The early and follow-up EEG characteristics of Rasmussen’s syndrome are reported in an 11-year-old girl studied at Ospedale Civile, Mantova, Italy. Delta activity localized to the left temporal region persisted in the EEG three days following an initial 15 min partial seizure characterized by staring, right arm parasthesias, and speech impairment. Neurologic exam and MRI were normal. SPECT scan showed left temporal hypoperfusion. Recurrent partial focal clonic seizures responded to steroid therapy but relapsed when treatment was discontinued after 2 months. Epilepsia partialis continua developed, and the EEG showed a spike focus complicating the continuous slow activity in the left rolandic region. A repeat MRI showed mild rolandic cortical atrophy. After 2 years, the patient was aphasic, hemiplegic, and mentally deteriorated, and seizures were refractory to antiepileptic drugs, corticosteroids, and alpha globulins. Plasmapheresis was of little benefit. [1]
COMMENT. The EEG may help in the early diagnosis of Rasmussen’s syndrome in a child with partial seizures complicated by speech impairment and normal MRI. Focal delta activity without epileptiform spikes may preceed the onset of epilepsia partialis continua by several months.