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先天性颈段脊髓性肌萎缩症:一种上肢非家族性、非进行性疾病。

Congenital cervical spinal muscular atrophy: a non-familial, non progressive condition of the upper limbs.

作者信息

Hageman G, Ramaekers V T, Hilhorst B G, Rozeboom A R

机构信息

Department of Neurology, Medical Spectrum Twente Hospital Enschede, The Netherlands.

出版信息

J Neurol Neurosurg Psychiatry. 1993 Apr;56(4):365-8. doi: 10.1136/jnnp.56.4.365.

Abstract

Two patients with congenital cervical spinal muscular atrophy had symmetrical severe muscle weakness and wasting confined to the upper limbs, areflexia and congenital contractures. The shoulders were internally rotated, elbows extended and wrists flexed. There were no sensory or bulbar symptoms, scoliosis, long tract signs or lower limb involvement. This condition should be regarded as a neurogenic type of arthrogryposis, limited to the upper limbs.

摘要

两名先天性颈段脊髓性肌萎缩症患者出现对称性严重肌无力和肌肉萎缩,局限于上肢,腱反射消失及先天性挛缩。肩部内旋,肘部伸展,腕部屈曲。无感觉或延髓症状、脊柱侧弯、长束征或下肢受累。这种情况应被视为关节挛缩症的神经源性类型,仅限于上肢。

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