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一例表现为臂丛神经病的遗传性压力易感性周围神经病(HNPP)报告:完整电诊断检测的价值

A report of hereditary neuropathy with liability to pressure palsy (HNPP) presenting with brachial plexopathy: the value of complete electrodiagnostic testing.

作者信息

Bulusu Srinivas, McMillan Hugh J

机构信息

Division of Neurology, University of Ottawa, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada.

出版信息

Am J Electroneurodiagnostic Technol. 2011 Sep;51(3):183-90.

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.

摘要

遗传性压力易感性周围神经病(HNPP)患者通常表现为单神经病(尤其是腓总神经或尺神经麻痹)或臂丛神经病。仔细的电诊断测试在确立HNPP诊断、将该疾病与其他遗传性或获得性神经病相鉴别以及避免不必要的手术方面具有重要作用。我们报告一例表现为无痛性臂丛神经病的患者,其神经传导研究发现有多个节段性脱髓鞘部位,符合HNPP。我们回顾了HNPP的临床和电诊断特征,包括筛查该疾病的关键电诊断结果。

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