Neurological manifestations of Wilson's disease

ABSTRACT

To study the frequency of various neurological manifestations in patients with Wilson's Disease.Patients suspected of having Wilson's disease were admitted to the Neurology unit of Postgraduate Medical Institute, Lady Reading hospital, Peshawar, for a full work up for Wilson's disease. Only those patients who had neurological signs or symptoms of Wilson's disease were included in this study. Out of 15 patients 9 were female and 6 were male, ranging in age from 8 to 26 years, with mean age of 15.6 years [SD +/- 5.82]. Most common neurological features were tremors followed by rigidity, dysphagia/drooling, speech or gait problems. All patients had low serum ceruloplasmin level and Kayser-Fleischer corneal rings. Liver involvement in the form of cirrhosis was found in 13 [86.7%] patients on ultrasonography, since no liver biopsy was done it could not be determined whether the remaining two patients had liver involvement or not. Neurological manifestations are not uncommon in Wilson's disease. It should be considered in patients of any age presenting with unusual liver, neurologic or psychiatric abnormalities. It should especially be looked for in children and other young patients with extrapyramidal signs or symptoms