Rasmussen encephalitis with dual pathology in a patient without seizures: case report and literature review

Childs Nerv Syst. 2015 Nov;31(11):2165-71. doi: 10.1007/s00381-015-2757-8. Epub 2015 Jun 2.

Abstract

Purpose: Rasmussen encephalitis without seizures is rare. We report a case of Rasmussen encephalitis and cortical dysplasia without epilepsy as well as describe the imaging, pathology, and clinical course and review the literature to investigate whether this may represent a rare subset of Rasmussen encephalitis.

Case report: We report the case of a 12-year-old girl with a history of cognitive decline and right arm weakness. Magnetic resonance imaging demonstrated diffuse left hemispheric cortical and subcortical atrophy suggestive of Rasmussen encephalitis. The patient had no clinical history of seizures, and electroencephalography did not demonstrate epileptiform abnormalities. Craniotomy for open brain biopsy was performed, and histopathologic evaluation identified Rasmussen encephalitis with cortical dysplasia (dual pathology).

Conclusions: To the best of our knowledge, this is the third case of Rasmussen encephalitis diagnosed by both imaging and histopathology that had no clinical or electroencephalographic evidence of seizures and is the only case of Rasmussen encephalitis with cortical dysplasia without epilepsy.

Keywords: Cortical dysplasia; Electroencephalography; Rasmussen encephalitis; T-cell dominant.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Child
  • Encephalitis / pathology*
  • Encephalitis / surgery*
  • Female
  • Follow-Up Studies
  • Humans
  • Magnetic Resonance Imaging
  • Neurosurgical Procedures / methods*