Dados do Trabalho

Título

Comparing hormonal effect in symptoms in myasthenia gravis and congenital myasthenic syndromes

Resumo

Introduction: Fluctuations due to hormonal alterations are known features of myasthenic syndromes. In autoimmune myasthenia gravis a reasonable explanation of this phenomenon is hormonal influence in autoimmune response, as usually seen in other autoimmune disorders. Congenital myasthenic patients also experience these fluctuations, but their syndrome is not associated with autoimmune mechanisms. Therefore, the influence of symptoms might be due to other mechanisms of neuromuscular junction impairment. Goals: Evaluate whether there are differences in menstrual period, pregnancy, or menopause in patients with congenital myasthenic syndromes and autoimmune myasthenia gravis. Methods: data of patients were all obtained by phone call and outpatients visits, and then compared between two groups by Fisher’s test. Results: A total of 34 participants with autoimmune myasthenia gravis (22 seropositivity for anti-acetylcholine antibody was present in 22 patients, and two for anti-Musk) and 27 participants with congenital myasthenic syndromes (15 CHRNE, 2 COL13A1, 2 GFPT1, 2 GMPPB, 1 RAPSN, 1 COLQ, 2 DOK7, 1 CHRNA1, 1 MUSK) were interviewed. Mean age was 42.8, MG-ADL median score was 4.7 and pregnancy rate 1.5, in the autoimmune group. In the congenital group the mean age was 34.6, MG-ADL median score 5.1 and pregnancy rate 1.5. There were no statistical differences of worsening rates during menstrual period and anticonception medications or improvement after menopause. Pregnancy in patients with congenital myasthenia resulted in improvement in 55% of patients compared to 70% of worsening symptoms in myasthenia gravis. Congenital myasthenic patients were significantly more likely to have symptoms improved during pregnancy compared with autoimmune patients (p = 0.018). Conclusion: Most results showed that no statistical differences could be observed in patients with congenital myasthenia or myasthenia gravis. An exception was the improvement in symptoms during pregnancy in congenital myasthenic women. This finding suggests that hormonal changes related to pregnancy may favor neuromuscular junction function but may also have an unfavorable effect on immunological attack to neuromuscular junction. These conflicting events would have a neutral, or even deleterious resulting effect on symptoms of autoimmune myasthenic patients. On the other hand, congenital patients, who do not present immunological disorder, would be subject only to the beneficial effects.