Subcortical neurofibrillary tangles and argyrophilic grains in a case of familial frontotemporal dementia with parkinsonism.
A case of familial frontotemporal dementia with parkinsonism (FTDP) similar to progressive supranuclear palsy (PSP) was reported. A 58-year-old man developed personality change followed by parkinsonism and dementia. Three family members showed similar symptoms. Cerebral atrophy was marked on the anterior frontotemporal lobes. The substantia nigra, hippocampus, peri-aqueductal gray matter and pontine nucleus were affected with globose neurofibrillary tangles (NFT) and glial tangles. Argyrophilic grains were distributed in the CA1-CA2. NFT, glial tangles and argyrophilic grains expressed four-repeat microtubule-associated protein tau (MAPT). MAPT gene had no mutation. Familial occurrence of FTDP with PSP-like tauopathy is rare.
Kobayashi
Katsuji
K
Department of Psychiatry and Neurobiology, Graduate School of Medical Sciences, Kanazawa University, Kanazawa, Japan. k-koba@ta2.so-net.ne.jp
Sudo
Satoru
S
Matsubara
Rokurou
R
Nakano
Hiroyuki
H
Koshino
Yoshifumi
Y
D001344:Autopsy; D001921:Brain / Q000473:pathology; D002540:Cerebral Cortex / Q000473:pathology; D003704:Dementia / Q000150:complications / Q000473:pathology* / Q000523:psychology; D006801:Humans; D002479:Inclusion Bodies / Q000473:pathology*; D008297:Male; D008869:Microtubule-Associated Proteins / Q000235:genetics; D008875:Middle Aged; D009154:Mutation / Q000235:genetics; D016874:Neurofibrillary Tangles / Q000473:pathology*; D010300:Parkinson Disease / Q000150:complications / Q000473:pathology*; D016875:tau Proteins / Q000235:genetics
D008869:Microtubule-Associated Proteins; D016875:tau Proteins