FAMILIAL ALTERNATING EPILEPSIA PARTIALIS CONTINUA WITH CHRONIC ENCEPHALITIS - ANOTHER VARIANT OF RASMUSSEN-SYNDROME

Two brothers had infantile epilepsia partialis continua alternately in volving both sides of the body. The children rapidly developed severe psychomotor regression and cerebral atrophy. A brain biopsy specimen s howed cadence of chronic inflammatory changes. Extensive investigation did not provide evidence of a specific viral pathogenesis, mitochondr ial disorder, or any identifiable neurodegenerative genetically determ ined disorder. This illness has the features of Rasmussen chronic ence phalitis, in which bilateral involvement is quite unusual. Although fe w patients with bilateral hemispheral involvement have been described, to our knowledge there have been no reported cases involving affected siblings. The familial disorder described herein may represent yet an other variant of the classically sporadic and unilateral childhood for m. This group of disorders is probably immunologically determined.