Abstract
Neuroachantocytosis, a form of hereditary chorea, is a degenerative multisystem disease presenting with several types of abnormal movements such as dystonia and chorea together with other neurological problems including neuropathy, amyotrophy, seizure and ataxia. Here, we report a 52-year old male with recurrent oromandibular dystonic attacks together with progressive chorea at limbs, seizure, ataxia, and depression. With diagnosis of neuroachantocytosis, the patient received clozapine and botulinum toxin injection resulting in partial response.