Case study shows the use of ILF Neurofeedback in patients with Dravet syndrome

This case study investigates how an intervention with ILF-neurofeedback training (ILF-NFT) can affect the symptoms of an eight-year-old patient with Dravet syndrome (DS), a rare and severely debilitating form of epilepsy.

Schmidt, C.; Laugesen, H. (2023): Infra-low frequency training in Dravet Syndrome: a case study. Epilepsy and Behaviour reports. Science Direct; 1-10.

You can read the full study here.

Background

Dravet syndrome (DS) is a rare and severe form of epilepsy that occurs in one in 40,000 epilepsy patients (Dhamija et al. 2014). Dravet syndrome is caused by the mutation of a gene responsible for the coding of sodium channels in the central nervous system. This genetic mutation is associated with severe neurological impairments in several areas of the brain (Wheless et al. 2020).  Particularly challenging for DS patients are the frequent epileptic seizures, which are largely resistant to treatment. Sleep disorders are also a significant challenge in DS. In addition, DS is characterized by a high degree of heterogeneity in the EEG. According to the international consensus on the diagnosis and treatment of DS, treatment options are limited. (Bureau/ Bernardina 2011). As a result, seizure remission can only rarely be achieved, which is why persistent developmental disorders and sleep problems are likely (Wheless et al. 2020).

Clinically, the core symptoms in particular are treated as part of a DS disorder. However, despite the numerous pharmacological treatment options, there is still no sufficiently effective treatment for DS patients to reduce neurodevelopmental disorders, sleep disorders and epileptic seizures (Moore 2022; Lyons et al. 2020).

Case Study

This case study presents an 8-year-old DS patient who presented with febrile seizures at the age of 5 months with increasing seizure frequency, up to 3 times per week and a seizure duration of up to 30 minutes. In the course of her illness, the epileptic seizures were increasingly triggered not only by fever, but also by low temperatures, physical activity and sensory stimulation. Until the start of ILF neurofeedback therapy, the patient had increasing sleep problems and was awake for two to four hours a night five nights a week.

During a neuropsychological examination in a Danish epilepsy hospital, the girl's mental development was assessed as delayed by around one year. The girl had tried various pharmacological therapies, which had already caused initial damage to her liver function. The use of benzodiazepines also increased the frequency of lethargic moods.
 

Methods

ILF neurofeedback training was started in December 2019. Since then, the patient has trained almost daily for approx. 30 minutes per session. The NeuroAmp II and Cygnet software are used for this.

Results

The results of the case study consist primarily of the parents' observations, including their records of seizures in the digital app, which they share with the Danish Epilepsy Hospital. After the first three training sessions, there was a clear improvement in sleep, and the waking phases during the night also became significantly shorter. With regard to the epileptic seizures, it was found that they could be reduced both in frequency and intensity. In general, the girl has developed positively since starting ILF-NFT. Her cognitive and motor skills are also developing well, even if they are not age-appropriate.

Resources

Dhamija, R.; Erickson, MK.; St. Louis, EK.; Wirrell, E.; Kotagal, S. Sleep abnormalities in children with Dravet syndrome, Pediatr. Neurol 2014; 50(5): 474-478.
Wheless, JW.; Fulton, SP.; Mudigoudar, BD. Dravet syndrome: a review of current management. Pediatr Neurol 2020; 107: 28-40.
Bureau, M.; Bernardina, BD. Electroencophalographic characteristics of Dravet syndrome. Epilepsia 2011; 52: 13-23.
Moore, PT. Infra-low frequency neurofeedback and insomnia as a model of CNS dysregulation. Front Hum Neurosci 2022; 514. 
Lyons, L.; Schoeler, NE.; Langan, D.; Cross, JH. Use of ketogenic diet therapy in infants with epilepsy: a systematic review meta-analysis. Epilepsia 2020; 61(6): 1261-1281.