Meier C, Bischoff A
J Neurol. 1977 May 13;215(2):103-14.
Clinical and sural nerve biopsy findings are described in four hypothyroid patients with manifestations of a diffuse peripheral neuropathy. Clinical examination revealed distal sensory inpairment, complaints of burning and lancinating extremity pains, ataxia and a decrease of deep tendon reflexes with total ankle jerk loss. In one patient, signs of the peripheral neuropathy comprised the leading symptomatology of the underlying metabolic disorder. Light and electron microscopic investigations of the sural nerve biopsies revealed a marked reduction of myelinated fibers, affecting mainly the large myelinated axons. The histograms showed a pathological unimodal spectrum of myelinated fiber diameters. Dystrophic and degenerative changes in axons of all fiber classes and the presence of clusters of thinly myelinated small fibers suggest that neuroaxonal degeneration and incomplete regeneration may represent the major pathogenetic mechanism of the neuropathy in these hypothyroid patients.
本文描述了4例表现为弥漫性周围神经病变的甲状腺功能减退患者的临床及腓肠神经活检结果。临床检查发现有远端感觉障碍、肢体灼痛和刺痛、共济失调以及深腱反射减弱,完全没有踝反射。在1例患者中,周围神经病变的体征构成了潜在代谢紊乱的主要症状。腓肠神经活检的光镜和电镜检查显示有髓纤维显著减少,主要影响大的有髓轴突。直方图显示有髓纤维直径呈病理性单峰分布。所有纤维类别的轴突出现营养不良和退行性改变,以及存在薄髓鞘小纤维束,提示神经轴突变性和不完全再生可能是这些甲状腺功能减退患者神经病变的主要发病机制。