AUTOSOMAL RECESSIVE PARAPARESIS WITH AMYOTROPHY OF HANDS AND FEET ANDWHITE-MATTER LESIONS

We report two siblings with a hitherto undescribed syndrome of autosom al recessive spastic paraparesis accompanied by amyotrophy of hands an d feet, and mental deterioration. Laboratory tests showed signs of low er motoneuron involvement in the four limbs, more accentuated in the d istal regions. Brain MR showed bilateral symmetrical white matter lesi ons. We discuss the nosological status of this syndrome in relation to other similar forms of ''complicated'' spastic paraparesis.