title: Progressive nonfluent aphasia: a rare clinical subtype of FTLD-TDP in Japan.
abstract: Progressive nonfluent aphasia (PNFA) is a clinical subtype of frontotemporal lobar degeneration (FTLD). FTLD with tau accumulation (FTLD-tau) and FTLD with TDP-43 accumulation (FTLD-TDP) both cause PNFA. We reviewed clinical records of 29 FTLD-TDP cases in the brain archive of our institute and found only one case of PNFA. The patient was an 81-year-old male at death. There was no family history of dementia or aphasia. He presented with slow, labored and nonfluent speech at age 75. Behavioral abnormality and movement disorders were absent. MRI at age 76 demonstrated atrophy of the perisylvian regions, including the inferior frontal gyrus, insular gyrus and superior temporal gyrus. The atrophy was more severe in the left hemisphere than the right. On post mortem examinations, neuronal loss was evident in these regions as well as in the substantia nigra. There were abundant TDP-43-immunoreactive neuronal cytoplasmic inclusions and round or irregular-shaped structures in the affected cerebral cortices. A few dystrophic neurites and neuronal intranuclear inclusions were also seen. FTLD-TDP showing PNFA seems to be rare but does exist in Japan, similar to that in other countries.
authors: Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan.
authors: Naoya
authors: N
authors: Aoki
authors: Kuniaki
authors: K
authors: Tsuchiya
authors: Zen
authors: Z
authors: Kobayashi
authors: Tetsuaki
authors: T
authors: Arai
authors: Takashi
authors: T
authors: Togo
authors: Hiroshi
authors: H
authors: Miyazaki
authors: Hiromi
authors: H
authors: Kondo
authors: Hideki
authors: H
authors: Ishizu
authors: Hirotake
authors: H
authors: Uchikado
authors: Omi
authors: O
authors: Katsuse
authors: Yoshio
authors: Y
authors: Hirayasu
authors: Haruhiko
authors: H
authors: Akiyama
mesh_terms: D000369:Aged, 80 and over; D001284:Atrophy; D001921:Brain / Q000473:pathology; D018450:Disease Progression; D017809:Fatal Outcome; D057174:Frontotemporal Lobar Degeneration / Q000473:pathology* / Q000523:psychology; D007839:Functional Laterality / Q000502:physiology; D006801:Humans; D002479:Inclusion Bodies / Q000473:pathology; D007564:Japan; D008279:Magnetic Resonance Imaging; D008297:Male; D016501:Neurites / Q000473:pathology; D009474:Neurons / Q000473:pathology; D009483:Neuropsychological Tests; D057178:Primary Progressive Nonfluent Aphasia / Q000473:pathology* / Q000523:psychology; D057177:TDP-43 Proteinopathies / Q000473:pathology; D016707:Tissue Fixation; D025801:Ubiquitin / Q000378:metabolism
chemical_list: D025801:Ubiquitin