Sano Hironori, Maeda Toshihiko, Omoto Masatoshi, Ogasawara Jun-Ichi, Koga Michiaki, Kawai Motoharu, Kanda Takashi
Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine.
Rinsho Shinkeigaku. 2017 Oct 27;57(10):573-578. doi: 10.5692/clinicalneurol.cn-001064. Epub 2017 Sep 28.
We herein report the findings of a 67-year-old woman with steroid-responsive multiple mononeuropathy associated with chronic natural killer (NK) cell lymphocytosis. The patient developed progressive, asymmetric weakness and numbness in all four extremities in the course of a three-month period. Nerve conduction studies revealed asymmetric demyelination in both the motor and sensory nerves, and a biopsy specimen of the sural nerve showed a conspicuous difference in the demyelination between the neighboring fascicles and the infiltration of NK cells in the endoneurium. We considered the multiple mononeuropathy in this patient to have been caused by NK cell infiltration in the endoneurium, and the observed asymmetry might have been due to differences in the NK cell intrusion among the fascicles. Corticosteroid administration resulted in a rapid neurological, electrophysiological and hematological improvement. The rapid clinical amelioration that was observed after corticosteroid therapy suggested that the neuropathy in this case had been mainly caused by the mechanical compression of the endoneurial NK cells or the inflammatory cytokines that had been released by them.
我们在此报告一名67岁女性的病例，该患者患有与慢性自然杀伤（NK）细胞淋巴细胞增多症相关的类固醇反应性多灶性单神经病。患者在三个月内出现四肢进行性、不对称的无力和麻木。神经传导研究显示运动和感觉神经均存在不对称性脱髓鞘，腓肠神经活检标本显示相邻束之间脱髓鞘存在明显差异，且神经内膜有NK细胞浸润。我们认为该患者的多灶性单神经病是由神经内膜NK细胞浸润引起的，观察到的不对称性可能是由于各束之间NK细胞侵入情况不同所致。给予皮质类固醇后，患者的神经功能、电生理和血液学状况迅速改善。皮质类固醇治疗后观察到的快速临床改善表明，该病例中的神经病变主要是由神经内膜NK细胞的机械压迫或它们释放的炎性细胞因子所致。
