Ce. Thomas et al., MYASTHENIC CRISIS - CLINICAL-FEATURES, MORTALITY, COMPLICATIONS, AND RISK-FACTORS FOR PROLONGED INTUBATION, Neurology, 48(5), 1997, pp. 1253-1260
We retrospectively reviewed the hospital records of 53 patients admitt
ed for 73 episodes of myasthenic crisis at Columbia-Presbyterian Medic
al Center over a period of 12 years, from 1983 to 1994. Median age at
the onset of first crisis was 55 (range, 20 to 82), the ratio of women
to men was 2:1, and the median interval from onset of symptoms to fir
st crisis was 8 months. Infection (usually pneumonia or upper respirat
ory infection) was the most common precipitating factor (38%), followe
d by no obvious cause (30%) and aspiration (10%). Twenty-five percent
of patients were extubated at 7 days, 50% at 13 days, and 75% at 31 da
ys; the longest crisis exceeded 5 months. Using survival analysis and
backward stepwise Cox regression, we identified three independent pred
ictors of prolonged intubation: (I) pre-intubation serum bicarbonate g
reater than or equal to 30 mg/dl (p = 0.0004, relative hazard 4.5), (2
) peak vital capacity day 1 to 6 post-intubation <25 ml/kg (p = 0.001,
relative hazard 2.9), and (3) age >50 (p = 0.01, relative hazard 2.4)
. The proportion of patients intubated longer than 2 weeks was 0% amon
g those with no risk factors, 21% with one risk factor, 46% with two r
isk factors, and 88% with three risk factors (p = 0.0004). Complicatio
ns independently associated with prolonged intubation included atelect
asis (p = 0.002), anemia treated with transfusion (p = 0.03), Clostrid
ium difficile infection (p = 0.01), and congestive heart failure (p =
0.03). Three episodes of crisis were fatal, for a mortality rate of 4%
(3/73); four additional patients died after extubation. All seven dea
ths were due to overwhelming medical comorbidity. Over half of those w
ho survived were functionally dependent (home or institutionalized) at
discharge. In addition to prospective controlled studies of immunothe
rapies, the prevention and treatment of medical complications offers t
he best opportunity for further improving the outcome of myasthenic cr
isis.