TY - JOUR
T1 - An autopsy case involving a 12-year history of amyotrophic lateral sclerosis with CIDP-like polyneuropathy
AU - Akaishi, Tetsuya
AU - Tateyama, Maki
AU - Kato, Kazuhiro
AU - Miura, Emiko
AU - Izumi, Rumiko
AU - Endo, Kaoru
AU - Sugeno, Naoto
AU - Suzuki, Naoki
AU - Baba, Toru
AU - Misu, Tatsuro
AU - Kikuchi, Akio
AU - Hasegawa, Takafumi
AU - Konosu-Fukaya, Sachiko
AU - Fujishima, Fumiyoshi
AU - Suzuki, Hiroyoshi
AU - Nakashima, Ichiro
AU - Aoki, Masashi
PY - 2014
Y1 - 2014
N2 - 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.
AB - 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.
KW - Amyotrophic lateral sclerosis
KW - Autopsy
KW - Chronic inflammatory demyelinating polyneuropathy
KW - Onion bulb formation
KW - TAR DNA-binding protein of 43 kDa
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U2 - 10.2169/internalmedicine.53.0774
DO - 10.2169/internalmedicine.53.0774
M3 - Article
C2 - 24930660
AN - SCOPUS:84902479854
SN - 0918-2918
VL - 53
SP - 1371
EP - 1375
JO - Internal Medicine
JF - Internal Medicine
IS - 12
ER -