Atrophy of bilateral extraocular muscles - CT and clinical features of seven patients

Swelling of the extraocular muscles is a common orbital abnormality that is easily demonstrated by computed tomography (CT). However, muscle atrophy is more difficult to identify and is rarely reported in the literature. Bilateral atrophy is extremely rare. We report the CT and clinical features of seven patients showing bilateral extraocular muscle atrophy: four with mitochondrial myopathy (MM) and three with myasthenia gravis (MG). Six patients had clinical histories of muscle involvement >20 years. An incorrect diagnosis of MG was made initially in two patients with MM because of mildly positive Tensilon testing. The ocular motor abnormalities failed to improve after thymectomy in the myasthenic patients. Orbital appearance on neuroimaging is similar in these disorders. Differentiation between these two disorders is impossible with orbital CT and magnetic resonance imaging (MR) alone.