Rabiy El Qadiry, Imane Fetoui*, Houda Nassih, Aicha Bourrahouat, Iman Ait Sab
Wilson's disease is the consequence of a congenital error in copper’s metabolism. In children, its basic symptom is liver damage. It can be combined with various extra-intestinal manifestations, including neurological ones, which must suggest the diagnosis. We report two cases of dysarthria, with asymptomatic liver injury, revealing Wilson's disease and we detail clinical signs that should make this diagnosis in the child.
Case 1: 12-years-old boy, the first child of a non-consanguineous couple, had normal psychomotor development and schooling. He had consulted for dysarthria, hyper-salivation and tremor of the upper limbs associated with a micrographic started two months before hospitalization. Clinical examination revealed an extrapyramidal syndrome, abnormal movements were noted, associating a kinetic-rigid Parkinson syndrome and a dystonia of the limbs without other signs in particular neither hepatomegaly nor jaundice. Wilson's disease was suspected and confirmed by biological tests, ophthalmologic examination and encephalic MRI.
Case 2: An 11-years-old female patient from first degree marriage with good psychomotor development and normal schooling, who had experienced gait abnormalities, dysarthria and dystonia for the last 6 months. The neurological examination had found an extrapyramidal syndrome, tremor of the extremities and pseudobulbar signs such as dysarthria. Cerebral MRI showed signal abnormalities of the basal ganglia. In front of the starting age, clinical data and radiological appearance, Wilson's disease was suspected. Ophthalmological examination revealed the presence of Kayser fleischer ring and copper assessment confirmed the diagnosis of Wilson's disease.
Conclusion: The diagnosis of Wilson's disease with neurological symptoms is difficult. It must be thought of in front of any dysarthria in young patients.
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