A CASE REPORT OF NEUROACANTHOCYTOSIS

Neuroacanthocytosis (Levin-Critchly syndrome or chorea acanthocytosis) is multi system neurodegenerative disorder characterized by acanthocytes, normal plasma lipids and lipoproteins and variable neurological manifestations. Onset is usually in the fourth and fifth decade. The most consistent clinical feature is a hyperkinetic movement disorder, dementia and personality changes. An axonal neuropathy and absent tendon reflexes occurs in most patients. About 40% have epileptic seizure. The course is progressive and only symptomatic therapy is available. In this article a case neroacanthocytosis was presented. The patient was a 34-year old man with two years history of abnormal body movement, tongue and lips biting and seizure. Acanthocytosis was found in his peripheral blood smear, caudate atrophy in radiological examination, normal lipoprotein and axonal neuropathy in E.M.G-N.C.V.The patient was treated with tetrabenazine, haloperiodol, trihexiphenydril and carbamazepin. His seizure was completely controlled and abnormal movements were significantly reduced.