以臂丛病为表现的遗传性神经性压迫性麻痹(HNPP):完全电诊断试验的价值

S. Bulusu, H. McMillan
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引用次数: 12

摘要

摘要遗传性神经病变伴压迫性麻痹(HNPP)的患者通常表现为单神经病变(特别是腓骨或尺神经麻痹)或臂丛病。仔细的电诊断测试在确定HNPP的诊断,将其与其他遗传或获得性神经病变区分开来以及避免不必要的手术需要方面具有重要作用。我们报告了一例无痛性臂丛病患者,在神经传导研究中发现有多处节段性脱髓鞘,与HNPP一致。我们回顾了HNPP的临床和电诊断特征,包括筛选这种疾病的关键电诊断结果。
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A Report of Hereditary Neuropathy with Liability to Pressure Palsy (HNPP) Presenting with Brachial Plexopathy: The Value of Complete Electrodiagnostic Testing
ABSTRACT. Patients with hereditary neuropathy with liability to pressure palsy (HNPP) typically present with a mononeuropathy (particularly peroneal or ulnar palsy) or a brachial plexopathy. Careful electrodiagnostic testing has an important role in establishing the diagnosis of HNPP, differentiating this condition from other inherited or acquired neuropathies as well as obviating the need for unnecessary surgeries. We present a case of a patient who presented with a painless brachial plexopathy who was found to have multiple sites of segmental demyelination on nerve conduction studies, consistent with HNPP. We review the clinical and electrodiagnostic features of HNPP including the key electrodiagnostic findings to screen for this disorder.
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