electron microscopic study. Acta Neuropathol (Berl)  1987; 73:62—66
[CrossRef]
 
Spatz H: Über den eisennachweis im gehirn, besonders in zentren des extrapyramidal-motorischen systems [On demonstrating iron in the brain, especially in extrapyramidal motor system centers]. Zentralbl Gesamte Neurol Psychiatry  1922; 77:261
[CrossRef]
 
Bouras C, Giannakopoulos P, Good PF, et al: A laser microprobe mass analysis of trace elements in brain mineralizations and capillaries in Fahr's disease. Acta Neuropathol (Berl)  1996; 92:351—357
[CrossRef]
 
Danowski TS, Lasser EC, Wechsler RL: Calcification of basal ganglia in post-thyroidectomy hypoparathyroidism. Metabolism  1960; 9:1064—1065
[PubMed]
 
Basser LS, Neale FC, Ireland AW, et al: Epilepsy and electroencephalographic abnormalities in chronic surgical hypoparathyroidism. Ann Intern Med  1969; 71:507—515
[PubMed]
 
Brannan TS, Burger AA, Chaudhary MY: Bilateral basal ganglia calcifications visualised on CT scan. J Neurol Neurosurg Psychiatry  1980; 43:403—406
[CrossRef] | [PubMed]
 
Avrahami E, Cohn DF, Feibel M, et al: MRI demonstration and CT correlation of the brain in patients with idiopathic intracerebral calcification. J Neurol  1994; 241:381—384
[CrossRef] | [PubMed]
 
Kulczycki J, Boguslawska-Staniaszczyk R, Kozlowski P: [The image of intracerebral calcification in CT and MR studies: a case report of Fahr syndrome.] Neurol Neurochir Pol  1994; 28:915—920
[PubMed]
 
Uygur GA, Liu Y, Hellman RS, et al: Evaluation of regional cerebral blood flow in massive intracerebral calcifications. J Nucl Med  1995; 36:610—612
[PubMed]
 
Ogata A, Ishida S, Wada T: A survey of 37 cases with basal ganglia calcification (BGC): CT-scan findings of BGC and its relationship to underlying diseases and epilepsy. Acta Neurol Scand  1987; 75:117—124
[CrossRef] | [PubMed]
 
Schmid H, Haller R, Konig P: [Value of EEG in parathyroid gland disorders and/or symmetrical calcinosis of the basal ganglia (Fahr syndrome): review of the literature with personal cases.] Wien Klin Wochenschr  1986; 98:486—490
[PubMed]
 
Nakayama T, Sakakihara Y, Hanaoka S, et al: Calcification of basal ganglia in a patient with partial trisomy 5q and partial monosomy 18q. Acta Paediatr Jpn  1993; 35:340—344
[PubMed]
 
Okano S, Takeuchi Y, Kohmura E, et al: Globus pallidus calcification in Down syndrome with progressive neurologic deficits. Pediatr Neurol  1992; 8:72—74
[CrossRef] | [PubMed]
 
Mann DM: Calcification of the basal ganglia in Down's syndrome and Alzheimer's disease. Acta Neuropathol (Berl)  1988; 76:595—598
[CrossRef]
 
Takashima S, Becker LE: Basal ganglia calcification in Down's syndrome. J Neurol Neurosurg Psychiatry  1985; 48:61—64
[CrossRef] | [PubMed]
 
Thase ME: Basal ganglia calcification and psychosis in Down's syndrome. Postgrad Med J  1984; 60:137—139
[CrossRef] | [PubMed]
 
Wisniewski KE, French JH, Rosen JF, et al: Basal ganglia calcification (BGC) in Down's syndrome (DS): another manifestation of premature aging. Ann N Y Acad Sci  1982; 396:179—189
[CrossRef] | [PubMed]
 
Jakab I: Basal ganglia calcification and psychosis in mongolism. Eur Neurol  1978; 17:300—314
[CrossRef] | [PubMed]
 
Salva E, Savoldi F: Contributo alla conoscenzo della forme ereditarie della malattia di Fahr [Contribution to the understanding of the hereditary form of Fahr's disease]. Sist Nerv  1959; 11:46—73
[PubMed]
 
Strobos RRJ, De La Torre E, Martin JF: Symmetrical calcification of the basal ganglia with familial ataxia and pigmentary macular degeneration. Brain  1957; 80:313—318
[CrossRef] | [PubMed]
 
Puvanendran K, Low CH, Boey HK, et al: Basal ganglia calcification on computer tomographic scan: a clinical and radiological correlation. Acta Neurol Scand  1982; 66:309—315
[PubMed]
 
Francis AF: Familial basal ganglia calcification and schizophreniform psychosis. Br J Psychiatry  1979; 79:360—362
 
Francis A, Freeman H: Psychiatric abnormality and brain calcification over four generations. J Nerv Ment Dis  1984; 172:166—170
[CrossRef] | [PubMed]
 
Martinelli P, Giuliani S, Ippoliti M, et al: Familial idiopathic strio-pallido-dentate calcifications with late onset extrapyramidal syndrome. Mov Disord  1993; 8:220—222
[CrossRef] | [PubMed]
 
Tokoro K, Chiba Y, Ohtani T, et al: Pineal ganglioglioma in a patient with familial basal ganglia calcification and elevated serum alpha-fetoprotein: case report. Neurosurgery  1993; 33:506—511
[CrossRef] | [PubMed]
 
Billard C, Dulac O, Bouloche J, et al: Encephalopathy with calcifications of the basal ganglia in children: a reappraisal of Fahr's syndrome with respect to 14 new cases. Neuropediatrics  1989; 20:12—19
[CrossRef] | [PubMed]
 
Rysz A, Gajkowski K, Empel A: [Familial occurrence of calcinosis of the basal ganglia.] Neurol Neurochir Pol  1988; 22:459—462
[PubMed]
 
Frydman M, Bar-Ziv J, Preminger-Shapiro R, et al: Possible heterogeneity in spondyloenchondrodysplasia: quadriparesis, basal ganglia calcifications, and chondrocyte inclusions. Am J Med Genet  1990; 36:279—284
[CrossRef] | [PubMed]
 
Rossi M, Morena M, Zanardi M: [Calcification of the basal ganglia and Fahr disease: report of two clinical cases and review of the literature.] Recenti Prog Med  1993; 84:192—198
[PubMed]
 
Ellie E, Julien J, Ferrer X: Familial idiopathic striopallidodentate calcifications. Neurology  1989; 39:381—385
[PubMed]
 
Garcia Urra D, Barquero Jimenez MS, Varela de Seijas E, et al: [Calcification of the basal ganglia and hypoparathyroidism: Fahr disease. Study of a family.] Arch Neurobiol (Madr)  1990; 53:18—22
[PubMed]
 
Yokota S, Mori T, Kosuge K, et al: [Basal ganglia calcification in two children with systemic lupus erythematosus and neuropsychiatric manifestations.] Ryumachi  1985; 25:115—122
[PubMed]
 
Nordstrom DM, West SG, Andersen PA: Basal ganglia calcifications in central nervous system lupus erythematosus. Arthritis Rheum  1985; 28:1412—1416
[CrossRef] | [PubMed]
 
Legido A, Zimmerman RA, Packer RJ, et al: Significance of basal ganglia calcification on computed tomography in children. Pediatr Neurosci  1988; 14:64—70
[CrossRef] | [PubMed]
 
Eleopra R, Accurti I, Neri W, et al: Unusual case of Fahr syndrome with motoneuron disease. Ital J Neurol Sci  1991; 12:597—600
[CrossRef] | [PubMed]
 
Kusunose Y, Taniguchi T, Yamada M, et al: [Two siblings of myotonic muscular dystrophy associated with basal ganglia calcification.] Rinsho Shinkeigaku  1987; 27:1276—1279
[PubMed]
 
Markesbery WR: Lactic acidemia, mitochondrial myopathy, and basal ganglia calcification. Neurology  1979; 29:1057—1060
[PubMed]
 
Robertson WC Jr, Viseskul C, Lee YE, et al: Basal ganglia calcification in Kearns-Sayre syndrome. Arch Neurol  1979; 36:711—713
[PubMed]
 
Carboni P, Giacanelli M, Porro G, et al: Kearns-Sayre syndrome: a case of the complete syndrome with encephalic leukodystrophy and calcification of basal ganglia. Ital J Neurol Sci  1981; 2:263—268
[CrossRef] | [PubMed]
 
Grotemeyer KH, Lehmann HJ, Jörg J, et al: [Familial basal ganglia calcinosis, mitochondrial myopathy and epilepsy: result of a single metabolic disorder?] Nervenarzt  1984; 55:202—207
 
