Dravet Syndrome Information Page

Dravet Syndrome Information Page


What research is being done?

The NINDS conducts and supports a broad program of basic and clinical research on all types of epilepsy, including Dravet syndrome.  Study of the genetic defects responsible for Dravet syndrome and related disorders is expected to lead to the development of effective drug therapies.

Information from the National Library of Medicine’s MedlinePlus
Epilepsy

×
What research is being done?

The NINDS conducts and supports a broad program of basic and clinical research on all types of epilepsy, including Dravet syndrome.  Study of the genetic defects responsible for Dravet syndrome and related disorders is expected to lead to the development of effective drug therapies.

Information from the National Library of Medicine’s MedlinePlus
Epilepsy

The NINDS conducts and supports a broad program of basic and clinical research on all types of epilepsy, including Dravet syndrome.  Study of the genetic defects responsible for Dravet syndrome and related disorders is expected to lead to the development of effective drug therapies.

Information from the National Library of Medicine’s MedlinePlus
Epilepsy

Search Disorders

Definition
Definition
Treatment
Treatment
Prognosis
Prognosis
Clinical Trials
Clinical Trials
Organizations
Organizations
Publications
Publications
Definition
Definition

Dravet syndrome, also called severe myoclonic epilepsy of infancy (SMEI), is a severe form of epilepsy.  It appears during the first year of life with frequent febrile seizures – fever-related seizures that, by definition, are rare beyond age 5.  Later, other types of seizures typically arise, including myoclonus (involuntary muscle spasms).  Status epilepticus – a state of continuous seizure requiring emergency medical care – also may occur.  Children with Dravet syndrome typically experience poor development of language and motor skills, hyperactivity, and difficulty relating to others. 

In 30 to 80 percent of cases, Dravet syndrome is caused by defects in a gene required for the proper function of brain cells.  Borderline SMEI (SMEB) and another type of infant-onset epilepsy called generalized epilepsy with febrile seizures plus (GEFS+) are caused by defects in the same gene.  In GEFS+, febrile seizures may persist beyond age 5.

×
Definition

Dravet syndrome, also called severe myoclonic epilepsy of infancy (SMEI), is a severe form of epilepsy.  It appears during the first year of life with frequent febrile seizures – fever-related seizures that, by definition, are rare beyond age 5.  Later, other types of seizures typically arise, including myoclonus (involuntary muscle spasms).  Status epilepticus – a state of continuous seizure requiring emergency medical care – also may occur.  Children with Dravet syndrome typically experience poor development of language and motor skills, hyperactivity, and difficulty relating to others. 

In 30 to 80 percent of cases, Dravet syndrome is caused by defects in a gene required for the proper function of brain cells.  Borderline SMEI (SMEB) and another type of infant-onset epilepsy called generalized epilepsy with febrile seizures plus (GEFS+) are caused by defects in the same gene.  In GEFS+, febrile seizures may persist beyond age 5.

Treatment
Treatment

Seizures in Dravet syndrome are difficult to control, but can be reduced by anticonvulsant drugs.  A ketogenic diet, high in fats and low in carbohydrates, also may be beneficial. 

×
Treatment

Seizures in Dravet syndrome are difficult to control, but can be reduced by anticonvulsant drugs.  A ketogenic diet, high in fats and low in carbohydrates, also may be beneficial. 

Definition
Definition

Dravet syndrome, also called severe myoclonic epilepsy of infancy (SMEI), is a severe form of epilepsy.  It appears during the first year of life with frequent febrile seizures – fever-related seizures that, by definition, are rare beyond age 5.  Later, other types of seizures typically arise, including myoclonus (involuntary muscle spasms).  Status epilepticus – a state of continuous seizure requiring emergency medical care – also may occur.  Children with Dravet syndrome typically experience poor development of language and motor skills, hyperactivity, and difficulty relating to others. 

In 30 to 80 percent of cases, Dravet syndrome is caused by defects in a gene required for the proper function of brain cells.  Borderline SMEI (SMEB) and another type of infant-onset epilepsy called generalized epilepsy with febrile seizures plus (GEFS+) are caused by defects in the same gene.  In GEFS+, febrile seizures may persist beyond age 5.

Treatment
Treatment

Seizures in Dravet syndrome are difficult to control, but can be reduced by anticonvulsant drugs.  A ketogenic diet, high in fats and low in carbohydrates, also may be beneficial. 

Prognosis
Prognosis

As children with Dravet syndrome get older, their decline in cognitive function stabilizes, and in many, it improves slightly.  However, most teenagers with Dravet syndrome are dependent on caregivers.  The degree of cognitive impairment appears to correlate with the frequency of seizures.

×

As children with Dravet syndrome get older, their decline in cognitive function stabilizes, and in many, it improves slightly.  However, most teenagers with Dravet syndrome are dependent on caregivers.  The degree of cognitive impairment appears to correlate with the frequency of seizures.

Prognosis
Prognosis

As children with Dravet syndrome get older, their decline in cognitive function stabilizes, and in many, it improves slightly.  However, most teenagers with Dravet syndrome are dependent on caregivers.  The degree of cognitive impairment appears to correlate with the frequency of seizures.

Definition

Dravet syndrome, also called severe myoclonic epilepsy of infancy (SMEI), is a severe form of epilepsy.  It appears during the first year of life with frequent febrile seizures – fever-related seizures that, by definition, are rare beyond age 5.  Later, other types of seizures typically arise, including myoclonus (involuntary muscle spasms).  Status epilepticus – a state of continuous seizure requiring emergency medical care – also may occur.  Children with Dravet syndrome typically experience poor development of language and motor skills, hyperactivity, and difficulty relating to others. 

In 30 to 80 percent of cases, Dravet syndrome is caused by defects in a gene required for the proper function of brain cells.  Borderline SMEI (SMEB) and another type of infant-onset epilepsy called generalized epilepsy with febrile seizures plus (GEFS+) are caused by defects in the same gene.  In GEFS+, febrile seizures may persist beyond age 5.

Treatment

Seizures in Dravet syndrome are difficult to control, but can be reduced by anticonvulsant drugs.  A ketogenic diet, high in fats and low in carbohydrates, also may be beneficial. 

Prognosis

As children with Dravet syndrome get older, their decline in cognitive function stabilizes, and in many, it improves slightly.  However, most teenagers with Dravet syndrome are dependent on caregivers.  The degree of cognitive impairment appears to correlate with the frequency of seizures.

What research is being done?

The NINDS conducts and supports a broad program of basic and clinical research on all types of epilepsy, including Dravet syndrome.  Study of the genetic defects responsible for Dravet syndrome and related disorders is expected to lead to the development of effective drug therapies.

Information from the National Library of Medicine’s MedlinePlus
Epilepsy

Patient Organizations
Dravet Syndrome Foundation
P.O. Box 16536
West Haven
CT
West Haven, CT 06516
Tel: 203-392-1950
Epilepsy Foundation
8301 Professional Place East, Suite 200
Landover
MD
Landover, MD 20785-2353
Tel: 301-459-3700; 800-EFA-1000 (332-1000)
Intractable Childhood Epilepsy Alliance
PO Box 365
6360 Shallowford Road
Lewisville
NC
Lewisville, NC 27023
Tel: 336-946-1570
Patient Organizations