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Updated: Jul 14, 2025

Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis

Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis

Published on: July 4, 2007

Atypical Rasmussen's Encephalitis.

Maria A Alfonso¹, Martha C Piñeros-Fernández^2,3, Luisa F Jaimes⁴

¹Pediatrics, Los Cobos Medical Center, Bogotá, COL.

|October 9, 2023

Summary

This summary is machine-generated.

This case study presents a rare instance of Rasmussen's encephalitis (RE) in a child, diagnosed via imaging despite the absence of typical epilepsy. Early steroid treatment showed a positive clinical response.

Keywords:

autoimmune encephalitis brain magnetic resonance images case report encephalitis focal motor deficit hemiplegia language regression rasmussen syndrome refractory epilepsy seizure

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Last Updated: Jul 14, 2025

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Published on: March 26, 2019

Area of Science:

Neurology
Pediatric Neurology
Neuroimmunology

Background:

Rasmussen's encephalitis (RE) is a rare, chronic inflammatory neurological disease affecting one cerebral hemisphere, typically presenting with intractable epilepsy and progressive neurological deficits.
Diagnosis often relies on clinical presentation, electroencephalography (EEG), and neuroimaging, with epilepsy being a hallmark symptom.
Atypical presentations of RE, particularly those without prominent or continuous epileptic seizures, pose diagnostic challenges.

Observation:

A 3-year-old female presented with subacute fever, vomiting, persistent mydriasis, facial palsy, and hemiparesis.
Brain MRI revealed encephalitis in multiple brain regions and significant volume loss in the left hemisphere, consistent with imaging features of Rasmussen's encephalitis.
The patient notably lacked epilepsy or continuous partial epilepsy, classifying this as an atypical presentation of RE based on imaging criteria.

Findings:

The case highlights imaging findings suggestive of Rasmussen's encephalitis in the subacute stage.
The absence of typical epileptic seizures in this patient underscores the variability in RE's clinical manifestations.
Intravenous methylprednisolone bolus therapy resulted in an adequate clinical response, suggesting a potential role for immunomodulatory treatment in atypical RE.

Implications:

This case expands the understanding of atypical Rasmussen's encephalitis, emphasizing the importance of neuroimaging in diagnosis, even without classic epilepsy.
The findings suggest that prompt diagnosis and treatment with corticosteroids may be beneficial in managing subacute, atypical RE.
Further research into the diagnostic and therapeutic strategies for non-epileptic or minimally epileptic forms of RE is warranted to improve patient outcomes.