Muscle & nerve · 2019
Treatment outcome in juvenile-onset myasthenia gravis.
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Jastrzębska Aleksandra, Jastrzębski Miłosz, Ryniewicz Barbara, Kostera-Pruszczyk Anna
Аннотация
INTRODUCTION: Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction. Approximately 10%-15% of MG patients have juvenile (<18 years of age) onset. We aimed to assess the clinical course, outcome, and subjectively perceived health status of a cohort of juvenile MG patients.
METHODS: This was a retrospective analysis of medical records of 101 patients followed by a cross-sectional questionnaire study.
RESULTS: The mean age of patients was 12.8 years at onset and 13.7 years at diagnosis. Ninety percent of the patients were seropositive. Over 40% of the patients were treated with immunosuppression and over 80% underwent thymectomy. The mean Myathenia Gravis Activities of Daily Living (MG-ADL) scale score was 2.48. At last follow-up, 30.9% of patients were in complete, stable remission; 77.8% perceived their health as good.
DISCUSSION: The treatment outcome for juvenile MG is favorable, with a marked reduction of symptoms and good day-to-day activity achieved for most patients. Muscle Nerve 59:549-549, 2019.
METHODS: This was a retrospective analysis of medical records of 101 patients followed by a cross-sectional questionnaire study.
RESULTS: The mean age of patients was 12.8 years at onset and 13.7 years at diagnosis. Ninety percent of the patients were seropositive. Over 40% of the patients were treated with immunosuppression and over 80% underwent thymectomy. The mean Myathenia Gravis Activities of Daily Living (MG-ADL) scale score was 2.48. At last follow-up, 30.9% of patients were in complete, stable remission; 77.8% perceived their health as good.
DISCUSSION: The treatment outcome for juvenile MG is favorable, with a marked reduction of symptoms and good day-to-day activity achieved for most patients. Muscle Nerve 59:549-549, 2019.
Предметные рубрики MeSH
Activities of Daily LivingAdolescentAdultAge of OnsetAutoantibodiesChildCholinergic AntagonistsCohort StudiesCross-Sectional StudiesDisease ProgressionElectromyographyFemaleFollow-Up StudiesHealth StatusHumansImmunoglobulins, IntravenousImmunologic FactorsImmunosuppressive AgentsMaleMyasthenia GravisPlasmapheresisReceptors, CholinergicRetrospective StudiesSurveys and QuestionnairesThymectomyTreatment OutcomeYoung Adult