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Lamotrigine in two cases of Rett syndrome
S Kumandas1, H Caksen, A Ciftçi
1Department of Pediatric Neurology, Erciyes University Faculty of Medicine, 38039, Kayseri, Turkey. rektorl@erciyes.edu.tr
Insights
Lamotrigine effectively controlled seizures in two girls with Rett syndrome (RS). The treatment also reduced autistic behaviors and stereotyped hand movements, suggesting potential broader benefits for this neurodevelopmental disorder.
Area of Science:
- Neuroscience
- Pediatrics
- Genetics
Background:
- Rett syndrome (RS) is a rare neurodevelopmental disorder affecting girls, characterized by developmental regression after a period of normal early growth.
- Core features include loss of acquired motor and language skills, stereotyped hand movements, and autistic behaviors.
- Seizures are a common and challenging comorbidity in RS.
Observation:
- Two pediatric patients diagnosed with classic Rett syndrome presented with seizures, developmental regression, and autistic features.
- Lamotrigine was administered to manage their convulsive episodes.
- Following lamotrigine treatment, both patients experienced successful seizure control.
Findings:
- Lamotrigine therapy not only resolved seizures but also led to a marked reduction in stereotyped hand movements and autistic behaviors in the affected girls.
- This suggests a potential therapeutic effect of lamotrigine beyond seizure management in Rett syndrome.
Implications:
- Lamotrigine may offer a dual benefit in managing Rett syndrome, addressing both epilepsy and key behavioral symptoms.
- Further controlled clinical trials are warranted to validate these preliminary observations and establish lamotrigine's efficacy in treating the broader spectrum of RS symptoms.
Abstract:
Rett syndrome (RS) is a particular neurodevelopmental disorder in which symptoms develop in early infancy and manifests particular symptoms at certain ages. Two girls aged 4.5 and 2.5-years-old were admitted with complaints of seizures, regression of language, psychomotor development, and autistic behavior. They had fulfilled the criteria of classic RS. Lamotrigine was used in the treatment of the convulsions. After lamotrigine therapy, the convulsions were successfully controlled and the stereotyped hand movements and autistic behaviors markedly decreased. We would like to stress that, aside from convulsion, abnormal signs of the syndrome such as stereotyped hand movements and autistic behaviors might also be improved with lamotrigine, but detailed and controlled studies should be performed to identify whether lamotrigine has any positive effects in RS.