Continuous, Generalized, High-Voltage Fast Activity and FIRDA in Two Children

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.