Abstracts

Rationale: Absence Status Epilepticus of Later Life is an uncommon presentation within the idiopathic generalized epilepsies (IGE) that can be challenging to diagnose and treat. This case series of three subjects highlights the medication refractory nature of this condition. Methods: A chart review was performed for three subjects greater than age 60 fitting a proposed diagnosis of Absence Status (AS) Epilepsy [1]: recurrent episodes of unprovoked AS, onset of AS in adult age, AS as the main seizure type, clinical and laboratory findings consistent with IGE but not suggesting one of the recognized IGE syndromes. Results: Subject 1 presented at age 60 with trouble at work due to “slow moments”. Video electroencephalogram (VEEG) revealed these to be 1-4-hour episodes of AS. She had a prior diagnosis of IGE since age 32, with well controlled absence and generalized-tonic-clonic (GTC) seizures on Valproate and Levetiracetam. She underwent trials of six antiepileptic drugs (AEDs) with temporary improvement in AS frequency but never long-term control. She opted to remain on Valproate monotherapy, as it provided the best control. Subject 2 presented at age 64 with worsening frequency of “bad days at work”. He initially experienced a “bad day” once a week, but this progressed to a near daily problem. He had a history of IGE with GTC seizures since age 12 and absence seizures with presumed onset in his teens. He required four AEDs to gain control of his seizures in his 20s with subsequent good control until age 64. VEEG revealed near daily episodes of AS lasting 30 minutes to 4 hours. He trialed four AEDs with best control of AS on a combination of Valproate and Ethosuximide. Subject 3 presented at age 61 with recurrent episodes of appearing confused. He was hospitalized six times for AS requiring high dose benzodiazepines. Two of the episodes culminated in GTC seizures. He had a history of IGE with three GTC seizures from ages 14-18, although afterwards he remained seizure free and medications were stopped without recurrence for 40 years. VEEG revealed multiple hour-long episodes of AS. He trialed three AEDs with best control on a combination of Valproate and Ethosuximide. All three subjects had 2-4 Hz polyspike wave discharges on EEG. All three subjects obtained improved seizure control on a combination of AEDs including Valproate, but none have obtained prolonged seizure freedom. Most importantly, all three subjects had to retire early from professional careers due to frequent hospitalizations, medication side effects, and confusion during frequent AS episodes. Conclusions: In this three subject case series of Absence Status Epilepticus of Later Life, all three subjects eventually obtained improved seizure frequency on a combination of medications including Valproate but never obtained complete seizure freedom. Most importantly from a patient perspective, they were unable to return to prior professional functional levels. This condition, while uncommon, is an example of medication refractory IGE and highlights our need for advancements in medications and other treatment options for this patient population.Reference1. Bilo L, Pappata S, De Simone R, Meo R. The syndrome of absence status epilepsy: review of the literature. Epilepsy Research and Treatment, pp. 1-8, 2014. Funding: Not applicable