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EPILEPSY WITH MYOCLONIC ATONIC SEIZURES (EMAtS)

Background

The background is normal at onset. Biparietal monomorphic theta rhythms may be seen. With increased seizure frequency, generalized slowing may be seen.

eeg
Example of bi-parietal theta.

CAUTION Focal slowing consistently over one area right arrow consider structural brain abnormality.

Interictal

2-6Hz generalized spike- and polyspike-wave is seen.

CAUTION Generalized paroxysmal fast (≥10Hz) activity in sleep right arrow consider Lennox-Gastaut syndrome.

Activation

Hyperventilation may elicit generalized spike-wave and absence seizures (if present clinically), photosensitivity is rare.

EEG abnormality is enhanced by sleep deprivation, in drowsiness and in sleep. Generalized spike-wave often becomes fragmented with sleep deprivation or in sleep. Fragmented generalized spike-wave can appear focal or multi-focal but usually is not consistently seen in one area. The morphology of the focal spike-wave typically appears similar to the generalized spike-wave.

CAUTION If photoparoxysmal response at low flash frequency right arrow consider CLN2-disease.

Ictal

The myoclonic component of a myoclonic-atonic seizure is associated with a generalized spike or polyspike. The atonic component is associated with the aftergoing high voltage slow wave. During non convulsive status, the EEG shows long runs of high amplitude 2-3Hz irregular generalized spike-wave with background slowing.

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