Two children with Rasmussen’s syndrome complicated by asymptomatic uveitis are reported from the Royal Children’s Hospital, Melbourne, Australia, and Montreal Neurological Institute, Montreal, Canada. A 10-year-old girl developed somatosensorimotor seizures affected her right leg and left sided acute uveitis. Epilepsia partialis continua, with onset 9 months later, was associated with right hemiparesis and cortical sensory loss, and dysphasia. EEG showed epileptic discharges in the left central region. CT revealed atrophy of the left hemisphere. Left frontal corticectomy failed to control seizures. The cortex showed chronic encephalitis. The second patient, a 5-year-old girl, had complex partial status epilepticus which responded to IV gammaglobulin, left facial weakness, and right sided uveitis. Right anterior temporal lobectomy revealed leptomeningeal inflammation. Seizures became intractable, and a dense left hemiparesis developed. She died 2 years 7 months after the onset of seizures. [1]
COMMENT. A viral cause seemed likely but was not confirmed by serology or tissue culture. Slit-lamp examination of the eye should be included in the evaluation of children with Rasmussen’s syndrome.