What You Need To Know About Dravet Syndrome

Epilepsy is the 4th most common neurological disorder in the United States. With children, around 400,000 have epilepsy and most are able to control their seizures and lead normal lives. Dravet Syndrome is a rare form, of epilepsy found in children. Symptoms include, developmental delays, sleeping conditions, and chronic infections. Here are 20 facts you shoud know about Davet Syndrome.

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  • Charlotte Dravet first described severe myoclonic epilepsy of infancy in France, 1978.
  • The name was later changed in 1989.
  • It is also called Severe Myoclonic Epilepsy of Infancy (SMEI)
  • It is a rare genetic disorder affecting 1 in every 20,000-40,000 children
  • It appears during the first year of life.
  • Developmental disabilities and abnormal EEG’s are often not evident until the second of third year of life.
  • Seizures are often fever-related.
  • It is rare to develop beyond the age of 5.
  • Children often experience poor development of language and motor skills
  • Is caused by defects in a gene in 79% of cases.
  • Affects 2-5% of children in North America
  • It is induced by prolonged exposure to warm weather
  • It is associated with sleep disorder including insomnia
  • Seizures are frequently resistant to treatment
  • There is a higher risk of Sudden Unexplained Death in Epilepsy (SUDEP) (15-20%)
  • The onset of signs and symptoms is around¬†6 months old
  • Children diagnosed with Dravet Syndrome have a 85% chance of survival.
  • Development is typically normal.
  • Episodes may be frequent and prolonged
  • Certain seizure medications are likely to worsen the seizures such as Dilantin, Cerebyx and Tegretol.

Resources

Dravet Syndrome Foundation
P.O. Box 16536
West Haven, CT
Tel. 203-392-1950

Dravet Syndrome UK
P.O. Box 756
Chesterfield
5439EB
Tel. 07874 866937

Epilepsy Foundation- Provides online resources on information about epilepsy and seizures, locating assistance, advocacy and public awareness.

ICE Epilepsy Alliance– Provides resources on refractory patients (anti-epileptic drug therapy which is ineffective). Site includes information on advocacy, childhood epilepsy, and Dravet syndrome.

NINDS Dravet Syndrome Information Page– Medical information page on Dravet syndrome, diagnosis, treatment, research and organizations.

WebMD– Medical website providing information on diagnosis, signs and symptoms, and treatment.

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Childhood Disintegrative Disorder Resources

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Childhood disintegrative disorder (CDD), also known as Heller’s Syndrome¬†is a developmental disorder that is characterized by aggression of previously acquired skills such as language and social abilities. Symptoms include:

  • Lack of play
  • Loss of motor skills
  • Loss of social skills
  • Delay in language

The following resources provide an overview of Childhood disintegrative disorder including prevalence, symptoms, diagnosis, and treatment.

Encyclopedia of Mental Disorders
Mayo Clinic
Medline Plus
WebMD
Wikipedia

Books

The Blessing of Autism: one family’s journey through childhood disintegrative disorder

When Autism Strikes: Families Cope with Childhood Disintegrative Disorder