Schizophrenia-Like Symptoms in the Westphal-Strümpell Form of Wilson's Disease
SIR: I read with great interest the recent article about lenticulostriatal diseases.1 Although Wilson's disease (WD) is a well-known illness, even today diagnosis often is difficult in cases of early and untypical onset.
One-fifth of patients with Wilson's disease present initially with psychiatric features in isolation. Diagnosis is missed in two-thirds of neurologic initial presentations; the missed diagnosis rate of the psychiatric cases is even worse. I would like to present a typical missed-diagnosis story concerning a patient with psychiatric symptoms since the age of 13 years; despite the symptoms, diagnosis was missed for about 7 years.
Case Report
Until the age of 10, the patient was an average pupil. After a thigh fracture at age 13, in 1986, he failed his classes and finally quit school without participating in final examinations. He was a laborer from 1991 to 1993, but since then he has been unemployed. In 1992 the patient made his first suicide attempt by an overdose of tablets, as he felt tyrannized at work and deceived by his girlfriend. Aggressive episodes and frequent changes in mood increased. Another suicide attempt occurred when he failed his driver's test.
In January 1993 a CT scan was normal. Because of his attacks of temper loss, autoaggressive behavior, and a recurrent aphonia, the diagnosis of “psychological maladaption” was made. During that year he had a manic episode. Zuclopenthixol was added for possible psychosis.
In 1994 the patient was admitted to a ward for the first time to clarify the cause of several symptoms: loss of appetite, intermittent wakefulness, tendency to withdraw, lack of drive, loss of interest, and apathetic change of personality. The patient was reduced in concentration and memory. He was reserved and seemed to have flattened affects. CT scan now showed symmetrical hypodensities in the basal ganglia area. MRI scan showed distinct signal deviations in the putamen and thalamus and finally led to a diagnosis of “change of personality due to an unclear cerebral process.“
After the termination of care the patient expressed fears of having AIDS, of his flat being burned down, and of his family being murdered. In December 1994, because of feelings of pursuit, thought insertion, and sleep disturbance, he was readmitted to the hospital. He felt he was being controlled and poisoned. This time the diagnosis was “delusional disorder, persecutory type with organic influence.” Therapy consisted of haloperidol, pipamperone, and later thioridazine, biperiden, and alprazolam. In March 1995 he made another suicide attempt by consuming fluid detergents.
In September 1995, because of explosive disorders expressing aggressive impulses, the patient was readmitted with the diagnosis of “minimal cerebral dysfunction.” Because of parkinsonism, biperiden was added. In October 1995 the patient finally was taken to a neurology department to clear up any organic causes. Because of an exacerbation of aggressive outbursts, compulsive acts, inadequate expression of mood, and nihilistic delusions, the patient was transferred to a psychiatric ward after 12 days. At the psychiatric ward, the diagnosis of WD at last was made. Therapy with d-penicillamine was initiated but was too late to improve symptoms significantly.
When confronted with an unclear, irritating illness, thinking of WD is the most important thing for a neuropsychiatrist to do. Diagnosis is seldom missed because of a lack of knowledge; mostly, it is clinical routine that prevents us from seeing atypical courses. Case reports could help us to be more aware of WD.
1 Lauterbach EC, Cummings JL, Duffy J, et al: Neuropsychiatric correlates and treatment of lenticulostriatal diseases: a review of the literature and overview of research opportunities in Huntington's, Wilson's, and Fahr's diseases. J Neuropsychiatry Clin Neurosci 1998; 10:249–266Link, Google Scholar
