We tried to investigate the incidence and the clinical profile of intractab
le epilepsy with hippocampal atrophy and ictal onset zones located in areas
other than the hippocampus (extra-medial-temporal epilepsy; EMTE). We incl
uded patients who had hippocampal atrophy confirmed by MRI but with extra-m
edial-temporal ictal onset zones as verified by invasive intracranial elect
rodes or video-EEG monitoring. The case histories, interictal EEG, ictal se
miology, other MRI findings in addition to hippocampal atrophy, and results
of ictal SPECT and PET scans were evaluated. Results were compared with th
ose of surgically proven medial temporal lobe epilepsy with hippocampal atr
ophy recruited during the same period. 8.5% of the intractable epilepsy pat
ients with hippocampal atrophy had extra-medial temporal epileptogenic zone
s. A history of encephalitis and hemiconvulsion-hemiparesis were significan
tly common in the EMTE group. Most of the interictal EEGs of EMTE patients
showed extratemporal irritative zones. MRI, ictal SPECT, and FDG-PET seemed
to be helpful at localizing the true epileptogenic zones. The predominant
EMTE seizure type was focal motor seizure with secondary generalization. So
me portion of intractable epilepsy patients with hippocampal atrophy had ex
tra-medial-temporal epileptogenic fool and careful analysis of semiology an
d neuroimagings could yield clues to correct diagnosis.