Frontotemporal dementia with co-occurrence of astrocytic plaques and tufted astrocytes, and severe degeneration of the cerebral white matter: a variant of corticobasal degeneration?
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.
Tan
Chun-Feng
CF
Department of Pathology, Brain Research Institute, University of Niigata, 1-757 Asahimachi, 951-8585, Niigata, Japan.
Piao
Yue-Shan
YS
Kakita
Akiyoshi
A
Yamada
Mitsunori
M
Takano
Hiroki
H
Tanaka
Masaharu
M
Mano
Atsushi
A
Makino
Kunihiko
K
Nishizawa
Masatoyo
M
Wakabayashi
Koichi
K
Takahashi
Hitoshi
H
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
D016229:Amyloid beta-Peptides; D005904:Glial Fibrillary Acidic Protein; D009419:Nerve Tissue Proteins; D051843:Synucleins; D014326:Tropanes; D016875:tau Proteins; C100236:homatropine