Neurológia pre prax 3/2012

Acute neuropsychiatric symptoms of Wilson´s disease, treatment and the issue of non-compliance a case report of a young patient

Wilson’s disease (WD) has to be diagnosed early and the treatment regimen must be followed very carefully. Non-compliance may have fatal consequences. We report a case of a young man with neuropsychiatric symptoms of WD that developed in association with drug abuse. The dominating neurological symptoms included dystonia and even status dystonicus, dysmetria, rigidity, akinesia, motor and vocal tics, impairment of speech and gait. A gradual increase of the penicilamine daily dose led to overall clinical improvement which was reflected by the fact that the patient got married and had a child. Later on, the patient withdrew the medication which was followed by an abrupt worsening of neuropsychiatric symptoms of WD. Despite the re-installment of treatment and repeated changes of penicilamine dosage over the time the clinical status remained unchanged.

Keywords: Wilson´s disease, substance abuse, penicillamine, non compliance, status dystonicus.