title: Ubiquitin-positive frontotemporal lobar degeneration presenting with progressive Gogi (word-meaning) aphasia. A neuropsychological, radiological and pathological evaluation of a Japanese semantic dementia patient.
abstract: A patient with progressive anomia and alexia with agraphia for kanji (Japanese morphograms) is described. The patient showed a deficit in single-word comprehension and on-reading (a type of reading that conveys phonetic value) dominance in kanji reading, i.e. on-preceding (pronouncing first with on-reading, irrespective of its preferred reading) and kun-deletion (inability to recall and recognize kun-reading [another type of reading that conveys meaning]) when reading a single-character kanji. These features were due to loss of lexico-semantic information and thus the patient was regarded as having progressive Gogi (word-meaning) aphasia by Imura, a Japanese manifestation of semantic dementia. Macroscopically, neuropathological examination disclosed atrophy of the left frontotemporal lobe with accentuation in the anterior portion of the temporal lobe. Histologically, there was neuronal loss in the cerebral cortex, hippocampus, parahippocampal gyrus, amygdala, caudate nucleus, and putamen. Ubiquitin-immunoreactive neuronal inclusions were present in the hippocampal dentate granular cells. This case demonstrates that progressive Gogi aphasia is semiologically identical to semantic dementia, and our patient clinicopathologically resembled those of Rossor et al. [Rossor, M.N., Revesz, T., Lantos, P.L., Warrington, E.K. Semantic dementia with ubiquitin-positive tau-negative inclusion bodies. Brain 2000; 123: 267-76.] and Hodges et al. [Hodges, J.R., Davies, R.R., Xuereb, J.H., Casey, B., Broe, M., Bak, T.H., et al. Clinicopathological correlates in frontotemporal dementia. Ann Neurol 2004; 56: 399-406.].
authors.affiliation: Department of Neurology, Mitsui Memorial Hospital, 1 Kanda-Izumi-cho, Chiyoda-ku, Tokyo 101-8643, Japan. ysakurai-tky@umin.ac.jp
authors.forename: Yasuhisa
authors.initials: Y
authors.lastname: Sakurai
authors.forename: Kuniaki
authors.initials: K
authors.lastname: Tsuchiya
authors.forename: Tatsuro
authors.initials: T
authors.lastname: Oda
authors.forename: Koji
authors.initials: K
authors.lastname: Hori
authors.forename: Itaru
authors.initials: I
authors.lastname: Tominaga
authors.forename: Haruhiko
authors.initials: H
authors.lastname: Akiyama
authors.forename: Mitsuaki
authors.initials: M
authors.lastname: Bando
authors.forename: Chie
authors.initials: C
authors.lastname: Haga
authors.forename: Makoto
authors.initials: M
authors.lastname: Iwata
authors.forename: Toru
authors.initials: T
authors.lastname: Mannen
mesh_terms: D000368:Aged; D001037:Aphasia / Q000175:diagnosis* / Q000209:etiology* / Q000503:physiopathology; D001284:Atrophy / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D001921:Brain / Q000473:pathology* / Q000503:physiopathology; D003704:Dementia / Q000150:complications* / Q000175:diagnosis* / Q000503:physiopathology; D003937:Diagnosis, Differential; D018450:Disease Progression; D004411:Dyslexia, Acquired / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D006801:Humans; D002479:Inclusion Bodies / Q000378:metabolism / Q000473:pathology; D007564:Japan; D007807:Language Tests; D008297:Male; D009474:Neurons / Q000378:metabolism / Q000473:pathology; D009483:Neuropsychological Tests; D020774:Pick Disease of the Brain / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology; D011237:Predictive Value of Tests; D014057:Tomography, X-Ray Computed; D025801:Ubiquitin / Q000378:metabolism*; D014705:Verbal Behavior / Q000502:physiology
chemical_list: D025801:Ubiquitin