Vagus nerve stimulation for treatment of epilepsy in Rett syndrome
Wilfong AA, Schultz RJ.
Dev Med Child Neurol. 2006 Aug;48(8):683-6.
https://doi.org/10.1017/S0012162206001435. PMID: 16836782.
Rett syndrome (#RTT) is a rare genetic disorder that primarily affects girls, usually appearing between 6 and 18 months of age. The genetic mutations responsible for Rett syndrome occur randomly, often resulting from a mutation on the X chromosome's #MECP2 gene. Male individuals with this genetic mutation experience severe consequences, with the majority dying before birth or in early infancy. #Epilepsy is a frequent clinical characteristic in RTT, occurring in 50-90% of the patients.
In 2006, Wilfong and Schultz conducted a retrospective study on seven females with RTT and drug-resistant epilepsy (#DRE) who had failed to achieve seizure freedom after at least two anti-epileptic drugs (AEDs). These patients were implanted with a vagus nerve stimulator (#VNS) and monitored for a minimum of one year. The group included five females with classic RTT (MECP2+) and two females with atypical RTT (MECP2-), with a median age of 9 years at the time of VNS implantation.
Median seizure frequency before VNS implantation was 150 per month, ranging from 12 seizures a month to several seizures per hour. The ≥50% responder rate was 71% at 3 months follow-up and 86% at 12 months follow-up. Further, all patients reported increased alertness at 12 months follow-up.
At one-year follow-up, all patients had VNS generator settings programmed to an output current of 1.50 mA, frequency of 30 Hz and pulse width of 500 µsec, with most patients having a 35% duty cycle (30s ON, 1.1 min OFF), and none of the patients remaining at the 10% factory setting.
Link to publication:
https://lnkd.in/g82aNX4k
