title: Continuous, generalized, high-voltage fast activity and FIRDA in two children.
abstract: The EEG pattern of continuous, generalized, high-voltage fast rhythms without any reaction to eye-opening/closure, photic stimulation, or the sleep-awaking cycle was previously reported to be characteristic of infantile neuroaxonal dystrophy (INAD). However, we have observed such fast activity in one child with INAD and one with Menkes' kinky-hair syndrome. They both exhibited severe psychomotor disturbance, and their EEGs also included "frontal intermittent rhythmic delta activity (FIRDA)," a nonspecific EEG finding suggestive of organic encephalopathy. Since the continuous, generalized, high-voltage fast activity had features suggestive of spindles in both children, this EEG pattern is thought to actually represent "extreme spindles," and nonspecifically to indicate widespread organic brain damage.
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Kyoto University, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Mutoh
authors.forename: T
authors.initials: T
authors.lastname: Okuno
authors.forename: M
authors.initials: M
authors.lastname: Ito
authors.forename: T
authors.initials: T
authors.lastname: Fujii
authors.forename: H
authors.initials: H
authors.lastname: Mikawa
mesh_terms: D002540:Cerebral Cortex / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D003700:Delta Rhythm; D004569:Electroencephalography; D004829:Epilepsy, Generalized / Q000175:diagnosis / Q000503:physiopathology*; D005071:Evoked Potentials / Q000502:physiology; D005625:Frontal Lobe / Q000503:physiopathology*; D006801:Humans; D008297:Male; D007706:Menkes Kinky Hair Syndrome / Q000175:diagnosis / Q000503:physiopathology*; D006211:Pantothenate Kinase-Associated Neurodegeneration / Q000175:diagnosis / Q000503:physiopathology*; D010775:Photic Stimulation