A previously healthy 19-year-old woman is brought to the emergency department (ED) for a witnessed “convulsion.” She had suddenly stopped talking and stared into space in the middle of a conversation with her roommates. She then exhibited stereotypical picking behavior of her clothes followed by head deviation to the right and generalized tonic-clonic arm and leg movements lasting approximately 3 minutes. Emergency medical services (EMS) were activated and immediately transferred her to the closest ED, where she is found to be lethargic, with a temperature of 101.3°F. The rest of her vital signs are within normal limits. Her pupils are bilaterally reactive to light, and she has an intact oculocephalic reflex and intact corneal reflexes. She localizes to painful stimulation with the left arm but does not move the right side. The remainder of her neurological examination is unremarkable. Admission chest radiograph and head computed tomography (CT) are unrevealing. On returning from the CT scanner, she has a second generalized tonic-clonic seizure witnessed by the ED staff that lasts slightly more than 5 minutes.
What is the most likely diagnosis in this patient?
The clinical presentation is most consistent with secondary generalized convulsive status epilepticus (GCSE). Current opinion holds that any convulsive seizure lasting greater than > 5 minutes or 2 or more convulsions in a 5-minute period without a return to preconvulsive neurological baseline is defined as status epilepticus (SE).1-6 This definition is based on animal and human data suggesting irreversible neuronal injury and pharmacoresistance7-9 after 5 minutes of prolonged seizures. Furthermore, observations have shown that most isolated clinical and electrographic seizures last less than 5 minutes, and prolonged seizures beyond this time typically are not self-resolving.10-12
SE is a relatively common neurologic emergency with an overall estimated incidence of 41 to 61 cases per 100 000 patients per year.13 Despite its prevalence, it continues to be associated with significant morbidity and mortality because of previously discussed associations with irreversible neuronal injury and treatment pharmacoresistance. Subsequently, rapid recognition, diagnosis, and treatment are crucial, and these multidisciplinary efforts begin in the prehospital setting to optimize the management of SE. Classic clinical features can range from obvious positive signs of rhythmic jerking and posturing, to more subtle positive symptoms of twitching, nystagmus, automatisms, and eye deviation. Focal weakness may occur in the setting of Todd’s paralysis. Negative symptoms of seizures include staring, coma, lethargy, confusion, and aphasia. Negative symptoms may be confused with a postictal state; however, a high suspicion must be maintained that these negative symptoms represent the presence of nonconvulsive SE (NCSE) as nearly half of GCSE patients will continue to have electrographic seizures after the clinical manifestations resolve.14
When should the treatment of GCSE be started?
The most important factor in the successful treatment of SE is initiating treatment as early as possible.15,16 In one ...