Kaur S, Kumar B, Chopra J S, Lamba G, Narang A
Clin Neurol Neurosurg. 1980;82(1):45-56. doi: 10.1016/0303-8467(80)90059-1.
Two siblings suffering from acro-osteolysis, a rare disorder, are discussed. Although three distinct types of acro-osteolysis, namely familial, idiopathic, and due to vinyl-chloride, are clearly defined, these patients did not fit into any category. They had intact nails, preserved tactile and sensory system, plantar ulceration and so sensory deficit. Nerve conduction studies showed prolongation of motor distal latencies in median, ulnar, posterior tibial and lateral popliteal nerves. Nerve biopsies from both patients showed severe loss of myelinated fibres of all sizes, but more so of small ones.
本文讨论了两名患有罕见疾病肢端骨质溶解症的兄弟姐妹。尽管肢端骨质溶解症有三种明确类型,即家族性、特发性和氯乙烯所致,但这两名患者不属于任何一种类型。他们指甲完好,触觉和感觉系统正常,但有足底溃疡和感觉缺失。神经传导研究显示,正中神经、尺神经、胫后神经和腘外侧神经的运动远端潜伏期延长。两名患者的神经活检均显示各种大小的有髓纤维严重缺失,尤其是小纤维。