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FAMILIAL FOCAL EPILEPSY WITH VARIABLE FOCI (FFEVF)

  Mandatory Alerts Exclusionary
Seizures Focal seizures   Generalized seizures
EEG   Generalized epileptiform abnormality  
Age at onset   Neonatal onset  
Development at onset     Moderate or greater intellectual impairment
Neurological exam   Abnormal exam  
Imaging Normal or focal cortical dysplasia    
Other Family history of individuals with focal seizures arising from different cortical regions in different family members   Family history of focal seizures exclusively seen <20 months of age
Are MRI or ictal EEG required for diagnosis?
An MRI is required for diagnosis to exclude other causes
An ictal EEG is not required for diagnosis
Syndrome without laboratory confirmation: in resource-limited regions, this syndrome can be diagnosed without EEG in a patient if other mandatory and exclusionary criteria are met. However, an MRI or CT is required to exclude other structural causes

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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