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儿童以顽固性局部瘙痒为唯一表现的脊髓内肿瘤:病例报告及文献复习。

Intractable localized pruritus as the sole manifestation of intramedullary tumor in a child: case report and review of the literature.

机构信息

Department of Dermatology, University of Utah, Salt Lake City, UT 84109, USA.

出版信息

JAMA Dermatol. 2013 Apr;149(4):446-9. doi: 10.1001/jamadermatol.2013.2032.

DOI:10.1001/jamadermatol.2013.2032
PMID:23715513
Abstract

IMPORTANCE

Persistent localized pruritus is a rare manifestation of central nervous system tumors. Delayed diagnosis can lead to devastating complications.

OBSERVATIONS

We report an otherwise healthy 19-month-old girl who presented with signs of localized intractable pruritus of 6 months' duration on the left side of the neck, shoulder, and arm, resistant to systemic antihistamines and topical corticosteroids. Findings from skin biopsy, viral culture for varicella-zoster virus, and skin prick test to common food and animal allergens were nondiagnostic. Neurologic examination results were unremarkable. After several months of localized intractable pruritus, magnetic resonance imaging of the cervical spine with and without contrast was performed, which revealed an intramedullary spinal cord tumor extending from just above the foramen magnum to C6. The tumor was surgically resected and found to be a ganglioglioma. Within a week after the surgery her pruritus completely resolved.

CONCLUSIONS AND RELEVANCE

We recommend a detailed neurologic examination in any case of persistent localized pruritus, in the absence of primary dermatologic causes. Given the challenges of performing a reliable neurologic examination in children, neuroimaging might be considered in children with intractable localized pruritus of unknown etiology of the head and neck or upper extremity, even in the absence of focal neurologic deficits.

摘要

重要性

持续性局部瘙痒是中枢神经系统肿瘤的罕见表现。延迟诊断可能导致严重的并发症。

观察结果

我们报告了一例 19 个月大的健康女孩,其左侧颈部、肩部和手臂出现局部顽固性瘙痒,持续 6 个月,对全身抗组胺药和局部皮质类固醇无反应。皮肤活检、水痘-带状疱疹病毒的病毒培养以及常见食物和动物过敏原的皮肤点刺试验结果均无诊断意义。神经系统检查结果无异常。在局部顽固性瘙痒持续数月后,进行了颈椎的磁共振成像(包括和不包括对比剂),结果显示从枕骨大孔上方延伸至 C6 的脊髓内肿瘤。肿瘤被手术切除,发现为神经节神经胶质瘤。手术后一周,她的瘙痒完全消失。

结论和相关性

我们建议在任何持续性局部瘙痒的情况下,即使没有原发性皮肤病原因,也要进行详细的神经系统检查。鉴于在儿童中进行可靠的神经系统检查存在挑战,对于病因不明的头颈部或上肢顽固性局部瘙痒且无局灶性神经缺损的儿童,即使无局灶性神经缺损,也可考虑进行神经影像学检查。

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