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Abstract Details

Progressive Unilateral Cortical Atrophy, Aphasia, and Seizures in a Postmenopausal Female with a History of Alcoholism
Autoimmune Neurology
P1 - Poster Session 1 (12:00 PM-1:00 PM)
1-031

To describe a case of a 62-year-old female former alcoholic with epilepsy, worsening aphasia, and progressive left hemispheric atrophy.

Rasmussen’s encephalitis is a rare clinical entity which includes focal seizures, unihemispheric inflammation, and progressive cortical atrophy. Only 10% of diagnoses are late onset, but not extending beyond early adulthood. Rasmussen-like presentations are poorly understood in older adults. Subacute encephalopathy and seizures in alcoholism (SESA) can be unilateral; however, existing literature lacks longitudinal outcomes. A recent case series described a group of older autoimmune encephalitis patients with Rasmussen encephalitis-like progressive unilateral cortical atrophy. One third had a history of alcohol abuse. Importantly, they improved with immunotherapy.

N/A
Our patient, a heavy drinker with no previous history of seizures, was hospitalized in 2019 at the age of 55 in status epilepticus, with left temporal seizures after experiencing flu-like symptoms. A brain MRI was unremarkable, but over time, as her seizures continued, she developed slowly progressive expressive aphasia, and her MRI evolved from left sided inflammation to progressive left hemispheric atrophy. Anti-seizure medications (ASMs) were continued after a 2022 EEG exhibited left frontotemporal sharp waves. Her seizures are currently controlled on 3 ASMs. The patient was referred to our neuroimmunology clinic to determine an etiology for her radiographic and clinical progression. Workup is currently underway.

Unilateral cortical inflammation preceding progressive atrophy is rare. While Rasmussen’s encephalitis is seen in childhood, such a presentation in the older adult population remains a diagnostic challenge. In alcoholic patients, SESA is on the differential. A recent case series reported Rasmussen-like changes in older individuals with autoimmune encephalitis, including patients with a history of alcohol use disorder. Our case can help to expand the literature on late onset unilateral cortical encephalitis, with the subsequent development of progressive atrophy, aphasia, and epilepsy.

Authors/Disclosures
Jenny Linnoila, MD, PhD (University Neurology Associates, UPMC)
PRESENTER
Dr. Linnoila has received personal compensation in the range of $10,000-$49,999 for serving as a expert respondent on autoimmune encephalitis with U.S. government/DHHS/Vaccine Injury Compensation Program. Dr. Linnoila has a non-compensated relationship as a member of the Medical Advisory Board, Research Subcommittee, with Autoimmune Encephalitis Alliance that is relevant to AAN interests or activities.
Angelo J. Marino, PA Mr. Marino has nothing to disclose.