Tredici G, Petruccioli M G, Cavaletti G, Marmiroli P, Crespi V, Pioltelli P
Istituto di Anatomia Umana, Università di Milano, Italy.
Clin Neuropathol. 1992 May-Jun;11(3):121-7.
Peripheral neuropathy often occurs in cryoglobulinemia but the pathogenesis of the peripheral nerve involvement is not completely understood, so that the relation between the reported endoneural changes and neuropathy is not clear. In this study we compared the sural nerve biopsies of 6 cryoglobulinemic patients with or without signs of peripheral neuropathy and all affected by the essential mixed type II form (ECII) and, moreover, of 8 age-matched controls. We found that in all the patients with neuropathy, axonopathy occurred and it was invariably associated with endoneural vessel damage. Moreover, the fiber losses were patchily distributed within the nerve fascicles. On the contrary both nerve fibers and vessels were normal in the patients without clinical and neurophysiological evidence of neuropathy and in controls. Our results support the hypothesis that the endoneural damage observed during ECII is not simply coincidental, but is relevant in the pathogenesis of cryoglobulinemic neuropathy. They also favor the assumption that ischemic damage of the nerve fibers occurs during ECII.
周围神经病变常发生于冷球蛋白血症，但周围神经受累的发病机制尚未完全明确，因此所报道的神经内膜改变与神经病变之间的关系尚不清楚。在本研究中，我们比较了6例冷球蛋白血症患者的腓肠神经活检结果，这些患者均患有原发性混合型II型（ECII），其中部分有周围神经病变体征，部分没有，此外还比较了8例年龄匹配的对照者的活检结果。我们发现，所有有神经病变的患者均出现轴索性神经病，且其总是与神经内膜血管损伤相关。此外，神经纤维的丢失在神经束内呈斑片状分布。相反，在没有临床和神经生理学证据表明存在神经病变的患者以及对照者中，神经纤维和血管均正常。我们的结果支持这样一种假说，即在ECII期间观察到的神经内膜损伤并非仅仅是巧合，而是与冷球蛋白血症性神经病变的发病机制相关。它们还支持这样一种假设，即在ECII期间神经纤维发生了缺血性损伤。
