Hyperventilation induced abnormalities in the electroencephalogram of children with Moyamoya disease

Two cases of Moyamoya disease in children are presented. The EEG of each child demonstrated minimal abnormalities at rest. However, during 3 min of hyperventilation, the record developed rhythmic delta activity which persisted in excess of 5 min after termination of over breathing. This activity did not respond to glucose administration and was maximal in the areas correlating with the clinical symptoms. It appears to represent a physiologic correlate of an abnormal vascular response to hypocapnia. Although admittedly a non-specific response, the persistence of delta activity easily provoked by hyperventilation, the topographic correspondence to the ultimately demonstrated vascular anomaly, and the lack of response to glucose ingestion represent a combination of factors which, in the appropriate clinical setting, are distinctive and of diagnostic value. Currently, invasive arteriography is required for definitive diagnosis of Moyamoya. Improved selection of children for invasive studies may be accomplished by EEG screening.