Sujets)
Humains , Mâle , Adulte d'âge moyen , Amyotrophie/diagnostic , Muscles de la face/anatomopathologie , Myasthénie/diagnostic , Autoanticorps/sang , Biopsie , Amyotrophie/étiologie , Récepteurs cholinergiques/sang , Électromyographie , Muscles de la face/physiopathologie , Myasthénie/complicationsRésumé
Myasthenia gravis (MG) in childhood is rare comprising 10 to 20 percent of all myasthenic patients. We studied 18 patients with MG whose first symptoms started from 1 to 12 years of age, followed at the Department of Neurology of the UNIFESP-EPM, from January 1983 to August 1997. There were 10 girls and 8 boys (1.2:1). Eleven patients (61 percent) presented moderate or severe generalized disease and 4 (22 percent) had at least one myasthenic crisis. EMG with supramaximal repetitive nerve stimulation was diagnostic in 8 (47 percent) out of 17 patients, and chest CT was normal in 14 patients. Seropositivity to acetylcholine receptor antibodies was found in 81.6 percent (9 out of 11 tested) and the levels had no relation to clinical severity. Nine out of 16 patients (56 percent) worsened with pyridostigmine alone and were treated with prednisone. Four out of those nine continued worsening despite steroids and were subjected to thymectomy (all showed thymic lymphoid follicular hyperplasia). Three patients (75 percent) improved markedly after thymectomy and one (25 percent) worsened, eventually getting better with intravenous immunoglobulin and oral azathioprine. MG treatment, using all resources available, has to be individualized for each child
Sujets)
Humains , Mâle , Femelle , Nourrisson , Enfant d'âge préscolaire , Enfant , Myasthénie/diagnostic , Myasthénie/chirurgie , Âge de début , Anticholinestérasiques , Études de suivi , Myasthénie/traitement médicamenteux , Myasthénie/immunologie , Prednisone/usage thérapeutique , Bromure de pyridostigmine/usage thérapeutique , Récepteurs cholinergiques/sang , Récepteurs cholinergiques/immunologie , Thymectomie , TomodensitométrieRésumé
Clinical, immunological and genetic parameters were studied in 73 Jamaican patients with myasthenia gravis (Mg). The reported biomodal clinical distribution of females with early onset of disease and males with late onset was not observed. The female to male ratio was 2:1. The most frequent manifestations of disease were ptosis (84.9%), general muscle weakness (68.5%), bulbar symptoms (41.1%) and diplopia (32.9%). Unusual presenting features such as unilateral ptosis, recurrent chest infection and stumbling while walking resulted in diagnosis being missed in 5.8%of cases. The sensitivity of radiommunoassay in detecting acetylcholine receptor antibody (AchR-Ab) in sera from a subgroup of 35 MG patients was 71.4%whilst that of the ELISA was only 14.2%. There was no correlation between HLA-type, thymic pathology and course of disease. HTLV-I could not be implicated in the pathogenesis of this disease. There was a paucity of other associated autoimmune conditions among MG patients. Thymectomy was an important therapeutic modality in that improvement was observered in 22 cases and remission in 11.