FAMILIAL FOCAL EPILEPSY WITH VARIABLE FOCI (FFEVF)

Overview
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Seizure Classification
Generalized onset seizure
- Motor Onset
- Non-Motor Onset
Focal Onset Seizure
- By Onset Feature
- Focal to Bilateral Tonic-Clonic
- Hemispheric lateralization
- Lobar localization
  - Frontal
  - Temporal
  - Parietal
  - Occipital
Unknown Onset Seizure
Neonatal Seizure
Epilepsy Classification
Generalized Epilepsy
Focal Epilepsy
Generalized and Focal Epilepsy
Unknown Epilepsy
Epilepsy Syndromes
Neonate/Infant
- Self-limited
- With developmental and epileptic encephalopathy
Childhood
- Self-limited focal epilepsy syndromes
  - Remission expected in all cases by adolescence
    - Self-limited epilepsy with centrotemporal spikes
    - Self-limited epilepsy with autonomic seizures
  - Remission expected in most cases by adolescence
    - Childhood occipital visual epilepsy
    - Photosensitive occipital lobe epilepsy
- Genetic generalised epilepsy syndromes
  - Epilepsy with eyelid myoclonia
  - Epilepsy with myoclonic absence
- Idiopathic generalised epilepsy syndrome
  - Childhood absence epilepsy
- With developmental and/or epileptic encephalopathy
Variable Age
- Idiopathic generalised epilepsy syndromes
- Focal epilepsy syndromes
- Combined generalised and focal epilepsy syndrome
  - Epilepsy with reading-induced seizures
- With developmental and/or epileptic encephalopathy or progressive neurological deterioration
  - Progressive myoclonus epilepsies
  - Rasmussen syndrome
Epilepsy Etiologies
Genetic Etiologies
- Chromosomal abnormalities
- Gene abnormalities
Structural Etiologies
- Malformations of cortical development
- Vascular malformations
- Hippocampal sclerosis
- Hypoxic-Ischemic
- Traumatic brain injury
- Tumors
- Porencephalic Cyst
Metabolic Etiologies
Immune Etiologies
Infectious Etiologies
Unknown Etiologies
Encephalopathy
Epilepsy imitators

FAMILIAL FOCAL EPILEPSY WITH VARIABLE FOCI (FFEVF)

Overview

Familial focal epilepsy with variable foci (FFEVF, previously known as familial partial epilepsy with variable foci) is a familial focal epilepsy syndrome characterized by focal seizures arising from different focal regions (most commonly frontal or temporal) in different members in a family, and with variable severity, but with each individual in a family having a single focal seizure type. It has both genetic and structural causes.

Clinical context

Age of focal seizure onset is highly variable (from infancy to adulthood, peak age is 12-13.5 years). Both males and females are affected. Antecedent and birth history are unremarkable. Head size and neurological examination are normal. Development and cognition are typically normal, although mild intellectual impairment, neuropsychiatric disorders (including autism) and behaviour disorders have been reported.