Doose syndrome (myoclonic-atonic epilepsy)
A childhood generalized epilepsy with 'drop' seizures — often strikingly responsive to the ketogenic diet, with a variable but frequently good outlook.
Doose syndrome — now usually called epilepsy with myoclonic-atonic seizures — is a generalized epilepsy that begins in early childhood, typically between 1 and 5 years, in a previously well-developing child. Its hallmark is myoclonic-atonic seizures: a brief jerk followed by a sudden loss of muscle tone causing a fall ('drop attack'). It is presumed genetic, brain imaging is normal, and the outlook varies widely — many children do well, sometimes after a difficult initial period, and the ketogenic diet is notably effective.
At a glance
- Also called
- Epilepsy with myoclonic-atonic seizures
- Onset
- 1–5 years, in a previously well child
- Hallmark
- Myoclonic-atonic 'drop' seizures
- Imaging
- Normal MRI; presumed genetic
- Notable
- Often responds well to the ketogenic diet
What it is
Doose syndrome is a genetic generalized epilepsy of early childhood. Its defining seizure is the myoclonic-atonic seizure — a quick jerk immediately followed by loss of tone, causing the child to drop — but children may also have generalized tonic–clonic, absence and myoclonic seizures. Development is usually normal before onset, and the MRI is normal.
Diagnosis
Diagnosis rests on the seizure pattern and EEG (generalized spike- and polyspike-wave), with normal imaging. Genetic testing is increasingly used, as some children have variants in genes such as SLC6A1 or SCN1A; this can also help distinguish Doose syndrome from conditions it can resemble, such as Lennox–Gastaut syndrome or Dravet syndrome.
Treatment
Broad-spectrum medicines are used — valproate, ethosuximide, lamotrigine and levetiracetam among them — and the ketogenic diet is particularly effective and often considered early. As in other generalized epilepsies, certain sodium-channel-blocking medicines (such as carbamazepine, oxcarbazepine and vigabatrin) can make seizures worse and are generally avoided.
Prognosis
The outlook is variable but often encouraging: many children eventually become seizure-free with normal or near-normal development, sometimes after a stormy first year or two. A minority have drug-resistant seizures and learning difficulties, which is why early, well-chosen treatment matters.
How an educational review can help
An educational review can clarify the diagnosis (and distinguish it from look-alike syndromes), explain EEG and genetic findings, and discuss where options such as the ketogenic diet fit — helping you prepare questions for your treating team. It is educational and does not replace your clinician's care.
Selected sources
- ILAE classification and reviews of epilepsy with myoclonic-atonic seizures (Doose syndrome).
- Evidence for the ketogenic diet in myoclonic-atonic epilepsy.
Last reviewed: 2026-05-22
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