Uncini A, Di Muzio A, De Angelis M V, Gioia S, Lugaresi A
Center for Neuromuscular Diseases, University G d'Annunzio, Chieti, Italy.
Clin Neurophysiol. 1999 Apr;110(4):694-8. doi: 10.1016/s1388-2457(98)00070-4.
Show the chronic inflammatory demyelinating polyneuropathy (CIDP) is not only clinically heterogeneous but extremely variable in severity.
Three patients were referred for mild distal paresthesiae lasting more than 6 months and one for inguinal and thigh pain later ascribed to coxarthrosis. Strength was normal in all patients and tactile sensation reduced distally only in one. Tendon jerks were absent, except the knee jerks in one patient, reduced in lower limbs in two and normal in one.
Electrophysiology showed a demyelinating neuropathy without motor conduction block. CSF protein content was increased in all patients. Nerve biopsies showed de-remyelination with varying degrees of axonal loss. Genetic studies excluded a demyelinating neuropathy associated with duplication or deletion of the 17p.11.2 segment.
CIDP patients with pure sensory clinical presentation have been described but are generally more severely impaired. However, because of the mildness of symptoms and the unequivocal electrophysiological involvement of motor fibers, we think that in these cases the term minimal CIDP is more appropriate than sensory CIDP. These cases represent the most benign end of the CIDP spectrum. In our series minimal or even asymptomatic CIDP encompasses 8% of cases.
表明慢性炎症性脱髓鞘性多发性神经病(CIDP)不仅在临床上具有异质性,而且在严重程度上变化极大。
三名患者因轻度远端感觉异常持续超过6个月前来就诊,一名患者因腹股沟和大腿疼痛后来被诊断为髋关节病。所有患者肌力正常,仅一名患者远端触觉减退。除一名患者膝反射正常外,其余患者腱反射均消失,两名患者下肢腱反射减弱,一名患者正常。
电生理检查显示为脱髓鞘性神经病,无运动传导阻滞。所有患者脑脊液蛋白含量均升高。神经活检显示有脱髓鞘改变,并伴有不同程度的轴突丢失。基因研究排除了与17p.11.2节段重复或缺失相关的脱髓鞘性神经病。
虽然已有关于纯感觉临床表现的CIDP患者的描述,但这些患者通常受损更严重。然而,由于症状较轻且运动纤维在电生理上有明确受累,我们认为在这些病例中,“轻度CIDP”这一术语比“感觉性CIDP”更合适。这些病例代表了CIDP谱系中最良性的一端。在我们的系列研究中,轻度甚至无症状的CIDP占病例的8%。