scholarly journals Natural History of Absence Epilepsy in Children

Author(s):  
Elaine C. Wirrell

Absence seizures may be seen in a variety of epileptic syndromes in childhood. Identification of the specific syndrome is important to determine medical prognosis. With childhood absence epilepsy, approximately two thirds of children can be expected to enter long-term remission, while in juvenile absence epilepsy, seizure control is often achieved, however, lifelong treatment is usually required. Other absence syndromes have a poorer prognosis, with lower rates of seizure control and remission. Psychosocial outcome is often poor, even in patients with more benign forms of absence epilepsy. Remission of epilepsy does not preclude psychosocial morbidity.

Author(s):  
Anita N. Datta ◽  
Laura Wallbank ◽  
Peter K. H. Wong

AbstractObjectiveBenign epilepsy of childhood with central temporal spikes (BECTS) and absence epilepsy are common epilepsy syndromes in children with similar age of onset and favorable prognosis. However, the co-existence of the electrocardiogram (EEG) findings of rolandic spike and 3 Hz generalized spike-wave (GSW) discharges is extremely rare, with few cases reported in the literature. Our objective was to characterize the EEG findings of these syndromes in children in our center and review the electro-clinical features.MethodsAll EEGs at BC Children’s Hospital are entered in a database, which include EEG findings and clinical data. Patients with both centro-temporal spikes and 3 Hz GSW discharges were identified from the database and clinical data were reviewed.ResultsAmong the 43,061 patients in the database from 1992 to 2017, 1426 with isolated rolandic discharges and 528 patients with isolated 3 Hz GSW discharges were identified, and 20 (0.05%) patients had both findings: 3/20 had BECTS, and subsequently developed childhood absence epilepsy and 17/20 had no seizures characteristic for BECTS. At follow-up, 17 (85%) were seizure-free, 1 (5%) had rare, and 2 (10%) had frequent seizures.ConclusionsThis is the largest reported group of patients to our knowledge with the co-existence of rolandic and 3 Hz GSW discharges on EEGs in one institution, not drug-induced. As the presence of both findings is extremely rare, distinct pathophysiological mechanisms are likely. The majority had excellent seizure control at follow-up, similar to what would be expected for each type of epilepsy alone.


Neurology ◽  
1996 ◽  
Vol 47 (4) ◽  
pp. 912-918 ◽  
Author(s):  
E. C. Wirrell ◽  
C. S. Camfield ◽  
P. R. Camfield ◽  
K. E. Gordon ◽  
J. M. Dooley

Author(s):  
Friederike Moeller ◽  
Ronit M. Pressler ◽  
J. Helen Cross

This chapter provides an overview of generalized epilepsies (GGE), which comprises a group of epilepsy syndromes of presumed genetic origin. They are classified into several syndromes according to their age, depending on clinical manifestation and associated electroencephalogram (EEG) features. The chapter introduces the concept of GGE before addressing different GGE syndromes, describing their clinical presentation, EEG features, treatment, prognosis, and underlying genetics. The following GGE syndromes are discussed in order of their age of onset—myoclonic astatic epilepsy, childhood absence epilepsy, epilepsy with myoclonic absences, eyelid myoclonia with absences, juvenile absence epilepsy, juvenile myoclonic epilepsy, and epilepsy with generalized tonic seizures on awakening. This is followed by an overview on pathophysiological mechanisms underlying GGE.


2012 ◽  
Vol 7 (1) ◽  
pp. 62
Author(s):  
Sylvain Rheims ◽  
Philippe Ryvlin ◽  
◽  

Childhood absence epilepsy (CAE) is one of the most common forms of paediatric epilepsy. However, there is still a gap between the prevalence of CAE in paediatric epilepsies and the paucity of available data regarding its therapeutic management. Only nine randomised controlled trials have been published in the field over the past four decades, with many suffering from major methodological limitations. A recent large randomised double-blind controlled trial reported that ethosuximide and sodium valproate are the most effective anti-epileptic dugs in CAE and that cognitive performance appears to be better with ethosuximide than with sodium valproate. Although lamotrigine also demonstrated anti-absence properties in the same trial, it proved to be significantly less efficacious than ethosuximide or sodium valproate. Despite these recent advances, several questions, including long-term outcomes, management of refractory CAE and treatment duration, remain unanswered and further studies are required to refine therapeutic decisions.


