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  • Title: Childhood-onset oculopharyngodistal myopathy with chronic intestinal pseudo-obstruction.
    Author: Amato AA, Jackson CE, Ridings LW, Barohn RJ.
    Journal: Muscle Nerve; 1995 Aug; 18(8):842-7. PubMed ID: 7630344.
    Abstract:
    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.
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