K. Silver et al., FAMILIAL ALTERNATING EPILEPSIA PARTIALIS CONTINUA WITH CHRONIC ENCEPHALITIS - ANOTHER VARIANT OF RASMUSSEN-SYNDROME, Archives of neurology, 55(5), 1998, pp. 733-736
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.