An Autopsy Case Involving a 12-year History of Amyotrophic Lateral Sclerosis with CIDP-like Polyneuropathy

作者：Akaishi Tetsuya; Tateyama Maki^*; Kato Kazuhiro; Miura Emiko; Izumi Rumiko; Endo Kaoru; Sugeno Naoto; Suzuki Naoki; Baba Toru; Misu Tatsuro; Kikuchi Akio; Hasegawa Takafumi; Konosu Fukaya Sachiko; Fujishima Fumiyoshi; Suzuki Hiroyoshi; Nakashima Ichiro; Aoki Masashi

来源：Internal Medicine, 2014, 53(12): 1371-1375.

DOI：10.2169/internalmedicine.53.0774

• amyotrophic lateral sclerosis
• chronic inflammatory demyelinating polyneuropathy
• autopsy
• onion bulb formation
• TAR DNA-binding protein of 43 kDa

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.

• 出版日期2014