The records of twenty-two children, ages 4.5 months to 18 years, treated for refractory status epilepticus (RSE) between 1992 and 2000, were reviewed retrospectively at Children’s Hospital, Boston. Treatment consisted of high-dose anesthetic agents, including pentobarbital, midazolam, propofol, phenobarbital, alone or in combination, for periods ranging from 2 to 146 days (mean 31 days). Outcome correlated with duration of treatment: of 8 treated for <7 days, only 2 died and 4 returned to baseline neurologic state, whereas of 7 requiring treatment of RES for >31 days, 4 died and only 1 returned to baseline. A total mortality of 32% (7 cases) was related to etiology, age, and EEG. The most frequent etiology was presumed or known viral encephalitis affecting 10 patients, of whom 4 died. Of 4 children <3 years of age, none returned to baseline, 3 died, and one had new neurologic deficits after RSE. Of 6 children older than 10 years, 4 returned to baseline, and only one died. Mortality was 25% in 12 children with focal EEG abnormalities at onset of SE, and 40% in 10 with multifocal or generalized EEG abnormalities. No deaths occurred in 5 seizure susceptible children with remote symptomatic seizures not due to an acute provocation; their diagnoses were mental retardation, Rett’s syndrome, old intracranial hemorrhage, atypical Rassmussen’s encephalitis, and developmental delay. [1]

COMMENT. Refractory status epilepticus in childhood carries a high mortality (32%) and morbidity that are related to the etiology, age, EEG, and duration of suppressive treatment. Younger patients with presumed encephalitis and multifocal EEGs require prolonged suppressive therapy and the outcome is especially poor.

The findings were similar in a study of 346 adolescent and adult patients with status epilepticus reported from the Netherlands [2]. A poor outcome was related especially to the underlying cause and status symptomatic of an acute illness, a duration of >4 hours, the occurrence of medical complications, and inadequate therapy. Of 38 patients who died, 44% had received insufficient therapy.

A study in 74 adults with 83 episodes of status epilepticus at the Neurologial Institute, New York, found a mortality of 21% which was correlated with increased age and acute symptomatic seizures. Increased length of hospitalization and acute symptomatic seizures were predictors of functional disability in 23% of patients with nonfatal episodes. [3]

Regarding long-term outcome, the occurrence of status with recovery had no adverse effects in a cohort of 342 children, when followed prospectively for a mean of 72 months [4]. It appears that mortality with status is related particularly to the cause of the status and the association of an acute illness such as encephalitis or encephalopathy, and sometimes to inadequate therapy. Although the majority of single, generalized tonic-clonic seizures are self-limiting and no more than 2 to 4 minutes duration, a seizure lasting 5 minutes or more may progress to status and should be treated aggressively. The prevention of status and the effective treatment of status are dependent on the avoidance of delay in initiating anticonvulsant therapy, and the adherence to a well-defined protocol outlined for emergency room physicians to follow. (Leppik IE, 2000; see Progress in Pediatric Neurology III, PNB Publ, 1997;102).