Doose syndrome , also known as myoclonic - astatic epilepsy , is a rare descriptor of epilepsy that typically begins in former childhood .
Doose syndrome usually depart causing symptoms in childrenbetween the ages of 2 and 6 . It involves a variety of seizure types , most notably myoclonic ( musculus jerks ) and astatic raptus , which take a sudden loss of muscle tone leading to a downfall . A German baby doctor call Dr. Hermann Doose first describe it in the 1970s .
Doose syndrome answer for forapproximately 1 % to 2%of all childhood epilepsies . It has a male predominance , occurring more oftentimes in boy than girls .
Symptoms of Doose syndrome
The symptom of Doose syndromeinclude :
What causes Doose syndrome?
scientist consider that Doose syndromemay havea genetic basis , though the precise cause is unknown . expert consider that it may relate to a mutation in genes involved in brain function . In some cases , a family history of epilepsy or other neurologic disorders may be present .
Dr. regard the condition idiopathic , meaning it arises spontaneously or from an unsung or unidentified cause .
How is Doose syndrome diagnosed?
diagnose Doose syndrome involves a combination of clinical valuation , seizure history , and diagnostic run .
Doctors typically start by take a elaborated history of the youngster ’s seizure , include the age of onset , types of seizures , relative frequency , duration , and any form . They will also critique the kid ’s developmental history , include any delays or retroversion in motor skills , manner of speaking , or cognitive ( thinking ) purpose .
Diagnostic run may involve :
How is Doose syndrome treated?
The treatment of Doose syndrome normally demand antiepileptic drug ( AEDs ) , also known asanticonvulsants . normally used AEDsinclude :
Other treatment alternative include :
Complications of Doose syndrome
knottiness of Doose syndrome can vary widely , affecting cognitive and developmental progress , behavior , emotional well - being , strong-arm safety , and overall character of life .
They can include :
Children with Doose syndrome areat riskofstatus epilepticus . This is a potentially life peril condition where seizures last for an extended period ( unremarkably more than 5 min ) or occur in speedy taking over without recovery in between . This is a aesculapian emergency brake that requires immediate intervention .
When to contact a doctor
People need to contact a doctor immediately if a child experiences a gaining control for the first time . This initial installment requires prompt evaluation to settle the cause and appropriate action .
Parents or caregivers should tell the child ’s doctor if there is an step-up in the relative frequency or rigorousness of raptus in a child with Doose syndrome . A rise in seizure activity may point that the current discussion is not manipulate the condition the right way , and alteration may be necessary .
People should alsocontact a doctorin cases of :
Epilepsy resources
Visit our dedicated hub for more research - backed information and in - deepness resource onepilepsy and ictus .
The outlook for Doose syndrome
The outlook for children with Doose syndrome varies . Some childrenmay respond wellto treatment and experience few or no seizures , while others may carry on to have frequent seizures despite treatment .
Developmental outcomes can also vary , with some child maintaining distinctive cognitive social occasion . However , others may experience developmental time lag or regression , particularly if seizures are not well controlled .
Frequently asked questions
At what age do atonic seizures start?
unaccented seizures , which involve sudden loss of heftiness tone , typically start in early puerility , most commonlybetween the geezerhood of 2 and 6 years .
What are the warning signs of atonic seizures?
unaccented seizures often fall out without warn , but some childrenmay experiencea brief champion of weakness or a sudden drop of the head or consistency before the seizure . However , in many cases , the capture occurs suddenly with no noticeable word of advice .
Do you lose consciousness in atonic seizure?
Yes , during an atonic capture , the persontypicallyloses consciousness , although the loss may be very brief . This can run to sudden tumble , known as fall attack .
Summary
Doose syndrome is a rare form of epilepsy that start out during puerility . The precise cause is unknown , although a genetic sensitivity belike plays a character . Due to its rarity and variable presentment , Doose syndrome can be challenging to diagnose and cover , while the mentality also widely varies .
