A Clinical and EEG Study on Idiopathic Partial Epilepsies with Evolution into ESES Spectrum Disorders

Abstract

Summary:Purpose: Questioning the presence of any possible prognostic predictors, this study includes a long-term follow-up of clinical and EEG characteristics of 16 patients with idiopathic partial epilepsy (IPE) who subsequently developed epilepsy with electrical status epilepticus during slow sleep (ESES) spectrum disorders.Methods: Epilepsy, cognitive and behavioral parameters, and waking and non-rapid eye movement (NREM) EEG data were evaluated and scored for initial stage (i.e., IPE stage), preESES, ESES, and ESES remission periods, individually, on a chronologic basis. Data from 25 healthy subjects who had had IPE at the appropriate ages served for comparison with the patients' data during the IPE stage.Results: Results revealed a higher incidence in seizure frequency and variability in the ESES group and a resistance to a single antiepileptic drug (AED), as compared with controls, during the IPE stage. Mean age at onset of epilepsy was younger in the ESES group versus controls (5.5 and 7.3 years, respectively).At least one of the premonitory clinical features for development of ESES [an increase in the seizure frequency and/or addition of new types of seizures (93%), appearance of cognitive and/or behavioural changes (81.2%), or a progression in EEG abnormalities (66%)] was present in all patients. Epilepsy remitted in patients within the ESES spectrum at a similar age as in controls in 81.2%, as ESES findings in the EEG disappeared by age 13 years in 94%. Seizure prognosis proved to be the most favorable among the questioned parameters.Conclusions: An increase in seizure frequency or development of new seizure types, a deviance in behavior or decrease in cognitive performance, or a spreading tendency of the previously focal abnormalities in control EEGs may be premonitory features of a developing ESES and necessitate close follow-ups with sleep EEGs in children with IPEs.

Encephalopathy with Electrical Status Epilepticus in Slow Wave Sleep – a review with an emphasis on regional (perisylvian) aspects

“…Other reports have shown that patients with a previous diagnosis of idiopathic partial epilepsy account for about one-third of the Electrical Status ES-ES cases (Saltik et al, 2005;Kramer et al, 2009).…”

“…Similarly, Tassinari et al (2012) relies on the secondary bilateral synchrony mechanism which was assumed to work classically (Tükel and Jasper, 1952) by involving secondarily thalamic and thalamo-cortical circuitry. However, asymmetric, unilateral, or more focal/regional discharges were frequently reported (Bureau, 1995;Saltik et al, 2005;Van Hirtum-Das et al, 2006;Kramer et al, 2009;Liukkonen et al, 2010;Sánches Fernández et al, 2012). The sleep discharges in LKS were also found almost always asymmetric, with a dominant hemispheric and regional distribution congruently to the language disturbances.…”

“…The required amount of discharges in ESES and the methods of estimating it are different; "a significant proportion" of the non-REM sleep is defined from 25% to 85% (Beaumanoir, 1995;Boel and Casaer, 1989;Bureau, 1995;Dalla Bernardina et al, 1978;Hirsch et al, 1990;Inutsuka et al, 2006;Laurette and Arfel, 1976;Loddenkemper et al, 2009;Patry et al, 1971;Saltik et al, 2005;Tassinari et al, 2000, Van Hirtum-Das et al, 2006Yan Liu and Wong, 2000;Yasuhara et al, 1991).…”

Landau Kleffner Syndrome and Misdiagnosis of Autism Spectrum Disorder: A Mini-Review

“…22 LKS may cause prolonged and severe loss of linguistic ability due to its impact on language areas. However, it became widely recognized when pediatric neurologists and psychiatrists realized the fact that there is a high percentage of the autism children might have a language and social deterioration in their past medical history.…”

Neurodevelopmental disorders in children with epilepsy

“…Saltik и соавт. [34] показали, что имеется ряд клини-ческих признаков, которые могут указывать на появление ESES при идиопатических фокальных эпилепсиях: увели-чение частоты приступов, появление новых типов присту-пов, развитие когнитивных и поведенческих нарушений, нарастание изменений на ЭЭГ. В группе наблюдавшихся ими 16 пациентов детского возраста расстройства поведе-ния и психические нарушения имели место у 81% (13 из 16) и включали тревогу, депрессию, отвлекаемость, гипер-активность, импульсивность, повышенную раздражи-тельность.…”