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

Abstract

Background:The present understanding of the clinical course, complications, and outcome of myasthenic crisis (MC) is based chiefly on observational studies and retrospective case series.Aim:To study the baseline demographic and clinical variables, risk factors, complications, outcome, and mortality in patients of MC.Materials and Methods:All patients of myasthenia gravis (MG) who presented with myasthenic crisis between July 2009 and December 2010 were included.Results:Ten patients of MC were included in this study. The median age of the patients was 40.5 years (range 14-71 years). Seven were females and three were males. Nine had generalized MG and one patient had oculobulbar involvement only. Median duration of disease was 3 years (range 1 month to17 years). Two patients had thymoma. Two patients had history of thymectomy in the past. Infection was the most common triggering factor accounting for five cases (50%) followed by inadequate treatment/drug withdrawal in three (30%) and steroid initiation and hypokalemia in the remaining two patients (20%). Median duration of MC was 12 days (range 3-28 days). Mortality was in 3 out of 10 (30%) during MC. Management in the intensive care unit (ICU) and treatment with plasma exchange/intravenous immunoglobulins were associated with good outcome.Conclusions:Ventilator support and management in intensive care unit are the most important components in the management of MC. The high mortality rate seen in present study may be more reflective of the actual ground reality in resource constrained developing countries, however, larger prospective studies are needed to confirm these findings.

Myasthenic Crisis Treated in a Neurological Intensive Care Unit: Clinical Features, Mortality, Outcomes, and Predictors of Survival

“…In the present study, we found that first MC affecting people younger than 50 years affected women disproportionately, most of whom were aged 20-50; in contrast, first MC affecting people older than 50 did not show gender bias. These results are consistent with other studies [3,6,8,9]. The average interval from onset of MG to first MC requiring ICU managemsnt was 24.08 months in our cohort, much longer than the 8 months reported in another study [3].…”

“…The average interval from onset of MG to first MC requiring ICU managemsnt was 24.08 months in our cohort, much longer than the 8 months reported in another study [3]. Our results are consistent with recent reports of a median interval from onset to crisis of 3 years [9] and mean duration of MG prior to ICU admission of 3.8 years [10]. A longer interval from MG onset to first MC probably reflects recent improvements in recognition of the disease, management of respiratory and bulbar conditions, and greater access to newer treatment modalities.…”

“…Infection, especially lower respiratory tract infection, was the most common identifiable precipitant of MC, followed by medication, and inadequate treatment/drug withdrawal. Other studies have also identified respiratory tract infection as the most frequent cause of MC, accounting for about half of crises resulting in ICU admission [9,10,14]. Failure to comply with treatment or drug withdrawal was a frequent cause of MC accounting for 11 patients out of 115 in our study.…”

“…By the end of follow-up, 21 of 115 patients in our cohort had died (18.26%).This mortality rate is near the high end of the range of 6-30% reported for MC patients in several studies [3,9,[23][24][25]. However, the mortality rate of MC fell from 42% in the early 1960s to contemporary rates of 4 to 10% with the improvement of the advent of IVIg and plasma exchange and ICU management [3,6].…”

Myasthenic crisis treated in a neurological intensive care unit: clinical features, mortality, outcomes, and predictors of survival

“…By the end of follow-up, 21 of 113 patients in our cohort had died (18.58%).This mortality rate is near the high end of the range of 6-30% reported for MC patients in several studies [3,9,[23][24][25]. In a Chinese cohort from Hong Kong, 35 MG patients experienced crisis and 2 died (5.71%) [26], but in a cohort from India, mortality was in 3 out 10 (30%) during MC (30%) [9]. However, the mortality rate of MC fell from 42% in the early 1960s to contemporary rates of 4 to 10% with the improvement of the advent of IVIg and plasma exchange and ICU management [3,6].…”