Title

The Prevalence and clinical outcome of Rasmussen's Encephalitis in children

Description

Rasmussen encephalitis is a rare but severe disorder characterised by unilateral cerebral atrophy with associated intractable seizures and progressive hemiparesis, hemianopia and cognitive decline. There are many unanswered questions in relation to this distressing and disabling disorder, not least the aetiology, the role of autoimmune therapies and timing of hemispherectomy. This study aims to improve our understanding of the management of and outcome for these children in order to improve care and identify potential areas for future aetiological and therapeutic research

Lead Clinician

Prof Helen Cross (UCL Institute of Child Health and Gt Ormond St Hospital)

Disease name

Rasmussen's Encephalitis

Case Definition

Diagnostic criteria

(Need all 3 from part A or 2 of 3 from part B for diagnosis)*
A  
Clinical Focal seizures +/- EPC and unilateral cortical deficit(s)
EEG Unihemisphereic slowing with or without epileptiform activity and unilateral seizure onset
MRI Unihemispheric focal cortical atrophy and at least one of the following:
  • Grey or white matter
  • T2/flair hyperintense signal
  • Hyperintense signal
  • Atrophy of ipsilateral caudate head
B  
Clinical EPC or progressive unilateral cortical deficit (s).**
MRI Progressive unihemisphereic focal cortical atrophy***
Histopathology T cell dominated encephalitis with activated microglial cells and reactive astrogliosis. (Numerous parenchymal macrophages, B cells or plasma cells or viral inclusion bodies exclude the diagnosis)
*Ref: Pathogenesis, diagnosis and treatment of Rasmussen encephalitis; A European consensus statement. Brain 2005, 128, 454-471.
** Has had at least 2 sequential clinical examinations which document a neurological deficit which gas increased over time.
*** Sequential MRI scans show hemiatrophy  which has increased over time

Inclusion Criteria

All patients between 1 to 16 years old who fit the case definition.

Exclusion Criteria

Brain biopsy histology that excludes the diagnosis of Rasmussen encephalitis or diagnosis of alternative progressive neurological condition