title: Frontotemporal dementia with co-occurrence of astrocytic plaques and tufted astrocytes, and severe degeneration of the cerebral white matter: a variant of corticobasal degeneration?
abstract: We report two patients who exhibited frontotemporal dementia (FTD) with unusual neuropathological features. The ages of the patients at death were 65 and 67 years, the disease durations were 6 and 5 years, and the clinical diagnoses were Pick's disease and corticobasal degeneration (CBD), respectively. At autopsy, both cases exhibited neuropathological findings compatible with those of CBD, including atrophy of the frontal and parietal lobes, neuronal loss and gliosis in the cortical and subcortical regions, and presence of cortical ballooned neurons and astrocytic plaques (APs). In both cases, immunoblotting of insoluble tau exhibited the pattern of selective accumulation of four-repeat tau, a finding that is also compatible with CBD. However, severe degeneration was evident in the frontal and parietal white matter in both cases. Moreover, a striking finding was the widespread presence in the affected cortex of tufted astrocytes (TAs), which are characteristic of progressive supranuclear palsy (PSP). Neither co-occurrence of APs and TAs nor severe degeneration of the cerebral white matter is a feature of either CBD or PSP. No mutations were found in the tau gene in either case. In conclusion, the possibility that these two cases represent a new neuropathological phenotype of non-familial FTD rather than simply a variant of CBD cannot be completely excluded.
authors.affiliation: Department of Pathology, Brain Research Institute, University of Niigata, 1-757 Asahimachi, 951-8585, Niigata, Japan.
authors.forename: Chun-Feng
authors.initials: CF
authors.lastname: Tan
authors.forename: Yue-Shan
authors.initials: YS
authors.lastname: Piao
authors.forename: Akiyoshi
authors.initials: A
authors.lastname: Kakita
authors.forename: Mitsunori
authors.initials: M
authors.lastname: Yamada
authors.forename: Hiroki
authors.initials: H
authors.lastname: Takano
authors.forename: Masaharu
authors.initials: M
authors.lastname: Tanaka
authors.forename: Atsushi
authors.initials: A
authors.lastname: Mano
authors.forename: Kunihiko
authors.initials: K
authors.lastname: Makino
authors.forename: Masatoyo
authors.initials: M
authors.lastname: Nishizawa
authors.forename: Koichi
authors.initials: K
authors.lastname: Wakabayashi
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Takahashi
mesh_terms: D016229:Amyloid beta-Peptides / Q000378:metabolism; D001253:Astrocytes / Q000378:metabolism / Q000473:pathology*; D001284:Atrophy / Q000473:pathology; D001480:Basal Ganglia Diseases / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D015153:Blotting, Western / Q000379:methods; D003704:Dementia / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D005260:Female; D005625:Frontal Lobe / Q000473:pathology*; D005904:Glial Fibrillary Acidic Protein / Q000378:metabolism; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000378:metabolism; D009474:Neurons / Q000473:pathology; D020774:Pick Disease of the Brain / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D058225:Plaque, Amyloid / Q000473:pathology*; D051843:Synucleins; D014326:Tropanes; D016875:tau Proteins / Q000235:genetics / Q000378:metabolism
chemical_list: D016229:Amyloid beta-Peptides; D005904:Glial Fibrillary Acidic Protein; D009419:Nerve Tissue Proteins; D051843:Synucleins; D014326:Tropanes; D016875:tau Proteins; C100236:homatropine