Adult-onset Rasmussen's encephalitis: Anatomical-electrographic-clinical features of 7 Italian cases

Flavio Villani, Alessandro Pincherle, Carlo Antozzi, Luisa Chiapparini, Tiziana Granata, Roberto Michelucci, Guido Rubboli, Isa Simone, Rosella Bellomo, Roberto Spreafico

Research output: Contribution to journalArticlepeer-review

Abstract

Purpose: A limited number of cases of adult-onset Rasmussen's encephalitis (A-RE) have been reported, but the features of the syndrome are still unclear. The aim of this study was to verify the clinical features of A-RE, and outline a noninvasive approach that may allow its early diagnosis and treatment. Methods: Retrospective evaluation of extensive noninvasive work-up of seven patients with A-RE, including repeat clinical, neurophysiological, and neuroimaging investigations. Results: The study identified two distinct patterns of disease presentation, one characterized by focal motor epilepsy (the "epileptic" phenotype), and the other by focal cortical myoclonus (the "myoclonic" phenotype). Unilateral neurological deficits and brain atrophy were progressive in both phenotypes, but they were more prominent and were detected earlier in the "epileptic" phenotype. Conclusions: The anatomo-electroclinical features of these patients allowed a noninvasive diagnosis of A-RE and identification of two distinct disease phenotypes. Early noninvasive diagnosis can allow faster initiation of treatment.

Original languageEnglish
Pages (from-to)41-46
Number of pages6
JournalEpilepsia
Volume47
Issue numberSUPPL. 5
DOIs
Publication statusPublished - Dec 2006

Keywords

  • Epilepsia partialis continua
  • Focal motor epilepsy
  • Myoclonus
  • Rasmussen's encephalitis

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

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