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What Neuropsychiatrists Would Like to See in DSM-5

Abstract

The foundation of neuropsychiatry rests upon the relationship between the brain and behavior. Neuropsychiatrists evaluate patients with neurological disorders and general medical conditions who are experiencing psychiatric symptoms. Knowledge of functional neuroanatomy of the central nervous system is essential to offer accurate diagnoses and treatments.

The practical nature of neuropsychiatric practice usually requires the clinician to offer a diagnosis found in the Diagnostic and Statistical Manual of Mental Disorders.1 Given the realistic limitations of neuroscience as it applies to the neurological basis of psychiatric illness, the clinician is often challenged to provide an accurate diagnosis based on DSM-IV-TR terminology. This article explores the issues encountered by neuropsychiatrists regarding proper diagnosis based on the criteria established in the DSM-IV-TR. It is our hope that these issues will be addressed in the new DSM-5.

The DSM is a useful reference that relies on empirical foundations to identify discrete psychiatric disorders. It is designed to be a helpful guide to clinicians, and it provides sets of clinical criteria and statements of the constructs embodied in the clinical criteria. Each section in the DSM-IV-TR includes diagnostic features; associated features and disorders; specific cultural, age, and gender features; prevalence, course, familial pattern, differential diagnosis, recording procedures, and diagnostic criteria.

The DSM-IV-TR section on Disorders of Infancy, Childhood, or Adolescence defines several conditions that could be confidently identified as neuropsychiatric. These include Pervasive Developmental Disorders (299.X), Tic Disorders (307.2X), Mental Retardation (317, 318.X), Learning Disorders (315.X), Motor Skills Disorder (315.4), Communication Disorders (307.X, 315.3X), and Attention-Deficit and Disruptive Behavior Disorders (312.X, 313.81, 314.X).

From the perspective of the neuropsychiatrist, the DSM-IV-TR classification for disorders of infancy, childhood, and adolescence are generally comprehensive in scope and reflect the connection between abnormal neurological development and the presence of neuropsychiatric signs and symptoms. They present a very good example of a neuropsychiatric nosology. The text identifies useful elements of neurological and neuropsychological evaluation and identifies associated neurological signs and symptoms. This section of the DSM-IV-TR represents an ideal template for adult neuropsychiatric disorders.

Templates for Adult Neuropsychiatric Disorders

Unfortunately, ideal templates for adult neuropsychiatric disorders are lacking in DSM-IV-TR. Many syndromes encountered in neuropsychiatric practice are not identified in the DSM-IV-TR. The diagnostic criteria often rely on the clinician to definitively link the psychiatric symptoms to concurrent neurologic conditions in the absence of conclusive neuroscientific proof. Furthermore, the diagnostic nomenclature is often lengthy and clumsy.

This article will review several common neuropsychiatric disorders and syndromes as seen in clinical practice and illustrate the challenges and limitations of DSM-IV-TR criteria.

I find the DSM-IV-TR section on Delirium (293.0) to be a helpful clinical and diagnostic guide. It provides useful clinical examples to assist the clinician in recognizing the varied presentations of delirium. Meagher et al.2 have proposed improving the DSM-5 classification of delirium by expanding the diagnosis to include subtypes “With Hyperactive Features” (Table 1) “With Hypoactive Features” (Table 2), and “With Mixed Hyperactive/Hypoactive Features.”

TABLE 1. Delirium With Hyperactive Features
Hyperactive Subtype
Definite evidence in the previous 24 hours of at least 2 of the following:
  • Increased quality of motor activity
  • Loss of control of activity
  • Restlessness
  • Wandering

From Meagher et al.2 Used by permission.

TABLE 1. Delirium With Hyperactive Features
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TABLE 2. Delirium With Hypoactive Features
Hypoactive Subtype
Definite evidence in the previous 24 hours of two or more of the following:
  • Decreased amount of activity
  • Decreased speed of actions
  • Reduced awareness of surroundings
  • Decreased amount of speech
  • Decreased speed of speech
  • Listlessness
  • Reduced alertness/withdrawal

From Meagher et al.2 Used by permission.

