Department of Neurology, Washington University in St Louis, St Louis, Missouri.
Division of Oncology, Department of Medicine, Washington University in St Louis, St Louis, Missouri.
JAMA Neurol. 2015 Dec;72(12):1519-23. doi: 10.1001/jamaneurol.2015.2390.
A 29-year-old woman presented with blurred vision and distal paresthesias. Her initial evaluation revealed severe bilateral optic disc edema with distal lower-extremity sensory and motor deficits and electrodiagnostic evidence of a length-dependent mixed demyelinating and axonal polyneuropathy. The results of routine diagnostic testing, including laboratory tests, magnetic resonance imaging, and lumbar puncture, were nondiagnostic. A targeted biopsy was ultimately required for diagnosis. In this article, we discuss the differential diagnosis and outline the clinical evaluation indicated for a patient presenting with demyelinating polyneuropathy and concurrent papilledema.
一位 29 岁女性因视力模糊和远端感觉异常就诊。她的初步评估显示双侧视盘严重水肿,伴有下肢远端感觉和运动功能障碍,电诊断结果显示为长度依赖性混合脱髓鞘和轴索性多发性神经病。常规诊断性检查(包括实验室检查、磁共振成像和腰椎穿刺)结果无明确诊断。最终需要进行靶向活检以明确诊断。本文讨论了这种脱髓鞘性多发性神经病伴并发视乳头水肿患者的鉴别诊断,并概述了提示性的临床评估方法。