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

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• 作者：Taíssa P. F. Ferrari (1) (4)
  Ana P. A. Hamad (1)
  Luís Otávio S. F. Caboclo (1)
  Ricardo S. Centeno (1)
  Ana Luiza Zaninotto (1)
  Monica Scattolin (1)
  Henrique Carrete Junior (2)
  Carmem L. P. Lancellotti (3)
  Elza Márcia T. Yacubian (1)
  
• 关键词：Rasmussen encephalitis ; late ; onset spasms ; epileptic spasms ; periodic spasms ; focal cortical dysplasia
• 刊名：Epileptic Disorders
• 出版年：2011
• 出版时间：September 2011
• 年：2011
• 卷：13
• 期：3
• 页码：321-325
• 全文大小：524KB
• 参考文献：1. Auvin S, Lamblin MD, Pandit F, Vallée L, Bouvet-Mourcia A. Infantile epileptic encephalopathy with late-onset spasms: report of 19 patients. / Epilepsia 2010; 51: 1290-296. CrossRef
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• 作者单位：Taíssa P. F. Ferrari (1) (4)
  Ana P. A. Hamad (1)
  Luís Otávio S. F. Caboclo (1)
  Ricardo S. Centeno (1)
  Ana Luiza Zaninotto (1)
  Monica Scattolin (1)
  Henrique Carrete Junior (2)
  Carmem L. P. Lancellotti (3)
  Elza Márcia T. Yacubian (1)
  
  1. Departamento de Neurologia e Neurocirurgia, Universidade Federal de S?o Paulo, S?o Paulo, Brazil
  4. Unidade de Pesquisa e Tratamento de Epilepsia (UNIPETE), Hospital S?o Paulo, Departamento de Neurologia e Neurocirurgia, Universidade Federal de S?o Paulo, Rua Napole?o de Barros, 737, 13o andar, CEP-04024-002, S?o Paulo-SP, Brazil
  2. Departamento de Diagnóstico por Imagem, Universidade Federal de S?o Paulo, S?o Paulo, Brazil
  3. Departamento de Patologia, Santa Casa de S?o Paulo, S?o Paulo, Brazil
  
• ISSN：1950-6945

文摘

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 under went 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.