Dominant psychiatric manifestations in Wilson's disease: a diagnostic and therapeutic challenge!
BACKGROUND
Recognition of psychiatric manifestations of Wilson's disease (WD) has diagnostic and therapeutic implications.


OBJECTIVE
To describe the clinical features and psychopathology of patients with WD who had initial or predominant psychiatric manifestations.


METHODS
Records of 15 patients with WD (M:F: 11:4), from a large cohort of 350 patients, with predominant psychiatric manifestations at onset were reviewed. Their initial diagnosis, demographic profile, family history, pre-morbid personality, clinical manifestations, treatment and outcome were recorded.


RESULTS
Their mean age at diagnosis was 19.8+/-5.8 years. Six patients were born to consanguineous parentage and two patients each had family history of WD and past history of psychiatric illness. Diagnosis of WD was suspected by detection of KF rings (all), observing sensitivity to neuroleptics (n=2), history of jaundice (n=2) and family history suggestive of WD (n=9). Psychiatric manifestations could be classified as affective disorder spectrum (n=11) and schizophreniform-illness (n=3). While the psychiatric symptoms improved in five patients with de-coppering therapy, seven patients needed symptomatic treatment as well. Three of the four patients who responded to de-coppering therapy were sensitive to neuroleptics. Long-term follow up of 10 patients revealed variable recovery.


CONCLUSIONS
Young patient with psychiatric manifestations with clues like history of jaundice, family history of neuropsychiatric manifestations and sensitivity to neuroleptics should be evaluated for WD to avoid delay in diagnosis and associated morbidity.


CONCLUSIONS
The study reemphasizes the importance of behavioral manifestations in Wilson disease in terms of diagnosis and management difficulties.


CONCLUSIONS
Retrospective nature of the study.
Srinivas
K
K
Department of Neurology and Psychiatry, India.
Sinha
S
S
Taly
A B
AB
Prashanth
L K
LK
Arunodaya
G R
GR
Janardhana Reddy
Y C
YC
Khanna
S
S
D000293:Adolescent; D000328:Adult; D002614:Chelating Agents / Q000627:therapeutic use; D015331:Cohort Studies; D003300:Copper / Q000037:antagonists & inhibitors / Q000097:blood / Q000652:urine; D003866:Depressive Disorder / Q000209:etiology / Q000523:psychology; D005260:Female; D005500:Follow-Up Studies; D006527:Hepatolenticular Degeneration / Q000175:diagnosis / Q000523:psychology* / Q000628:therapy; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D001523:Mental Disorders / Q000209:etiology / Q000523:psychology*; D019964:Mood Disorders / Q000209:etiology / Q000523:psychology; D010396:Penicillamine / Q000627:therapeutic use; D011618:Psychotic Disorders / Q000209:etiology / Q000523:psychology; D012559:Schizophrenia / Q000209:etiology; D019287:Zinc Sulfate / Q000627:therapeutic use
D002614:Chelating Agents; D019287:Zinc Sulfate; D003300:Copper; D010396:Penicillamine