Myasthenic crisis: report of 24 cases

Abstract

-Myasthenic crisis (MC) is a life-threatening complication of myasthenia gravis (MG) with a high mortality rate. The aim of our study was to review the different therapeutics approaches in the treatment of MC and their impact in the final outcome. We reviewed the medical files of patients diagnosed with MG admitted between February 1993 and October 1997, who developed MC. Sex, mean age, disease's duration, functional scale, symptoms preceding the crisis, crisis therapy in each set and mortality were then analysed. There were 24 patients who developed MC, 21 females and 3 males, with 1 neonatal, 1 congenital sporadic, 17 juvenile/adult, 3 over 50 years and 2 with thymoma. Dysphagia, dysphonia and dysartria were the most common symptoms preceding the crisis. A precipitating factor was elicited in 8 cases and the most common was infection (upper airway infection, urinary tract infection and pneumonia). 16 patients needed a nasogastric tube and 9 had a tracheostomy performed. 24 patients used anticholinesterase drugs, 21 prednisone, 7 immunosuppressive agents, 5 plasmapheresis, 3 human hyperimune gamma immunoglobulin and 12 had thymectomy. A good response was obtained in 13, satisfactory in 7 and there were 4 deaths. We concluded that in spite of all the therapeutics options, there were non statistically significant differences in the outcome of patients that underwent thymectomy and those who did not.KEY WORDS: myasthenia gravis, myasthenic crisis, thymectomy, neuromuscular disorders, muscle diseases.Crise miastênica: relato de 24 casos Crise miastênica: relato de 24 casos Crise miastênica: relato de 24 casos Crise miastênica: relato de 24 casos Crise miastênica: relato de 24 casos RESUMO -A crise miastênica (CM) é uma complicação preocupante da miastenia grave (MG) que apresenta altos índices de mortalidade. Neste estudo revisamos as diferentes abordagens no tratamento da CM e seu impacto no resultado final. Levantamos os dados dos pacientes com MG que desenvolveram CM admitidos entre fevereiro de 1993 e outubro de 1997. Foram analisados as interrelações do sexo, idade media, duração da doença, escala funcional, sintomas e procedimentos precedendo as crises, terapêutica empregada e mortalidade. Foram encontrados 24 casos que desenvolveram CM, sendo 21 do sexo feminino e 3 do masculino. Em relação à apresentação clínica, 1 era da forma neonatal, 1 congênita esporádica, 17 juvenil/adulta, 3 acima de 50 anos e 2 com timoma. Os sintomas principais que precederam a CM foram disfagia, disfonia e disartria. Foi possível identificar um fator desencadeante em 8 casos e o mais comum foi infecção (vias aéreas, pneumonia e trato urinário). Necessitaram de sonda nasogastrica 16 casos e a traqueostomia, 9. Usaram medicações anticolinesterásicas todos os 24 casos, prednisona 21, imunossupressivos 7, plasmaferese 5, gamaglobulina hiperimune 3 e foram submetidos a timectomia 12 casos antes ou após a CM. Obtivemos bom resultado em 13, satisfatório em 7 e ocorreram 4 óbitos, sendo 3 não relacionados com a CM. Concluímos que...

Clinical features and impact of myasthenia gravis disease in Australian patients

“…While it is generally accepted that external factors can cause exacerbation of disease there is little data about this. However, there are studies of the triggers of myasthenic crisis and the most common precipitant is infection [24]. This effect could be due to the infection increasing inflammatory processes.…”

Clinical profile and outcome of myasthenic crisis in a tertiary care hospital: A prospective study

“…Other studies have reported estimates of mortality in MG crisis as high as 6-16.7%. [3121324] Among the Indian studies Aggarwal et al . [14] reported no mortality in their case series of six patients, Murthy et al .…”

Plasmapheresis in the treatment of myasthenia gravis: retrospective study of 26 patients

“…A multicenter study from Taiwan showed that 34.9% of PE was indicated for MG patient 16 . A restricted number of national publications about PE and MG was encountered [17][18][19][20] . Seggia et al 17 from Rio de Janeiro analyzed costs and benefits of PE in the preparation of patients for thymectomy.…”

“…PE was indicated due to myasthenic crisis in seven cases, 26.9 % of the casuistic. Werneck et al 20 used PE as specific treatment in 16.6 % of 24 cases of these potentially fatal condition.…”