Abnormal auditory cortex with giant N100m signal in patients with autosomal dominant lateral temporal lobe epilepsy.

OBJECTIVE

Lateralization of functionally abnormal cortical area in autosomal dominant lateral temporal lobe epilepsy (ADLTE).

METHODS

A sound pulse of pure tone was delivered monaurally to the ears alternately. Auditory evoked magnetic fields (AEF) were measured by using whole-head magnetoencephalography (MEG) system.

RESULTS

Significantly large N100m signals (a magnetic counterpart of N1/N100 in EEG) were detected in three out of five patients, either in the left or in the right hemisphere, contralateral to the auditory stimulation. The peak latency, location and orientation of distinct N100m exhibited no clear difference from those of normal controls.

CONCLUSIONS

Unilateral cortical abnormality exists in some of the patients in ADLTE. Patients with abnormally large N100m had seizures apparently provoked by auditory stimuli, suggesting that the appearance of significantly large N100m is associated with the epileptogenicity. Based on the detailed examination using MRI and FDG-PET for two of the patients, the authors hypothesize hyperexcitability caused by the decreased inhibitory functions, larger number of synchronously activated neurons, or the elongation of neuronal firing in the pathological temporal cortex in ADLTE.

SIGNIFICANCE

The present study revealed clear abnormalities in the auditory cortex that have not been well detected by conventional EEG in patients with ADLTE.