MYASTHENIA-GRAVIS IN CHILDHOOD

Citation
B. Anlar et al., MYASTHENIA-GRAVIS IN CHILDHOOD, Acta paediatrica, 85(7), 1996, pp. 838-842
Citations number
21
Categorie Soggetti
Pediatrics
Journal title
ISSN journal
08035253
Volume
85
Issue
7
Year of publication
1996
Pages
838 - 842
Database
ISI
SICI code
0803-5253(1996)85:7<838:MIC>2.0.ZU;2-O
Abstract
Clinical features, serum acetylcholine receptor antibody (AChRAb) titr es and course were reviewed in a series of 25 congenital (CMG) and 30 juvenile (JMG) myasthenia gravis cases to recognize characteristics of childhood-onset myasthenia and its subgroups. The initial symptom for CMG is ptosis accompanied or followed by generalized weakness; myasth enic crises do not occur and spontaneous remissions are rare. In JMG, the distribution of weakness remains the same, but the severity fluctu ates: spontaneous remissions (6 patients) and myasthenic crises (10 pa tients) are observed. Good response to anticholinesterase drugs is sli ghtly more frequent in JMG (62 versus 41%). AChRAbs were present in 9/ 26 JMG tested, girls with onset after 11 years being more likely to be Ab-positive. Since patients with autoimmune myasthenia and a young ag e of onset are often seronegative, clinical features such as changing distribution of weakness, fluctuating severity, or response to treatme nt might be considered as supportive criteria for differentiating JMG from CMC.