Two Peruvian brothers, ages 16 months and 5 years, with alternating epilepsia partialis continua are reported from the Montreal Neurological Hospital, McGill University, Canada, and Loyola University, Chicago. Seizures began at 6 months in one child and at 4 months in the other, both associated with a febrile illness. Severe psychomotor regression and cerebral atrophy developed rapidly. A brain biopsy in one child revealed chronic encephalitis with changes compatible with Rasmussen syndrome. This familial disorder may represent a variant of the classically sporadic and unilateral Rasmussen syndrome. [1]
COMMENT. A familial syndrome is described involving two brothers with symptoms and pathology resembling Rasmussen syndrome but with unusual characteristics of bilateral seizure and paresis involvement, early age of onset, and rapid deterioration. A history of parental consanguinity suggests an autosomal recessive inheritance.