Yoda S, Terauchi A, Kitahara F, et al: Neurologic deterioration with progressive CT changes in a child with Kearns-Shy syndrome. Brain Dev  1984; 6:323—327
[PubMed]
 
Pavlakis SG, Phillips PC, Di Mauro S, et al: Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes: a distinctive syndrome. Ann Neurol  1984; 16:481—488
[CrossRef] | [PubMed]
 
Bastiaensen LAK, Stadhouders AM, Ter Laak HJ, et al: Kearns-Sayre syndrome. Neuro-Ophthalmology  1984; 4:55—63
[CrossRef]
 
Federico A, Cornelio F, Di Donato S, et al: Mitochondrial encephalo-neuro-myopathy with myoclonus epilepsy, basal nuclei calcification and hyperlactacidemia. Ital J Neurol Sci  1988; 9:65—71
[PubMed]
 
Mousa AM, Muhtaseb SA, Reddy RR, et al: The high rate of prevalence of CT-detected basal ganglia calcification in neuropsychiatric (CNS) brucellosis. Acta Neurol Scand  1987; 76:448—456
[CrossRef] | [PubMed]
 
Fenelon G, Gray F, Paillard F, et al: A prospective study of patients with CT detected pallidal calcifications. J Neurol Neurosurg Psychiatry  1993; 56:622—625
[CrossRef] | [PubMed]
 
Arranz Perez M, Ergueta Martin P, Gonzalez Sarmiento E, et al: [Fahr's disease and hypocalcemic syndromes: presentation of a clinical case.] An Med Interna  1992; 9:495—497
[PubMed]
 
Arias Mayorga J, Gonzalez Martin T, Escorial Miguel C, et al: [Intracranial calcifications in the differential diagnosis of epileptic disease.] Rev Clin Esp  1991; 189:425—427
[PubMed]
 
Chiu HF, Lam LC, Shum PP, et al: Idiopathic calcification of the basal ganglia. Postgrad Med J  1993; 69:68—70
[CrossRef] | [PubMed]
 
Lauterbach EC, Spears TE, Prewett MJ, et al: Neuropsychiatric disorders, myoclonus, and dystonia in calcification of basal ganglia pathways. Biol Psychiatry  1994; 35:345—351
[CrossRef] | [PubMed]
 
Trenkwalder C, Schwarz J, Gebhard J, et al: Starnberg trial on epidemiology of parkinsonism and hypertension in the elderly: prevalence of Parkinson's disease and related disorders assessed by a door-to-door survey of inhabitants older than 65 years. Arch Neurol  1995; 52:1017—1022
[PubMed]
 
Matsui K, Yamada M, Kobayashi T, et al: [An autopsy case of Fahr disease (infantile form).] No To Hattatsu  1992; 24:358—363
[PubMed]
 
Tolmie JL, Shillito P, Hughes-Benzie R, et al: The Aicardi-Goutieres syndrome (familial, early onset encephalopathy with calcifications of the basal ganglia and chronic cerebrospinal fluid lymphocytosis). J Med Genet  1995; 32:881—884
[CrossRef] | [PubMed]
 
Rosenberg DR, Neylan TC, El-Alwar M, et al: Neuropsychiatric symptoms associated with idiopathic calcification of the basal ganglia. J Nerv Ment Dis  1991; 179:48—49
[CrossRef] | [PubMed]
 
Cummings JL: Clinical Neuropsychiatry. Orlando, FL, Grune and Stratton, 1985, p 154
 
Seidler GH: [Psychiatric and psychological aspects of Fahr syndrome.] Psychiatr Prax  1985; 12:203—205
[PubMed]
 
Shibayama H, Kobayashi H, Nakagawa M, et al: Non-Alzheimer non-Pick dementia with Fahr's syndrome. Clin Neuropathol  1992; 11:237—250
[PubMed]
 
Kosaka K: Diffuse neurofibrillary tangles with calcification: a new presenile dementia. J Neurol Neurosurg Psychiatry  1994; 57:594—596
[CrossRef] | [PubMed]
 
Cummings JL, Gosenfeld LF, Houlihan JP, et al: Neuropsychiatric disturbances associated with idiopathic calcification of the basal ganglia. Biol Psychiatry  1983; 18:591—601
[PubMed]
 
Wodarz N, Becker T, Deckert J: Musical hallucinations associated with post-thyroidectomy hypoparathyroidism and symmetric basal ganglia calcifications (letter). J Neurol Neurosurg Psychiatry  1995; 58:763—764
[CrossRef] | [PubMed]
 
Fernandez-Bouzas A, Angrist B, Hemdal P, et al: Basal ganglia calcification in schizophrenia (letter). Biol Psychiatry  1990; 27:682—685
[CrossRef] | [PubMed]
 
Forstl H, Krumm B, Eden S, et al: Neurological disorders in 166 patients with basal ganglia calcification: a statistical evaluation. J Neurol  1992; 239:36—38
[CrossRef] | [PubMed]
 
Forstl H, Krumm B, Eden S, et al: What is the psychiatric significance of bilateral basal ganglia mineralization? Biol Psychiatry  1991; 29:827—833
 
Chow KS, Lu DN: [Primary hypoparathyroidism with basal ganglia calcification: report of a case.] Acta Paediatr Sin  1989; 30:129—133
[PubMed]
 
Abe S, Tojo K, Ichida K, et al: A rare case of idiopathic hypoparathyroidism with varied neurological manifestations. Intern Med  1996; 35:129—134
[CrossRef] | [PubMed]
 
Gubbay SS, Hankey GJ, Tan NT, et al: Mitochondrial encephalomyopathy with corticosteroid dependence. Med J Aust  1989; 151:100—103
[PubMed]
 
Skvortsov IA, Rudenskaia GE, Karaseva AN, et al: [Effectiveness of the therapeutic use of complexones in various diseases of the extrapyramidal system in children.] Zh Nevropatol Psikhiatr  1987; 87:1457—1462
[PubMed]
 
Vermersch P, Leys D, Pruvo JP, et al: Parkinson's disease and basal ganglia calcifications: prevalence and clinico-radiological correlations. Clin Neurol Neurosurg  1992; 94:213—217
[CrossRef] | [PubMed]
 
Vaamonde J, Legarda I, Jimenez-Jimenez J, et al: Levodopa-responsive parkinsonism associated with basal ganglia calcification and primary hypoparathyroidism (letter). Mov Disord  1993; 8:398—400
[CrossRef] | [PubMed]
 
Tambyah PA, Ong BK, Lee KO: Reversible parkinsonism and asymptomatic hypocalcemia with basal ganglia calcification from hypoparathyroidism 26 years after thyroid surgery. Am J Med  1993; 94:444—445
[CrossRef] | [PubMed]
 
Uncini A, Tartaro A, Di Stefano E, et al: Parkinsonism, basal ganglia calcification and epilepsy as late complications of postoperative hypoparathyroidism. J Neurol  1985; 232:109—111
[CrossRef] | [PubMed]
 
Munir KM: The treatment of psychotic symptoms in Fahr's disease with lithium carbonate. J Clin Psychopharmacol  1986; 6:36—38
[PubMed]
 
+

References

Nauta WJ: Limbic innervation of the striatum. Adv Neurol  1982; 35:41—47
[PubMed]
 
Haber SN, Groenewegen HJ, Grove EA, et al: Efferent connections of the ventral pallidum: evidence of a dual striato pallidofugal pathway. J Comp Neurol  1985; 235:322—335
[CrossRef] | [PubMed]
 
Alexander GE, DeLong MR, Strick PL: Parallel organization of functionally segregated circuits linking basal ganglia and cortex. Annu Rev Neurosci  1986; 9:357—381
[CrossRef] | [PubMed]
 
Cummings JL: Frontal subcortical circuits and human behavior. Arch Neurol  1993; 50:873—880
[PubMed]
 
Robinson RG: Psychiatric management in stroke, in Psychiatric Management in Neurological Diseases—Major Basal Ganglia—Cortical Disorders, edited by Lauterbach EC. Washington, DC, American Psychiatric Press (in press)
 
Caplan LR, Schmahmann JD, Kase CS, et al: Caudate infarcts. Arch Neurol  1990; 47:133—143
[PubMed]
 
Huntington's Disease Collaborative Research Group: A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell  1993; 72:917—983
 
MacDonald ME, Gusella JF: Huntington's disease: translating a CAG repeat into a pathogenic mechanism. Curr Opin Neurobiol  1996; 6:638—643
[CrossRef] | [PubMed]
 
Folstein SE, Chase GA, Wahl WE, et al: Huntington disease in Maryland: clinical aspects of racial variation. Am J Hum Genet  1987; 41:168—179
[PubMed]
 
