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非系统性血管炎性神经病:一种神秘的临床病症。

Non-Systemic Vasculitic Neuropathy: An Enigmatic Clinical Entity.

作者信息

Lubana Sandeep Singh, Singh Navdeep, Sanelli-Russo Susan, Abrudescu Adriana

机构信息

Department of Internal Medicine, Icahn School of Medicine at Mount Sinai, Queens Hospital Center, Queens, NY, USA.

Department of Neurology, Icahn School of Medicine at Mount Sinai, Queens Hospital Center, Queens, NY, USA.

出版信息

Am J Case Rep. 2015 Jul 13;16:449-53. doi: 10.12659/AJCR.894601.

Abstract

BACKGROUND

Non-systemic vasculitic peripheral neuropathy is a rare condition characterized by necrotizing inflammation resulting in luminal narrowing of the vasa nervorum, leading to ischemic injury to peripheral nerves. Here, we present the case of 63-year-old woman with subacute onset of severe hyperesthesia of the lower extremities accompanied by foot drop.

CASE REPORT

A 63-year-old woman with prolonged history of uncontrolled diabetes mellitus presented with subacute onset of severe bilateral lower extremity hyperesthesia and motor weakness along with left-sided foot drop. She had multiple emergency room visits with no relief of her symptoms. High doses of analgesics were insufficient to control pain. Laboratory tests were positive only for high erythrocyte sedimentation rate and C-reactive protein. A skin biopsy obtained 5 cm above the left lateral malleolus revealed medium-sized dermal vasculitis with dense mononuclear infiltrate. Electromyography showed peripheral neuropathy. A nerve biopsy was needed to reveal the exact diagnosis.

CONCLUSIONS

Diagnosis of non-systemic vasculitic peripheral neuropathy can be delayed or missed in patients with uncontrolled diabetes mellitus, leading to significant morbidity. Elevated markers of inflammation in the absence of a possible explanation should prompt the clinician to perform a nerve biopsy; however, it is an invasive procedure and is associated with complications of post-neuropathic pain and delayed wound healing. Magnetic resonance angiography of the lower limbs, if combined with skin biopsy, can save the patient from undergoing nerve biopsy.

摘要

背景

非系统性血管炎性周围神经病是一种罕见疾病,其特征为坏死性炎症,导致神经滋养血管管腔狭窄,进而引起周围神经缺血性损伤。在此,我们报告一例63岁女性,其下肢出现亚急性严重感觉过敏并伴有足下垂。

病例报告

一名有长期未控制的糖尿病病史的63岁女性,出现双侧下肢亚急性严重感觉过敏及运动无力,并伴有左侧足下垂。她多次前往急诊室就诊,但症状均未缓解。高剂量镇痛药不足以控制疼痛。实验室检查仅红细胞沉降率和C反应蛋白升高。在左外踝上方5厘米处进行的皮肤活检显示为中型真皮血管炎,伴有密集单核细胞浸润。肌电图显示周围神经病变。需要进行神经活检以明确诊断。

结论

在未控制的糖尿病患者中,非系统性血管炎性周围神经病的诊断可能会延迟或漏诊,从而导致严重的发病率。在没有其他可能解释的情况下,炎症指标升高应促使临床医生进行神经活检;然而,这是一种侵入性操作,与神经性疼痛和伤口愈合延迟等并发症相关。下肢磁共振血管造影术若与皮肤活检相结合,可避免患者接受神经活检。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a741/4504409/eb7299663eda/amjcaserep-16-449-g001.jpg

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