TABLE 2. Delirium With Hypoactive Features
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Caregivers of patients with delirium are often confused by the noteworthy differences in activity levels manifested by the patient. Consequently, patients with diminished activity are frequently overlooked as having delirium. Acknowledging the existence of varied presentations of delirium would improve our ability to educate caregivers, such as nursing staff in the general hospital, and thus improve detection and treatment of delirium.

The DSM-IV-TR section on Cognitive Disorders is problematic. The “A” diagnostic criteria for Dementia of the Alzheimer Type (294.1X) represent the prototype for all specified DSM-IV-TR dementia diagnoses (Table 3). These include Vascular Dementia (290.4X), Dementia Due to Other General Medical Conditions (294.1X), and Substance-Induced Persisting Dementia (292.82); yet many well-established dementia syndromes do not present in this way, for example the Progressive Aphasias or Posterior Cortical Dementia. Consequently, the diagnostic nomenclature for DSM-IV-TR dementias departs from the typical terminology utilized by clinicians.

TABLE 3. Prototype for All Specified DSM-IV-TR Dementia Diagnoses
294.1X Dementia of the Alzheimer Type
A. The development of multiple cognitive impairments manifested by both:
1. memory impairment (impaired ability to learn new information or to recall previously-learned information
2. one (or more) of the following cognitive disturbances:
 a. aphasia
 b. apraxia
 c. agnosia
 d. disturbance in executive functioning
B. The cognitive deficits in criteria A1 and A2 each cause significant impairment in social or occupational functioning and represent a significant decline from previous level of functioning.
C. The course is characterized by gradual onset and continuing cognitive decline.
D. The cognitive deficits in Criteria A1 and A2 are not due to any of the following:
 1. other central nervous system conditions that cause progressive deficits in memory and cognition
 2. systemic conditions that are known to cause dementia
 3. substance-induced conditions
E. The deficits do not occur exclusively during the course of a delirium.
F. The disturbance is not better accounted for by another Axis I disorder.
TABLE 3. Prototype for All Specified DSM-IV-TR Dementia Diagnoses
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Table 4 illustrates DSM-IV-TR terminology for some of the more common dementias. It clearly demonstrates the limitations of the DSM-IV-TR diagnostic terminology for diagnosing dementias in clinical practice. I believe the criteria would be improved by defining the specific presentation of the various dementias as “syndromes” and providing consensus criteria for each syndrome in the DSM-5. The best current illustration for this would be the McKeith criteria3 for Dementia with Lewy Bodies (which would be reclassified as the Dementia With Lewy Body Syndrome) and the DSM-IV-TR criteria for Dementia of the Alzheimer Type (which would be reclassified as the Dementia Syndrome of the Alzheimer Type).

TABLE 4. Neuropsychiatric Dementia Syndromes in DSM-IV-TR Terminology
Dementia SyndromeDSM-IV-TR Diagnosis
• Mild Cognitive Impairment294.0 Amnestic Disorder Due to …
• Dementia of the Alzheimer Type294.1X Dementia of the Alzheimer Type
• Frontotemporal Dementia294.1X Dementia Due to Frontotemporal Atrophy
• Primary Progressive Aphasia294.9 Cognitive Disorder, Not Otherwise Specified
• Corticobasal Syndrome294.1X Dementia Due to Corticobasal Degeneration
• Posterior Cortical Dementia294.9 Cognitive Disorder, Not Otherwise Specified
• Dementia With Lewy Bodies294.1X Dementia Due to (Presumed) Lewy Bodies
• Subcortical Dementia294.0 Amnestic Disorder Due to Parkinsonism
TABLE 4. Neuropsychiatric Dementia Syndromes in DSM-IV-TR Terminology
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In this way, we can align the DSM-5 criteria with the typical nomenclature used by clinicians and also eliminate the conundrum of certainty that is required for dementia diagnoses in the DSM-IV-TR because, by definition, syndromes are not diseases. For example, clinicopathologic studies of dementia have demonstrated that the correlation between bedside diagnosis and histological findings ranges widely, from 65% to 95%.4 Londos et al.5 found that two-thirds of patients carefully diagnosed with probable Alzheimer’s disease (AD) by NINCDS-ADRDA criteria6 also met McKeith criteria3 for Dementia with Lewy Bodies (DLB). Furthermore, the neuropathology for each group of patients was remarkably similar. Both the AD and the DLB groups had histologically-identified neuritic plaques, neurofibrillary tangles, and Lewy bodies. This problem with diagnostic specificity is thoroughly explored by Dubois et al.4

