John Libbey Eurotext

Epileptic Disorders

The Educational Journal of the

Faciobrachial dystonic seizures expressed as epileptic spasms, followed by focal seizures in anti-LGI1 encephalitis: a video-polygraphic study Volume 20, issue 6, December 2018

Video

  • Faciobrachial dystonic seizures expressed as epileptic spasms, followed by focal seizures in anti-LGI1 encephalitis: a video-polygraphic study

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Authors
Epilepsy Centre - Clinic of Nervous System Diseases, Riuniti Hospital, Foggia, Italy
* Correspondence: Giuseppe d’Orsi Department of Neurological Sciences, Epilepsy Center, Clinic of Nervous System Diseases, Riuniti Hospital, Via Luigi Pinto 1, 71100 Foggia, Italy
  • Key words: anti-LGI1 encephalitis, faciobrachial dystonic seizure, video-EEG/polygraphy, epileptic spasm, basal ganglia
  • DOI : 10.1684/epd.2018.1010
  • Page(s) : 525-9
  • Published in: 2018

The origin of faciobrachial dystonic seizures in anti-LGI1 encephalitis is controversial due to a lack of neurophysiological characterization. We report a 68-year-old man with subacute anterograde memory loss and involuntary faciobrachial movements. Video-polygraphic recordings disclosed repetitive events characterized by sudden, short contraction of the upper limbs and ipsilateral hemiface. A focal contralateral EEG slow wave from frontal or central electrodes was accompanied by increased muscle activity, often with a diamond-shaped configuration, on the orbicularis oris muscle, deltoid muscle, and extensor muscle of the hand. This EEG/EMG pattern (resembling a tonic epileptic spasm) was always followed by oral and gestural automatisms with dystonic posturing of the upper limbs, compatible with a temporal lobe seizure. Brain MRI showed hyperintensity in the bilateral mesial temporal lobes, while 18FDG-PET revealed basal ganglia hypermetabolism with extensive cortical hypometabolism. Serum and CSF were both positive for anti-LGI antibodies. The patient was treated with intravenous methylprednisolone (1 g/day for five days) with seizure freedom within four days after initiation of the immunotherapy. In this case, a video-EEG/polygraphic study disclosed that faciobrachial dystonic seizures may resemble epileptic spasms, and the occurrence in close temporal association with focal seizures as a single ictal event is suggestive of a peculiar cortical-subcortical interaction. [Published with video sequence on www.epilepticdisorders.com]