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Rasmussen's encephalitis
Etiology:
- idiopathic
Pathology:
- unilateral hemispheric atrophy
- intractable seizures
Microscopic pathology:
- chronic encephalitis
- predominance of T lymphocytes
- prominent microglial activation with microglial nodules
- neuronophagia
- gliosis
Clinical manifestations:
- chronic, progressive course
- progressive unilateral weakness
- intractable seizures
Special laboratory:
- electroencephalography may reveal unilateral discharges
- lumbar puncture: CSF may be normal
- brain biopsy
Radiology:
- MRI neuroimaging may reveal unilateral lesion in motor area
Management:
- neurosurgery: hemispheric disconnection
General
encephalitis
References
- Thibert RL et al
Case 34-2014 - A 7-year-Old Boy with Focal Seizures and
Progressive Weakness.
N Engl J Med 2014; 371:1737-1746. October 30, 2014
http://www.nejm.org/doi/full/10.1056/NEJMcpc1305993