title: An autopsy case involving a 12-year history of amyotrophic lateral sclerosis with CIDP-like polyneuropathy.
abstract: Demyelinating polyneuropathy associated with amyotrophic lateral sclerosis (ALS) is quite rare. We herein present the case of a woman patient with a 12-year history of chronic inflammatory demyelinating polyneuropathy (CIDP)-like polyneuropathy who later developed bulbar palsy and respiratory failure. The autopsy findings revealed neuronal loss in the anterior horn and primary motor cortex with degeneration of the corticospinal tracts. Diffuse phosphorylated TAR DNA-binding protein of 43 kDa inclusions were observed in the anterior horn and cerebral cortices, including the temporal lobe. The final diagnosis was ALS with CIDP-like polyneuropathy. Compared with other reports of ALS with CIDP-like polyneuropathy, the present patient was younger and followed a relatively long clinical course, with no upper motor neuron signs.
authors.affiliation: Department of Neurology, Tohoku University School of Medicine, Japan.
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Akaishi
authors.forename: Maki
authors.initials: M
authors.lastname: Tateyama
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Kato
authors.forename: Emiko
authors.initials: E
authors.lastname: Miura
authors.forename: Rumiko
authors.initials: R
authors.lastname: Izumi
authors.forename: Kaoru
authors.initials: K
authors.lastname: Endo
authors.forename: Naoto
authors.initials: N
authors.lastname: Sugeno
authors.forename: Naoki
authors.initials: N
authors.lastname: Suzuki
authors.forename: Toru
authors.initials: T
authors.lastname: Baba
authors.forename: Tatsuro
authors.initials: T
authors.lastname: Misu
authors.forename: Akio
authors.initials: A
authors.lastname: Kikuchi
authors.forename: Takafumi
authors.initials: T
authors.lastname: Hasegawa
authors.forename: Sachiko
authors.initials: S
authors.lastname: Konosu-Fukaya
authors.forename: Fumiyoshi
authors.initials: F
authors.lastname: Fujishima
authors.forename: Hiroyoshi
authors.initials: H
authors.lastname: Suzuki
authors.forename: Ichiro
authors.initials: I
authors.lastname: Nakashima
authors.forename: Masashi
authors.initials: M
authors.lastname: Aoki
mesh_terms: D000328:Adult; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D001344:Autopsy; D004268:DNA-Binding Proteins / Q000378:metabolism; D005260:Female; D006801:Humans; D008875:Middle Aged; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000150:complications / Q000378:metabolism / Q000473:pathology*
chemical_list: D004268:DNA-Binding Proteins