Neurology ◽  
1997 ◽  
Vol 49 (4) ◽  
pp. 1187-1187 ◽  
Author(s):  
E. Wirrell ◽  
C. Camfield ◽  
P. Camfield ◽  
K. Gordon ◽  
J. Dooley

2009 ◽  
Vol 83 (2-3) ◽  
pp. 249-256 ◽  
Author(s):  
Petra M.C. Callenbach ◽  
Paul A.D. Bouma ◽  
Ada T. Geerts ◽  
Willem Frans M. Arts ◽  
Hans Stroink ◽  
...  

2019 ◽  
Vol 34 (4) ◽  
pp. 224-228
Author(s):  
C. Martínez-Ferrández ◽  
E. Martínez-Salcedo ◽  
C. Casas-Fernández ◽  
H. Alarcón-Martínez ◽  
S. Ibáñez-Micó ◽  
...  

Neurosurgery ◽  
2012 ◽  
Vol 70 (6) ◽  
pp. 1406-1414 ◽  
Author(s):  
Derek G. Southwell ◽  
Paul A. Garcia ◽  
Mitchel S. Berger ◽  
Nicholas M. Barbaro ◽  
Edward F. Chang

Abstract BACKGROUND: Gangliogliomas are rare glioneuronal tumors that typically cause refractory seizures during the first 3 decades of life. OBJECTIVE: To determine the prognosticators of seizure outcome after surgery for ganglioglioma. METHODS: We reviewed the cases of 66 patients who underwent resection of gangliogliomas at the University of California, San Francisco. Demographic, seizure history, and operative data were examined for statistical association with postoperative seizure outcomes. RESULTS: Of the 66 patients who underwent surgical resection of ganglioglioma, 49 patients (74%) presented with a history of seizures. Of those 49 patients, 50% presented with intractable epilepsy. Temporal lobe gangliogliomas were present in 76% of the patients who presented with a history of seizures. Electrocorticography was performed on 35% of the patients, and of those patients, 82% underwent extended lesionectomy to remove abnormally epileptogenic extralesional tissue. The median follow-up duration was 6.9 years, during which tumor progression occurred in 38% of patients who underwent subtotal resection and in 8% of patients who underwent gross total resection (P = .02). Overall, 85% of patients were seizure free (International League Against Epilepsy class I or II) 5 years after surgery. Subtotal resection was associated with poor seizure outcomes 1 year after resection (odds ratio = 14.6; 95% confidence interval = 2.4-87.7): rates of seizure freedom were 54% after subtotal resection, 96% after gross total resection, and 93% after gross total resection with intraoperative electrocorticography-guided extended lesionectomy. CONCLUSION: We report excellent long-term seizure control outcomes after surgery for gangliogliomas. Intraoperative electrocorticography may be a useful adjunct for guiding extended resection in certain pharmacoresistant epilepsy patients with gangliogliomas. Subtotal resection is associated with higher rates of tumor progression and nonoptimal seizure outcomes.


2012 ◽  
Vol 7 (4) ◽  
pp. 234
Author(s):  
Sylvain Rheims ◽  
Philippe Ryvlin ◽  
◽  

Childhood absence epilepsy (CAE) is one of the most common forms of paediatric epilepsy. However, there is still a gap between the prevalence of CAE in paediatric epilepsies and the paucity of available data regarding its therapeutic management. Only nine randomised controlled trials have been published in the field over the past four decades, with many suffering from major methodological limitations. A recent large randomised double-blind controlled trial reported that ethosuximide and sodium valproate are the most effective anti-epileptic dugs in CAE and that cognitive performance appears to be better with ethosuximide than with sodium valproate. Although lamotrigine also demonstrated anti-absence properties in the same trial, it proved to be significantly less efficacious than ethosuximide or sodium valproate. Despite these recent advances, several questions, including long-term outcomes, management of refractory CAE and treatment duration, remain unanswered and further studies are required to refine therapeutic decisions.


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