Demyelinating neuropathy

脱髓鞘性神经病
  • 文章类型: Journal Article
    据报道,在有限数量的患者中,表皮神经纤维参与了慢性炎症性脱髓鞘性神经病(CIDP)。我们量化了典型CIDP和CIDP变异患者混合队列中的小纤维受累,以评估不同疾病阶段与临床结局指标的关系。
    通过皮肤穿刺活检对23名CIDP患者和13名前臂健康对照进行表皮内神经纤维密度(IENFD)评估,大腿,和远端腿。在16CIDP患者和10个年龄和性别匹配的健康对照的所有三个区域中,皮肤切片均得到了最佳解释。在这些受试者中进行统计分析。
    前臂的IENFD,大腿,在七个典型的CIDP和nineCIDP变体中,远端腿相似。与健康对照组相比,这些地区的IENFD在CIDP中显著降低,与国际神经病病因和治疗(INCAT)上肢功能残疾量表评分呈中度负相关。与对照组相比,IENFD的减少在远端腿中更为明显。在临床上不稳定的CIDP患者中,与stableCIDP患者和对照组相比,腿远端和前臂的IENFD显著减少.与对照组相比,StableCIDP患者腿远端和前臂的IENFDs明显减少。
    在这项探索性研究中,我们确认小纤维在CIDP中也受到影响。需要更大规模的研究来探索IENFDinCIDP的纵向变化及其与疾病分期的关系。
    Epidermal nerve fiber involvement in chronic inflammatory demyelinating neuropathy (CIDP) has been reported in a limited number of patients. We quantified small-fiber involvement in a mixed cohort of patients with typical CIDP and CIDP variants to evaluate relationships with clinical outcome measures at different disease stages.
    Intraepidermal nerve fiber densities (IENFDs) were evaluated by skin punch biopsies of 23 patients with CIDP and 13 healthy controls at the forearm, thigh, and distal leg. Skin sections were optimally interpreted in all three regions in 16 CIDP patients and 10 age- and sex-matched healthy controls. Statistical analysis was performed in these subjects.
    The IENFDs in forearm, thigh, and distal leg were similar among seven typical CIDP and nine CIDP variants. IENFDs in those regions were significantly reduced in CIDP compared with healthy controls, with a moderate negative correlation with scores on the International Neuropathy Cause and Treatment (INCAT) Upper Limb Functional Disability Scale. The reduction in IENFD compared with controls was more remarkable in the distal leg. In clinically unstable CIDP patients, the IENFDs of distal leg and forearm were significantly reduced compared with stable CIDP patients and controls. Stable CIDP patients had significantly reduced IENFDs in distal leg and forearm compared with controls.
    In this exploratory study, we confirm that small fibers are also affected in CIDP. Larger studies are needed to explore longitudinal changes of IENFD in CIDP and its relation to disease stage.
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  • 文章类型: Journal Article
    A distal-predominant demyelinating symmetric pattern is most frequent in patients with neuropathy associated with anti-myelin-associated glycoprotein (MAG) antibodies. The literature however lacks longitudinal data to describe whether this is consistent over time.
    From the Ottawa Neuromuscular Center database, we identified 23 patients with both immunoglobulin M gammopathy and anti-MAG antibodies. For median, ulnar and fibular motor conduction studies, we analyzed distal latency and amplitude, negative peak duration, terminal latency index (TLI), and conduction velocity. For median, ulnar, sural, and superficial fibular sensory conduction studies, we analyzed distal latency and amplitude. Results were compared for the earliest and the latest data sets.
    The mean time interval between the two assessment points was 6.5 years. Median and ulnar motor nerve conduction studies did not show a significant change for any of the parameters tested. There was disproportionate prolongation of median distal motor latency and reduction in TLI, compared to the ulnar nerve. Deep fibular motor conduction studies showed a marked reduction in amplitudes over time. Sensory potentials were recordable in the upper limb in less than 50% at the first study and less than 25% on the most recent study. There was an even larger attrition of recordable sural and superficial fibular sensory potentials.
    Our results highlight the stability of median and ulnar motor conduction study results over a mean observation period of 6.5 years. In contrast, lower limb motor and all sensory potentials show a marked trend toward becoming unrecordable.
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