Faciobrachial dystonic seizures expressed as epileptic spasms, followed by focal seizures in anti‐LGI1 encephalitis: a video‐polygraphic study. Issue 6 (27th December 2018)

• Record Type: Journal Article
• Authors: d'Orsi, Giuseppe; Martino, Tommaso; Lalla, Alessandra; Claudio, Maria Teresa Di; Carapelle, Elena; Avolio, Carlo
• Is Part Of: Epileptic disorders. Volume 20:Issue 6(2018)
• Page Start: 525
• Page End: 529
• Publication Date: 2018-12-27
• Subjects: anti‐LGI1 encephalitis -- faciobrachial dystonic seizure -- video‐EEG/polygraphy -- epileptic spasm -- basal ganglia; Epilepsy -- Periodicals; 616.853
• DOI: 10.1684/epd.2018.1010
• Languages: English
• ISSNs: 1294-9361

Abstract

ABSTRACT: 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 ]