Nishiyama K, Kurisaki H, Masuda N, Kusunoki S, Takatsu M
Department of Neurology, Tokyo National Hospital, Japan.
Neuromuscul Disord. 1993 May;3(3):227-9. doi: 10.1016/0960-8966(93)90064-q.
A 76-yr-old male patient with carcinomatous neuropathy associated with hepatic cell carcinoma, whose initial symptom was deep sensory disturbance followed by muscle weakness is described. The onset was subacute, followed by slow progression. Sural nerve biopsy, as well as electrophysiological examinations, revealed severe axonal degeneration without any evidence of demyelination. The autopsy findings were similar to findings described in the literature on carcinomatous neuropathy. Although carcinomatous neuropathy is usually associated with lung cancer, this report describes an association with hepatic cell carcinoma. The patient also had motor nerve involvement with positive serum anti-GM1 ganglioside antibody which decreased after immunosuppressant therapy in parallel with recovery of muscle weakness. The anti-GM1 ganglioside antibody may be involved in the pathogenesis of motor disturbance in the present case.
本文描述了一位76岁男性患者,其患有与肝细胞癌相关的癌性神经病变,初始症状为深感觉障碍,随后出现肌肉无力。起病亚急性,随后缓慢进展。腓肠神经活检及电生理检查显示严重轴索性变性,无脱髓鞘证据。尸检结果与文献中描述的癌性神经病变相符。尽管癌性神经病变通常与肺癌相关,但本报告描述了其与肝细胞癌的关联。该患者还存在运动神经受累,血清抗GM1神经节苷脂抗体阳性,免疫抑制治疗后该抗体水平下降,同时肌肉无力症状有所恢复。抗GM1神经节苷脂抗体可能参与了本例运动障碍的发病机制。