Duyao M, Ambrose C, Myers R, et al: Trinucleotide repeat length instability and age of onset in Huntington's disease. Nat Genet  1993; 4:387—392
[CrossRef] | [PubMed]
 
Kremer B, Goldberg P, Andrew SE, et al: A worldwide study of the Huntington's disease mutation: the sensitivity and specificity of measuring CAG repeats. N Engl J Med  1994; 330:1401—1406
[CrossRef] | [PubMed]
 
Sieradzan K, Mann DM, Dodge A: Clinical presentation and patterns of regional cerebral atrophy related to the length of trinucleotide repeat expansion in patients with adult onset Huntington's disease. Neurosci Lett  1997; 28:45—48
 
Folstein SE: Huntington's Disease: A Disorder of Families. Baltimore, The Johns Hopkins University Press, 1989
 
Jones AL, Wood JD, Harper PS: Huntington disease: advances in molecular and cell biology. J Inherit Metab Dis  1997; 20:125—138
[CrossRef] | [PubMed]
 
Gusella JF, MacDonald ME: Huntington's disease: CAG genetics expands neurobiology. Curr Opin Neurobiol  1995; 5:656—662
[CrossRef] | [PubMed]
 
Li X-J, Li S-H, Sharp AH, et al: A Huntington-associated protein enriched in brain with implications for pathology. Nature  1995; 378:398—402
[CrossRef] | [PubMed]
 
Ross CA, Becher MW, Colomer V, et al: Huntington's disease and dentatorubral-pallidoluysian atrophy: proteins, pathogenesis and pathology. Brain Pathol  1997; 7:1003—1016
[CrossRef] | [PubMed]
 
Nasir J, Goldberg YP, Hayden MR: Huntington disease: new insights into the relationship between CAG expansion and disease. Hum Mol Genet 1996; 5(special number):1431—1435
 
Beal MF, Ferrante RJ, Swartz KJ, et al: Chronic quinolinic acid lesions in rats closely resemble Huntington's disease. J Neurosci  1991; 11:1649—1659
[PubMed]
 
Brouillet E, Hantraye P, Ferrante RJ, et al: Chronic mitochondrial energy impairment produces selective striatal degeneration and abnormal choreiform movements in primates. Proc Natl Acad Sci USA  1995; 92:7105—7109
[CrossRef] | [PubMed]
 
Wellington CL, Brinkman RR, O'Kusky JR, et al: Toward understanding the molecular pathology of Huntington's disease. Brain Pathol  1997; 7:979—1002
[CrossRef] | [PubMed]
 
Roos RAC: Neuropathology of Huntington's chorea, in Handbook of Clinical Neurology, vol 5(49): Extrapyramidal Disorders, edited by Vinken PJ, Bruyn GW, Klawans HL. Amsterdam, Elsevier Science, 1986, pp 315—326
 
Nagel JS, Ichise M, Holman BL: The scintigraphic evaluation of Huntington's disease and other movement disorders using single photon emission computed tomography perfusion brain scans. Semin Nucl Med  1991; 21:11—23
[CrossRef] | [PubMed]
 
Turjanski N, Weeks R, Dolan R, et al: Striatal D1 and D2 receptor binding in patients with Huntington's disease and other choreas: a PET study. Brain  1995; 118:689—696
[CrossRef] | [PubMed]
 
Aylward EH, Anderson NB, Bylsma FW, et al: Frontal lobe volume in patients with Huntington's disease. Neurology  1998; 50:252—258
[PubMed]
 
Brandt J, Bylsma FW, Gross R, et al: Trinucleotide repeat length and clinical progression in Huntington's disease. Neurology  1996; 46:527—531
[PubMed]
 
Kieburtz K, MacDonald M, Shih C, et al: Trinucleotide repeat length and progression of illness in Huntington's disease. J Med Genet  1994; 31:872—874
[CrossRef] | [PubMed]
 
Brandt J, Quaid KA, Folstein SE, et al: Presymptomatic diagnosis of delayed onset disease with linked DNA markers: the experience in Huntington's disease. JAMA  1989; 261:3108—3114
[CrossRef] | [PubMed]
 
Wiggins S, Whyte P, Huggins M, et al: The psychological consequences of predictive testing for Huntington's disease. N Engl J Med  1992; 327:1401—1405
[CrossRef] | [PubMed]
 
Wexler NS: The Tiresias complex: Huntington's disease as a paradigm for testing for late-onset disorders. FASEB J  1992; 6:2820—2825
[PubMed]
 
International Huntington Association and World Federation of Neurology Research Group on Huntington's Chorea: Guidelines for the molecular genetics predictive test in Huntington's disease. Neurology  1992; 44:1533—1536
 
Hayden MR, Bloch M, Wiggins S: Psychological effects of predictive testing for Huntington's disease. Adv Neurol  1995; 65:201—210
[PubMed]
 
Folstein SE, Jensen B, Leigh RJ, et al: The measurement of abnormal movement: methods developed for Huntington's disease. Neurobehav Toxicol Teratol  1983; 5:605—609
[PubMed]
 
Huntington Study Group: The Unified Huntington Disease Rating Scale: reliability and consistency. Mov Disord  1996; 4:136—142
 
Albin RL, Reiner A, Anderson KD, et al: Striatal and nigral neuron subpopulations in rigid Huntington's disease: implications for the functional anatomy of chorea and rigidity-akinesia. Ann Neurol  1990; 27:357—365
[CrossRef] | [PubMed]
 
Pearson SJ, Heathfield KW, Reynolds GP: Pallidal GABA and chorea in Huntington's disease. J Neural Transm Gen Sect  1990; 81:241—246
[CrossRef] | [PubMed]
 
Penney JB Jr, Young AB, Shoulson I, et al: Huntington's disease in Venezuela: 7 years of follow-up on symptomatic and asymptomatic individuals. Mov Disord  1990; 5:93—99
[CrossRef] | [PubMed]
 
Brandt J, Strauss ME, Larus J, et al: Clinical correlates of dementia and disability in Huntington's disease. J Clin Neuropsychol  1984; 6:401—412
[CrossRef] | [PubMed]
 
Morris M: Psychiatric aspects of Huntington's disease, in Huntington's Disease, edited by Harper PS. Philadelphia, WB Saunders, 1991, pp 81—126
 
Ranen NG: Psychiatric management in Huntington's disease, in Psychiatric Management in Neurological Diseases—Major Basal Ganglia—Cortical Disorders, edited by Lauterbach EC. Washington, DC, American Psychiatric Press (in press)
 
Mayeux R, Stern Y, Herman A, et al: Correlates of early disability in Huntington's disease. Ann Neurol  1986; 20:727—731
[CrossRef] | [PubMed]
 
Brandt J: Cognitive investigations in Huntington's disease, in Neuropsychological Explorations of Memory and Cognition: Essays in Honor of Nelson Butters, edited by Cermak LS. New York, Plenum, 1994, pp 4135—4136
 
Brandt J, Folstein SE, Folstein MF: Differential cognitive impairment in Alzheimer's disease and Huntington's disease. Ann Neurol  1988; 23:555—561
[CrossRef] | [PubMed]
 
Butters N, Wolfe J, Granholm E, et al: An assessment of verbal recall, recognition and fluency abilities in patients with Huntington's disease. Cortex  1986; 22:11—32
[PubMed]
 
Brandt J, Corwin J, Krafft L: Is verbal recognition memory really different in Huntington's and Alzheimer's disease? J Clin Exp Neuropsychol  1992; 14:773—784
 
Morris M: Dementia and cognitive changes in Huntington's disease. Adv Neurol  1995; 65:187—200
[PubMed]
 
Reynolds GP, Pearson SJ: Neurochemical-clinical correlates in Huntington's disease: applications of brain banking techniques. J Neural Transm Suppl  1993; 39:207—214
[PubMed]
 
Cummings JL: Behavioral and psychiatric symptoms associated with Huntington's disease. Adv Neurol  1995; 65:179—186
[PubMed]
 
McHugh PR, Folstein MR: Psychiatric syndromes of Huntington's chorea: a clinical and phenomenological study, in Psychiatric Aspects of Neurologic Disease, edited by Benson DF, Blumer D. New York, Grune and Stratton, 1975, pp 267—286
 
Shiwach R: Psychopathology in Huntington's disease patients. Acta Psychiatr Scand  1994; 90:241—246
[CrossRef] | [PubMed]
 
Burns A, Folstein SE, Brandt J, et al: Clinical assessment of irritability, aggression, and apathy in Huntington and Alzheimer disease. J Nerv Ment Dis  1990; 178:20—26
[CrossRef] | [PubMed]
 
Kuwert T, Lange HW, Langen K-J, et al: Cerebral glucose consumption measured by PET in patients with and without psychiatric symptoms of Huntington's disease. Psychiatry Res  1989; 29:361—362
[CrossRef] | [PubMed]
 
