Teaching Video Neuro Images: Faciobrachial dystonic seizures

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Abstract

A 58-year-old man had a 6-month history of daily brief (5-10 seconds) jaw-pulling and arm-flexion episodes (video 1 on the Neurology® Web site at Neurology.org). At-home videos were interpreted as "stress-induced" (video 2). These stereotypical episodes with altered awareness lacked electrographic correlate or any abnormalities on brain MRI, as previously reported. 1 The clinical diagnosis of faciobrachial dystonic seizures was supported by elevated voltage-gated potassium channel antibodies (4.11 nmol/L) and positive LGi1 (leucine-rich glioma inactivated-1) protein. Methylprednisolone and plasmapheresis abolished the episodes. Recognition of faciobrachial dystonic seizures is important to reorient treatment from antiepileptics (ineffective) to immunotherapy, preventing progression to cognitive impairment. 1-3 Besides pathognomonic ipsilateral face-arm contractions (figure), our patient manifested unilateral and alternating face-only episodes.

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Schmerler, D. A., Roller, S., & Espay, A. J. (2016, February 9). Teaching Video Neuro Images: Faciobrachial dystonic seizures. Neurology. Lippincott Williams and Wilkins. https://doi.org/10.1212/WNL.0000000000002359

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