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A 30 year old male presenting with involuntary oral movements

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Presentation on theme: "A 30 year old male presenting with involuntary oral movements"— Presentation transcript:

1 A 30 year old male presenting with involuntary oral movements
Teaching NeuroImages Neurology Resident and Fellow Section © 2013 American Academy of Neurology

2 Case Vignette A 30 year old male with a history of generalized epilepsy presented with involuntary movements for 2 years. 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. He had no family history of movement disorders Blood smear showed 20% acanthocytes. MRI was negative for white matter changes and caudate atrophy Nerve conduction demonstrated sensory axonal neuropathy Gooneratne et al © 2013 American Academy of Neurology

3 Gooneratne et al. Video legend
The patient demonstrates intermittent oral, facial, and neck movements and mimicked sucking and grimacing movements. These are associated with intermittent rapid brief eye closings and occasional flexion of his neck. Kissing and sucking sounds are also audible. Oral mucosal ulcers are also demonstrated at the end of the video Gooneratne et al. © 2013 American Academy of Neurology

4 Orofacial dyskinesia and oral ulceration due to involuntary biting in Neuroacanthocytosis  
Orofacial dyskinesia as demonstrated, oral mutilation and feeding dystonia is typical of chorea-acanthocytosis[1]. Generalized seizures and axonal sensory neuropathy are associated [2]. Gooneratne et al. References Bader B, Walker RH, Vogel M, Prosiegel M, McIntosh J, Danek A: Tongue protrusion and feeding dystonia: A hallmark of chorea-acanthocytosis. Mov Disord 2010, 25(1): Jung HH, Danek A, Walker RH: Neuroacanthocytosis Syndromes. Orphanet Journal of Rare Diseases :68. © 2013 American Academy of Neurology

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