Abstract Details

Title Bilaterally Synchronized Faciobrachial Dystonic Seizures in Anti-LGI1 Encephalitis: A Case Report

Topic Multiple Sclerosis

Presentation(s) P14 - Poster Session 14 (8:00 AM-9:00 AM)

Poster/Presentation
Number 9-011

Objective To report a case presenting as bilaterally synchronized faciobrachial dystonic seizures (FBDS) mimicking generalized convulsive status epilepticus (GCSE).

Background Faciobrachial dystonic seizures (FBDS) are a hallmark of autoimmune encephalitis with leucine-rich glioma inactivated-1 (LGI1) antibodies (anti-LGI1 encephalitis), and usually present as brief dystonic contraction of unilateral face and ipsilateral arm lasting less than 3 seconds. However, bilaterally synchronized FBDS has not been reported yet.

Design/Methods A case report.

Results A 75-year-old previously healthy woman presented with gradual memory impairment with visual and auditory hallucination over one month. One week before admission, she started to have rhythmically synchronized bilateral dystonic posturing of her face, arms, and legs 1-3 times a day. Each attack lasted about four minutes without aura or automatism, followed by postictal drowsiness for 10-30 minutes. CSF examination was unremarkable, but oligoclonal bands were detected. Autoantibodies against classical paraneoplastic neuronal intracellular antigen were not detetected in serum. Autoantibodies against neuronal cell-surface antigens or synaptic proteins were examined in serum/CSF and determined to be negative for NMDAR, AMPAR, GABA_AR, GABA_BR, DPPX, mGluR5, and Caspr2, but positive for LGI1; the serum was weakly positive while the CSF was strongly positive. A brain MRI showed right-side predominant increased T2/FLAIR signals in the medial temporal lobes and insular cortex. No tumor was found. A post-ictal EEG did not show paroxysmal discharges. The patient was treated with intravenous high-dose methylprednisolone, followed by prednisolone, resulting in dramatic improvement in her psychiatric symptoms. She was discharged on day 30, without neurologic deficits or seizures.

Conclusions Bilaterally synchronized FBDS mimicking GCSE in our case are phenotypically different from classic LGI1-associated FBDS in terms of duration and bilateral involvements. The findings of the study will expand the phenotypic spectrum of LGI1-associated FBDS. Further studies are required to elucidate the origin of LGI1-associated bilaterally synchronizing FBDS.

Authors/Disclosures
Takahisa Mikami, MD (Massachusetts General Hospital) PRESENTER	Dr. Mikami has nothing to disclose.
	No disclosure on file
Takahiro Iizuka, MD (Department of Neurology, Kitasato University School of Medicine)	The institution of Dr. Iizuka has received research support from EUROIMMUN Japan Co., Ltd.
Akira Machida	No disclosure on file

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Abstract Details