title: An autopsy case of hemiconvulsion-hemiplegia-epilepsy syndrome manifesting as cerebral hemiatrophy in an elderly man.
abstract: We report an autopsy case of hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome in a 79-year-old man. HHE syndrome usually occurs in children younger than 4 years of age. Although most HHE syndrome patients live into adult life, only a few cases of the syndrome have been reported in the elderly. In our case, cerebral hemiatrophy, left mesial temporal sclerosis and crossed cerebellar atrophy were observed. Because this is the oldest case ever reported, we further investigated age-related neuropathological changes and found an interhemispheric difference in amyloid-β-related neuropathologic changes. There were almost no senile plaques or amyloid-laden vessels in the left hemisphere. As far as we know, this is the first report of age-related neuropathology in a brain manifesting HHE syndrome.
authors.affiliation: Department of Neurology, Nagoya City Koseiin Medical Welfare Center, Aichi, Japan.
authors.forename: Tamaki
authors.initials: T
authors.lastname: Iwase
authors.affiliation: Department of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Aichi, Japan.
authors.forename: Mari
authors.initials: M
authors.lastname: Yoshida
authors.affiliation: Department of Neurology, Nagoya City Koseiin Medical Welfare Center, Aichi, Japan.
authors.forename: Tomoyuki
authors.initials: T
authors.lastname: Mizuno
authors.affiliation: Department of Experimental Pathology and Tumor Biology, Nagoya City University Graduate School of Medical Sciences, Aichi, Japan.
authors.forename: Shinya
authors.initials: S
authors.lastname: Sato
authors.affiliation: Department of Neurology, Ban Buntane Hotokukai Hospital, School of Medicine, Fujita Health University, Aichi, Japan.
authors.forename: Kazuya
authors.initials: K
authors.lastname: Nokura
mesh_terms: D000368:Aged; D001284:Atrophy / Q000209:etiology / Q000473:pathology; D001344:Autopsy; D001921:Brain / Q000473:pathology*; D004827:Epilepsy / Q000150:complications / Q000473:pathology*; D006429:Hemiplegia / Q000150:complications / Q000473:pathology*; D006801:Humans; D008297:Male; D012640:Seizures / Q000150:complications / Q000473:pathology*; D013577:Syndrome
keywords: cerebral amyloid angiopathy; cerebral hemiatrophy; hemiconvulsion-hemiplegia-epilepsy syndrome; senile plaques; temporal lobe epilepsy