EEG abnormalities preceding the epilepsy onset in tuberous sclerosis complex patients – A prospective study of 5 patients

Domańska-Pakieła, Dorota; Kaczorowska, Magdalena; Jurkiewicz, Elżbieta; Kotulska, Katarzyna; Dunin-Wąsowicz, Dorota; Jóźwiak, Sergiusz

doi:10.1016/j.ejpn.2013.12.006

Cited by 58 publications

(65 citation statements)

References 29 publications

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“…Furthermore, similar to results of the Domańska-Pakieła et al study 16 , classic or modified hypsarrhythmia was not found in any infant prior to the onset of focal seizures or epileptic spasms. This would suggest that classic or modified hypsarrhythmia, reported to occur in up to 71% of TSC patients with TSC and clinical epileptic spasms 17 , occurs after seizure onset and corresponds to later events in the epileptogenesis process.…”

Section: Discussionsupporting

confidence: 87%

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Clinical Electroencephalographic Biomarker for Impending Epilepsy in Asymptomatic Tuberous Sclerosis Complex Infants

Peters

Goyal

et al. 2016

Pediatric Neurology

107

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PURPOSE To assess the clinical utility of routine electroencephalograms (EEGs) in the prediction of epilepsy onset in asymptomatic infants with Tuberous Sclerosis Complex (TSC) PROCEDURES This multicenter prospective observational study recruited infants less than 7 months of age, seizure-free on no antiepileptic drugs at enrollment, who all underwent serial physical examination and video-EEGs throughout the study. Parental education on seizure recognition was completed at initial enrollment. Once seizure onset occurred, standard of care was applied, and subjects were followed until 24 months. FINDINGS 40 patients were enrolled, 28 over age 12 months with completed EEG evaluation at the time of this interim analysis. Of those, 19 (67.8%) developed seizures. Epileptic spasms occurred in 10 (52.6%), focal seizures occurred in 5 (26.3%), generalized tonic-clonic seizure in 1 (5.3%), and a combination of epileptic spasms and focal seizures in 3 (15.7%). Fourteen infants (73.6%) had the first emergence of epileptiform abnormalities on EEG at an average age of 4.2 months, preceding seizure onset by a median of 1.9 months. Hypsarrhythmia or modified hypsarrhythmia was not found in any infant prior to onset of epileptic spasms. All children with epileptiform discharges subsequently developed epilepsy (100% positive predictive value), and the negative predictive value for not developing epilepsy after a normal EEG was 64%. CONCLUSIONS Serial routine EEGs in TSC infants is a feasible strategy to identify those at high risk for epilepsy. The most frequent clinical presentation was epileptic spasms followed by focal seizures, and then a combination of both seizure types.

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Section: Discussionsupporting

confidence: 87%

“…Only one prior published study has evaluated EEG findings in TSC infants prior to the onset of seizures, consisting of 5 subjects 16 . Patients were enrolled from 9 days to 9 weeks of age and had serial EEGs at 4 week intervals.…”

Section: Discussionmentioning

confidence: 99%

Clinical Electroencephalographic Biomarker for Impending Epilepsy in Asymptomatic Tuberous Sclerosis Complex Infants

Peters

Goyal

et al. 2016

Pediatric Neurology

107

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“…New methods to improve the outcome of epilepsy in TSC are needed. Recent insights suggest that close EEG monitoring of children newly diagnosed with TSC can pick up epileptic discharges before the onset of clinical seizures . Treatment of children with electrographic epilepsy with AEDs before the onset of clinical seizures may result in better seizure control and better cognitive development .…”

Section: Discussionmentioning

confidence: 99%

Epilepsy in children with tuberous sclerosis complex: Chance of remission and response to antiepileptic drugs

et al. 2015

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Iris E. Overwater is a doctoral student in pediatric neurology at the ENCORE TSC Expertise Center. SUMMARYObjective: To describe treatment and outcome of epilepsy in children with tuberous sclerosis complex (TSC). Methods: Seventy-one children with TSC and epilepsy treated at the ENCORE TSC Expertise Center between 1988 and 2014 were included. Patient characteristics and duration and effectiveness of antiepileptic treatments were extracted from our clinical database. Correlations were made between recurrence of seizures after response to treatment, and several patient characteristics. Results: Median age at time of inclusion was 9.4 years (range 0.9-18.0). Seizure history showed that 55 children (77%) of 71 became seizure-free for longer than 1 month, and 21 (30%) of 71 for longer than 24 months. Remission of seizures was associated with higher IQ, and a trend was observed between seizure remission and age at onset of seizures. A total of 19 antiepileptic drugs (AEDs) were used. Valproic acid, vigabatrin, levetiracetam, and carbamazepine were used most frequently. Nonpharmacologic therapies (ketogenic diet, epilepsy surgery, and vagus nerve stimulation) were used 13 times. Epilepsy surgery was most effective, with four of five children becoming seizurefree. AEDs prescribed as first and second treatment were most effective. Valproic acid was prescribed most frequently as first and second treatment, followed by vigabatrin. Thirty-one children had infantile spasms, preceded by focal seizures in 18 children (58%). Vigabatrin was used by 29 children (94%), and was first treatment in 15 (48%). Vigabatrin was more effective than other AEDs when prescribed as first treatment. Significance: We showed that, although 77% of children with epilepsy due to TSC reached seizure remission, usually after their first or second AED, this was sustained for at least 24 months in only 38%. Almost half of those with 24 months of remission later had relapse of seizures. Our results support vigabatrin as first choice drug, and show the need for better treatment options for these children.

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“…In case of drug resistance, candidacy for epilepsy surgery should be evaluated at specialized centers, as both seizure freedom and favorable developmental outcomes can be achieved in well-selected patients [23,24]. According to recent studies, AED treatment already started at the onset of epileptiform discharges in the EEG and prior to the occurrence of clinically overt seizures seems to be more efficient in preventing developmental deficits [25].…”

Section: School Age To Adolescencementioning

confidence: 99%

Tuberous Sclerosis Complex: new criteria for diagnostic work-up and management

Samueli¹,

Abraham

Dressler

et al. 2015

Wien Klin Wochenschr

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Tuberous sclerosis complex (TSC) is a rare genetic multisystem disorder, characterized by predominantly benign tumors in potentially all organ systems. System involvement, severity of clinical symptoms and the response to treatment are age-dependent and heterogeneous. Consequently, the disorder is still not recognized in a considerable number of patients. The diagnostic criteria and the guidelines for surveillance and management of patients with TSC were revised, and the establishment of specialized TSC-centers was strongly recommended during an International Consensus Conference in 2012. TOSCA (TuberOus SClerosis registry to increase disease Awareness), an international patient registry, was started to allow new insights into the causes of different courses. Finally, there are-since the approval of the mTOR inhibitor Everolimus-promising new therapeutic approaches.This review focuses on the various TSC related symptoms occurring at different ages, the novel recommendations for diagnosis and treatment as well as the need for multidisciplinary follow-up.

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EEG abnormalities preceding the epilepsy onset in tuberous sclerosis complex patients – A prospective study of 5 patients

Cited by 58 publications

References 29 publications

Clinical Electroencephalographic Biomarker for Impending Epilepsy in Asymptomatic Tuberous Sclerosis Complex Infants

Clinical Electroencephalographic Biomarker for Impending Epilepsy in Asymptomatic Tuberous Sclerosis Complex Infants

Epilepsy in children with tuberous sclerosis complex: Chance of remission and response to antiepileptic drugs

Tuberous Sclerosis Complex: new criteria for diagnostic work-up and management

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