Department of Neurology, Neurological Institute, Columbia University, 710 W 168th Street, Box 163, New York, New York 10032 USA.
Muscle Nerve. 2013 Feb;47(2):292-6. doi: 10.1002/mus.23629. Epub 2012 Nov 21.
Distal acquired demyelinating symmetric polyneuropathy (DADS) is proposed as a distinct entity from classic chronic inflammatory demyelinating polyneuropathy (CIDP).
We report a 58-year-old woman with DADS that progressed to a severe case of classic CIDP.
She had distal numbness and paresthesias, minimal distal weakness and impaired vibratory sensation. She had anti-MAG antibodies, negative Western blot, and lacked a monoclonal gammopathy. There were prolonged distal motor latencies. She remained stable for 6 years until developing proximal and distal weakness. Nerve conduction studies showed multiple conduction blocks. She developed quadriparesis despite first-line treatment for CIDP. She was started on cyclophosphamide and fludarabine. Twenty-five months after receiving chemotherapy, she had only mild signs of neuropathy off all immunotherapy.
DADS may progress to classic CIDP and is unlikely to be a separate disorder. Fludarabine and cyclophosphamide may be effective for refractory CIDP.
远端获得性脱髓鞘对称性多发性神经病（DADS）被认为是一种与经典慢性炎症性脱髓鞘性多发性神经病（CIDP）不同的实体疾病。
我们报告了一例 58 岁女性患有 DADS，后来进展为严重的经典 CIDP。
她有远端麻木和感觉异常，轻微的远端无力和振动感觉受损。她有抗 MAG 抗体，Western blot 阴性，没有单克隆丙种球蛋白病。存在远端运动潜伏期延长。她在 6 年内保持稳定，直到出现近端和远端无力。神经传导研究显示多处传导阻滞。尽管对 CIDP 进行了一线治疗，她还是出现了四肢瘫痪。她开始接受环磷酰胺和氟达拉滨治疗。在接受化疗 25 个月后，她停止了所有免疫治疗，仅有轻微的神经病变迹象。
DADS 可能进展为经典 CIDP，不太可能是一种单独的疾病。氟达拉滨和环磷酰胺可能对难治性 CIDP 有效。
