Dubas F, Bertrand P, Emile J
Service de Neurologie et Laboratoire de Neuro-immunologie et Neuropathologie, INSERM U.298, CHR, Angers.
Rev Neurol (Paris). 1989;145(1):65-8.
A 82 year-old man died 6 years after the onset of a progressive spinal muscular atrophy. Post-mortem examination disclosed a parathyroid adenoma. Weakness and wasting were prominent in the proximal lower limbs. There were no fasciculations. Involvement of the medulla was mild and late. These clinical features were also present in 16 reported cases, which were improved by treatment of primary or secondary hyperparathyroidism. Our patient differs by the involvement of the hand muscles and the loss of tendon reflexes. Neuropathological study, as in one other reported case, showed a loss of anterior horn cells. Such cases underline that calcium metabolism must be studied in syndromes of spinal muscular atrophy.
一名82岁男性在进行性脊髓性肌萎缩发病6年后死亡。尸检发现甲状旁腺腺瘤。近端下肢无力和消瘦明显。无肌束震颤。延髓受累轻微且较晚。16例报告病例也有这些临床特征,经原发性或继发性甲状旁腺功能亢进治疗后病情改善。我们的患者不同之处在于手部肌肉受累和腱反射消失。神经病理学研究与另一例报告病例一样,显示前角细胞丧失。这些病例强调,在脊髓性肌萎缩综合征中必须研究钙代谢。