Review Article

The Syndrome of Absence Status Epilepsy: Review of the Literature

Table 1

Patients with recurrent AS with onset in adolescent/adult age.

AuthorsSexAge at AS onset (yrs)Other seizuresEpilepsy family historyPhoto sensitivityAED therapyResponse of AS to AEDsNotes

Definite cases of ASE

Nightingale and Welch, 1982 [6]F56GTCSNSNSPRI, PHTPersistence of seizures

Zambrelli et al., 2006 [7]M>20GTCSNoNoLTG, VPASeizure free

Genton et al., 2008 [5]F14GTCSNoNoVPASeizure free
F34ANoNoVPA, LTGSeizure free
F16GTCSNoNoVPA, ESM, and CZPPersistence of seizures
M35GTCSNoNoVPA, ESMSeizure free
M15GTCSNoNoVPASeizure free
M26GTCSNoNoVPASeizure free
F26GTCSNoNoVPA, PB, and LEVPersistence of seizures
F65GTCSNoNoVPASeizure free
M16A, GTCSNoNoVPA, LTGPersistence of seizures
M36ANoNoLEV, TPMSeizure free
M36GTCSNoNoLTG, ESMSeizure free

Bilo et al., 2010 [8]M14GTCSNoNoLTG, VPAPersistence of seizures

Pro et al., 2011 [9]F54NoYesYesLTGSeizure free

Possible cases of ASE (atypical features)

Terzano et al., 1978 [11]F76GTCSNoYes??Atypical feature: prominent myoclonic jerks during AS

Possible cases of ASE (insufficient data)

Lee 1985 [12] Three patients in this series presented recurring AS, but it is not clear if a defined IGE syndrome and/or other seizures were the main seizure type and/or if AS episodes were situation related.

Berkovic et al., 1989* [13] Fourteen patients in this series had recurring AS with onset after the age of 15. An undetermined number of these 14 patients belonged to “the unusual but well-recognized group of IGEs beginning in middle to later life, where AS is a particular prominent seizure type.”

Tomson et al., 1992 [14] AS was the only seizure type in 5 patients with undetermined IGE. However, it is not clear whether or not AS was recurring in any of these patients.

Agathonikou et al., 1998 [15]
(patients 18, 19, and 20)
M48GTCSNSNSVPA, CBZ, and PHTSeizure freeIt is not clear if AS is the main seizure type.
F36A, GTCSNSNSVPASeizure freeIt is not clear if AS is the main seizure type.
F43A, GTCSNSNSPB, PHT, ESM, and CZPSeizure freeIt is not clear if AS is the main seizure type.

Szucs et al., 2008 [16]
(patient 2)
F45GTCSNSNSNSSeizure freeIt is not clear if AS is the main seizure type.

Mireles and O’Donovan, 2010 [17]
(patients 1, 2, and 3)
NS49ANSNSLEVSeizure freePossible diagnosis of CAE
NS60GTCSNSNSLEV, LTG, PTH, and VPAPersistence of seizures Insufficient clinical data
NS47NoNSNSVPASeizure freeInsufficient clinical data

Possible cases of ASE (prompt diagnosis may have altered natural history)

Iivanainen et al., 1984 [18]
(patient 2)
F61A, GTCSNoNoVPASeizure freeThis patient had few seizures in her life, among which AS does not stand as the main seizure type. However, its low recurrence may be due to prompt diagnosis and treatment.

Fernández-Torre and Rebollo, 2009 [19]F68GTCSNoYesVPASeizure freeConcomitant seizures were rare and disappeared after childhood. AS is not recurrent, but this may be due to prompt diagnosis and treatment.

Cases of AS not fulfilling criteria for ASE

Iivanainen et al., 1984 [18]
(patient 1)
F55A, GTCSNoNoVPA, PBConcomitant seizures were very frequent.

Michelucci et al., 1996 [20] (patients 1 and 4)F28A, GTCSNoNSVPANSAS was not the main seizure type.
M41A, GTCSNoNSPHT, PBNSDiagnosis of JAE.

Agathonikou et al., 1998 [15] Patients 1–17 had defined IGE syndromes; moreover, patients 9, 10, 16, and 17 had presented only one AS episode.

Baykan et al., 2002 [21] All 8 patients had recurrent AS but all of them were diagnosed with defined IGE syndromes.

Nguyen Michel et al., 2011 [22]
(patient 5)
F71A, GTCSNSNSLTG, LEV?Diagnosis of CAE.

AS: absence status; ASE: absence status epilepsy.
This series includes also the patients presented in Andermann and Robb, 1972 [4].
GTCS: generalized tonic clonic seizures; A: absences.
NS: not specified.
AED: antiepileptic drugs; PRI: primidone; PHT: phenytoin; LTG: lamotrigine; VPA: valproate; ESM: ethosuximide; CZP: clonazepam; PB: phenobarbital; LEV: levetiracetam; TPM: topiramate; CBZ: carbamazepine.
IGE: idiopathic generalized epilepsy; JAE: juvenile absence epilepsy; CAE: childhood absence epilepsy.