Ophthalmoplegia - plus syndrome

ABSTRACT

Two patients, with chronic progressive external Ophthalmoplegia, are described. The first case, in addition, had involvement of the facial, palatopharyngeal and proximal muscles of upper limbs, while the second patient had wasting and weakness of the shoulder girdle muscles. Electromyography revealed myopathic pattern in both the patients and muscle biopsy in the second case was consistent with a primary muscle disease. A brief review on the controversy over the nomenclature of the term "Ophthalmoplegia-plus" is presented