Outcome after Functional Hemispherectomy in Children
with Rasmussen Encephalitis: A Report of 20 Years’
Experience at King Chulalongkorn Memorial Hospital
Niemtant T, MD¹, Desudchit T, MD¹
Affiliation : ¹ Pediatric Neurologist, Division of Pediatric Neurology, Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand
Background: Rasmussen’s encephalitis (RE) is characterized by unilateral cerebral inflammation of the cerebral cortex and drug-
resistant epilepsy. Functional hemispherectomy (FH) remains the only effective cure. There is no prior Thai children-case-report.
Objective: To review King Chulalongkorn Memorial Hospital (KCMH) pediatric clinical, magnetic resonance imaging (MRI), and treatment-outcome of RE.
Materials and Methods: This report is a retrospective review of pediatric RE treated with FH. The authors described twenty- year of experience and outcome after FH with three years follow-up at KCMH. Demographic, clinical data, seizures duration, electroencephalogram (EEG), and neuroimaging before FH were collected. Seizure frequency and functional deficit after FH were collected. All RE patients diagnosed by criteria of the European consensus criteria.
Results: Seven patients were diagnosed with RE and had FH. The mean epilepsy-onset age was 73.29±31.39 months, mean diagnosis-age was 91.43±31.15 months, and mean surgery-age was 95.57±31.89 months. Duration between seizure-onset to surgery was 21.29±12.05 months. All EEG showed focal seizures at the right-hemisphere 3 from 7 (42.9%). At the time of surgery, patients had right-hemiparesis 5 from 7 (71.4%) and epilepsia partialis continua (EPC) 4 from 7 (57.1%). Comorbid diseases were attention deficit hyperactivity disorder (ADHD), tics, learning disability, and possible systemic lupus erythematosus (SLE). MRI showed caudate atrophy 3 from 7 (42.9%), T2-hyperintensity 5 from 7 (71.4%), progressive atrophy 4 from 7 (57.1%), and cerebral-hemisphere atrophy 6 from 7 (85.7%). All patients had regression in activity of daily living (ADL), school performance, and quality of life. Outcome after FH were seizure-reduction in 99.44% (95% CI –109.68 to –75.1, p=0.018) and antiepileptic drug (AED) decrease (95% CI 0.61 to 3.1, p=0.047). Regression stopped with better ADL and decrease in seizure-frequency with no worsening of seizure.
Conclusion: RE should be planned in focal intractable epilepsy with EPC and progressive hemiparesis. Appropriate surgical treatment such as FH improves clinical seizures, quality of life, and stop clinical regression.
Received 21 Oct 2019 | Revised 24 Dec 2019 | Accepted 26 Dec 2019
Keywords : Pediatric, Rasmussen encephalitis, Hemispherectomy, Magnetic resonance imaging (MRI), Outcome, Quality of life
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