CHILDHOOD-ONSET OCULOPHARYNGODISTAL MYOPATHY WITH CHRONIC INTESTINAL-PSEUDO-OBSTRUCTION
CHILDHOOD-ONSET OCULOPHARYNGODISTAL MYOPATHY WITH CHRONIC INTESTINAL-PSEUDO-OBSTRUCTION
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DOI：
10.1002/mus.880180807
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发表时间：
1995-08-01
期刊：
影响因子：
3.4
通讯作者：
BAROHN, RJ
中科院分区：
文献类型：
--
作者：
AMATO, AA;JACKSON, CE;BAROHN, RJ
眼咽远端肌病的特征是成人发病的上睑下垂、眼外肌麻痹、吞咽困难和远端无力。虽然吞咽困难是常见的，其他胃肠道受累尚未被描述。我们报告一个儿童发病谁发展为慢性假性肠梗阻。排除了与眼肌麻痹和肠假性梗阻相关的其他肌病，如线粒体细胞病变。眼咽远端肌病是眼咽肌营养不良的一种变体还是一种独特的神经肌肉疾病尚不清楚，需要进一步研究。(C)1995年，John Wiley and Sons，Inc.
Oculopharyngodistal myopathy is characterized by the adult onset of ptosis, external ophthalmoplegia, dysphagia, and distal weakness. Although dysphagia is common, other gastrointestinal involvement has not been described. We report a case with childhood onset who developed chronic intestinal pseudo-obstruction. Other myopathies associated with ophthalmoplegia and intestinal pseudo-obstruction such as mitochondrial cytopathies were excluded, Whether oculopharyngodistal myopathy is a variant of oculopharyngeal muscular dystrophy or a distinct neuromuscular disorder is unknown and requires further study. (C) 1995 John Wiley and Sons, Inc.