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Pan Afr Med J
March 2017
Service de Réanimation Médicale, Pôle Anesthésie-Réanimation, Hôpital Militaire Med V, Rabat, Maroc.
Chorea-acanthocytosis (ChAc) is an extremely rare autosomal recessive disorder caused by mutations in the VSP13A gene on chromosome 9q21. It is characterized by neurological symptoms, psychiatric manifestations and multisystem involvement resulting in myopathy, axonal neuropathy and presence of spiculated red blood cells or acanthocytes. Rarely, epilepsy may be the early symptom in these patients.
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