Myasthenic crisis: report of 24 cases

Author:

Werneck Lineu Cesar1,Scola Rosana Herminia1,Germiniani Francisco Manoel Branco1,Comerlato Enio A.1,Cunha Francisco Marcos Bezerra1

Affiliation:

1. Universidade Federal do Paraná, Brazil

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.

Publisher

FapUNIFESP (SciELO)

Subject

Neurology,Neurology (clinical)

Reference18 articles.

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4. Long-term cyclosporine treatment in a group of severe myasthenia gravis patients;Bonifati DM;J Neurol,1997

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