We report clinical and video-EEG findings in 11 adults with the syndro me of eyelid myoclonia with absences (EMA). Prevalence was 2.7% among all epilepsies and 12.9% among idiopathic generalized epilepsies (IGE) with typical absences. All patients with EMA were women, with a mean age of 30.9 years and a mean age of 7.8 years at reported onset of eyelid myoclonia. The characteristic seizures, studied with video-EEG in 10 patients, began with and were mainly manifested by eyelid myoclonia, either alone if brief (< or = 2 s) or with associated mild impairment of consciousness if longer. The ictal EEG was characterized by polyspike and slow waves at 3-6 Hz. Ictal clinical and EEG manifestations occurred mainly after eye closure and were inhibited by total darkness. All patients were photosensitive, but photosensitivity decreased with age; nonpatient had self-induced seizures. Infrequent generalized tonic-clonic seizures (GTCS) occurred in all but the youngest patient; they were usually infrequent and were precipitated mainly by flickering lights, sleep deprivation, fatigue, and menstruation. Mild myoclonic jerks of the upper limbs occurred in 6 patients. Eyelid myoclonia was resistant to medication and persisted despite control of other seizures.

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.

Fifteen patients who experienced epileptic seizures while playing video games are described together with a review of 20 cases in the English literature. Nine of the 15 cases and all but two of the reported cases experienced their first seizure while playing video games.

Background: Tuberous sclerosis complex (TSC)-associated neuropsychiatric disorders (TAND) have unique, individual patterns that pose significant challenges for diagnosis, psycho-education, and intervention planning. A recent study suggested that it may be feasible to use TAND Checklist data and data-driven methods to generate natural TAND clusters. However, the study had a small sample size and data from only two countries. Here, we investigated the replicability of identifying natural TAND clusters from a larger and more diverse sample from the TOSCA study. Methods: As part of the TOSCA international TSC registry study, this embedded research project collected TAND Checklist data from individuals with TSC. Correlation coefficients were calculated for TAND variables to generate a correlation matrix. Hierarchical cluster and factor analysis methods were used for data reduction and identification of natural TAND clusters.