title: Clinicopathological characterization of Pick's disease versus frontotemporal lobar degeneration with ubiquitin/TDP-43-positive inclusions.
abstract: Although frontotemporal lobar degeneration with ubiquitin/TDP-43-positive inclusions (FTLD-TDP) and Pick's disease are common pathological substrates in sporadic FTLD, clinical differentiation of these diseases is difficult. We performed a retrospective review of medical records and semiquantitative examination of neuronal loss of 20 sporadic FTLD-TDP and 19 Pick's disease cases. Semantic dementia as the first syndrome developed only in FTLD-TDP patients. Impaired speech output in the early stage was five times more frequent in Pick's disease than in FTLD-TDP. The total frequency of asymmetric motor disturbances (e.g., parkinsonism, pyramidal signs, and contracture) during the course was significantly more frequent in FTLD-TDP (78%) than in Pick's disease cases (14%). Asymmetric pyramidal signs were found in 7 of 13 FTLD-TDP cases with corticospinal tract degeneration similar to primary lateral sclerosis. Frontotemporal dementia as the first syndrome was noted in both FTLD-TDP (28%) and Pick's disease cases (64%); however, only FTLD-TDP cases subsequently developed asymmetric motor disturbances, and some of the cases further exhibited hemineglect. Concordant with these clinical findings, degeneration in the temporal cortex, caudate nucleus, putamen, globus pallidus, substantia nigra, and corticospinal tract was significantly more severe in FTLD-TDP, and degeneration in the frontal cortex tended to be more severe in Pick's disease. Given these findings, the initial impairment of semantic memory or comprehension and subsequent asymmetric motor disturbances in sporadic FTLD patients predict sporadic FTLD-TDP rather than Pick's disease, while initial behavioral symptoms or non-fluent aphasia without subsequent asymmetric motor disturbances predict Pick's disease rather than sporadic FTLD-TDP.
authors.affiliation: Department of Neuropathology, Tokyo Institute of Psychiatry, 2-1-8 Kamikitazawa, Setagaya-ku, Tokyo, 156-8585, Japan. oyokota1@yahoo.co.jp
authors.forename: Osamu
authors.initials: O
authors.lastname: Yokota
authors.forename: Kuniaki
authors.initials: K
authors.lastname: Tsuchiya
authors.forename: Tetsuaki
authors.initials: T
authors.lastname: Arai
authors.forename: Saburo
authors.initials: S
authors.lastname: Yagishita
authors.forename: Osamu
authors.initials: O
authors.lastname: Matsubara
authors.forename: Akihide
authors.initials: A
authors.lastname: Mochizuki
authors.forename: Akira
authors.initials: A
authors.lastname: Tamaoka
authors.forename: Mitsuru
authors.initials: M
authors.lastname: Kawamura
authors.forename: Hidetoshi
authors.initials: H
authors.lastname: Yoshida
authors.forename: Seishi
authors.initials: S
authors.lastname: Terada
authors.forename: Hideki
authors.initials: H
authors.lastname: Ishizu
authors.forename: Shigetoshi
authors.initials: S
authors.lastname: Kuroda
authors.forename: Haruhiko
authors.initials: H
authors.lastname: Akiyama
mesh_terms: D001921:Brain / Q000473:pathology; D001923:Brain Chemistry; D016923:Cell Death; D004268:DNA-Binding Proteins / Q000032:analysis*; D003704:Dementia / Q000150:complications / Q000175:diagnosis / Q000473:pathology* / Q000503:physiopathology*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D002479:Inclusion Bodies / Q000737:chemistry* / Q000648:ultrastructure; D007806:Language Disorders / Q000209:etiology; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology; D009069:Movement Disorders / Q000209:etiology; D009410:Nerve Degeneration / Q000209:etiology; D009457:Neuroglia / Q000502:physiology; D009474:Neurons / Q000473:pathology; D020774:Pick Disease of the Brain / Q000150:complications / Q000175:diagnosis / Q000473:pathology* / Q000503:physiopathology*; D012189:Retrospective Studies; D013116:Spinal Cord / Q000473:pathology; D025801:Ubiquitin / Q000032:analysis*
chemical_list: D004268:DNA-Binding Proteins; D025801:Ubiquitin