title: Argyrophilic grain disease presenting with frontotemporal dementia: a neuropsychological and pathological study of an autopsied case with presenile onset.
abstract: A right-handed Japanese man with no consanguinity exhibited personality changes, speech disorder and abnormal behaviors, such as stereotypical, running-away, environment-dependent, and going-my-way behaviors, since the age of 49 years. At age 52 years, neuropsychological examination revealed frontal lobe dysfunctions, mild memory impairment, and transcortical sensory aphasia. MRI showed symmetrical severe atrophy of the anterior part of the temporal and frontal lobes. The clinical diagnosis was FTD. He died at age 54 years after a clinical illness of approximately 5 years. Numerous argyrophilic grains were observed throughout the limbic system, temporal lobe, frontal lobe and brainstem. In addition, there were many tau-positive neurons and glial cells. These findings are all compatible with argyrophilic grain disease (AGD). Our case, however, is atypical AGD because of the young age of onset of the disease and sharply circumscribed cortical atrophy exhibiting severe neuronal loss and gliosis. Our case, together with some other similar cases of atypical AGD, gives rise to the possibility that this type of AGD would constitute a part of pathological background of FTD.
authors.affiliation: Department of Neurology, Ushioda General Hospital, Kanagawa, Japan. k-ishihara@mvj.biglobe.ne.jp
authors.forename: Kenji
authors.initials: K
authors.lastname: Ishihara
authors.forename: Shigeo
authors.initials: S
authors.lastname: Araki
authors.forename: Nami
authors.initials: N
authors.lastname: Ihori
authors.forename: Jun-ichi
authors.initials: J
authors.lastname: Shiota
authors.forename: Mitsuru
authors.initials: M
authors.lastname: Kawamura
authors.forename: Mari
authors.initials: M
authors.lastname: Yoshida
authors.forename: Yoshio
authors.initials: Y
authors.lastname: Hashizume
authors.forename: Imaharu
authors.initials: I
authors.lastname: Nakano
mesh_terms: D000328:Adult; D001921:Brain / Q000473:pathology*; D003704:Dementia / Q000209:etiology / Q000473:pathology*; D003937:Diagnosis, Differential; D005625:Frontal Lobe / Q000473:pathology*; D006801:Humans; D002479:Inclusion Bodies / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D019636:Neurodegenerative Diseases / Q000150:complications / Q000473:pathology*; D013702:Temporal Lobe / Q000473:pathology*