Andrade R, González I, Machado A, García A
Hospital Universitario Arnaldo Milián Castro. Hosp. Municipal de Remedios, Santa Clara. Villa Clara, Cuba.
Rev Neurol. 2001;33(11):1005-9.
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired neuropathy characterized by demyelination of the peripheral nerves and roots. The course of the illness is progressively chronic or of relapses and remissions. Biopsy of the sural nerve is not essential for diagnosis. It is therefore not necessary to carry it out on all occasions, but probably permits a more rational approach to treatment.
To show whether biopsy of the sural nerve is useful for orientation of the treatment required in cases of CIDP.
We studied a total of 16 patients admitted to hospital with a diagnosis of CIDP. They had neurophysiological studies, sural nerve biopsy and other studies to rule out other diagnoses. The patients were assigned to the therapeutic protocols recommended. During 8 years of follow up we compared the response to treatment with intacglobin/plamapheresis, steroids and cytostatic drugs.
It was highly significant (p< 0.001) that the patients with axon lesions on sural nerve biopsy did not respond to treatment with intac globin/plasmapheresis or steroids but did respond to cyclophosphamide, without any serious adverse effects being seen.
Axonal histopathological lesions of CIDP at the onset of the disease may require cytostatic treatment since they do not usually improve on standard treatment. Sural biopsy is usually invaluable in such cases.
慢性炎症性脱髓鞘性多发性神经病(CIDP)是一种获得性神经病,其特征为周围神经和神经根的脱髓鞘。病程呈进行性慢性或复发缓解型。腓肠神经活检并非诊断所必需。因此并非所有情况下都有必要进行该检查,但它可能有助于采取更合理的治疗方法。
探讨腓肠神经活检对CIDP患者所需治疗的指导作用。
我们共研究了16例诊断为CIDP的住院患者。他们接受了神经生理学检查、腓肠神经活检及其他检查以排除其他诊断。患者被分配至推荐的治疗方案。在8年的随访期间,我们比较了患者对免疫球蛋白/血浆置换、类固醇和细胞毒性药物治疗的反应。
腓肠神经活检显示有轴突病变的患者对免疫球蛋白/血浆置换或类固醇治疗无反应,但对环磷酰胺有反应,且未见任何严重不良反应,差异具有高度显著性(p<0.001)。
CIDP疾病初发时的轴索性组织病理学病变可能需要细胞毒性治疗,因为标准治疗通常无法使其改善。在这种情况下,腓肠神经活检通常非常有价值。