Fukuyama type congenital muscular dystrophy with central-temporal EEG foci (rolandic spikes)

Central-temporal EEG foci (rolandic spikes) in three patients who satisfied all the diagnostic criteria for Fukuyama type congenital muscular dystrophy (FCMD) are reported. Clinically one case remained silent and two cases were accompanied by epileptic seizures, characterized as follows: 1) partial seizures with elementary motor symptomatology; 2) brief and rare seizures, but occasionally with frequent or prolonged convulsions; 3) seizures were easily induced by defined precipitating factors such as fever; 4) seizures could be quickly controlled by routine antiepileptic treatment. Electroencephalographically, the appearance of repetitive stereotyped diphasic spikes over central and mid-temporal areas were quite similar to those described in rolandic epilepsy. We believe that the EEG findings in our cases corroborate the postulation that the prognosis is as good in these severely brain-damaged children as in normal children in terms of recovery from the epilepsy.