|Case 27 :|
male with epilepsy. CSF shows mild pleocytosis with elevated protein count
that is not consistent with a viral encephalitis. What is the diagnosis
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Findings reveal hyperintensity of bilateral perisylvian temporal and frontal grey and subcortial grey mater with evidence of focal atropy. Rest of the neuro parenchyma is normal.
It is a neurodegenerative disorder characterized by intractable focal motor seizures and progressive neurologic deterioration (dementia, hemiparesis). It is also known as Chronic Focal Encephalitis or Chronic Progressive Epilepsia Paritalis Continua of Childhood. This may represent an etiologically diverse disorder inwhich the common clinicopathologic features represent the terminal stage of expression of various viral brain infections, i.e., CMV, EBV. Etiolopathogenesis is believed to be autoimmune:
IGlutamate Receptor R3 (GluR3):there may be an abnormal immune attack against GluR3 as antibodies directed against GluR3 have been identified in patients: this hypothesis suggests that a focal interruption of the blood-brain barrier must occur for the GluR3 antibodies to enter the brain and thus must look for a previous history of head injury or vascular malformations in each patient
Deepak Patkar, MRI Centre, Nanavati Hospital, Mumbai