Publication: Juvenile Generalized Myasthenia Gravis: Presented as Unilateral Blepharoptosis and Successfully Managed with Pulse Intravenous Methylprednisolone
Date
2021
Journal Title
Journal ISSN
Volume Title
Publisher
Kathmandu University
Abstract
ABSTRACT
Myasthenia Gravis is a rare autoimmune disorder of childhood and this is rarer in South
Asia. We present a pre-pubertal 7 year old female child of seropositive Generalized
Juvenile Myasthenia Gravis. She presented with unilateral blepharoptosis and
later generalized symptoms appeared. Ice-pack test, Neostigmine challenge test
and acetylcholine receptor antibody test were positive. Serum muscle specific
tyrosine kinase antibody test was normal. She did not have thymic abnormalities.
She did not respond to high dose (26 mg/kg/day) of Pyridostigmine and oral
Prednisolone (2 mg/kg/day), but was successfully treated with a combination of
pulse intravenous Methylprednisolone (30 mg/kg once a month for 6 months) and
daily doses of oral Prednisolone (2 mg/kg/day) along with Pyridostigmine without
significant side effects. This combination can be considered a potential inexpensive
treatment for Juvenile Myasthenia Gravis in a resource limited area where other
immunosuppressive treatments such as intravenous immunoglobulin is expensive
and unaffordable.
KEY WORDS
Blepharoptosis, Child, Methylprednisolone, Muscle weakness, Myasthenia gravis
Description
Poudel P, Anand A, Ghosh S
Department of Paediatrics and Adolescent Medicine
B.P. Koirala Institute of Health Sciences,
Dharan, Nepal
Keywords
Blepharoptosis, Child, Methylprednisolone, Muscle weakness, Myasthenia gravis