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Neurophysiologic evaluations provide information concerning the developmental aspects of Rett syndrome and the character and extent of involvement of the central, peripheral, and autonomic nervous system pathways. Evoked potentials typically demonstrate intactness of peripheral auditory and visual pathways and suggest dysfunction of central or \u201chigher\u201d cortical pathways. Somatosensory evoked potentials can be characterized by \u201cgiant\u201d responses, suggesting cortical hyperexcitability. Cortical hyperexcitability is further suggested by the findings of the electroencephalogram (EEG), which are primarily characterized by a loss of expected developmental features; the appearance of focal, multifocal, and generalized epileptiform abnormalities; and the occurrence of rhythmic slow (theta) activity, primarily in the frontal-central regions. Epileptic seizures are reported to occur frequently in Rett syndrome. However, many events presumed to be seizures have no EEG correlate during video-EEG monitoring. Impairment of the autonomic nervous system in Rett syndrome is suggested by an increased incidence of long Q\u2014T intervals during electrocardiographic recordings and diminished heart rate variability. Autonomic nervous system dysfunction can contribute to the increased incidence of sudden unexpected death in Rett syndrome. 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