Nokura Kazuya, Nagamatsu Masaaki, Inagaki Toshiaki, Yamamoto Hiroko, Koga Hiroshi, Sugimura Kimiya, Yoshida Mari, Hashizume Yoshio
Department of Neurology, Second Teaching Hospital, Ban Buntane Hotokukai Hospital, School of Medicine, Fujita Health University, Nagoya, Japan.
Neuropathology. 2006 Aug;26(4):329-37. doi: 10.1111/j.1440-1789.2006.00698.x.
A 48-year-old Chinese woman developed ascending motor paralysis while visiting Japan, leading to tetraplegia and respiratory failure over 2 weeks. The patient's course was complicated by anoxic encephalopathy. Nerve conduction studies revealed a severely decreased amplitude of compound muscle action potentials and a sural nerve biopsy specimen showed findings consistent with axonal-form Guillain-Barr6 syndrome. An autopsy, excluding the brain, demonstrated small-cell lung cancer that was not detected clinically, axonal-dominant degeneration in the nerve roots and distal peripheral nerves, and the loss of both myelin and axons in the dorsal spinal column. The spinal anterior horn cells were severely decreased and were accompanied by astrocytic reaction in all spinal segments with lymphocytic infiltration. A limited examination of the dorsal root ganglia did not show Nageotte nodules, but the infiltration of T cells was observed. Although the clinical course mimicked axonal-form Guillain-Barré syndrome, the autopsy demonstrated both sensory and motor neuronal involvement, as well as small-cell lung cancer. Although anti-Hu and antiganglioside antibodies were negative in the patient's serum, the para-neoplastic mechanism might have damaged the anterior horn and dorsal root ganglia cells, which subsequently led to secondary axonal degeneration. There has been a report on a case of paraneoplastic subacute motor neuronopathy, but the acute course described here has not been reported before.
一名48岁的中国女性在访问日本期间出现进行性上升性运动麻痹,2周内发展为四肢瘫痪和呼吸衰竭。患者病程并发缺氧性脑病。神经传导研究显示复合肌肉动作电位幅度严重降低,腓肠神经活检标本显示与轴索性吉兰-巴雷综合征一致的表现。尸检(不包括脑部)显示临床未检测到的小细胞肺癌、神经根和远端周围神经的轴索性为主的变性以及脊髓背柱中髓鞘和轴突的丧失。脊髓前角细胞严重减少,所有脊髓节段均伴有星形细胞反应和淋巴细胞浸润。对背根神经节的有限检查未发现纳热奥特小结,但观察到T细胞浸润。尽管临床病程类似轴索性吉兰-巴雷综合征,但尸检显示感觉和运动神经元均受累,以及小细胞肺癌。尽管患者血清中的抗Hu和抗神经节苷脂抗体均为阴性,但副肿瘤机制可能损害了前角和背根神经节细胞,随后导致继发性轴索变性。有一篇关于副肿瘤性亚急性运动神经元病病例的报告,但此处描述的急性病程此前未见报道。