4例原发性甲旁亢伴肌萎缩临床分析

4 cases of primary hyperparathyroidism with amyotrophy

目的:探讨原发性甲旁亢伴肌萎缩临床特点,提高临床医师对本病认识,降低误诊率和漏诊率。方法:回顾性分析1990年至今在我科诊治的以肌萎缩和肌无力为首发症状的甲旁亢病例共4例,就其临床表现、实验室检查、电生理特点、组织学变化以及治疗预后结合文献进行分析。结果:4例均以双下肢无力起病,逐渐进展为四肢对称性以近端为主的肌无力和肌肉萎缩,下肢重于上肢,其中2例同时有上运动神经元损害表现。4例肌酶均正常,血钙浓度和血清甲状旁腺激素测定都明显升高。肌电图和肌活检结果可表现为肌源性或神经源性。3例患者采用手术治疗,切除甲状旁腺腺瘤后肌无力症状有所改善。结论:原发性甲旁亢对神经肌肉系统的影响错综复杂,肌电图和肌肉活检结果缺乏特异性,血钙浓度测定对疾病诊断有较大帮助．手术是治疗的重要手段。本病存在类似肌萎缩侧索硬化症状的少见类型。

Objective： To investigate the clinical characteristics of primary hyper-parathyroidism with amyotrophy. Methods： Medical records, including clinical presentations, lab analysis, electromyography, muscle biopsy, therapy and prognosis, of 4 primary hyperparathyroidism with amyotrophy patients admitted in our hospital from 1990 to 2006, were retrospectively reviewed in comparison with the literatures. Results： All 4 patients presented symmetric proximal weakness et atrophy, most severe in lower limbs, 2 patients had also the presentation of damage in the upper motor neuron. The muscular enzyme was normal in all patients, the concentration of calcium and PTH were high. EMG may neurogenic or myopathic changcs. 3 patients had a improved clinic presentation after the parathyroid surgery. Conclusions： Neuromuscular symptoms of primary hyperparathyrodism were complex. The EMG and biopsy result were atypical. It is very useful of the concentration of calcium in diagnosis. The parathyroid surgery is the most avail for the therapy. It has a rare type of resemble ALS.