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[Atypical evolutions of benign partial epilepsy of infancy with centro-temporal spikes]
N Fejerman1, R Caraballo, S N Tenembaum
1Servicio de Neurología, Hospital de Pediatría Dr. J.P. Garrahan, Buenos Aires, Argentina. nfejerman@iname.com
Insights
Benign childhood epilepsy with centro-temporal spikes (BCECTS) can sometimes evolve into more severe conditions, including Landau-Kleffner syndrome. Identifying predictive clinical and EEG markers is crucial for managing these atypical epilepsy cases.
Area of Science:
- Pediatric Neurology
- Epileptology
- Neurodevelopmental Disorders
Background:
- Benign partial epilepsies of childhood (BPEC) are typically characterized by specific clinical and electroencephalographic (EEG) features.
- However, a subset of patients may exhibit atypical evolutions with significant neurological sequelae.
Purpose of the Study:
- To highlight the occurrence of diverse atypical evolutions in children initially diagnosed with benign childhood epilepsy with centro-temporal spikes (BCECTS).
- To encourage the identification of clinical and EEG indicators that may predict these unfavorable outcomes.
Main Methods:
- A longitudinal follow-up study of 26 pediatric patients initially presenting with typical BCECTS features.
- Data collection included repeated neurological examinations, EEG recordings, neuropsychological evaluations, and brain imaging (CT/MRI) over a period of up to 14 years.
Main Results:
- Four patient groups emerged: atypical benign partial epilepsy of childhood (ABPEC), Landau-Kleffner syndrome (LKS), status epilepticus, and continuous spike-wave during slow sleep (CSWS).
- While most patients recovered, some experienced learning difficulties, persistent language impairments (LKS), or required intensive management for status epilepticus or CSWS.
Conclusions:
- A small fraction of children with initial BCECTS diagnosis can develop ABPEC, LKS, status epilepticus, or CSWS, indicating that BCECTS is not invariably benign.
- Further research is needed to establish reliable clinical and EEG markers for predicting these atypical, potentially severe, epilepsy evolutions.
Objectives:
To stress that different atypical evolutions occur in the course of disease in some patients with the so called benign partial epilepsies of childhood (BPEC). To promote interest in finding clinical and/or electroencephalographic clues to recognize which patients might be prone to present these risks.
Methods:
Follow up of 26 pediatric patients who started with typical clinical and EEG features of benign childhood epilepsy with centro-temporal spikes (BCECTS) but presented reversible or persistent serious epileptic events including status epilepticus and language, cognitive or behavioral impairments. Repeated neurological examinations, EEG records and neuropsychological evaluations were done in the course of up to 14 years. Brain imaging studies (CAT and/or MRI) were obtained in all patients.
Results:
Four groups of patients were recognized: 11 children with atypical benign partial epilepsy of childhood (ABPEC) followed during 4 to 13 years. All have finally recovered and attend normal schools, although five have learning difficulties. Three patients met diagnostic criteria for Landau-Kleffner syndrome (LKS). Two of them recovered from aphasia and in one, some language difficulties persist. Seven children showed status epilepticus of BCECTS but all of them are now normal after 3 to 14 years of follow-up. Five children showed mixed features of the three groups mentioned above and three of them fulfilled the criteria for diagnosis of the syndrome of continuous spike-wave during slow sleep (CSWS).
Conclusions:
A small proportion of cases starting with BCECTS evolve into either ABPEC, LKS, status of BCECTS or the syndrome of CSWS. Then, BCECTS is not always benign. Clinical and EEG markers should be sought to predict these atypical evolutions.