Naganuma M, Doi S, Shima K, Matsumoto A, Tashiro K
Department of Neurology, Hokkaido University School of Medicine.
Rinsho Shinkeigaku. 1991 Nov;31(11):1186-91.
We reported a 29-year-old woman who had chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) associated with multifocal nerve hypertrophy. She developed chronic progressive muscular weakness of distal part of her extremities and sensory ataxia of limbs and trunk for five years. Steroid therapy improved most of her signs and symptoms. No recurrence has occurred for the subsequent four years until present time. Magnetic resonance imaging (MRI) revealed multifocal and nodular hypertrophy of her peripheral nerves in the extremities. MRI also detected the hypertrophy of posterior nerve ganglia and extradural nerve roots. Hypertrophic nerve trunk comprised of multifocal and nodular hypertrophy of the nerve fiber fascicles which were detected as abnormal high signal intensity area in MR T2-weighted images. Her sural nerve contained no onion-bulb formations and presented normal low signal in MR T2-weighted images. We concluded that the hypertrophic changes occurred multifocally in and among nerve fascicles predominantly at the proximal part of the nerves of the extremities. MRI is useful method to detect the hypertrophic change of peripheral nerves deep in the trunk and the extremities in CIDP.
我们报告了一名29岁女性，她患有与多灶性神经肥大相关的慢性炎症性脱髓鞘性多发性神经根神经病（CIDP）。她出现四肢远端慢性进行性肌肉无力以及四肢和躯干感觉性共济失调达五年之久。类固醇治疗改善了她的大部分体征和症状。直至目前，在随后的四年里未出现复发情况。磁共振成像（MRI）显示其四肢周围神经存在多灶性和结节状肥大。MRI还检测到后神经节和硬膜外神经根肥大。肥大的神经干由神经纤维束的多灶性和结节状肥大组成，在MR T2加权图像中被检测为异常高信号区。她的腓肠神经未出现洋葱球样结构，在MR T2加权图像中呈正常低信号。我们得出结论，肥大性改变主要在四肢神经近端的神经束内及神经束之间多灶性发生。MRI是检测CIDP患者躯干和四肢深部周围神经肥大性改变的有用方法。
