Characteristics and Management of Children with Continuous Spikes and Waves during Slow Sleep
K Fatema1, M M Rahman, S Begum
1Dr Kanij Fatema, Assistant Professor, Pediatric Neurology Unit, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh.
Insights
Continuous spike and slow wave in sleep (CSWS) in children presents with diverse symptoms and often drug-resistant epilepsy. Midazolam drip showed significant efficacy in treating CSWS, offering a promising therapeutic option.
Area of Science:
- Pediatric Neurology
- Epileptology
- Clinical Neurophysiology
Background:
- Continuous spike and slow wave in sleep (CSWS) is an epilepsy syndrome characterized by continuous spike-and-wave discharges during sleep.
- CSWS often presents with cognitive and behavioral regression, posing significant management challenges in children.
- Previous treatment strategies have shown variable efficacy, necessitating exploration of alternative therapeutic options.
Purpose of the Study:
- To delineate the clinical spectrum and EEG characteristics of CSWS in a pediatric cohort.
- To evaluate the effectiveness of various treatment modalities for CSWS.
- To identify potentially efficacious treatments, particularly in cases resistant to conventional antiepileptic drugs (AEDs).
Main Methods:
- Retrospective analysis of ten pediatric patients diagnosed with CSWS treated between 2012 and 2013.
- Detailed review of clinical presentations, including seizures, regression, and behavioral changes.
- Assessment of EEG findings, specifically the percentage of sleep with spike-wave index (SWI), and evaluation of treatment responses to different AEDs and therapies.
Main Results:
- The study included 10 patients with a mean age of 6.9 years, predominantly with epilepsy as the underlying etiology.
- Presenting features included abnormal behavior, agitation, aggression, and regression (speech/motor).
- Eighty percent of patients responded positively to midazolam drip, while pulse methylprednisolone showed partial response in one case and failure in two. Valproate was also found to be efficacious.
Conclusions:
- Midazolam drip demonstrated significant efficacy in treating pediatric CSWS, even in cases resistant to multiple oral AEDs.
- This study highlights midazolam drip as a potentially valuable therapeutic option for CSWS, contrasting with findings from previous studies emphasizing other treatments.
- Further research is warranted to confirm these findings and establish optimal treatment protocols for CSWS.
Abstract:
This study was done to describe the clinical spectrum, EEG characteristics and treatment modalities in children with continuous spike and slow wave in sleep (CSWS). Ten patients with CSWS had been treated between 2012 and 2013. Mean age of the patients was 6.9 years; male female ratio was 3:2. The main etiologic group in this study was epilepsy (10), cerebral palsy (3) and brain lesion (arachnoid cyst). All the patients had prior seizure. Presenting features were abnormal behavior (4), agitation (4), aggression (4), eye blinking (2) and involuntary movement (2). Three patients had speech regression and 1 had motor regression. Regarding EEG finding, 7 out 10 cases had SWI>85% whereas, rest of them had SWI 50 to 80%. Most of the patients were resistant to two or more oral AED. The AED found to be efficacious were Midazolam drip, pulse methyl prednisolone and valproate. Eighty percent (80%) patient responded to midazolam drip. Methyl prednisolone caused 50% improvement in one patient but failed in 2 cases. In contrast to the previous studies where high dose valproic acid, levetiracetam, Injection ACTH was more efficacious, this study demonstrates significant positive result with midazolam drip.
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