In reality, the diseases that cause dementia syndromes are primarily limited to some combination of amyloidoses causing neuritic plaques or vascular lesions, tauopathies causing neurofibrillary tangles, synucleinopathies causing Lewy bodies, prionopathies causing spongiform changes, or cerebrovascular disease causing strokes or hemorrhages. Each of these diseases can present with any number of dementia syndromes, depending solely on the location of the pathology, as opposed to the specific type of histopathology. Thus, each dementia syndrome can reflect different underlying diseases.

The DSM-IV-TR criteria for vascular dementia are particularly problematic because they rely strongly on clinical judgment to decide whether the patient’s neurological signs and symptoms are “etiologically related to the disturbance” and are thus causative. I have seen many cases where patients are given the diagnosis of vascular dementia simply because they have radiologic evidence of cortical, white-matter, or lacunar infarcts, regardless of the location in relation to the cognitive findings. Furthermore, many of these patients have cortical atrophy, suggesting the presence of a cortical degenerative component. The certainty of identifying a stroke on neuroimaging usually exceeds the certainty that atrophy is disease-related rather than age-related. Thus, we will tend to overdiagnose vascular dementia at the expense of what is more likely a mixed cortical degenerative disease with a vascular component. Wallin et al.7 have proposed a more detailed and clinically useful set of criteria for vascular dementia by defining subtypes (Table 5).

TABLE 5. Criteria for Vascular Dementia by Subtype
Vascular Dementia Subtypes
Post-Stroke Dementia
  • Strategic Infarct Dementia
  • Multi-Infarct Dementia
  • Intracerebral Hemorrhage-Associated Dementia
Subcortical Vascular Dementia
  • Lacunar Infarcts
  • Ischemic White-Matter Lesions
Dementia of the Alzheimer type + Cerebrovascular Disease

From Wallin et al.7 Used by permission.

TABLE 5. Criteria for Vascular Dementia by Subtype
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DSM-IV-TR coding procedures for neuropsychiatric syndromes in the setting of dementia are inconsistent. Vascular Dementia (290.4) is the only type of dementia that has defined subtypes to indicate the presence of significant associated symptoms, such as delirium (290.41), delusions (290.42), or depressed mood (290.43). Any associated behavioral disturbance would be specified, but not coded. On the other hand, Dementia of the Alzheimer Type (290.1X) with associated behavioral disturbances would be coded as 290.11. But there would be no coding for an associated delirium, delusions, or depressed mood. This is illustrated in Table 6, where the DSM-IV-TR diagnoses for the very common presentation of an elderly patient with dementia, delusions, depressed mood, and intermittent aggression are provided. The DSM-5 criteria would be improved by standardizing these coding procedures.

