Lu sur :https://www.ncbi.nlm.nih.gov/pubmed/28877546
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Clinical Features and Evolution of Juvenile Myasthenia Gravis In a French Cohort.
Barraud C1,2, Desguerre I3,4, Barnerias C3, Gitiaux C2,3, Boulay C1,2, Chabrol B1,2.
Muscle Nerve. 2017 Sep 6. doi: 10.1002/mus.25965.
We determined the clinical, paraclinical, and treatment-related features of juvenile myasthenia gravis (JMG) as well as the clinical course in a cohort of French children.
We conducted a retrospective study of 40 patients with JMG at 2 French Pediatric Neurology departments from April 2004 to April 2014.
Among the patients, 70% had generalized JMG, 52% had positive acetylcholine receptor antibodies, 8% had muscle-specific-kinase antibodies, and 40% were seronegative. Treatment with acetylcholinesterase inhibitors was effective and sufficient in 47% of patients. The 6 patients with generalized JMG treated with rituximab and/or immunoadsorption showed improvement. Thirty percent of the patients required hospitalization in an intensive care unit during follow-up (mean 4.7years). Remission without treatment occurred in 18% of patients.
As with adults, JMG has high morbidity, particularly among children with generalized symptoms, and rituximab should be considered early in the course of the disease as a second line treatment.