Caine ED, Shoulson I: Psychiatric syndromes in Huntington's disease. Am J Psychiatry  1983; 140:728—733
[PubMed]
 
Bolt JMW: Huntington's chorea in the west of Scotland. Br J Psychiatry  1970; 116:259—270
[CrossRef] | [PubMed]
 
Baxter LR, Mazziotta JC, Pahl JJ, et al: Psychiatric, genetic, and positron emission tomographic evaluation of persons at risk for Huntington's disease. Arch Gen Psychiatry  1992; 49:148—154
[PubMed]
 
Dewhurst K, Oliver J, Trick KLK, et al: Neuro-psychiatric aspects of Huntington's disease. Confin Neurol  1969; 31:258—268
[CrossRef] | [PubMed]
 
Shiwach RS, Patel V: Aggressive behaviour in Huntington's disease: a cross-sectional study in a nursing home population. Behav Neurol  1993; 6:43—47
 
Dewhurst K, Oliver JE, McKnight AL: Socio-psychiatric consequences of Huntington's disease. Br J Psychiatry  1970; 116:255—258
[CrossRef] | [PubMed]
 
Rosenbaum D: Psychosis with Huntington's chorea. Psychiatr Q  1941; 15:93—99
[CrossRef]
 
Beckson M, Cummings JL: Psychosis in basal ganglia disorders. Neuropsychiatry Neuropsychol Behav Neurol  1992; 5:126—131
 
Vonsattel J-P, Myers RH, Stevens TJ, et al: Neuropathological classification of Huntington's disease. J Neuropathol Exp Neurology  1985; 44:559—577
[CrossRef]
 
Ranen NG, Peyser CE, Folstein SE: A Physician's Guide to the Management of Huntington's Disease: Pharmacologic and Non-Pharmacologic Interventions, New York, HDSA, 1993
 
Mayberg HS, Starkstein SE, Peyser CE, et al: Paralimbic frontal lobe hypometabolism in depression associated with Huntington's disease. Neurology  1992; 42:1791—1797
[PubMed]
 
Peyser CE, Folstein SE: Huntington's disease as a model for mood disorders: clues from neuropathology and neurochemistry. Mol Chem Neuropathol  1990; 12:99—119
[CrossRef] | [PubMed]
 
Schoenfeld M, Myers RH, Cupples LA, et al: Increased rate of suicide among patients with Huntington's disease. J Neurol Neurosurg Psychiatry  1984; 47:1283—1287
[CrossRef] | [PubMed]
 
Lipe H, Schultz A, Bird TD: Risk factors for suicide in Huntington's disease: a retrospective case controlled study. Am J Med Genet  1993; 48:231—233
[CrossRef] | [PubMed]
 
Pflanz S, Besson JA, Ebmeier KP, et al: The clinical manifestation of mental disorder in Huntington's disease: a retrospective case record study of disease progression. Acta Psychiatr Scand  1991; 83:53—60
[CrossRef] | [PubMed]
 
Cummings JL, Cunningham K: Obsessive-compulsive disorder in Huntington's disease. Biol Psychiatry  1992; 31:263—270
[CrossRef] | [PubMed]
 
Watt DC, Seller A: A clinico-genetic study of psychiatric disorder in Huntington's chorea. Psychol Med Suppl  1993; 23:1—46
[CrossRef]
 
Fedoroff JP, Peyser C, Franz ML, et al: Sexual disorders in Huntington's disease. J Neuropsychiatry Clin Neurosci  1994; 6:147—153
[PubMed]
 
Young AB: Role of excitotoxins in heredito-degenerative neurologic disease. Res Publ Assoc Res Nerv Ment Dis  1993; 71:175—189
[PubMed]
 
Shoulson I, Odoroff C, Oakes D, et al: A controlled trial of baclofen as protective therapy in early Huntington's disease. Ann Neurol  1989; 25:252—259
[CrossRef] | [PubMed]
 
Ranen NG, Peyser CE, Coyle JT, et al: A controlled trial of idebenone in Huntington's disease. Mov Disord 1996;11:549—554
 
Peyser CE, Folstein M, Chase GA, et al: Trial of d-α-tocopherol in Huntington's disease. Am J Psychiatry  1995; 152:1771—1775
[PubMed]
 
Koroshetz WJ, Jenkins BG, Rosen BR, et al: Energy metabolism defects in Huntington's disease and effects of coenzyme Q10. Ann Neurol  1997; 41:160—165
[CrossRef] | [PubMed]
 
Emerich DF, Winn SR, Hantraye PM, et al: Protective effect of encapsulated cells producing neurotrophic factor CNTF in a monkey model of Huntington's disease. Nature  1997; 386:395—399
[CrossRef] | [PubMed]
 
Barr AN, Fischer JH, Koller WC, et al: Serum haloperidol concentration and choreiform movements in Huntington's disease. Neurology  1988; 38:84—88
[PubMed]
 
Albanese A, Cassetta E, Carretta D, et al: Acute challenge with apomorphine in Huntington's disease: a double-blind study. Clin Neuropharmacol  1995; 18:427—434
[CrossRef] | [PubMed]
 
van Vugt JP, Siesling S, Vergeer M, et al: Clozapine versus placebo in Huntington's disease: a double blind randomised comparative study. J Neurol Neurosurg Psychiatry  1997; 63:35—39
[CrossRef] | [PubMed]
 
Mateo D, Gimenez-Roldan S: [The effect of piracetam on involuntary movements in Huntington's disease: a double-blind, placebo-controlled study.] Neurologia  1996; 11:16—19
[PubMed]
 
Consroe P, Laguna J, Allender J, et al: Controlled clinical trial of cannabidiol in Huntington's disease. Pharmacol Biochem Behav  1991; 40:701—708
[CrossRef] | [PubMed]
 
Giuffra ME, Mouradian MM, Chase TN: Glutamatergic therapy of Huntington's chorea. Clin Neuropharmacol  1992; 15:148—151
[CrossRef] | [PubMed]
 
Como PG, Rubin AJ, O'Brien CF, et al: A controlled trial of fluoxetine in nondepressed patients with Huntington's disease. Mov Disord  1997; 12:397—401
[CrossRef] | [PubMed]
 
Murman DL, Giordani B, Mellow AM, et al: Cognitive, behavioral, and motor effects of the NMDA antagonist ketamine in Huntington's disease. Neurology  1997; 49:153—161
[PubMed]
 
Ranen NG, Lipsey JR, Treisman G, et al: Sertraline in the treatment of severe aggressiveness in Huntington's disease. J Neuropsychiatry Clin Neurosci  1996; 8:338—340
[PubMed]
 
Stewart JT: Huntington's disease and propranolol. Am J Psychiatry  1993; 150:166—167
[PubMed]
 
Byrne A, Martin W, Hnatko G: Beneficial effects of buspirone therapy in Huntington's disease (letter). Am J Psychiatry  1994; 151:1097
 
Findling RL: Treatment of aggression in juvenile-onset Huntington's disease with buspirone (letter). Psychosomatics  1993; 34:460—461
[PubMed]
 
Sajatovic M, Verbanac P, Ramirez LF, et al: Clozapine treatment of psychiatric symptoms resistant to neuroleptic treatment in patients with Huntington's chorea. Neurology  1990; 41:156
 
Bonucelli U, Ceravolo R, Maremmani C, et al: Clozapine in Huntington's chorea. Neurology  1994; 44:821—823
[PubMed]
 
Korenyi C, Whittier JR: Drug treatment in 117 cases of Huntington's disease with special reference to fluphenazine (Prolixin). Psychiatr Q  1967; 41:203—210
[CrossRef] | [PubMed]
 
Ringel SP, Guthrie M, Klawans HL: Current treatment of Huntington's chorea. Adv Neurol  1973; 1:797—801
 
Whittier J, Haydu G, Crawford J: Effect of imipramine (Tofranil) on depression and hyperkinesia in Huntington's disease. Am J Psychiatry  1961; 118:79
[PubMed]
 
Ford MF: Treatment of depression in Huntington's disease with monoamine oxidase inhibitors. Br J Psychiatry  1986; 149:654—656
[CrossRef] | [PubMed]
 
Patel SV, Tariot PN, Asnis J: l-Deprenyl augmentation of fluoxetine in a patient with Huntington's disease. Ann Clin Psychiatry  1996; 8:23—26
[CrossRef] | [PubMed]
 
Ranen NG, Peyser CE, Folstein SE: ECT as a treatment for depression in Huntington's disease. J Neuropsychiatry Clin Neurosci  1994; 6:154—159
[PubMed]
 
