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The Important Role of Hemispherotomy for Rasmussen Encephalitis: Clinical and Functional Outcomes.
Ursula Thomé1, Larissa A Batista1, Renata P Rocha1
1Department of Neuroscience and Behavioral Sciences, Ribeirão Preto Medical School, University of São Paulo (USP), Ribeirão Preto, Brazil.
Pediatric Neurology
|November 22, 2023
Summary
Hemispheric surgery (HS) for Rasmussen encephalitis (RE) in children offers good seizure control and preserves function. This treatment is recommended for its manageable risks and improved quality of life in pediatric RE patients.
Area of Science:
- Neurology
- Pediatric Epilepsy Surgery
- Autoimmune Encephalitis
Background:
- Rasmussen encephalitis (RE) is a rare, progressive neurological disorder characterized by drug-resistant epilepsy and unilateral brain atrophy.
- While an autoimmune process is implicated, the exact cause of RE remains unknown.
- Hemispheric surgery (HS) is the primary treatment, despite evidence supporting early immunomodulatory therapies.
Purpose of the Study:
- To evaluate the clinical, electrographic, and neuroimaging features of pediatric patients with RE undergoing HS.
- To analyze seizure and functional outcomes following HS in children and adolescents with RE.
Main Methods:
- Retrospective review of 44 pediatric patients with RE who underwent HS between 1995 and 2020.
- Analysis of preoperative data (seizure semiology, EEG, MRI) and postoperative outcomes (seizure control, cognitive, and motor function).
- Histopathological confirmation of RE diagnosis in 95% of cases.
Main Results:
- At last follow-up, 68% of patients were seizure-free, with 70% achieving Engel Class I or II outcomes.
- Postoperative cognitive and gross motor function remained unchanged in 64% and 54% of patients, respectively.
- 74% of patients retained functional hand ability after HS, indicating preserved functional status.
Conclusions:
- Hemispheric surgery is a recommended treatment for pediatric Rasmussen encephalitis due to its progressive nature.
- HS offers manageable risks, good seizure control, and often preserves preoperative functional status, enhancing quality of life.
- Early surgical intervention should be considered for pediatric RE patients, even with left hemisphere involvement.