TABLE 6. Dementia Coding
A 75-year-old patient with dementia, delusions, depressed mood, and intermittent aggression
History of StrokeNo Stroke
290.42 Vascular Dementia With Delusions, With Behavioral Disturbance290.11 Dementia of the Alzheimer Type, With Late Onset, With Behavioral Disturbance
293.81 Psychotic Disorder Due to Dementia, With Delusions
290.43 Vascular Dementia With Depressed Mood293.83 Mood Disorder Due to Dementia, With Depressive Features
310.1 Personality Change Due to Vascular Dementia, Aggressive Type310.1 Personality Change Due to Dementia, Aggressive Type
TABLE 6. Dementia Coding
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Finally, the DSM-IV-TR section on the dementias offers proposals for the office examination of various cognitive domains. Coffey et al.8 identified excessive variation in the actual office assessment of cognition by clinicians who describe their practice as neuropsychiatric. Furthermore, the Institute of Medicine has identified variation in clinical practice as a significant impediment to achieving higher-quality care.9 Ideally, we can agree on a single, valid method for office evaluation of each cognitive domain and specify these tests in the DSM-5. The diagnostic criteria for each dementia syndrome could specifically include the findings on each test. For example, diagnosing Frontotemporal Dementia Syndrome would require the clinician to administer an agreed-upon office test of executive cognition and identify a cutoff score that would serve as one means of qualifying the patient for the diagnosis. In this way, we could eliminate the unacceptable variations with interrater reliability and ensure more consistency in diagnosing these often-confusing syndromes.

Neuropsychiatrists typically treat patients whose psychiatric symptoms appear in the setting of general medical conditions, such as traumatic brain injury, autoimmune disorders, striatal disorders, and developmental disorders. The DSM-IV-TR includes diagnostic criteria for these clinical presentations, which include psychotic disorders, mood disorders, anxiety disorders, personality changes, and cognitive impairment (Table 7). In order to offer the diagnosis, the clinician must conclude that the general medical condition is actually causing the psychiatric symptom. This degree of certainty is frequently not possible, given our current understanding of neuroscience and functional neuroanatomy. Bradford-Hill proposed specific criteria to assist the clinician in identifying causation.10

TABLE 7. Psychiatric Symptoms in the Setting of General Medical Conditions
Psychiatric Conditions Caused by General Medical Conditions
293.XX Psychotic Disorder Due to XXX
  • -.81 With Delusions / -0.82 With Hallucinations
293.83 Mood Disorder Due to XXX
  • With Depressive/Major Depressive-Like/Manic/Mixed Features
293.84 Anxiety Disorder Due to XXX
  • With Generalized Anxiety/Panic Attacks/Obsessive-Compulsive Symptoms
310.1 Personality Change Due to XXX
  • Labile/Disinhibited/Aggressive/Apathetic/Paranoid/Other/Combines/Unspecified
294.1 Dementia Due to XXX
TABLE 7. Psychiatric Symptoms in the Setting of General Medical Conditions
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Even under the best clinical circumstances, it is often not really possible to conclude that the general medical condition is causative. For example, a 50-year-old man develops a manic syndrome a few days after suffering a right frontal cortical infarct. His father and brother have been diagnosed with bipolar disorder, but the patient has never had a major mood disorder. Is the manic syndrome a sequela of right-hemisphere stroke (293.83 Mood Disorder Due to Stroke With Manic Features) or the new onset of mania in a man who is genetically predisposed and now experiencing significant stress because of his disability (296.4 Bipolar Disorder, Type I, Most Recent Episode Manic)?

In the absence of truly consistent and reproducible evidence of neuropsychiatric causation in many cases, I believe it is most reasonable at present to eliminate the need for certainty. When the psychiatric syndrome occurs in the setting of a known condition such as traumatic brain injury, stroke, or dementia, then the clinician could simply identify the DSM-IV-TR subtype (Table 7) and diagnose the patient with, for example, Right Hemisphere Stroke With Manic Features. This format would leave open the possibility of causation, but not insist on it, while acknowledging that there is a larger biologic context for the mood disorder in the patient.