Rich SS, Ovsiew F: Leuprolide acetate for exhibitionism in Huntington's disease. Mov Disord  1994; 9:353—357
[CrossRef] | [PubMed]
 
Lauterbach EC: Family management in neurological disorders, in Psychiatric Management in Neurological Diseases—Major Basal Ganglia—Cortical Disorders, edited by Lauterbach EC. Washington, DC, American Psychiatric Press (in press)
 
Frydman M: Genetic aspects of Wilson's disease. J Gastroenterol Hepatol  1990; 5:483—490
[CrossRef] | [PubMed]
 
Chowrimootoo GF, Andoh J, Seymour CA: Western blot analysis in patients with hypocaeruloplasminaemia. Q J Med  1997; 90:197—202
 
Mansouri A, Gaou I, Fromenty B, et al: Premature oxidative aging of hepatic mitochondrial DNA in Wilson's disease. Gastroenterology  1997; 113:599—605
[CrossRef] | [PubMed]
 
Nanji MS, Nguyen VT, Kawasoe JH, et al: Haplotype and mutation analysis in Japanese patients with Wilson disease. Am J Hum Genet  1997; 60:1423—1429
[CrossRef] | [PubMed]
 
Walshe JM: Copper: not too little, not too much, but just right. Based on the triennial Pewterers Lecture delivered at the National Hospital for Neurology, London, 23 March 1995. J R Coll Physicians Lond  1995; 19:280—288
 
Houwen RH, Juyn J, Hoogenraad TU, et al: H714Q mutation in Wilson disease is associated with late, neurological presentation. J Med Genet  1995; 32:480—482
[CrossRef] | [PubMed]
 
Hoogenraad TU, Houwen RHJ: Prevalence and genetics, in Wilson's Disease, edited by Hoogenraad TU. London, WB Saunders, 1996, pp 14—24
 
Chu NS, Hung TP: Geographic variations in Wilson's disease. J Neurol Sci  1993; 117:1—7
[CrossRef] | [PubMed]
 
Rodman R, Burnstine M, Esmaeli B, et al: Wilson's disease: presymptomatic patients and Kayser-Fleischer rings. Ophthalmic Genet  1997; 18:79—85
[CrossRef] | [PubMed]
 
Esmaeli B, Burnstine MA, Martonyi CL, et al: Regression of Kayser-Fleischer rings during oral zinc therapy: correlation with systemic manifestations of Wilson's disease. Cornea  1996; 15:582—588
[PubMed]
 
Magalhaes AC, Caramelli P, Menezes JR, et al: Wilson's disease: MRI with clinical correlation. Neuroradiology  1994; 36:97—100
[CrossRef] | [PubMed]
 
Starosta-Rubinstein S, Young AB, Kluin K, et al: Clinical assessment of 31 patients with Wilson's disease: correlations with structural changes on magnetic resonance imaging. Arch Neurol  1987; 44:365—370
[PubMed]
 
Mochizuki H, Kamakura K, Masaki T, et al: Atypical MRI features of Wilson's disease: high signal in globus pallidus on T1-weighted images. Neuroradiology  1997; 39:171—174
[CrossRef] | [PubMed]
 
Saatci I, Topcu M, Baltaoglu FF, et al: Cranial MR findings in Wilson's disease. Acta Radiol  1997; 38:250—258
[PubMed]
 
King AD, Walshe JM, Kendall BE, et al: Cranial MR imaging in Wilson's disease. Am J Roentgenol  1996; 167:1579—1584
 
van Wassenaer-van Hall HN, van den Heuvel AG, Algra A, et al: Wilson disease: findings at MR imaging and CT of the brain with clinical correlation. Radiology  1996; 198:531—536
[PubMed]
 
Jardim LB, Carneiro A, Hansel S, et al: CT hypodensity on cerebral white matter in Wilson's disease. Arq Neuropsiquiatr  1991; 49:211—214
[CrossRef] | [PubMed]
 
Kuwert T, Hefter H, Scholz D, et al: Regional cerebral glucose consumption measured by positron emission tomography in patients with Wilson's disease. Eur J Nucl Med  1992; 19:96—101
[PubMed]
 
Schlaug G, Hefter H, Engelbrecht V, et al: Neurological impairment and recovery in Wilson's disease: evidence from PET and MRI. J Neurol Sci  1996; 136:129—139
[CrossRef] | [PubMed]
 
van Den Heuvel AG, Van der Grond J, Van Rooij LG, et al: Differentiation between portal-systemic encephalopathy and neurodegenerative disorders in patients with Wilson disease: H-1 MR spectroscopy. Radiology  1997; 203:539—543
[PubMed]
 
Oder W, Brucke T, Kollegger H, et al: Dopamine D2 receptor binding is reduced in Wilson's disease: correlation of neurological deficits with striatal 123I-iodobenzamide binding. J Neural Transm  1996; 103:1093—1103
[CrossRef] | [PubMed]
 
Roach ES, Ford CS, Spudis EV, et al: Wilson's disease: evoked potentials and computed tomography. J Neurol  1985; 232:20—23
[CrossRef] | [PubMed]
 
Maier-Dobersberger T, Mannhalter C, Rack S, et al: Diagnosis of Wilson's disease in an asymptomatic sibling by DNA linkage analysis. Gastroenterology  1995; 109:2015—2018
[CrossRef] | [PubMed]
 
Oder W, Grimm G, Kollegger H, et al: Neurological and neuropsychiatric spectrum of Wilson's disease: a prospective study of 45 cases. J Neurol  1991; 238:281—287
[PubMed]
 
Brewer GJ, Yuzbasiyan-Gurkan V: Wilson disease. Medicine (Baltimore)  1992; 71:139—164
[PubMed]
 
Arendt G, Hefter H, Stremmel W, et al: The diagnostic value of multi-modality evoked potentials in Wilson's disease. Electromyogr Clin Neurophysiol  1994; 34:137—148
[PubMed]
 
Dening TR, Berrios GE, Walshe JM: Wilson's disease and epilepsy. Brain  1988; 111:1139—1155
[CrossRef] | [PubMed]
 
Walshe JM, Yealland M: Wilson's disease: the problem of delayed diagnosis. J Neurol Neurosurg Psychiatry  1992; 55:692—696
[CrossRef] | [PubMed]
 
Akil M, Brewer GJ: Psychiatric and behavioral abnormalities in Wilson's disease. Adv Neurol  1995; 65:171—178
[PubMed]
 
Dening TR, Berrios GE: Wilson's disease. Psychiatric symptoms in 195 cases. Arch Gen Psychiatry  1989; 46:1126—1134
[PubMed]
 
Scheinberg IH, Sternlieb I: Wilson's disease, in Major Problems in Internal Medicine, vol 23, edited by Smith LH Jr. Philadelphia, WB Saunders, 1984
 
Dening TR, Berrios GE: Wilson's disease: a longitudinal study of psychiatric symptoms. Biol Psychiatry  1990; 28:255—265
[CrossRef] | [PubMed]
 
Rathbun JK: Neuropsychological aspects of Wilson's disease. Int J Neurosci  1996; 85:221—229
[CrossRef] | [PubMed]
 
Akil M, Schwartz JA, Dutchak D, et al: The psychiatric presentations of Wilson's disease. J Neuropsychiatry Clin Neurosci  1991; 3:377—382
[PubMed]
 
Knehr CA, Bearn AG: Psychological impairment in Wilson's disease. J Nerv Ment Dis  1956; 124:251—255
[CrossRef] | [PubMed]
 
Littman E, Medalia A, Senior G, et al: Rate of information processing in patients with Wilson's disease. J Neuropsychiatry Clin Neurosci  1995; 7:68—71
[PubMed]
 
Dening TR, Berrios GE: Wilson's disease: a prospective study of psychopathology in 31 cases. Br J Psychiatry  1989; 155:206—213
[CrossRef] | [PubMed]
 
Dening TR: Psychiatric aspects of Wilson's disease. Br J Psychiatry  1985; 147:677—682
[CrossRef] | [PubMed]
 
Oder W, Prayer L, Grimm G, et al: Wilson's disease: evidence of subgroups derived from clinical findings and brain lesions. Neurology  1993; 43:120—124
[PubMed]
 
Walshe JM, Yealland M: Chelation treatment of neurological Wilson's disease. Q J Med  1993; 86:197—204
[PubMed]
 
Schlaug G, Hefter H, Nebeling B, et al: Dopamine D2 receptor binding and cerebral glucose metabolism recover after d-penicillamine-therapy in Wilson's disease. J Neurol  1994; 241:577—584
[CrossRef] | [PubMed]
 
Selwa LM, Vanderzant CW, Brunberg JA, et al: Correlation of evoked potential and MRI findings in Wilson's disease. Neurology  1993; 43:2059—2064
[PubMed]
 
