Juvenile Myasthenia Gravis: Frequent Presentation with Bulbar Symptoms

Abstract

Juvenile myasthenia gravis usually presents with ocular findings. Rarely, children may present with generalized weakness, sometimes with accompanying bulbar symptoms (dysphagia, facial weakness). However, presentation with isolated bulbar symptoms is extremely unusual. We present four consecutive cases of JMG presenting with predominant bulbar symptoms including voice nasality, dysarthria, and swallowing difficulty (even leading to significant weight loss). Our cases emphasize that primary care physicians should suspect neuromuscular disease such as JMG in children with bulbar symptoms and signs.

Keywords

• myasthenia gravis, children, bulbar, swallowing

References

1. Parr JR, Jayawant S. Childhood myasthenia: clinical subtypes and practical management. Dev Med Child Neurol 2007; 49: 629-635.
2. Chiang LM, Darras BT, Kang PB. Juvenile myasthenia gravis. Mus Nerve 2009; 39: 423-431.
3. Gadient P, Bolton J, Puri V. Juvenile myasthenia gravis: Three case reports and a literature review. J Child Neurol 2009; 24: 584-590.
4. Finnis MF, Jayawant S. Juvenile myasthenia gravis: a paediatric pespective. Autoimm Dis 2011; 2011: 1-7.
5. Osserman KE. Myasthenia Gravis. New York: Grune & Stratton, 1958.
6. Yang Z, Xiong H, Zhang Y, Bao X, Jiang Y, Wu Y, et al. Clinical characteristics and follow-up management of 135 children with myasthenia gravis. J Peking Univ (Health Sci) 2011; 43: 455-459 (in Chinese; abstract in English).
7. Andrews PI,Massey JM, Howard JF Jr, Sanders DB. Race, sex, and puberty influence onset, severity, and outcome in juvenile myasthenia gravis. Neurology 1994; 44: 1208-1214. 8- Farrugia ME, Robson MD, Clover L, Anslow P, Newsom-Davis J, Kennett R, et al. MRI and clinical studies of facial and bulbar muscle involvement in MuSK antibody-associated myasthenia gravis. Brain 2006; 129: 1481-1492.
8. Chiu HC, Vincent A, Newsom-Davis J, Hsieh KH, Hung T. Myasthenia gravis: population differences in disease expression and acetylcholine receptor antibody titers between Chinese and Caucasians. Neurology 1987; 37: 1854-1857. 10- Lindner A, Schalke B, Toyka KV. Outcome in juvenile-onset myasthenia gravis: a retrospective study with long-term follow-up of 79 patients. J Neurol 1997; 244: 515-520.