Kikkawa Y, Kuwabara S, Nakajima M, Asahina M, Hirayama K
Department of Neurology, School of Medicine, Chiba University.
Rinsho Shinkeigaku. 1996 May;36(5):661-4.
We report a 23-year-old woman, who had a relapsing-remitting multiple mononeuropathy with multifocal nerve enlargement. The patient was characterized by asymmetrical, marked enlargement of multiple nerves in the arms (median and ulnar nerves) and neck (accessory nerve) bilaterally. Sequential nerve conduction studies revealed persistent demyelinative abnormalities, especially across the segment of nerve thickening. The MRI of the proximal arm confirmed the markedly enlarged median and ulnar nerves which showed high signal intensity on T2-weighted images and partial enhancement after administration of gadolinium. We consider that the patient had the clinical features of "multifocal pseudohypertrophic neuropathy" described by Adams et al, and that chronic demyelination and inflammation associated with impairment of the blood-nerve barrier might be underlying mechanisms.
我们报告一名23岁女性，患有复发缓解型多灶性单神经病伴多灶性神经增粗。该患者的特征为双侧手臂（正中神经和尺神经）和颈部（副神经）的多条神经不对称、明显增粗。连续的神经传导研究显示持续存在脱髓鞘异常，尤其是在神经增粗节段。上臂近端的MRI证实正中神经和尺神经明显增粗，在T2加权图像上呈高信号强度，注射钆后有部分强化。我们认为该患者具有Adams等人描述的“多灶性假肥大性神经病”的临床特征，与血神经屏障受损相关的慢性脱髓鞘和炎症可能是其潜在机制。
