Table 1

Familial temporal lobe epilepsy (TLE) subtypes

Syndrome	ADPEAF^7–¹¹	Familial mesial (FM) TLE syndromes*			TLE in broader partial epilepsy syndromes syndrome
Syndrome	ADPEAF^7–¹¹	FMTLE no HS no FS^16–²⁰	FMTLE often HS+/−FS^22,²³	FMTLE with FS usually no HS^27,²⁸	FPEVF^32,³³	PEPS³⁴
*These divisions are preliminary and they are probably overlapping mesial TLE syndromes. †Range of onset ages reflects the majority of reported cases. Some “outliers” may begin earlier or later, but most family members fall within the stated ranges.
ADPEAF, autosomal dominant partial epilepsy with auditory features; HS, hippocampal sclerosis; FS, febrile seizures; FPEVF, familial partial epilepsy with variable foci; PEPS, partial epilepsy with pericentral spikes.
Typical age of onset†	8 years to 4th decade	10 years to 4th decade	1 year to 3rd decade	1 year to 2nd decade	1 year to 4th decade	2 years to 2nd decade
Characteristic features	Auditory, sensory aura	Psychic, autonomic aura			Seizures with different focal origin in family members	Multiple seizure types in the same individual
EEG	Rare temporal discharges	Rare temporal discharges	Frequent temporal discharges	Occasional temporal discharges	Occasional temporal discharges	Frequent peri-central spikes
MRI	Normal	Normal	HS	Normal	Normal	Normal
Outcome	Generally benign	Generally benign	Often refractory	Variable	Variable	Generally benign
Linkage	10q		–	18qt,1q (?)	22q,2q (?)	4p
Genes	LGI1	–	–	–	–	–