Myoclonic astatic epilepsy (MAE), also known as Doose syndrome, is
an epilepsy syndrome of early childhood
, most commonly appearing between ages 1 and 5 and featuring generalized seizures. Children will experience drop attacks and staring seizures, sometimes associated with falls.
What is a Doose?
Doose Syndrome, also known as Epilepsy with Myoclonic Atonic Seizures (EMAS), is
an epilepsy syndrome of childhood that is often resistant to medication
. Previously also known as Myoclonic Astatic Epilepsy (MAE), the syndrome is usually characterised by generalised seizures, which may vary in type and frequency.
Does Doose syndrome go away?
The long-term outcome for children with Doose syndrome is highly variable. The
spectrum ranges from complete remission and normal intellectual development
to therapy-resistant epilepsy which can result in mild to severe developmental delay.
What is the life expectancy of a person with Dravet syndrome?
Dravet syndrome is a rare, severe, and lifelong form of epilepsy (seizure disorder). Most people affected by this condition have
a good life expectancy
. The disease typically starts in the first year of life, and around 80-85% of the children survive into adulthood.
Is Doose Syndrome genetic?
Doose Syndrome (MAE) is
a presumably genetic epilepsy syndrome with a heterogeneous cause
. Disease-causing variants are present in 14% of children and are typically identified in children with additional neurodevelopmental features such as developmental delay or autism.
Is myoclonus a seizure?
Myoclonic epilepsy
causes the muscles in the body to contract
. This type of seizure causes quick jerking movements. Myoclonic seizures often happen in everyday life. This includes hiccups and a sudden jerk while falling asleep.
What is Rasmussen syndrome?
Rasmussen syndrome appears to be
an immune-mediated response that causes one hemisphere of the brain to become inflamed and deteriorate
. Inflammation may stop without treatment, but the damage is irreversible.
How common is Doose Syndrome?
It is an uncommon childhood epilepsy syndrome that accounts for
1 to 2 out of 100
(1 to 2%) of all childhood-onset epilepsies. Usually the first seizure occurs between 2 and 6 years of age. Boys tend to be affected more than girls (two thirds to three quarters of children with MAE will be boys).
What is the meaning of dozed off?
:
to fall asleep especially for a short period of time
A few students dozed off during the movie.
What is myoclonic atonic epilepsy?
Epilepsy with myoclonic-atonic seizures is
a rare epilepsy syndrome of early childhood
. It is characterized by seizures of many different types, most often myoclonic–atonic, astatic, or generalized tonic-clonic seizures. Seizures can be followed by drop attacks, which can lead to falls and injuries.
What is the rarest type of epilepsy?
- Dravet syndrome is a rare, drug-resistant epilepsy that begins in the first year of life in an otherwise healthy infant. …
- Most cases are due to severe SCN1A gene mutations.
- Most children develop varying degrees of developmental disability.
What is the most severe form of epilepsy?
Lennox-Gastaut syndrome (LGS)
is a severe form of epilepsy that typically becomes apparent during infancy or early childhood. Affected children experience several different types of seizures most commonly atonic, tonic and atypical absence seizures.
Who treats Dravet syndrome?
Children with Dravet syndrome are best treated by
specialist epilepsy doctors
, and often require up to three different types of anti-epileptic medication to control their symptoms.
What triggers myoclonic seizures?
The most common triggers are lack of sleep and too much stress.
Drinking alcohol
, which can lead to too little sleep and fatigue, is the strongest trigger of myoclonic jerks and tonic-clonic seizures. Flickering lights can also trigger seizures for some people.
What type of seizure is status epilepticus?
A seizure that lasts longer than 5 minutes, or having more than 1 seizure within a 5 minutes period
, without returning to a normal level of consciousness between episodes is called status epilepticus. This is a medical emergency that may lead to permanent brain damage or death.
Can myoclonic seizures cause brain damage?
It can be the most disabling form of myoclonus affecting the arms, legs, and face. One of the causes may be brain damage that results from
a lack of oxygen and blood flow to the brain
, or it can be secondary to other medical or neurological conditions.
