Rizzuto N, Moretto G, Monaco S, Martinelli P, Pazzaglia P
Acta Neuropathol. 1982;56(3):179-86. doi: 10.1007/BF00690633.
The clinico-pathologic findings in two patients with chronic relapsing polyneuritis are reported. The patients had several attacks of diffuse polyneuritis followed by partial recovery. The tendency to persisting disability increased following relapses and in the late stages there was a progressive motor deterioration suggesting a continuing activity of nerve damage. The biopsies of peripheral nerves, obtained in the advanced stages of the disease, showed severe loss of myelinated fibers and aspects of repeated demyelination and remyelination, with formation of onion bulb complexes. Completely demyelinated axons together with remyelinating fibers as well as remyelinated fibers invested by macrophages were seen at the same time. The clinical and pathological evidences suggest that in chronic relapsing polyneuritis, at least in the late stages of the disease, besides clinical relapses, there is a continuing activity of nerve damage. Thus, due to this ongoing activity of demyelination and remyelination, the elimination of the supernumerary Schwann cells does not take place satisfactorily and hypertrophic changes of the nerve fascicles are produced.
报告了两名慢性复发性多神经炎患者的临床病理 findings。患者有几次弥漫性多神经炎发作，随后部分恢复。复发后持续残疾的倾向增加，在疾病后期出现进行性运动恶化，提示神经损伤持续活动。在疾病晚期获取的周围神经活检显示，有髓纤维严重丧失，存在反复脱髓鞘和再髓鞘形成的表现，并形成洋葱球复合体。同时可见完全脱髓鞘的轴突以及再髓鞘化纤维，还有被巨噬细胞包绕的再髓鞘化纤维。临床和病理证据表明，在慢性复发性多神经炎中，至少在疾病后期，除了临床复发外，还存在神经损伤的持续活动。因此，由于这种持续的脱髓鞘和再髓鞘活动，多余施万细胞的清除不能令人满意地发生，从而导致神经束的肥大性改变。
