2024/25 : In Paris, research on the immunity of children with Norse Fires syndrome
Norse Fires syndrome is a severe form of epilepsy in children for which neither the cause nor the treatments are currently known. Every year, it brutally strikes children who were previously perfectly healthy. They then fall into an epileptic coma lasting several weeks or months. A third of children never wake up, the others suffer serious irreversible damage to their brain functions.
This is a medical emergency requiring intensive care. The mortality rate is 12% on average among children.
The mechanisms that trigger this syndrome remain unknown but research is progressing to propose new therapeutic strategies. Because only research can develop treatments to reduce the neurological after-effects suffered by children during the acute phase of the disease.
The Paratonnerre Association has already launched 2 programs : one at the Imagine/Necker Institute with the teams of Professor Rima Nabbout, and the other at the ICM Institut du Cerveau with the teams of Professor Vincent Navarro.
These programs were initiated and co-financed by private funds : Ipsen, Bouygues, Banque Postale, Tikéhau.
Paratonnerre is also studying a new pathway: intracerebral cholesterol metabolism. It has been demonstrated that status epilepticus is accompanied, in humans and animals, by excess synthesis of cholesterol, promoting its accumulation in the brain. The accumulation of cholesterol in neurons has been shown to be toxic, responsible for neuronal death and the emergence of epileptic seizures.
These results prompted the use of simvastatin, a safe drug approved in France in 2004 for the treatment of hypercholesterolemia. Daily treatment with simvastatin was observed to reduce the consequences of status epilepticus in an animal model.
We now wish to study its neuroprotective effect in humans.
La Fondation La Petite Etoile is participating in this project, the aim of which is to acquire better knowledge of status epilepticus in order to :
- prevent the appearance of brain damage
- study the impact of treatment on children (autonomy, cognitive after-effects) and on the development of sequelae epilepsy.
Places of intervention : Pitié-Salpêtrière Hospital (epilepsy unit), Bichat Hospital, Sainte Anne Hospital, Rothschild Foundation.
Total budget : € 276,000
Co-participants : Fondation La Petite Etoile, Institut du Cerveau, Doctor Navarro Laboratory, Paratonnerre Association
Budget provided by Fondation La Petite Etoile : € 5,000
Norse Fires syndrome is a severe form of epilepsy in children for which neither the cause nor the treatments are currently known. Every year, it brutally strikes children who were previously perfectly healthy. They then fall into an epileptic coma lasting several weeks or months. A third of children never wake up, the others suffer serious irreversible damage to their brain functions.
This is a medical emergency requiring intensive care. The mortality rate is 12% on average among children.
The mechanisms that trigger this syndrome remain unknown but research is progressing to propose new therapeutic strategies. Because only research can develop treatments to reduce the neurological after-effects suffered by children during the acute phase of the disease.
The Paratonnerre Association has already launched 2 programs : one at the Imagine/Necker Institute with the teams of Professor Rima Nabbout, and the other at the ICM Institut du Cerveau with the teams of Professor Vincent Navarro.
These programs were initiated and co-financed by private funds : Ipsen, Bouygues, Banque Postale, Tikéhau.
Paratonnerre is also studying a new pathway: intracerebral cholesterol metabolism. It has been demonstrated that status epilepticus is accompanied, in humans and animals, by excess synthesis of cholesterol, promoting its accumulation in the brain. The accumulation of cholesterol in neurons has been shown to be toxic, responsible for neuronal death and the emergence of epileptic seizures.
These results prompted the use of simvastatin, a safe drug approved in France in 2004 for the treatment of hypercholesterolemia. Daily treatment with simvastatin was observed to reduce the consequences of status epilepticus in an animal model.
We now wish to study its neuroprotective effect in humans.
La Fondation La Petite Etoile is participating in this project, the aim of which is to acquire better knowledge of status epilepticus in order to :
- prevent the appearance of brain damage
- study the impact of treatment on children (autonomy, cognitive after-effects) and on the development of sequelae epilepsy.
Places of intervention : Pitié-Salpêtrière Hospital (epilepsy unit), Bichat Hospital, Sainte Anne Hospital, Rothschild Foundation.
Total budget : € 276,000
Co-participants : Fondation La Petite Etoile, Institut du Cerveau, Doctor Navarro Laboratory, Paratonnerre Association
Budget provided by Fondation La Petite Etoile : € 5,000