Fujiwara T, Takagi S, Saitou T, Ueki A
1st Department of Sogo-Igaku, Jichi Medical School, Omiya Medical Center.
Rinsho Ketsueki. 1993 Nov;34(11):1452-7.
A case of Waldenström's macroglobulinemia (WM) (IgM-kappa type) associated with acute-onset demyelinating peripheral neuropathy is reported. A 49-year-old woman was admitted to our hospital because of general fatigue and recurrent syncope attacks. She was treated with vincristine, cyclophosphamide, epirubicin and prednisolone. By 10th hospital day, her clinical condition improved and serum viscosity was reduced. However, on the 21st hospital day, she suffered from rapidly progressive writing and gait disturbance. Neurological examination showed muscular atrophy and weakness in the distal part of four extremities. Deep tendon reflexes were diminished. There was no sensory deficit. Cerebrospinal fluid was normal. Anti-myelin associated glycoprotein activity of her serum was negative. Both motor and sensory nerve conduction velocities were markedly decreased. Biopsy of sural nerve revealed marked demyelination and onion bulb formation. There was IgM deposition on myelin sheath. Minimal axonal changes excluded the possibility of vincristine neuropathy. Plasmapheresis improved her symptoms, but nerve conduction velocities remained unchanged. Polyneuropathy associated with WM is usually gradual onset and sensory dominant. In this case, associated neuropathy was acute onset, progressive and motor dominant. This type of neuropathy in patients with WM is very rare.
报告了一例与急性起病的脱髓鞘性周围神经病相关的华氏巨球蛋白血症（WM）（IgM-κ型）病例。一名49岁女性因全身乏力和反复晕厥发作入院。她接受了长春新碱、环磷酰胺、表柔比星和泼尼松龙治疗。到住院第10天时，她的临床状况改善，血清黏度降低。然而，在住院第21天时，她出现了迅速进展的书写和步态障碍。神经系统检查显示四肢远端肌肉萎缩和无力。腱反射减弱。无感觉障碍。脑脊液正常。她血清中的抗髓鞘相关糖蛋白活性为阴性。运动和感觉神经传导速度均明显降低。腓肠神经活检显示明显的脱髓鞘和洋葱球形成。髓鞘上有IgM沉积。轻微的轴突改变排除了长春新碱性神经病的可能性。血浆置换改善了她的症状，但神经传导速度仍未改变。与WM相关的多发性神经病通常起病隐匿且以感觉为主。在该病例中，相关神经病起病急性、进展性且以运动为主。WM患者中这种类型的神经病非常罕见。
