Hussein Jafar Elhafyan1*, Abdelgadir Hussein M Osman2, Yahia Ownalla Younis
Background: Delineating the neuropsychiatric pattern and features of Wilson disease is essential step to better recognition and management of these presentations. To our knowledge this is the first large study of neuropsychiatric manifestations of Wilson disease in Sudan.
Method: A comprehensive case finding survey was conducted over a six month period, in the Capital of Sudan, covering all secondary and tertiary services that cater for patients with Wilson disease. Patients’ assessment was carried out using batteries of functional tests, including, ICD-10 research criteria for major psychiatric categories, Mini Mental State Examination, ADHD scale, school records, parental reports. All neurological findings were conducted and verified by two neurologists.
Results: Fifty cases of Wilson disease, of varying age and stage of illness, were thoroughly examined for neuropsychiatric symptoms and disorders. Most patients 29 (58%) showed psychological disturbance in form of psychiatric illness and significant behavioural disturbance. Mood and emotional disorders observed in 21 (42%) of cases, core symptoms of depressive disorder diagnosed in 19 cases (38%), behavioural and personality disturbances detected in 19 patients (38%), while cognitive impairments were found in 24 (48%) of all cases. Twenty-one cases (42%) presented with neurological abnormalities, predominantly consisting of posturing in 20 (40%), dystonia in 8 (16%), Parkinsonism was found in 10 (20%) patients, dysarthria in 9 (18%), athetosis in 3 (6%) cases and various forms of tremors in 21 (42%). Epilepsy was recorded in 3 cases (6%).
Conclusion: Neuropsychiatric symptoms and disorders are highly prevalent in Wilson disease patients regardless of age, or stage of illness. Even though, patients in our sample had had an average of 6 years follow up duration, non-had shown evidence of liver failure. This shows an early indication of possibly benign trajectory course of the illness in Sudan.