Session Title: Section Info: Annual Assembly Posters (Non Presentations)
Session Time: 11:15am-12:45pm
Location: Research Hub - Kiosk 8
Disclosures: Nicole Diaz-Segarra, MD: No disclosure data submitted.
Case Description: Leucine-rich glioma-inactivated 1 (LGI1) limbic encephalitis
Setting: Acute Care Hospital
Patient: A 68-year-old female with a history of metastatic colorectal adenocarcinoma without active disease presented with new onset generalized seizures and decreased level of consciousness. Brain magnetic resonance imaging showed left hippocampal T2 enhancement, and anticonvulsants were started. Electroencephalogram showed recurrent left posterior temporal region partial seizures accompanied by right facial and arm clonic movements, consistent with faciobrachial dystonic seizures (FBDS).
Assessment/Results: This raised concern for leucine-rich glioma-inactivated 1 limbic encephalitis (LGI1 LE) and autoimmune labs were obtained. She was given intravenous immunoglobulins (IVIG) and high dose steroids. The FDBS resolved and her level of arousal improved, but she continued to have right hemiparesis, aphasia and apraxia. Labs were positive for Anti-VGKC and Anti-LGI1, supporting the diagnosis. She was discharged to rehabilitation with a prolonged steroid taper.
Discussion: LGI1 LE is an uncommon, recently characterized disorder due to antibody formation against LGI1. Early signs include generalized seizures and FBDS. FBDS are partial seizures manifesting as involuntary movements of the unilateral arm and face. They are very specific for LGI1 LE, however they are only present in a minority of patients. FBDS can occur up to 100 times per day, last only seconds, and be easily misdiagnosed as a movement disorder. Later in the disease course, neurocognitive symptoms develop including behavioral, memory, and sleep disturbances. Aphasia and apraxia are rarely reported symptoms but can be present. Positive LGI1 antibodies are not required for diagnosis, and treatment with immunotherapy (IVIG and steroids) should be initiated immediately if LGI1 LE is suspected.
Conclusion: In patients presenting with clonic movements of the face and arm suggestive of FDBS, clinicians should have an increased suspicion for LGI-1 limbic encephalitis, especially if associated with generalized seizures or neuropsychologic symptoms. Early identification and intervention are essential for minimizing morbidity and mortality.
Level of Evidence: Level V
To cite this abstract in AMA style:Diaz-Segarra N, Averill A. Leucine-rich Glioma-inactivated 1 (LGI1) Limbic Encephalitis with Faciobrachial Dystonic Seizures: A Case Report [abstract]. PM R. 2019; 11(S2)(suppl 2). https://pmrjabstracts.org/abstract/leucine-rich-glioma-inactivated-1-lgi1-limbic-encephalitis-with-faciobrachial-dystonic-seizures-a-case-report/. Accessed September 20, 2021.
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