title: Hyaline protoplasmic astrocytopathy with associated focal cortical dysplasia and hippocampal sclerosis.
abstract: Hyaline protoplasmic astrocytopathy is a rare histopathologic finding in which there is an accumulation of filamin A within protoplasmic astrocytes. The condition has been reported in association with Aicardi syndrome and in patients with epilepsy. This case reports findings of hyaline protoplasmic astrocytopathy in a 4-year-old female who presented with febrile-onset, medically-intractable epilepsy. She underwent resection of the left lateral temporal lobe, hippocampus, and amygdala. In addition to characteristic eosinophilic, astrocytic cytoplasmic inclusions, the lateral temporal lobe showed changes consistent with ILAE (International League Against Epilepsy) type Ic focal cortical dysplasia/Palmini et al. [12] type IA focal cortical dysplasia. The hippocampal formation was marked by loss of neurons in the CA1 region accompanied by gliosis and a relative sparing of neurons in the CA4 region, consistent with ILAE type 2 hippocampal sclerosis or CA1 sclerosis. The literature on hyaline protoplasmic astrocytopathy and coexistent pathologies in the clinical setting of chronic epilepsy is reviewed.
authors.forename: Richard A
authors.initials: RA
authors.lastname: Prayson
mesh_terms: D001253:Astrocytes / Q000473:pathology*; D002675:Child, Preschool; D005260:Female; D006624:Hippocampus / Q000473:pathology*; D006801:Humans; D006818:Hyalin; D054220:Malformations of Cortical Development / Q000473:pathology*; D012598:Sclerosis