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
Department of Neurology, Tohoku University School of Medicine, Japan.
Intern Med. 2014;53(12):1371-5. doi: 10.2169/internalmedicine.53.0774. Epub 2014 Jun 15.
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.
与肌萎缩侧索硬化症（ALS）相关的脱髓鞘性多发性神经病相当罕见。我们在此报告一例女性患者，她有12年慢性炎症性脱髓鞘性多发性神经病（CIDP）样多发性神经病病史，后来发展为延髓麻痹和呼吸衰竭。尸检结果显示前角和初级运动皮层神经元丢失，皮质脊髓束变性。在前角和包括颞叶在内的大脑皮层中观察到弥漫性43 kDa磷酸化TAR DNA结合蛋白包涵体。最终诊断为伴有CIDP样多发性神经病的ALS。与其他伴有CIDP样多发性神经病的ALS报告相比，本患者年龄较轻，临床病程相对较长，且无上运动神经元体征。
