Teaching video neuroimages: orofacial dyskinesia and oral ulceration due to involuntary biting in neuroacanthocytosis.

A 30-year-old man with a history of generalized epilepsy presented with progressively worsening involuntary movements for 2 years. He had no family history of movement disorders. He had orofacial choreiform movements with sucking, grimacing, and neck flexion, which was exacerbated with eating. He also had oral ulcers due to involuntary biting. Blood smear showed 20% acanthocytes. Nerve conduction demonstrated sensory axonal neuropathy. MRI was negative for white matter changes and caudate atrophy. Orofacial dyskinesia (video on the Neurology (R) Web site at www.neurology.org), oral mutilation, and feeding dystonia are typical of chorea-acanthocytosis.(1) Generalized seizures and axonal sensory neuropathy are associated.(2)