Mandatory | Alerts | Exclusionary | |
---|---|---|---|
Seizures | Brief focal motor seizures with hyperkinetic or asymmetric tonic/dystonic features occurring predominantly from sleep | Seizures predominantly from the awake state | Seizures only during wakefulness Generalized onset seizures |
EEG | Frequent epileptiform abnormality
outside frontal regions Generalized epileptiform abnormality |
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Age at onset | <10 or >20 years | <2 months or >64 years | |
Development at onset | Moderate or greater intellectual impairment | ||
Neurological exam | Abnormal examination | ||
Imaging | |||
Are MRI or ictal EEG required for diagnosis? An MRI is not required for diagnosis but should be done to evaluate for underlying cause An ictal EEG is not required for diagnosis |
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Syndrome without laboratory confirmation: in resource-limited regions, this syndrome can be diagnosed if other mandatory and exclusionary criteria are met, and the patient has witnessed or video-recorded hyperkinetic seizures during sleep |
NOTE Alert criteria are absent in the vast majority of patients with the syndrome, but rarely can be seen. Their presence should result in caution in diagnosing the syndrome and consideration of other conditions