背景:腓肠神经活检对慢性炎性脱髓鞘性多神经根神经病(CIDP)的诊断价值存在争议。缺乏关于其执行的循证建议。我们调查了导致活检的因素,并分析了活检结果和后果,通过临床参数评估活检结果的可预测性,以避免不必要的活检,并将结果与电生理和临床严重程度进行比较,以确定其预后价值。
方法:在两个队列中分析了190例腓肠神经活检。一个包括163个活检,第二个来自前瞻性免疫介导的神经病生物材料和数据注册(INHIBIT)的72个活检。两者都有45名患者的交集。使用来自没有活检的患者的75个数据集。神经传导研究分析,治疗,总体残疾总和评分(ODSS),活检结果,并进行了诊断。
结果:51%的活检患者接受了诊断CIDP(77%符合EFNS/PNS标准),21%不是典型的DP,和27%是不具体的。与未活检的患者相比,活检的患者对免疫疗法的反应频率较低(p=0.003)。在活检后更频繁地开始免疫治疗(p<0.001),并且更经常地使用静脉内免疫球蛋白(p<0.0001)。所有活检患者中有76%符合CIDP的电生理标准。0µV的感觉神经动作电位幅度仍然提供了组织学诊断价值的73%。变性的组织学征象预测ODSS在1年后恶化(p=0.028),但疾病严重程度与组织学损害严重程度无关。
结论:神经活检的主要适应症是治疗难治性自身免疫性神经病,治疗开始或升级的治疗结果。硬膜活检也提供了预后信息。即使有熄灭的suralSNAP,活检仍有诊断价值。
BACKGROUND: The value of a sural nerve biopsy for the diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is controversial. Evidence-based recommendations for its implementation are lacking. We investigated factors leading to biopsy and analyzed biopsy outcomes and consequences, assessed the predictability of biopsy outcomes through clinical parameters to avoid unnecessary biopsies, and compared results with electrophysiological and clinical severity to determine their prognostic value.
METHODS: 190 sural nerve biopsies were analyzed in two cohorts. One consisted of 163 biopsies and the second of 72 biopsies from the prospective Immune-mediated Neuropathies Biomaterial and Data registry (INHIBIT). Both have an intersection of 45 patients. 75 data sets from patients without biopsy were used. Analysis of nerve conduction studies, treatment, overall disability sum score (ODSS), biopsy outcomes, and diagnosis was performed.
RESULTS: 51% of biopsied patients received the diagnosis CIDP (77% fulfilled EFNS/PNS criteria), 21% were not CIDP typical, and 27% were unspecific. Biopsied patients responded less frequently to immunotherapies at time of biopsy than non-biopsied patients (p = 0.003). Immunotherapy was initiated more frequently after biopsy (p < 0.001) and more often with intravenous immunoglobulins (p < 0.0001). 76% of all biopsied patients met the electrophysiological criteria for CIDP. Sensory nerve action potential amplitudes of 0 µV still provide 73% of histological diagnostic value. Histologic signs of degeneration predicted ODSS worsening after 1 year (p = 0.028) but disease severity did not correlate with histological damage severity.
CONCLUSIONS: The main indication for nerve biopsy was the treatment of refractory cases of autoimmune neuropathies with the therapeutic consequence of treatment initiation or escalation. Sural biopsy also provided prognostic information. Even with extinguished sural SNAP, the biopsy can still have diagnostic value.