title: Atypical presentation in Rasmussen encephalitis: delayed late-onset periodic epileptic spasms.
abstract: A five-and-a-half-year-old girl started experiencing progressive left hemiparesis at age two and a half years. At age five years and four months she started presenting clusters of asymmetric periodic epileptic spasms with no hypsarrhythmia. The ictal EEG showed periodic, constant and stereotyped complexes. Serial brain imaging revealed progressive atrophy of the right hemisphere with increased T2 signal on MRI. She underwent a right hemispherotomy, and histological examination showed signs of inflammation and features of focal cortical dysplasia (FCD). She has been seizure-free for 16 months. This case is unique in the following aspects: the presence of typical Rasmussen encephalitis features of progressive unilateral brain involvement without seizures, a delay of almost three years prior to seizure onset; an atypical seizure type presentation with periodic epileptic spasms and the presence of FCD associated with inflammatory changes. [Published with video sequences].
authors: Departamento de Neurologia e Neurocirurgia, Universidade Federal de São Paulo, Sao Paulo, Brazil. taissaferrari@uol.com.br
authors: Taíssa P F
authors: TP
authors: Ferrari
authors: Ana P A
authors: AP
authors: Hamad
authors: Luís Otávio S F
authors: LO
authors: Caboclo
authors: Ricardo S
authors: RS
authors: Centeno
authors: Ana Luiza
authors: AL
authors: Zaninotto
authors: Monica
authors: M
authors: Scattolin
authors: Henrique
authors: H
authors: Carrete Junior
authors: Carmem L P
authors: CL
authors: Lancellotti
authors: Elza Márcia T
authors: EM
authors: Yacubian
mesh_terms: D001921:Brain / Q000473:pathology; D002675:Child, Preschool; D004569:Electroencephalography; D004660:Encephalitis / Q000175:diagnosis* / Q000601:surgery / Q000628:therapy; D004827:Epilepsy / Q000175:diagnosis / Q000209:etiology*; D005260:Female; D038421:Hemispherectomy; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D019635:Neurosurgical Procedures; D010291:Paresis / Q000209:etiology; D013035:Spasm / Q000175:diagnosis / Q000209:etiology*; D013036:Spasms, Infantile / Q000175:diagnosis; D014057:Tomography, X-Ray Computed