Diagnosis:
A combination of clinical, MRI and EEG findings
Clinical features:
Childhood onset
Intractable partial seizures, worsen with time, epilepsia partialis continua, hemiplegia, aphasia
Findings on Investigations:
MRI:
- White matter hyperintensity and then atrophy
EEG:
- Focal and multifocal epileptiform discharges and slowing
Pathology:
Cortical atrophy, perivascular lymphocytic infiltrates with vascular injury, astorcytic gliosis, neuronal loss