Amato A A, Jackson C E, Ridings L W, Barohn R J
Department of Neurology, Wilford Hall Medical Center, Lackland AFB, Texas 78236-5300, USA.
Muscle Nerve. 1995 Aug;18(8):842-7. doi: 10.1002/mus.880180807.
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.
