Noguchi Moeko, Yoshita Mitsuhiro, Sakai Kenji, Matsumoto Yasuko, Arahata Masahisa, Ontachi Yasuo, Takami Akiyoshi, Nakao Shinji, Sato Katsuaki, Yamada Masahito
Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Science, 13-1 Takara-machi, Kanazawa 920-8641, Japan.
J Neurol Sci. 2005 May 15;232(1-2):119-22. doi: 10.1016/j.jns.2005.01.013.
We report a patient with steroid-responsive peripheral neuropathy which developed with chronic natural killer cell lymphocytosis (CNKL). A 70-year-old female with a 2-week history of progressive motor and sensory neuropathy showed a marked increase of natural killer (NK) cells in the blood, and was diagnosed as having CNKL. Nerve conduction studies (NCS) revealed a mixed axonal and demyelinating neuropathy. A sural nerve biopsy revealed infiltration of NK cells into the nerve fascicles, and demyelinating changes with axonal degeneration. The infiltrating NK cells were adjacent to myelinated fibers, showing damage of Schwann cell membrane. Treatment with oral prednisolone resulted in rapid improvement of the sensory disturbance and weakness with a significant decrease of NK cells in the blood and disappearance of the conduction blocks in NCS. This is the first case of CNKL associated neuropathy in which infiltration of NK cells was demonstrated in the nerve fascicles. Our observations suggest that the infiltrating NK cells may directly damage myelin and Schwann cells, thus causing demyelination.
我们报告了一例患有类固醇反应性周围神经病的患者，该疾病与慢性自然杀伤细胞淋巴细胞增多症（CNKL）相关。一名70岁女性，有2周进行性运动和感觉神经病病史，血液中自然杀伤（NK）细胞显著增多，被诊断为CNKL。神经传导研究（NCS）显示为轴索性和脱髓鞘性混合性神经病。腓肠神经活检显示NK细胞浸润神经束，伴有脱髓鞘改变和轴索变性。浸润的NK细胞与有髓纤维相邻，显示施万细胞膜受损。口服泼尼松龙治疗导致感觉障碍和无力迅速改善，血液中NK细胞显著减少，NCS中传导阻滞消失。这是首例在神经束中证实有NK细胞浸润的CNKL相关性神经病。我们的观察结果提示，浸润的NK细胞可能直接损伤髓鞘和施万细胞，从而导致脱髓鞘。
