Atypical presentation in Rasmussen encephalitis: delayed late-onset periodic epileptic spasms

Atypical presentation in Rasmussen encephalitis: delayed late-onset periodic epileptic spasms

Author Ferrari, Taissa Pinheiro Fernandes Autor UNIFESP Google Scholar
Hamad, Ana Paula Andrade Autor UNIFESP Google Scholar
Caboclo, Luis Otavio Sales Ferreira Autor UNIFESP Google Scholar
Centeno, Ricardo Silva Autor UNIFESP Google Scholar
Zaninotto, Ana Luiza Autor UNIFESP Google Scholar
Scattolin, Monica Autor UNIFESP Google Scholar
Carrete Junior, Henrique Autor UNIFESP Google Scholar
Lancellotti, Carmem Lucia Penteado Google Scholar
Yacubian, Elza Márcia Targas Autor UNIFESP Google Scholar
Institution Universidade Federal de São Paulo (UNIFESP)
Santa Casa São Paulo
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]
Keywords Rasmussen encephalitis
late-onset spasms
epileptic spasms
periodic spasms
focal cortical dysplasia
Language English
Date 2011-09-01
Published in Epileptic Disorders. Montrouge: John Libbey Eurotext Ltd, v. 13, n. 3, p. 321-325, 2011.
ISSN 1294-9361 (Sherpa/Romeo, impact factor)
Publisher John Libbey Eurotext Ltd
Extent 321-325
Access rights Open access Open Access
Type Article
Web of Science ID WOS:000296567000015

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