Sporadic oculopharyngeal myopathy with abnormal mitochondria

A case of late onset ophthalmoplegia and dysphagia is presented. Serum enzymes, ECG, EEG, thyroid function and edrophonium test were normal. Muscle biopsy revealed the presence at oxidative enzymes of 5% of fibres displaying subsarcolemmal hyperactivity and a coarse network pattern, mainly involving type II fibres. At electron microscopy mitochondrial abnormalities with paracrystalline inclusions were detected. The case is discussed in the light of the heterogeneity of ocular myopathies and their overlapping both with one another and with the so called mitochondrial myopathies.