[A case of Aicardi syndrome: neurophysiological investigation]

A Japanese girl developed tonic spasms in series at two months after birth. When she was admitted at 4 months, she had generalized muscle hypotonia and could not control her head. She had atotal agenesis of the corpus callosum on MRI, reticulo-lacunitis revealed by ophthalmologic examination, and hypsarrhythmia in EEG. Brain stem auditory evoked potentials and flash-visual evoked potentials exhibited abnormally asymmetrical findings. The cortical components of somatosensory evoked potentials were not elicited on admission, but some components became to be recognized on median nerve stimulation in accordance with improvement of EEG findings after various therapeutic trials. We concluded that these neurophysiological examinations were useful to investigate the abnormal lesions of the central nervous system in patients with Aicardi syndrome.