title: Constant and severe involvement of Betz cells in corticobasal degeneration is not consistent with pyramidal signs: a clinicopathological study of ten autopsy cases.
abstract: This report concerns a clinicopathological study of three additional patients with corticobasal degeneration (CBD), described here for the first time, and a clinicopathological correlation between pyramidal signs and upper motor neuron involvement, in ten autopsy cases of CBD, including seven cases reported by us previously. We investigated pyramidal signs, including hyperreflexia, Babinski sign, and spasticity, and involvement of the primary motor cortex and pyramidal tract, focusing on the astrocytosis of the fifth layer of the primary motor cortex. Pyramidal signs were observed in six (60%) of the ten cases. Hyperreflexia was evident in six patients (60%), with spasticity being observed in three patients (30%). Loss of Betz cells associated with prominent astrocytosis and presence of ballooned neurons in the fifth layer of the primary motor cortex was observed in all ten cases. In all cases, involvement of the pyramidal tract was obvious in the medulla oblongata, without involvement of the pyramidal tract in the midbrain. Constant and severe involvement of the fifth layer of the primary motor cortex, including the Betz cells, has not previously been reported in CBD. We suggest that the pyramidal signs in CBD have been disregarded.
authors.affiliation: Department of Laboratory Medicine and Pathology, Tokyo Metropolitan Matsuzawa Hospital, 2-1-1, Kamikitazawa, Setagaya-ku, 156-0057 Tokyo, Japan. ktsuchi@jcom.home.ne.jp
authors.forename: Kuniaki
authors.initials: K
authors.lastname: Tsuchiya
authors.forename: Shigeo
authors.initials: S
authors.lastname: Murayama
authors.forename: Kazuko
authors.initials: K
authors.lastname: Mitani
authors.forename: Tatsuro
authors.initials: T
authors.lastname: Oda
authors.forename: Kunimasa
authors.initials: K
authors.lastname: Arima
authors.forename: Masaru
authors.initials: M
authors.lastname: Mimura
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Nagura
authors.forename: Chie
authors.initials: C
authors.lastname: Haga
authors.forename: Haruhiko
authors.initials: H
authors.lastname: Akiyama
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Yamanouchi
authors.forename: Hidehiro
authors.initials: H
authors.lastname: Mizusawa
mesh_terms: D000368:Aged; D001253:Astrocytes / Q000378:metabolism / Q000473:pathology; D001344:Autopsy / Q000379:methods; D001479:Basal Ganglia / Q000473:pathology* / Q000503:physiopathology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009044:Motor Cortex / Q000473:pathology* / Q000503:physiopathology; D009046:Motor Neurons / Q000473:pathology*; D009128:Muscle Spasticity / Q000209:etiology; D016874:Neurofibrillary Tangles / Q000473:pathology; D011712:Pyramidal Tracts / Q000473:pathology* / Q000503:physiopathology; D012021:Reflex, Abnormal / Q000502:physiology; D001405:Reflex, Babinski / Q000209:etiology; D013194:Staining and Labeling / Q000379:methods