Benady S G
Dev Med Child Neurol. 1978 Dec;20(6):746-57. doi: 10.1111/j.1469-8749.1978.tb15306.x.
The clinical findings in 50 children with intermediate or mild spinal muscular atrophy are reviewed. An early age of onset and the presence of tongue fasciculation carry a poor prognosis for disability. Scoliosis appears early in nearly all children with the more severe forms of spinal muscular atrophy and early energetic treatment is indicated. Contractures are also found in both the more severe and the milder forms and are the main cause of progressive disability, since continuous deterioration of muscle power was not evident in the present series. Respiratory failure is rare but potentially fatal, and may occur even in mild forms of the disease. Clinically evident weakness of the axial musculature may give warning of this complication.
回顾了50例中度或轻度脊髓性肌萎缩症患儿的临床症状。发病年龄早以及存在舌肌束颤对残疾预后不利。脊柱侧弯在几乎所有病情较重的脊髓性肌萎缩症患儿中出现较早，因此需要早期积极治疗。挛缩在病情较重和较轻的患儿中均有发现，并且是导致残疾进展的主要原因，因为在本系列研究中未发现肌肉力量持续恶化的情况。呼吸衰竭虽罕见但可能致命，甚至在疾病的轻度形式中也可能发生。临床上明显的轴性肌肉无力可能提示这一并发症。