Grimm G, Oder W, Prayer L, et al: Evoked potentials in assessment and follow-up of patients with Wilson's disease. Lancet  1990; 336:963—964
[CrossRef] | [PubMed]
 
Nazer H, Brismar J, al-Kawi MZ, et al: Magnetic resonance imaging of the brain in Wilson's disease. Neuroradiology  1993; 35:130—133
[CrossRef] | [PubMed]
 
Goldstein NP, Ewert MA, Randall RV, et al: Psychiatric aspects of Wilson's disease (hepatolenticular degeneration): results of psychometric tests during long-term therapy. Am J Psychiatry  1968; 124:1155—1561
 
Kontaxakis V, Stefanis C, Markidis M, et al: Neuroleptic malignant syndrome in a patient with Wilson's disease (letter). J Neurol Neurosurg Psychiatry  1988; 51:1001—1002
[CrossRef] | [PubMed]
 
Rosselli M, Lorenzana P, Rosselli A, et al: Wilson's disease, a reversible dementia: case report. J Clin Exp Neuropsychol  1987; 9:399—406
[CrossRef] | [PubMed]
 
Schwarz J, Antonini A, Kraft E, et al: Treatment with d-penicillamine improves dopamine D2-receptor binding and T2-signal intensity in de novo Wilson's disease. Neurology  1994; 44:1079—1082
[PubMed]
 
McDonald LV, Lake CR: Psychosis in an adolescent patient with Wilson's disease: effects of chelation therapy. Psychosom Med  1995; 57:202—204
[PubMed]
 
Glass JD, Reich SG, DeLong MR: Wilson's disease: development of neurological disease after beginning penicillamine therapy. Arch Neurol  1990; 47:595—596
[PubMed]
 
Brewer GJ: Interactions of zinc and molybdenum with copper in therapy of Wilson's disease. Nutrition 1995; 11(suppl 1):114—116
 
Brewer GJ, Johnson V, Dick RD, et al: Treatment of Wilson disease with ammonium tetrathiomolybdate, II: initial therapy in 33 neurologically affected patients and follow-up with zinc therapy. Arch Neurol  1996; 53:1017—1025
[PubMed]
 
Lang CJ, Rabas-Kolominsky P, Engelhardt A, et al: Fatal deterioration of Wilson's disease after institution of oral zinc therapy. Arch Neurol  1993; 50:1007—1008
 
Heckmann JM, Eastman RW, De Villiers JC, et al: Wilson's disease: neurological and magnetic resonance imaging improvement on zinc treatment (letter). J Neurol Neurosurg Psychiatry  1994; 57:1273—1274
[CrossRef] | [PubMed]
 
Brewer GJ, Yuzbasiyan-Gurkan V, Johnson V, et al: Treatment of Wilson's disease with zinc, XI: interaction with other anticopper agents. J Am Coll Nutr  1993; 12:26—30
[PubMed]
 
Saito H, Watanabe K, Sahara M, et al: Triethylene-tetramine (trien) therapy for Wilson's disease. Tohoku J Exp Med  1991; 164:29—35
[CrossRef] | [PubMed]
 
Dahlman T, Hartvig P, Lofholm M, et al: Long-term treatment of Wilson's disease with triethylene tetramine dihydrochloride (trientine). Q J Med  1995; 88:609—616
 
Scheinberg IH, Sternlieb I: Wilson disease and idiopathic copper toxicosis. Am J Clin Nutr  1996; 63:842S—845S
 
Nunns D, Hawthorne B, Goulding P, et al: Wilson's disease in pregnancy. Eur J Obstet Gynecol Reprod Biol  1995; 62:141—143
[CrossRef] | [PubMed]
 
Schilsky ML, Scheinberg IH, Sternlieb I: Prognosis of Wilsonian chronic active hepatitis. Gastroenterology  1991; 100:762—767
[PubMed]
 
Santos Silva EE, Sarles J, Buts JP, et al: Successful medical treatment of severely decompensated Wilson disease. J Pediatr  1996; 128:285—287
[CrossRef] | [PubMed]
 
Rothfus WE, Hirsch WL, Malatack JJ, et al: Improvement of cerebral CT abnormalities following liver transplantation in a patient with Wilson disease. J Comput Assist Tomogr  1988; 12:138—140
[CrossRef] | [PubMed]
 
Bellary S, Hassanein T, Van Thiel DH: Liver transplantation for Wilson's disease. J Hepatol  1995; 23:373—381
[CrossRef] | [PubMed]
 
Schumacher G, Platz KP, Mueller AR, et al: Liver transplantation: treatment of choice for hepatic and neurological manifestation of Wilson's disease. Clin Transplant  1997; 11:217—224
[PubMed]
 
Lauterbach EC: Psychiatric management in Wilson's disease (progressive hepatolenticular degeneration), in Psychiatric Management in Neurological Diseases—Major Basal Ganglia—Cortical Disorders, edited by Lauterbach EC. Washington, DC, American Psychiatric Press (in press)
 
Hoogenraad TU: Wilson's Disease. London, WB Saunders, 1996
 
Negro PJ Jr, Louzã Neto MR: Results of ECT for a case of depression in Wilson's disease (letter). J Neuropsychiatry Clin Neurosci  1995; 7:384
[PubMed]
 
Lowenthal A: Striopallidodentate calcifications, in Handbook of Clinical Neurology, vol 5 (49): Extrapyramidal Disorders, edited by Vinken PJ, Bruyn GW, and Klawans HL. Amsterdam, Elsevier Science, 1986, pp 417—436
 
Trautner RJ, Cummings JL, Read SL, et al: Idiopathic basal ganglia calcification and organic mood disorder. Am J Psychiatry  1988; 145:350—353
[PubMed]
 
Beall SS, Patten BM, Mallette L, et al: Abnormal systemic metabolism of iron, porphyrin, and calcium in Fahr's syndrome. Ann Neurol  1989; 26:569—575
[CrossRef] | [PubMed]
 
Flint J, Goldstein LH: Familial calcification of the basal ganglia: a case report and review of the literature. Psychol Med  1992; 22:581—595
[CrossRef] | [PubMed]
 
Rasmussen MJ, Pilo L, Nielsen HR: [Basal ganglia calcifications demonstrated by CT. Is further investigation necessary when this is found incidentally?] Ugeskr Laeger  1991; 153:2051—2053
 
Nishiyama K, Honda E, Mizuno T, et al: [A case of idiopathic, symmetrical non-arteriosclerotic, intracerebral calcification (Fahr's disease) associated with M-proteinemia, followed by multiple myeloma.] Rinsho Shinkeigaku  1991; 31:781—784
[PubMed]
 
Manyam BV, Bhatt MH, Moore WD, et al: Bilateral striopallidodentate calcinosis: cerebrospinal fluid, imaging, and electrophysiological studies. Ann Neurol  1992; 31:379—384
[CrossRef] | [PubMed]
 
Lauterbach EC: Psychiatric management in Fahr's syndrome, in Psychiatric Management in Neurological Diseases—Major Basal Ganglia—Cortical Disorders, edited by Lauterbach EC. Washington, DC, American Psychiatric Press (in press)
 
Murphy MJ: Clinical correlations of CT scan-detected calcifications of the basal ganglia. Ann Neurol  1979; 6:507—511
[CrossRef] | [PubMed]
 
Vles JS, Lodder J, van der Lugt PJ: Clinical significance of basal ganglia calcifications detected by CT (a retrospective study of 33 cases). Clin Neurol Neurosurg  1981; 83:253—256
[CrossRef] | [PubMed]
 
Harrington MG, MacPherson P, McIntosh WB, et al: The significance of the incidental finding of basal ganglia calcification on computed tomography. J Neurol Neurosurg Psychiatry  1981; 44:1168—1170
[CrossRef] | [PubMed]
 
Stellamor K, Stellamor V: [Roentgen diagnosis of Fahr's disease.] Roentgenblatter  1983; 36:194—196
 
Kazis AD: Contribution of CT scan to the diagnosis of Fahr's syndrome. Acta Neurol Scand  1985; 71:206—211
[CrossRef] | [PubMed]
 
Konig P: Psychopathological alterations in cases of symmetrical basal ganglia sclerosis. Biol Psychiatry  1989; 25:459—468
[CrossRef] | [PubMed]
 
Kendall B, Cavanagh N: Intracranial calcification in pediatric computed tomography. Neuroradiology  1986; 28:324—330
[CrossRef] | [PubMed]
 
Taxer F, Haller R, Konig P: [Clinical early symptoms and CT findings in Fahr syndrome.] Nervenarzt  1986; 57:583—588
[PubMed]
 
