Publication: Early onset myasthenia gravis with atypical features
Date
2008
Authors
Journal Title
Journal ISSN
Volume Title
Publisher
Kathmandu University
Abstract
Abstract
A 14 year old boy with atypical myasthenia gravis is reported. The interesting features of the case were the onset in first
decade with progressive weakness of limb muscles simulating limb girdle myopathy, presence of bilateral symmetrical
non fluctuating external ophthalmoplegia with ptosis and the absence of diplopia. Differential response to choline
esterase inhibitors was clinically apparent. In contrast to excellent improvement in limb weakness with choline esterase
inhibitors, there was no improvement in external ophthalmoplegia or ptosis. The possibilities are discussed and literature
is reviewed.
Key words: Myasthenia gravis (MG), External ophthalmoplegia, Acetylcholine receptor (Ach-R), Acetylcholine
receptor antibodies, (Ach-R ab), Ragged red fibers, Muscular dystrophy, Congenital myasthenia gravis, Choline esterase
inhibitors, Overlap syndrome
Description
Agarwal P1, Chapagain U 1 , Deewan KR 2 , Rana PVS 3
1 Intern, 2 Medical Officer, 3Neurologist, College of Medical Sciences, Bharatpur, Nepal