MY ASTHENIA CONGENITA: ELECTROMYOGRAPHIC FINDINGS

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Abstract
Congenital myasthenia is reported in two siblings born to a non‐myasthenic mother. The major symptoms were confined to the eyes (symmetrical ptosis and external ophthalmoplegia), and the disease tended to remain stationary. In a review of the literature. 14 similar cases were found. Electromyography showed evidence of myopathie involvement, particularly in the eye muscles; a muscle biopsy from the right biceps muscle in one case revealed normal findings. Repetitive nerve stimulation, combined with a Tensilon (edrophonium) test, was in favor of the diagnosis of myasthenia. Although the possibility exists that such cases represent, myopathy with myasthenic features, the most likely possibility seems to be that the myopathic electromyographic changes are secondary to the myasthenia. It is emphasized that repetitive nerve stimulation and Tensilon test are important aids in the diagnosis.

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