Juvenile myasthenia gravis: three case reports and a literature review.
ABSTRACT Juvenile myasthenia gravis is a rare disorder acquired in childhood, representing 10% to 15% of all cases of myasthenia gravis. Like the adult form, it is generally characterized by an autoimmune attack on acetylcholine receptors at the neuromuscular junction. Most patients present with ptosis, diplopia, and fatigability. More advanced cases may also have bulbar problems and limb weakness. Left untreated, the disease may progress to paralysis of the respiratory muscles. Early recognition of this disease helps avoid unnecessary testing, prevent undue parental anxiety, and stop the progression of symptoms. Here, we relate the clinical course and current status of 3 patients with juvenile myasthenia gravis, discuss the disease in general, and review current treatment modalities.
- SourceAvailable from: bmj.com[show abstract] [hide abstract]
ABSTRACT: A long term follow up study is presented of 73 patients with myasthenia gravis, living in Amsterdam between 1926 and 1965. In the period 1961-65 the annual incidence was 3.1, the prevalence 53 per million. Maximum severity of the disease occurred during the first seven years after onset in 87%. Eighteen (29%) patients died, of whom eight had a thymoma (TH). Spontaneous improvement or remission occurred at any time during the follow up. At the end of the study (1985) 16 (22%) patients were in a complete clinical remission, 13 (18%) had improved considerably (3 with prednisone), 12 (16%) had improved moderately, 12 (16%) had remained unchanged and two had deteriorated. If the early deaths are excluded the outcome is similar in the early and the late onset group without TH. Patients with TH had a less favourable course. Associated autoimmune diseases were diagnosed in 25% (n = 58). Because most of these patients were treated with anticholinesterases only, the evolution of their clinical state represents the natural course of MG.Journal of Neurology Neurosurgery & Psychiatry 11/1989; 52(10):1121-7. · 4.92 Impact Factor
- [show abstract] [hide abstract]
ABSTRACT: Disorders of the neuromuscular junction have a wide range of clinical presentations, which frequently poses a diagnostic challenge to evaluating clinicians. This article describes the tests used in the diagnosis of diseases of the neuromuscular junction, reviews the evidence supporting the use of each test, and proposes guidelines for their efficient utilization. A focused review of the literature was employed. Reports from four main categories of diagnostic tests (pharmacologic, electrodiagnostic, immunologic, and miscellaneous tests) were reviewed, and the sensitivity and specificity of each test in the diagnosis of specific neuromuscular junction diseases were examined. The clinical presentation determines which diagnostic tests should be utilized in individual cases of suspected neuromuscular junction disease. However, knowledge of the sensitivity and specificity of each test can help to focus the diagnostic evaluation and maximize the diagnostic yield of each test.American Journal of Physical Medicine & Rehabilitation 09/2005; 84(8):627-38. · 1.73 Impact Factor
- [show abstract] [hide abstract]
ABSTRACT: Randomised and controlled treatment studies of juvenile-onset myasthenia gravis have not been published. We therefore report our retrospective analysis of 79 patients with juvenile-onset myasthenia gravis observed for as long as 30 years. The mean age at onset was 13.7 years and median follow-up 7.7 years. The initial presentation was generalised disease in 90% and ocular disease in the remaining patients. Sixty-five patients (82%) were thymectomised. In 14 of these, treatment consisted of a combination of azathioprine (2-3 mg/kg), corticosteroids (prednisolone up to 60 mg for a maximum duration of 12 months with subsequent tapering) and acetylcholinesterase (AChE) inhibitors, and of azathioprine and AChE inhibitors in 27 patients. One patient received azathioprine and 22 AChE inhibitors only; in another no further medication was necessary. In the severely affected group (n = 16), plasmapheresis was performed additionally before thymectomy and continued for some time after the operation. Treatment was started between 1 and 14 months (mean 2.4 months) after the onset of myasthenic symptoms. No thymectomy was done in 14 patients, and immunosuppressive treatment and AChE inhibitors were given in 9 of these cases. One patient received azathioprine only; 4 patients received AChE inhibitors only. The histology of the thymus gland showed follicular hyperplasia in 89% of the 65 thymectomised patients and normal findings in the remainder. Remission occurred in 60% of patients who underwent thymectomy and in 29% of those who were not thymectomised. Hyperthyroidism (6 patients, 8%), diabetes mellitus (2 patients, 3%) and rheumatoid arthritis (2 patients, 3%) were the most frequent associated immune-mediated diseases. Epileptic seizures and neoplasia were coincident diseases in 2 (3%) and 3 (4%) patients, respectively. There were no deaths from thymectomy or from immunosupression. This open, retrospective analysis suggests that juvenile-onset myasthenia gravis can be treated satisfactorily in most patients by the use of thymectomy and/or immunosupressive medication.Journal of Neurology 09/1997; 244(8):515-20. · 3.58 Impact Factor