Summary: Purpose: To study the electroclinical features of typical absence status (TAS) in adults with syndromes of idiopathic generalized epilepsies (IGEs).Methods: Twenty-one patients with one or more spells of TAS were identified among 136 consecutive adult patients with IGEs. All patients with TAS had comprehensive electroclinical investigations and EEG or video-EEG recorded absences.Results: TAS occurred in 24.4% of 86 patients who had IGEs with typical absences alone or in combination with other seizures presisting in adult life. The prevalence of TAS appeared to be syndrome related, ranging from as high as 57.1% in perioral myoclonia with absences and 46.2% in "phantom" absences with GTCS to as low as 6.7% in juvenile myoclonic epilepsy. A varying degree of impairment of cognition was the cardinal clinical symptom shared in all TAS, but corresponding syndromes of IGE were often betrayed by other symptoms such as eyelid or perioral myoclonia. In phantom absences with GTCS, TAS was more numerous (p S 0.05) and more frequently the first overt seizure type (p = 0.006) than in any other IGE. Only in the syndrome of eyelid myoclonia with absences, TAS was always situation related, mainly as a result of antiepileptic drug discontinuation.Conclusions: The clinical EEG semiology and prevalence of TAS appear to be syndrome related with the highest prevalence in the syndromes of perioral myoclonia with absences and phantom absences with GTCS (p = 0.0024). Key Words:Typical absence status-Nonconvulsive status epilepticusIdiopathic generalized epilepsies-Epileptic syndromesAdults.Absence status is the commone3t form of nonconvulsive status epilepticus, ranging from 53 to 94% in different studies (1-5). It is manifested with prolonged confusional states of varying severity and EEG continuous or repetitive generalized discharges of spikes, multiple lowing. classification of absence status:4. Absence status epilepticus occumng in other epileptic syndromes: progressive myoclonic epilepsies, electrical status epilepticus during slow sleep, Landau-Kleffner syndrome, metabolic or degenerative generalized epilepsies, electrographic gtatus. spikes, and slow waves. Shorvon (6,7) proposed the folTypical (idiopathic) absence status epilepticus occurring in the syndromes of idiopathic generalized epilepsies (IGEs). The EEG discharges are of >2.5 Hz. Atypical (cryptogenic and symptomatic) absence status epilepticus, mainly found in epilepsies, such as the Lennon-Gastaut syndrome. The EEG discharges are slow and <2.5 Hz. De novo absence status epilepticus of late onset, arising de novo in adults without a history of previous epileptic seizures, mainly as the result of drug discontinuation.Although it is well documented in the literature (1-16), absence status is frequently overlooked. It is erroneously considered as complex partial status or other conditions unrelated to epileptic seizures, such as dementia, psychogenic or other behavior disorder (1,10, 12).Furthermore, absence status is usually studied in a unitary fashion without ...

None of the patients had myoclonic jerks or photosensitivity. Two patients were father and daughter and another patient had a family history of infrequent generalised tonic clonic seizures. Conclusion-It seems that this is an idiopathic generalised epilepsy syndrome in adults which has not been previously recognised.

Summary:Purpose: EEG is widely used during positron emission tomography (PET) to confirm the interictal state of the patient and assist in scan interpretation when a seizure occurs. Ictal scans usually reflect mixed interictal-ictalpostictal metabolic activity as seizures are brief in comparison to the 30-min uptake period of the tracer. We wished to determine whether routine EEG is justified and if seizures commonly affect the diagnostic information of the PET scan.Methods: We examined the PET scans of 6 of 236 outpatients with intractable epilepsy with clinical and electrical evidence of a seizure during tracer uptake. We performed semiquantitative analysis in 2 patients who had "ictal" and control interictal scans. Conclusions: In this group of patients, seizures occurred infrequently during tracer uptake. The interpretation of the PET scan when single seizures occurred did not appear to be influenced by the continuous scalp EEG (CSEEG) recordings. The value of routine CSEEG in outpatients treated with medication should be reappraised, with potential cost savings. In rare circumstances in which a true ictal study occurs (complex partial status, epilepsia partialis continua, and repetitive CPS), PET scanning may be inconclusive and repeat interictal scanning should be pursued. Results

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