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Chronic limb-girdle myasthenia gravis

Department of Neurology, The University of Alabama at Birmingham, The Veterans Administration Medical Center, Birmingham, AL.

The existence of chronic "limb-girdle" form of myasthenia gravis (MG) has been questioned. We report here 12 such patients (10 women and two men) who constituted 3.8% of 314 MG patients in our study. The duration of disease ranged from 4 months to 7 years before the diagnosis. In almost all cases, the initial diagnosis was other than MG. None of the patients had any oculobulbar weakness. Acetylcholine receptor antibody was positive in five cases, although not all in the first assay. Repetitive nerve stimulation test was positive in all cases, although not necessarily the first time. Single-fiber EMG was positive in 11 cases. All patients responded to acetylcholinesterase inhibitors, and two thirds underwent immunotherapy. Diagnosis of limb-girdle MG requires a strong index of suspicion.

Address correspondence and reprint requests to Dr. Shin J. Oh, Department of Neurology, The University of Alabama at Birmingham, UAB Station, Birmingham, AL 35294.

Supported by a grant from the Alabama Chapter of Myasthenia Gravis Foundation.

Presented in part at the 43rd annual meeting of the American Academy of Neurology, Boston, MA, April 1991.

Received September 18, 1991. Accepted for publication in final form November 13, 1991.

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