Further EEG observations in children with the Rett syndrome

In four cases of the Rett syndrome (RS) (age 3 1/2, 6, 11 and 12 years), paroxysmal activity and especially spike discharges over the central region could be blocked or attenuated by passive finger movements. This response, however, is not demonstrable in the majority of children with RS. The blocking or attenuating effect of passive movements is compared with earlier observations of central spike discharges occurring in children with benign Rolandic epilepsy. In some of these children, central spikes could be blocked by active hand movements. It was assumed that such a positive blocking response underscores the "functional" ("dysfunctional") character of the Rolandic spikes and the lack of local structural damage. In children with cerebral palsy, seizures and central spike (and evidence of structural brain damage), such a blocking response was not obtainable. Taking into consideration these earlier observations, there is reason to presume that the central spike activity found in RS is based on dysfunction rather than on structural impairment.