Myoclonic-Astatic Epilepsy in Early Childhood (MAE)

    (Doose Syndrome)

 
 

 

 

  

  

 

Seizure types (generalised seizures)

An internationally accepted system of classification separates seizures into two groups - "generalised" and "partial" (also called focal). The seizures associated with MAE are generalised. Generalised seizures affect the whole brain instantly, not just one part, and they alter consciousness. There is no warning.

Seizure types seen in MAE

Cases

Myoclonic and/or myoclonic-astatic

100%

Absences

62%

Febrile convulsions (with fever)

28%

Generalised tonic-clonic

75%

 - at onset

34%

 - during course

41%

Non-convulsive status epilepticus

30%

 

 

 

Generalised seizures come in two sizes; large and small or convulsive and non-convulsive. Non-convulsive refers to alterations of consciousness without jerking movements. Convulsive means that there are muscle movements like jerking or stiffening.

MAE is a complex seizure disorder that presents as a mixed bag of the generalised seizure types. The myoclonic-astatic seizure (also called drop seizure or drop attack) is the core seizure type associated. All children with MAE will experience either myoclonic or myoclonic-astatic seizures - or both - but it is common for them to experience other generalised events including absence, atypical absence, myoclonic, tonic-clonic, episodes of non-convulsive status epilepticus (NCSE) and, in rare cases, tonic seizures.¨

Myoclonic seizures

Myoclonic-astatic seizures (atonic or drop attack)

Absence seizures (formerly petit mal)

Tonic-clonic seizures (formerly grand mal)

Non-convulsive status epilepticus (NCSE - formerly minor motor status)

Atypical absence seizures

Tonic seizures

Other terms

 

 

 
 
 
 
 
 
 
 
 
 
 
 

     

 In brief

 Why? and other FAQ

 Background

 The syndrome

 Seizure types

 Genetic link

 Onset

 Diagnosis

 Prognosis

 Recovery / remission

 Medical research

 

 

 

 
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