Evidence of Small-Fiber Polyneuropathy in Unexplained, Juvenile-Onset, Widespread Pain Syndromes
Evidence of Small-Fiber Polyneuropathy in Unexplained, Juvenile-Onset, Widespread Pain Syndromes
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DOI：
10.1542/peds.2012-2597
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发表时间：
2013-04-01
期刊：
影响因子：
8
通讯作者：
Klein, Max M.
中科院分区：
文献类型：
--
作者：
Oaklander, Anne Louise;Klein, Max M.
目的：我们检验了获得性小纤维多发性神经病（SVPN）的假设，以前在儿童中未被描述，导致无法解释的儿科广泛疼痛综合征。对41名21岁以前开始的不明原因广泛性疼痛的连续患者进行了关于SFPN客观诊断测试的全面分析（神经诊断皮肤活检、神经活检和自主神经功能测试），加上病史、症状、体征、其他测试和治疗。健康的，人口统计学匹配的志愿者提供正常对照SFPN tests.RESULTS：发病年龄平均为12.3 +/- 5.7岁，73%的多民族样本中的女性（P = 0.001）。68%的人患有慢性残疾，68%的人住院治疗。客观测试诊断明确的SFPN在59%，可能的SFPN在17%，和可能的SFPN在22%。41人中只有1人的SFPN测试结果完全正常。98%的患者有其他与SFPN自主神经功能障碍一致的躯体主诉（90%心血管，82%胃肠道和34%泌尿系统），83%报告慢性疲劳，63%有慢性头痛。神经系统检查发现68%感觉减退，55%血管异常，包括23%红斑性肢痛症。对SFPN因果关系的详尽调查仅确定了33%的自身免疫性疾病史和89%的免疫紊乱血清学标志物。皮质类固醇和/或静脉注射免疫球蛋白治疗客观和主观受益80%的患者（12/15）。结论：在一个大型系列的儿童期发病的，原因不明的慢性广泛性疼痛的患者中，超过一半符合严格的，多项测试，诊断标准的SFPN，获得性SFPN的年龄范围扩展到儿童早期。一些病例表现为免疫介导，并通过免疫调节治疗得到改善。
OBJECTIVE: We tested the hypothesis that acquired small-fiber polyneuropathy (SFPN), previously uncharacterized in children, contributes to unexplained pediatric widespread pain syndromes.METHODS: Forty-one consecutive patients evaluated for unexplained widespread pain beginning before age 21 had medical records comprehensively analyzed regarding objective diagnostic testing for SFPN (neurodiagnostic skin biopsy, nerve biopsy, and autonomic function testing), plus histories, symptoms, signs, other tests, and treatments. Healthy, demographically matched volunteers provided normal controls for SFPN tests.RESULTS: Age at illness onset averaged 12.3 +/- 5.7 years; 73% among this poly-ethnic sample were female (P = .001). Sixty-eight percent were chronically disabled, and 68% had hospitalizations. Objective testing diagnosed definite SFPN in 59%, probable SFPN in 17%, and possible SFPN in 22%. Only 1 of 41 had entirely normal SFPN test results. Ninety-eight percent of patients had other somatic complaints consistent with SFPN dysautonomia (90% cardiovascular, 82% gastrointestinal, and 34% urologic), 83% reported chronic fatigue, and 63% had chronic headache. Neurologic examinations identified reduced sensation in 68% and vasomotor abnormalities in 55%, including 23% with erythromelalgia. Exhaustive investigations for SFPN causality identified only history of autoimmune illnesses in 33% and serologic markers of disordered immunity in 89%. Treatment with corticosteroids and/or intravenous immune globulin objectively and subjectively benefited 80% of patients (12/15).CONCLUSIONS: More than half among a large series of patients with childhood-onset, unexplained chronic widespread pain met rigorous, multitest, diagnostic criteria for SFPN, which extends the age range of acquired SFPN into early childhood. Some cases appeared immune-mediated and improved with immunomodulatory therapies.