title: Constant involvement of the Betz cells and pyramidal tract in amyotrophic lateral sclerosis with dementia: a clinicopathological study of eight autopsy cases.
abstract: We investigated clinicopathologically pyramidal signs, including hyperreflexia, Babinski sign, and spasticity, and the involvement of the primary motor cortex and pyramidal tract, in eight Japanese autopsy cases of amyotrophic lateral sclerosis (ALS) with dementia. Pyramidal signs were observed in seven (88%) of the eight autopsy cases. Hyperreflexia and Babinski sign were evident in seven (88%) and three (38%) patients, respectively, but spasticity was not observed in any of the eight patients. Loss of Betz cells in the primary motor cortex was evident in the seven cases in which this structure was examined. Astrocytosis in the fifth layer of the primary motor cortex was noticed in three cases. In all eight cases, involvement of the pyramidal tract was obvious in the medulla oblongata, but no involvement of the pyramidal tract was found in the midbrain. Involvement of the pyramidal tract in the spinal cord, particularly of large myelinated fibers, was observed in all six cases in which the spinal cord was examined. In ALS with dementia, pyramidal signs were shown to be present more frequently than previously believed, and the clinicopathological correlation between pyramidal signs and involvement of the pyramidal tract was obvious. Constant involvement of Betz cells and the pyramidal tract in ALS with dementia has not been reported. Our clinicopathological findings may make a contribution to the understanding of the clinicopathological hallmarks of this disorder. Furthermore, we believe that this study will also contribute to the elucidation of the nosological status of ALS with dementia.
authors.affiliation: Department of Laboratory Medicine and Pathology, Tokyo Metropolitan Matsuzawa Hospital, 2-1-1 Kamikitazawa, Setagaya-ku, Tokyo 156-0057, Japan. ktsuchi@dd.mbn.or.jp
authors.forename: K
authors.initials: K
authors.lastname: Tsuchiya
authors.forename: K
authors.initials: K
authors.lastname: Ikeda
authors.forename: M
authors.initials: M
authors.lastname: Mimura
authors.forename: M
authors.initials: M
authors.lastname: Takahashi
authors.forename: H
authors.initials: H
authors.lastname: Miyazaki
authors.forename: M
authors.initials: M
authors.lastname: Anno
authors.forename: H
authors.initials: H
authors.lastname: Shiotsu
authors.forename: H
authors.initials: H
authors.lastname: Akabane
authors.forename: K
authors.initials: K
authors.lastname: Niizato
authors.forename: T
authors.initials: T
authors.lastname: Uchihara
authors.forename: I
authors.initials: I
authors.lastname: Tominaga
authors.forename: I
authors.initials: I
authors.lastname: Nakano
mesh_terms: D000328:Adult; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000473:pathology* / Q000503:physiopathology*; D005260:Female; D006801:Humans; D002479:Inclusion Bodies; D008297:Male; D008526:Medulla Oblongata / Q000473:pathology; D008875:Middle Aged; D009044:Motor Cortex / Q000473:pathology* / Q000503:physiopathology*; D009128:Muscle Spasticity / Q000503:physiopathology; D009474:Neurons / Q000473:pathology; D011712:Pyramidal Tracts / Q000473:pathology* / Q000503:physiopathology*; D012021:Reflex, Abnormal / Q000502:physiology; D001405:Reflex, Babinski / Q000503:physiopathology