Unprovoked Seizure Clusters

Patients with unprovoked seizures can present to the emergency room with a cluster of seizures and therefore need to be admitted to the hospital for initial work-up and treatment. Single seizures with return to baseline (much more common) do not usually require admission, unless these are new-onset seizures and where it is anticipated that there will be a significant delay in outpatient work-up (depending on the resource setting).

According to the International League Against Epilepsy (ILAE), a first unprovoked seizure is defined as a seizure or flurry of seizures all occurring within 24 h in a person older than 1 month of age with no prior history of unprovoked seizure. Diagnosing epilepsy requires a minimum of two unprovoked seizures [1].

Are clusters of seizures different from single seizures in terms of the management and the recurrence rate? Overall, about 35% of patients with a first seizure are at risk of having a second within the subsequent 3-5 years, although the risks varies from 23% to 71%, depending on the studies, exclusion criteria and the clinical characteristics [68, 69, 70]. Hauser et al. looked at recurrence risk after a first unprovoked seizure, and found that risks were 10%, 24% and 29% at 1, 3 and 5 years respectively for the idiopathic generalized group, contrasting with 26%, 41% and 46% for the remote symptomatic seizure group. Remote symptomatic seizures were defined as seizures in individuals with prior history of CNS insult known to substantially increase the risk for subsequent epilepsy such as head trauma, stroke or CNS infection [68].

Kho et al. studied adult patients presenting with multiple seizures occurring within 24 h (with recovery in between) and compared them with those presenting with single seizures. Patients with status epilepticus were excluded. They found that the recurrence rate at 1 year was almost identical (38% for the single seizure group vs. 40% for the multiple seizures group), regardless of the aetiology or whether or not they were treated [71]. The only variable that independently predicted the seizure recurrence at 1 year was a remote symptomatic aetiology [71, 72].

Patients who have unprovoked status epilepticus or a seizure cluster as their first seizure may have a lower threshold for deciding to begin chronic AEDs. In cryptogenic or idiopathic epilepsy, initial presentation with status epilepticus was not associated with increased risk of subsequent seizures [73] though there may be a greater risk that recurrence will be in the form of another prolonged seizure or cluster (personal communication, W. A. Hauser, 2007). Among remote symptomatic seizure patients, having status epilepticus or post-ictal Todd's paresis increased the risk for recurrence [68]. In patients with status epilepticus or multiple seizures (two or more within 24 h), recurrence risk was 37% at 1 year, 56% at 3 years and 56% at 5 years, compared with 21%, 34% and 43% at 1, 3 and 5 years for those presenting with single seizures. These patients who present with prolonged seizures or clusters should be offered agents for abortive treatment if they have a means of administering it during their seizure (long aura, clusters with recovery between seizures or a care-giver), such as rectal, nasal or buccal benzodiazepines. Rectal diazepam and nasal/buccal midazolam are the most commonly used choices.

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