There are two distinct forms of CEOP:23,24
The early-onset type is characterized by ictal vomiting and deviation of the eyes, often with impairment of consciousness and progression to generalized tonic-clonic seizures. The seizures are infrequent and often solitary, but in about one-third of the children the episodes evolve into partial status epilepticus. Two-thirds of the seizures occur during sleep.
The late-onset type consists of brief seizures with mainly visual symptoms such as elementary visual hallucinations, illusions, or amaurosis, followed by hemiclonic convulsions. Postictal migraine headaches occur in half of the patients.
The interictal EEG in both conditions is characterized by normal background activity and well-defined occipital discharges. The occipital spikes are typically high in voltage (200 to 300 mV) and diphasic, with a main negative peak followed by a relatively small positive peak and a negative slow wave. The discharges may be unilateral or bilateral and are increased during non-rapid eye movement (REM) sleep. An important feature in this syndrome is the prompt disappearance of the occipital discharges with eye opening and their reappearance 1 to 20 seconds after eye closure.
The prognosis in the early-onset type is excellent. The symptoms typically resolve with several years of onset.
The prognosis in the late-onset form varies. Although most patients have a benign course, seizure control may be difficult in some. Seizures may continue into adulthood, but many children outgrow them.
For another perspective on this disorder, see Benign occipital epilepsy of childhood with occipital paroxysms.
Reviewed and revised January 2004 by Gregory L. Holmes, MD, Dartmouth Medical School
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