Pharmacoresistent Partial Epilepsy Secondary to Progressive Inflammatory Poliodystrophy.
BACKGROUND
Epilepsy with progressive cortical volume loss is described secondary to energy failure such as mitochondrial disorders, infectious, or inflammatory etiologies and associated with temporal lobe epilepsy. Postmortem studies do not support that spontaneous seizures even if present for prolonged periods universally result in cortical volume loss.


RESULTS
We describe two children with extratemporal pharmacoresistent epilepsy, slowly progressive gray matter volume loss over several years, and evidence of central nervous system inflammation. Brain magnetic resonance imaging changes and antibody profiles were not typical of a well-defined, antibody-mediated central nervous system syndrome such as N-methyl-D-aspartate receptor encephalitis.


CONCLUSIONS
These patients illustrate a novel presentation of a subacute inflammatory central nervous system process with epilepsy and progressive cortical volume loss, supporting the role of sequential brain imaging in children with epilepsy.
Pediatric Neurology, Children's Hospital and Research Center, Oakland, California. Electronic address: rkuperman@mail.cho.org.
Rachel A
RA
Kuperman
Pediatric Radiology, Children's Hospital and Research Center, Oakland, California.
Kenneth W
KW
Martin
D000927:Anticonvulsants / Q000627:therapeutic use; D020274:Autoimmune Diseases of the Nervous System / Q000150:complications* / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D001921:Brain / Q000187:drug effects / Q000276:immunology / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D004828:Epilepsies, Partial / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D005260:Female; D066128:Gray Matter / Q000187:drug effects / Q000276:immunology / Q000473:pathology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D009929:Organ Size
D000927:Anticonvulsants; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors
Poliodystrophy; Rassmusen encephalitis; autoimmune encephalitis; paraneoplastic encephalitis; pharmacoresistant epilepsy