Apathy is a common neuropsychiatric syndrome that is not clearly identified in the DSM-IV-TR. Indeed, an OVID database search done in late 2010 revealed over 2,000 citations for the keyword “apathy.” It is often quite disabling to the patient and frustrating to caregivers who may confuse the apathy with depression or volitional, but undesirable, behavior. Starkstein et al.11 and Marin et al.12 have proposed that apathy syndromes be formally identified in the DSM (Table 8). Apathy can occur in various contexts, such as poststroke or frontotemporal dementia. Currently, apathy in these contexts would be diagnosed as 310.1 Personality Change Due to XXX, Apathetic Type. However, developing formal consensus criteria would support reliable case description and identification, facilitate clinical communication about the syndrome, and encourage research and treatment efforts.

TABLE 8. Proposed Criteria for Apathy
A. Lack of motivation relative to a patient’s previous level of functioning or the standards of his or her age and culture, as indicated by either subjective account or observation by others.
B. Presence for at least 4 weeks during most of the day, of at least 1 symptom belonging to each of the following three domains:
 Diminished Goal-Directed Behavior
  • Lack of effort or energy to perform everyday activities
  • Dependency on prompts from others to structure everyday activities
 Diminished Goal-Directed Cognition
  • Lack of interest in learning new things or in new experiences
  • Lack of concern about one’s personal problems
  Diminished concomitants of goal-directed behavior
  • Unchanging or flat affect
  • Lack of emotional responsivity to positive or negative events
C. Symptoms cause clinically significant distress or impairment in social, occupational, or other important areas of functioning.
D. Symptoms are not due to diminished level of consciousness or the direct effects of a substance.

From Starkstein et al.11 and Marin et al.12 Used by permission.

TABLE 8. Proposed Criteria for Apathy
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In addition to apathy, there are other common neuropsychiatric syndromes not clearly defined in the DSM-IV-TR. These are noted in Table 9.

TABLE 9. Other Neuropsychiatric Syndromes Not Formally Identified in DSM-IV-TR
Emotional Incontinence
  • (?) 312.30 Impulse Control Disorder, Not Otherwise Specified
Charles Bonnet Syndrome
  • (?) 293.82 Psychotic Disorder Due to Visual Impairment, With Hallucinations
Klein-Levin Syndrome
  • (?) 310.1 Personality Change Due to Klein-Levin Syndrome, Mixed Type
  • (?) 312.30 Impulse Control Disorder, Not Otherwise Specified
Gastaut-Geschwind Syndrome
  • (?) 310.1 Personality Change Due to Partial Epilepsy, Mixed Type
Pathological Yelling Secondary to Brain Injury or Dementia
  • (?) 294.1X Dementia Due to Brain Injury, With Behavioral Disturbance
  • (?) 312.30 Impulse Control Disorder, Not Otherwise Specified
REM Sleep Behavioral Disorder
  • 307.47 Parasomnia, Not Otherwise Specified
TABLE 9. Other Neuropsychiatric Syndromes Not Formally Identified in DSM-IV-TR
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The evolution of the DSM has reflected advances in the biological understanding of mental illness, and it also mirrors changes in our culture and how we perceive mental illness. The formal diagnostic criteria provided in the manual permit reliable diagnosis, which guides evidence-based treatment, epidemiologic studies, and clinical research. The DSM-IV-TR is, however, less useful to neuropsychiatrists for everyday clinical work. Most neuropsychiatric syndromes occur in the context of neurological and general medical illness, yet conclusive proof of causation is often lacking. Furthermore, the neuropsychiatric lexicon offered by the DSM-IV-TR is rather clumsy and often requires multiple diagnoses and codes (Table 6) to accurately diagnose common neuropsychiatric clinical presentations. It is my hope that these issues will be addressed in the DSM-5.

From Tufts University School of Medicine, Boston, MA; General Hospital Psychiatric Services, Maine Medical Center, Portland, ME.
Send correspondence to Dr. Campbell; e-mail:
References

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