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SLEEP-RELATED HYPERMOTOR (HYPERKINETIC) EPILEPSY (SHE)

  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
 
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
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

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