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Atypical Rasmussen's Encephalitis
Maria A Alfonso1, Martha C Piñeros-Fernández2,3, Luisa F Jaimes4
1Pediatrics, Los Cobos Medical Center, Bogotá, COL.
Abstract:
A three-year-old female patient was admitted to our institution due to subacute fever, intermittent vomiting, persistent bilateral mydriasis after cycloplegia, right central facial palsy, and mild right hemiparesis with hyperreflexia. Brain MRI shows encephalitis in frontal, parietal, insular, and left putamen course and loss of cortical volume and white matter of the entire left hemisphere which are features described in Rasmussen's encephalitis (RE). Therapy with intravenous methylprednisolone bolus was initiated, with adequate clinical response. We consider in this case the diagnosis of atypical RE by imaging criteria in the subacute stage. There are few reports of atypical RE without epilepsy or continuous partial epilepsy. Our purpose is to present a case of a patient with RE images without epilepsy seizures and review the diagnostic and therapeutic approach of RE.
Insights
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.
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.
