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Updated: May 5, 2026
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Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
Published on: July 4, 2007
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Unilateral cortical autoimmune encephalitis: A case series and comparison to late-onset Rasmussen's encephalitis
Sophia Damman1, Persen Sukpornchairak2, Amit Ahituv3
1Case Western Reserve University School of Medicine, 10900 Euclid Avenue, Cleveland, OH 44106, USA.
Journal of Neuroimmunology
|May 10, 2024
Summary
A novel pattern of unilateral autoimmune encephalitis (AE) is more common in older adults and often seronegative. This condition resembles Rasmussen's encephalitis but responds better to immunotherapy.
Area of Science:
- Neurology
- Immunology
- Neuroscience
Background:
- Autoimmune encephalitis (AE) encompasses a spectrum of inflammatory brain disorders.
- Rasmussen's encephalitis is a rare, chronic inflammatory neurological disease affecting one hemisphere of the brain.
- Late-onset neurological conditions present unique diagnostic and therapeutic challenges.
Purpose of the Study:
- To describe a newly identified anatomical pattern of autoimmune encephalitis (AE).
- To characterize the clinical presentation, etiology, and outcomes of strictly unilateral cortical inflammation in AE.
- To compare this pattern with late-onset Rasmussen's encephalitis.
Main Methods:
- Retrospective data collection from two tertiary referral academic centers.
- Inclusion criteria applied to identify relevant patient cases.
- Analysis of clinical data, neuroimaging (MRI), electroencephalography (EEG), and serological findings.
Main Results:
- Twelve cases of unilateral cortical AE identified (mean age 65 years, 58% female).
- Common manifestations included focal seizures, cognitive decline, hemicortical deficits, and unilateral MRI/EEG abnormalities.
- Etiologies included idiopathic (6), paraneoplastic (2), iatrogenic (2), and post-COVID-19 (2); most were seronegative.
Conclusions:
- Unilateral cortical AE is more prevalent in the elderly, often idiopathic and seronegative.
- This AE variant shares features with late-onset Rasmussen's encephalitis but shows better response to immunotherapy.
- Consideration of interventions for refractory Rasmussen's encephalitis, like hemispherectomy, may be relevant for refractory unilateral AE cases.
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