title: Clinical, electrophysiological, imaging, pathological and therapeutic observations among 18 patients with Rasmussen's encephalitis.
abstract: We studied the clinical, electrophysiological, imaging and pathological features of 18 patients with Rasmussen's encephalitis (RE). This descriptive study included 18 patients (six males, 12 females) with RE who were evaluated for demographic and phenotypic details, electroencephalogram (EEG) results, MRI results, pathological features, virological markers and outcome. Radiological staging as per Bien et al. and pathological staging in accordance with Robitaille et al. were performed. Simple partial seizures were the most common initial manifestation. During the disease course, epilepsia partialis continua (EPC) developed in 15/18 (83.3%) and hemiparesis in 17/18 (94.4%) patients. EEG revealed hemispheric slowing (100%), interictal epileptiform discharges (100%) and ictal pattern (44.4%). Brain MRI revealed unihemispheric focal cortical atrophy (100%), white matter changes (88.2%), basal ganglia-ipsilateral caudate and putamen involvement (50.0%) and progression of atrophy on serial MRI (100%). Unusual presentations in this series included late onset (n=1), and isolated lingual EPC (n=1). Diagnostic biopsies in two patients revealed Robitaille stage 3 disease. The six hemispherotomy specimens showed stage 2 disease in one, stage 3 in three and stage 4 in two cases. Heterogeneity in disease stage in the different neuroanatomical regions and within the same cortical segment reflected progression of immune-mediated damage. Immunomodulation provided only temporary benefit. Patients who underwent functional hemispherotomy had reduction in seizure frequency and improved quality of life. The clinical, EEG and MRI findings are in accordance with the established literature. MRI staging was concordant with Robitaille pathological staging. Immunomodulation did result in transient reduction in seizure frequency while surgery in six produced reasonable benefit.
authors.affiliation: Department of Neurology, National Institute of Mental Health and NeuroSciences, Bangalore 560 029, Karnataka, India.
authors.forename: K
authors.initials: K
authors.lastname: Pradeep
authors.affiliation: Department of Neurology, National Institute of Mental Health and NeuroSciences, Bangalore 560 029, Karnataka, India. Electronic address: sanjib_sinha2004@yahoo.co.in.
authors.forename: S
authors.initials: S
authors.lastname: Sinha
authors.affiliation: Department of Neuropathology, National Institute of Mental Health and NeuroSciences, Bangalore, India.
authors.forename: A
authors.initials: A
authors.lastname: Mahadevan
authors.affiliation: Department of Neuroimaging and Interventional Radiology, National Institute of Mental Health and NeuroSciences, Bangalore, India.
authors.forename: J
authors.initials: J
authors.lastname: Saini
authors.affiliation: Department of Neurosurgery, National Institute of Mental Health and NeuroSciences, Bangalore, India.
authors.forename: A
authors.initials: A
authors.lastname: Arivazhagan
authors.affiliation: Department of Neuroimaging and Interventional Radiology, National Institute of Mental Health and NeuroSciences, Bangalore, India.
authors.forename: R D
authors.initials: RD
authors.lastname: Bharath
authors.affiliation: Department of Neurology, National Institute of Mental Health and NeuroSciences, Bangalore 560 029, Karnataka, India.
authors.forename: P S
authors.initials: PS
authors.lastname: Bindu
authors.affiliation: Department of Psychology, National Institute of Mental Health and NeuroSciences, Bangalore, India.
authors.forename: R
authors.initials: R
authors.lastname: Jamuna
authors.affiliation: Department of Neurosurgery, National Institute of Mental Health and NeuroSciences, Bangalore, India.
authors.forename: M B
authors.initials: MB
authors.lastname: Rao
authors.affiliation: Xcyton Diagnostics, Bangalore, India.
authors.forename: S
authors.initials: S
authors.lastname: Govekar
authors.affiliation: Xcyton Diagnostics, Bangalore, India.
authors.forename: B V
authors.initials: BV
authors.lastname: Ravikumar
authors.affiliation: Department of Neurosurgery, National Institute of Mental Health and NeuroSciences, Bangalore, India.
authors.forename: B A
authors.initials: BA
authors.lastname: Chandramouli
authors.affiliation: Department of Neurology, National Institute of Mental Health and NeuroSciences, Bangalore 560 029, Karnataka, India.
authors.forename: P
authors.initials: P
authors.lastname: Satishchandra
mesh_terms: D000328:Adult; D001706:Biopsy; D018450:Disease Progression; D004569:Electroencephalography; D004660:Encephalitis / Q000473:pathology* / Q000503:physiopathology* / Q000628:therapy; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D055815:Young Adult
keywords: EPC; Epilepsy; Hemispherotomy; MRI; Rasmussen’s encephalitis; Virological markers