López-Villegas D, Kulisevsky J, Deus J, et al: Neuropsychological alterations in patients with computed tomography—detected basal ganglia calcification. Arch Neurol  1996; 53:251—256
[PubMed]
 
Adachi M, Hosoya T, Yamaguchi K: [Non-calcified line in calcification of the globus pallidus.] Nippon Igaku Hoshasen Gakkai Zasshi  1994; 54:1347—1351
[PubMed]
 
Bruyn GW, Bots GT, Staal A: Familial bilateral vascular calcification in the central nervous system. Psychiatry Neurol Neurochir  1964; 67:342—376
 
Kobayashi S, Yamadori I, Miki H, et al: Idiopathic nonarteriosclerotic cerebral calcification (Fahr's disease): an electron microscopic study. Acta Neuropathol (Berl)  1987; 73:62—66
[CrossRef]
 
Spatz H: Über den eisennachweis im gehirn, besonders in zentren des extrapyramidal-motorischen systems [On demonstrating iron in the brain, especially in extrapyramidal motor system centers]. Zentralbl Gesamte Neurol Psychiatry  1922; 77:261
[CrossRef]
 
Bouras C, Giannakopoulos P, Good PF, et al: A laser microprobe mass analysis of trace elements in brain mineralizations and capillaries in Fahr's disease. Acta Neuropathol (Berl)  1996; 92:351—357
[CrossRef]
 
Danowski TS, Lasser EC, Wechsler RL: Calcification of basal ganglia in post-thyroidectomy hypoparathyroidism. Metabolism  1960; 9:1064—1065
[PubMed]
 
Basser LS, Neale FC, Ireland AW, et al: Epilepsy and electroencephalographic abnormalities in chronic surgical hypoparathyroidism. Ann Intern Med  1969; 71:507—515
[PubMed]
 
Brannan TS, Burger AA, Chaudhary MY: Bilateral basal ganglia calcifications visualised on CT scan. J Neurol Neurosurg Psychiatry  1980; 43:403—406
[CrossRef] | [PubMed]
 
Avrahami E, Cohn DF, Feibel M, et al: MRI demonstration and CT correlation of the brain in patients with idiopathic intracerebral calcification. J Neurol  1994; 241:381—384
[CrossRef] | [PubMed]
 
Kulczycki J, Boguslawska-Staniaszczyk R, Kozlowski P: [The image of intracerebral calcification in CT and MR studies: a case report of Fahr syndrome.] Neurol Neurochir Pol  1994; 28:915—920
[PubMed]
 
Uygur GA, Liu Y, Hellman RS, et al: Evaluation of regional cerebral blood flow in massive intracerebral calcifications. J Nucl Med  1995; 36:610—612
[PubMed]
 
Ogata A, Ishida S, Wada T: A survey of 37 cases with basal ganglia calcification (BGC): CT-scan findings of BGC and its relationship to underlying diseases and epilepsy. Acta Neurol Scand  1987; 75:117—124
[CrossRef] | [PubMed]
 
Schmid H, Haller R, Konig P: [Value of EEG in parathyroid gland disorders and/or symmetrical calcinosis of the basal ganglia (Fahr syndrome): review of the literature with personal cases.] Wien Klin Wochenschr  1986; 98:486—490
[PubMed]
 
Nakayama T, Sakakihara Y, Hanaoka S, et al: Calcification of basal ganglia in a patient with partial trisomy 5q and partial monosomy 18q. Acta Paediatr Jpn  1993; 35:340—344
[PubMed]
 
Okano S, Takeuchi Y, Kohmura E, et al: Globus pallidus calcification in Down syndrome with progressive neurologic deficits. Pediatr Neurol  1992; 8:72—74
[CrossRef] | [PubMed]
 
Mann DM: Calcification of the basal ganglia in Down's syndrome and Alzheimer's disease. Acta Neuropathol (Berl)  1988; 76:595—598
[CrossRef]
 
Takashima S, Becker LE: Basal ganglia calcification in Down's syndrome. J Neurol Neurosurg Psychiatry  1985; 48:61—64
[CrossRef] | [PubMed]
 
Thase ME: Basal ganglia calcification and psychosis in Down's syndrome. Postgrad Med J  1984; 60:137—139
[CrossRef] | [PubMed]
 
Wisniewski KE, French JH, Rosen JF, et al: Basal ganglia calcification (BGC) in Down's syndrome (DS): another manifestation of premature aging. Ann N Y Acad Sci  1982; 396:179—189
[CrossRef] | [PubMed]
 
Jakab I: Basal ganglia calcification and psychosis in mongolism. Eur Neurol  1978; 17:300—314
[CrossRef] | [PubMed]
 
Salva E, Savoldi F: Contributo alla conoscenzo della forme ereditarie della malattia di Fahr [Contribution to the understanding of the hereditary form of Fahr's disease]. Sist Nerv  1959; 11:46—73
[PubMed]
 
Strobos RRJ, De La Torre E, Martin JF: Symmetrical calcification of the basal ganglia with familial ataxia and pigmentary macular degeneration. Brain  1957; 80:313—318
[CrossRef] | [PubMed]
 
Puvanendran K, Low CH, Boey HK, et al: Basal ganglia calcification on computer tomographic scan: a clinical and radiological correlation. Acta Neurol Scand  1982; 66:309—315
[PubMed]
 
Francis AF: Familial basal ganglia calcification and schizophreniform psychosis. Br J Psychiatry  1979; 79:360—362
 
Francis A, Freeman H: Psychiatric abnormality and brain calcification over four generations. J Nerv Ment Dis  1984; 172:166—170
[CrossRef] | [PubMed]
 
Martinelli P, Giuliani S, Ippoliti M, et al: Familial idiopathic strio-pallido-dentate calcifications with late onset extrapyramidal syndrome. Mov Disord  1993; 8:220—222
[CrossRef] | [PubMed]
 
Tokoro K, Chiba Y, Ohtani T, et al: Pineal ganglioglioma in a patient with familial basal ganglia calcification and elevated serum alpha-fetoprotein: case report. Neurosurgery  1993; 33:506—511
[CrossRef] | [PubMed]
 
Billard C, Dulac O, Bouloche J, et al: Encephalopathy with calcifications of the basal ganglia in children: a reappraisal of Fahr's syndrome with respect to 14 new cases. Neuropediatrics  1989; 20:12—19
[CrossRef] | [PubMed]
 
Rysz A, Gajkowski K, Empel A: [Familial occurrence of calcinosis of the basal ganglia.] Neurol Neurochir Pol  1988; 22:459—462
[PubMed]
 
Frydman M, Bar-Ziv J, Preminger-Shapiro R, et al: Possible heterogeneity in spondyloenchondrodysplasia: quadriparesis, basal ganglia calcifications, and chondrocyte inclusions. Am J Med Genet  1990; 36:279—284
[CrossRef] | [PubMed]
 
Rossi M, Morena M, Zanardi M: [Calcification of the basal ganglia and Fahr disease: report of two clinical cases and review of the literature.] Recenti Prog Med  1993; 84:192—198
[PubMed]
 
Ellie E, Julien J, Ferrer X: Familial idiopathic striopallidodentate calcifications. Neurology  1989; 39:381—385
[PubMed]
 
Garcia Urra D, Barquero Jimenez MS, Varela de Seijas E, et al: [Calcification of the basal ganglia and hypoparathyroidism: Fahr disease. Study of a family.] Arch Neurobiol (Madr)  1990; 53:18—22
[PubMed]
 
Yokota S, Mori T, Kosuge K, et al: [Basal ganglia calcification in two children with systemic lupus erythematosus and neuropsychiatric manifestations.] Ryumachi  1985; 25:115—122
[PubMed]
 
Nordstrom DM, West SG, Andersen PA: Basal ganglia calcifications in central nervous system lupus erythematosus. Arthritis Rheum  1985; 28:1412—1416
[CrossRef] | [PubMed]
 
Legido A, Zimmerman RA, Packer RJ, et al: Significance of basal ganglia calcification on computed tomography in children. Pediatr Neurosci  1988; 14:64—70
[CrossRef] | [PubMed]
 
Eleopra R, Accurti I, Neri W, et al: Unusual case of Fahr syndrome with motoneuron disease. Ital J Neurol Sci  1991; 12:597—600
[CrossRef] | [PubMed]
 
Kusunose Y, Taniguchi T, Yamada M, et al: [Two siblings of myotonic muscular dystrophy associated with basal ganglia calcification.] Rinsho Shinkeigaku  1987; 27:1276—1279
[PubMed]
 
Markesbery WR: Lactic acidemia, mitochondrial myopathy, and basal ganglia calcification. Neurology  1979; 29:1057—1060
[PubMed]
 
