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Myoclonic Astatic Epilepsy

Doose Syndrome

Epilepsy with Myoclonic Astatic Seizures

What is Myoclonic astatic epilepsy? It is a generalized idiopathic epilepsy. Generalized means that it affects both hemispheres of the brain. Idiopathic means the reason for the seizures is unknown and is most likely hereditary. It is characterized by the development of myoclonic seizures and/or myoclonic astatic seizures.

Types of seizures experienced

Tonic clonic A seizure often referred to as grand mal seizures. These seizures begin with stiffening of the extremities followed by jerking of the extremities and face.

Myoclonic A seizure with rapid, brief contractions of muscles, usually occurring at the same time on both sides of the body.

Myoclonic cluster Same as an individual Myoclonic seizure but happens repetitively fairly close together.

Atonic A seizure with a sudden loss of muscle tone, often resulting in a sudden collapse. These seizures are also known as drop seizures.

Absence A seizure with a brief lapse of awareness and or staring spell.

Myoclonic astatic A seizure that involves a myoclonic seizure followed immediately by an atonic seizure. This type of seizure is exclusive to MAE and is one of the defining characteristics of this syndrome.

Tonic Muscle stiffening or rigidity. This seizure is rare in this syndrome.

Initial onset

The onset of seizures is between the ages of 2 and 5. EEG shows regular and irregular bilaterally synchronous 2- to 3-Hz spike-waves and polyspike patterns with a 4- to 7-Hz background. 84% of affected children show normal development; the remainder show moderate psychomotor retardation mainly affecting speech. Boys (74%) are more often affected than girls (Doose and Baier 1987a).

Prognosis

Epilepsy with myoclonic-astatic seizures has a variable course and outcome. Spontaneous remission with normal development has been observed in a few untreated cases. Complete seizure control can be achieved in about half of the cases with antiepileptic drug treatment (Doose and Baier 1987b; Dulac et al 1990). In the remainder of cases, the level of intelligence deteriorates and the children become severely retarded. Other neurologic abnormalities such as ataxia, poor motor function, dysarthria, and poor language development may emerge (Doose 1992b). However, this proportion may not be representative because in this series the data were collected in an institution for children with severe epilepsy.

The outcome is unfavorable if generalized tonic-clonic, tonic, or clonic seizures appear at the onset or occur frequently during the course. Generalized tonic-clonic seizures usually occur during the daytime in this disorder, at least in the early stages. Nocturnal generalized tonic-clonic seizures, which may develop later, are another unfavorable sign. If tonic seizures appear, prognosis is poor.

Status epilepticus with myoclonic, astatic, myoclonic-astatic, or absence seizures is another ominous sign, especially when prolonged or appearing early.

Failure to suppress the EEG abnormalities (4- to 7-Hz rhythms and spike-wave discharges) during therapy and absence of occipital alpha-rhythm with therapy also suggest a poor prognosis (Doose 1992a).

Helpful websites

http://www.epilepsy.com
http://www.ILAE.org
http://www.epilepsymoms.com
http://.epilepsyfoundation.org/
http://www.facebook.com/pages/Myoclonic-Astatic-Epilepsy/190872344263079?v=wall