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Childhood myasthenia gravis: clinical features and outcomes
Somjit Sri-udomkajorn1, Patipat Panichai, Sahas Liumsuwan
1Division of Child Neurology, Queen Sirikit National Institute of Child Health, College of Medicine, Rangsit University, Bangkok, Thailand. Somjit3d@yahoo.com
Insights
Ocular myasthenia gravis (OMG) is common in children, often starting earlier than generalized myasthenia gravis (GMG). Early prednisolone treatment may help prevent OMG progression.
Area of Science:
- Pediatric Neurology
- Autoimmune Disorders
- Ophthalmology
Background:
- Myasthenia gravis (MG) is a rare autoimmune disorder affecting neuromuscular junctions.
- Pediatric MG presents unique challenges in diagnosis and management.
- Understanding disease subtypes and progression is crucial for effective treatment.
Purpose of the Study:
- To investigate the clinical characteristics of pediatric myasthenia gravis.
- To analyze treatment strategies and outcomes in children with MG.
- To identify factors influencing the prognosis of childhood MG.
Main Methods:
- Retrospective review of 119 children diagnosed with MG over 15 years.
- Analysis of demographic data, clinical presentations, treatments, and outcomes.
- Minimum follow-up of 6 months for all patients.
Main Results:
- Ocular MG (OMG) comprised 84% of cases, with an earlier age of onset than generalized MG (GMG).
- Ptosis was the predominant symptom (99%), with varying degrees of ophthalmoplegia.
- Pharmacological remission was achieved in 60.5%, and complete remission in 18.5%; no specific remission factors were identified.
Conclusions:
- Ocular myasthenia gravis is the most frequent form in children, with an earlier onset than GMG.
- Early prednisolone intervention may positively impact OMG progression and prevent further ocular involvement.
- Further research is needed to identify definitive factors associated with remission in pediatric MG.
Objective:
To study the clinical features, treatment, outcome and factors that affected the outcome of myasthenia gravis (MG) in children.
Material And Method:
Children aged 16 years or less with diagnosed myasthenia gravis (MG) seen at Queen Sirikit National Institute of Child Health over a 15-year period with a minimum follow-up of 6 months were reviewed. Demographic, clinical characteristics, treatment and the outcome were analyzed.
Results:
One hundred and nineteen MG patients, 100 patients (84%) were ocular MG (OMG) and 19 patients (16%) were generalized MG (GMG). Median age of onset was 4.1 years. OMG patients had the age of onset earlier than GMG patients (p = 0.01). Female to male ratio was 1.8: 1. Ptosis was a clinical feature in 99%, accompanied with ophthalmoplegia in 63%, diplopia in 19.3%, extremity weakness in 13.4%, respiratory muscle weakness in 9%, head tilt in 10.1%, dysphagia in 7.5%, hyperthyroidism in 3.4% and epilepsy in 2.5%. One hundred and six patients who had ptosis as the initial symptom 67% were bilateral ptosis, 33% were unilateral ptosis, 10 patients progressed to GMG in 2 years. Almost all patients were treated with pyridostigmine and prednisolone. At the end of follow-up, 60.5% had pharmacological remission for more than 3 months, 18.5% had complete remission without medication. No definite factors associated with the remission were identified.
Conclusion:
OMG is the majority of MG patients and the age of onset is earlier than GMG. Early treatment by prednisolone may have the favorable effect on OMG in the progression to GMG and subsequent involvement to the other eye.
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