Robertson WC Jr, Viseskul C, Lee YE, et al: Basal ganglia calcification in Kearns-Sayre syndrome. Arch Neurol  1979; 36:711—713
[PubMed]
 
Carboni P, Giacanelli M, Porro G, et al: Kearns-Sayre syndrome: a case of the complete syndrome with encephalic leukodystrophy and calcification of basal ganglia. Ital J Neurol Sci  1981; 2:263—268
[CrossRef] | [PubMed]
 
Grotemeyer KH, Lehmann HJ, Jörg J, et al: [Familial basal ganglia calcinosis, mitochondrial myopathy and epilepsy: result of a single metabolic disorder?] Nervenarzt  1984; 55:202—207
 
Yoda S, Terauchi A, Kitahara F, et al: Neurologic deterioration with progressive CT changes in a child with Kearns-Shy syndrome. Brain Dev  1984; 6:323—327
[PubMed]
 
Pavlakis SG, Phillips PC, Di Mauro S, et al: Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes: a distinctive syndrome. Ann Neurol  1984; 16:481—488
[CrossRef] | [PubMed]
 
Bastiaensen LAK, Stadhouders AM, Ter Laak HJ, et al: Kearns-Sayre syndrome. Neuro-Ophthalmology  1984; 4:55—63
[CrossRef]
 
Federico A, Cornelio F, Di Donato S, et al: Mitochondrial encephalo-neuro-myopathy with myoclonus epilepsy, basal nuclei calcification and hyperlactacidemia. Ital J Neurol Sci  1988; 9:65—71
[PubMed]
 
Mousa AM, Muhtaseb SA, Reddy RR, et al: The high rate of prevalence of CT-detected basal ganglia calcification in neuropsychiatric (CNS) brucellosis. Acta Neurol Scand  1987; 76:448—456
[CrossRef] | [PubMed]
 
Fenelon G, Gray F, Paillard F, et al: A prospective study of patients with CT detected pallidal calcifications. J Neurol Neurosurg Psychiatry  1993; 56:622—625
[CrossRef] | [PubMed]
 
Arranz Perez M, Ergueta Martin P, Gonzalez Sarmiento E, et al: [Fahr's disease and hypocalcemic syndromes: presentation of a clinical case.] An Med Interna  1992; 9:495—497
[PubMed]
 
Arias Mayorga J, Gonzalez Martin T, Escorial Miguel C, et al: [Intracranial calcifications in the differential diagnosis of epileptic disease.] Rev Clin Esp  1991; 189:425—427
[PubMed]
 
Chiu HF, Lam LC, Shum PP, et al: Idiopathic calcification of the basal ganglia. Postgrad Med J  1993; 69:68—70
[CrossRef] | [PubMed]
 
Lauterbach EC, Spears TE, Prewett MJ, et al: Neuropsychiatric disorders, myoclonus, and dystonia in calcification of basal ganglia pathways. Biol Psychiatry  1994; 35:345—351
[CrossRef] | [PubMed]
 
Trenkwalder C, Schwarz J, Gebhard J, et al: Starnberg trial on epidemiology of parkinsonism and hypertension in the elderly: prevalence of Parkinson's disease and related disorders assessed by a door-to-door survey of inhabitants older than 65 years. Arch Neurol  1995; 52:1017—1022
[PubMed]
 
Matsui K, Yamada M, Kobayashi T, et al: [An autopsy case of Fahr disease (infantile form).] No To Hattatsu  1992; 24:358—363
[PubMed]
 
Tolmie JL, Shillito P, Hughes-Benzie R, et al: The Aicardi-Goutieres syndrome (familial, early onset encephalopathy with calcifications of the basal ganglia and chronic cerebrospinal fluid lymphocytosis). J Med Genet  1995; 32:881—884
[CrossRef] | [PubMed]
 
Rosenberg DR, Neylan TC, El-Alwar M, et al: Neuropsychiatric symptoms associated with idiopathic calcification of the basal ganglia. J Nerv Ment Dis  1991; 179:48—49
[CrossRef] | [PubMed]
 
Cummings JL: Clinical Neuropsychiatry. Orlando, FL, Grune and Stratton, 1985, p 154
 
Seidler GH: [Psychiatric and psychological aspects of Fahr syndrome.] Psychiatr Prax  1985; 12:203—205
[PubMed]
 
Shibayama H, Kobayashi H, Nakagawa M, et al: Non-Alzheimer non-Pick dementia with Fahr's syndrome. Clin Neuropathol  1992; 11:237—250
[PubMed]
 
Kosaka K: Diffuse neurofibrillary tangles with calcification: a new presenile dementia. J Neurol Neurosurg Psychiatry  1994; 57:594—596
[CrossRef] | [PubMed]
 
Cummings JL, Gosenfeld LF, Houlihan JP, et al: Neuropsychiatric disturbances associated with idiopathic calcification of the basal ganglia. Biol Psychiatry  1983; 18:591—601
[PubMed]
 
Wodarz N, Becker T, Deckert J: Musical hallucinations associated with post-thyroidectomy hypoparathyroidism and symmetric basal ganglia calcifications (letter). J Neurol Neurosurg Psychiatry  1995; 58:763—764
[CrossRef] | [PubMed]
 
Fernandez-Bouzas A, Angrist B, Hemdal P, et al: Basal ganglia calcification in schizophrenia (letter). Biol Psychiatry  1990; 27:682—685
[CrossRef] | [PubMed]
 
Forstl H, Krumm B, Eden S, et al: Neurological disorders in 166 patients with basal ganglia calcification: a statistical evaluation. J Neurol  1992; 239:36—38
[CrossRef] | [PubMed]
 
Forstl H, Krumm B, Eden S, et al: What is the psychiatric significance of bilateral basal ganglia mineralization? Biol Psychiatry  1991; 29:827—833
 
Chow KS, Lu DN: [Primary hypoparathyroidism with basal ganglia calcification: report of a case.] Acta Paediatr Sin  1989; 30:129—133
[PubMed]
 
Abe S, Tojo K, Ichida K, et al: A rare case of idiopathic hypoparathyroidism with varied neurological manifestations. Intern Med  1996; 35:129—134
[CrossRef] | [PubMed]
 
Gubbay SS, Hankey GJ, Tan NT, et al: Mitochondrial encephalomyopathy with corticosteroid dependence. Med J Aust  1989; 151:100—103
[PubMed]
 
Skvortsov IA, Rudenskaia GE, Karaseva AN, et al: [Effectiveness of the therapeutic use of complexones in various diseases of the extrapyramidal system in children.] Zh Nevropatol Psikhiatr  1987; 87:1457—1462
[PubMed]
 
Vermersch P, Leys D, Pruvo JP, et al: Parkinson's disease and basal ganglia calcifications: prevalence and clinico-radiological correlations. Clin Neurol Neurosurg  1992; 94:213—217
[CrossRef] | [PubMed]
 
Vaamonde J, Legarda I, Jimenez-Jimenez J, et al: Levodopa-responsive parkinsonism associated with basal ganglia calcification and primary hypoparathyroidism (letter). Mov Disord  1993; 8:398—400
[CrossRef] | [PubMed]
 
Tambyah PA, Ong BK, Lee KO: Reversible parkinsonism and asymptomatic hypocalcemia with basal ganglia calcification from hypoparathyroidism 26 years after thyroid surgery. Am J Med  1993; 94:444—445
[CrossRef] | [PubMed]
 
Uncini A, Tartaro A, Di Stefano E, et al: Parkinsonism, basal ganglia calcification and epilepsy as late complications of postoperative hypoparathyroidism. J Neurol  1985; 232:109—111
[CrossRef] | [PubMed]
 
Munir KM: The treatment of psychotic symptoms in Fahr's disease with lithium carbonate. J Clin Psychopharmacol  1986; 6:36—38
[PubMed]
 
+
+

CME Activity

There is currently no quiz available for this resource. Please click here to go to the CME page to find another.
Submit a Comments
Please read the other comments before you post yours. Contributors must reveal any conflict of interest.
Comments are moderated and will appear on the site at the discertion of APA editorial staff.

* = Required Field
(if multiple authors, separate names by comma)
Example: John Doe



Web of Science® Times Cited: 53

Related Content
Articles
Books
Gabbard's Treatments of Psychiatric Disorders, 4th Edition > Chapter 11.  >
Textbook of Traumatic Brain Injury, 2nd Edition > Chapter 4.  >
The American Psychiatric Publishing Textbook of Psychiatry, 5th Edition > Chapter 11.  >
The American Psychiatric Publishing Textbook of Psychopharmacology, 4th Edition > Chapter 62.  >
The American Psychiatric Publishing Textbook of Psychiatry, 5th Edition > Chapter 26.  >
Topic Collections
Psychiatric News