FURTHER DELINEATION OF NEVO SYNDROME

Nevo syndrome is an autosomal recessive syndrome characterised by pren atal overgrowth, joint laxity, kyphosis, wrist drop, spindle shaped fi ngers, and volar oedema. Four children from two families have been rep orted previously. We report two further children from two unrelated Ar ab families from two different tribes. Both presented at birth with hy potonia, joint laxity, kyphosis, wrist drop, spindle shaped fingers, a nd volar oedema. Both have delayed motor development at the ages of 2 years 10 months and 3 months respectively. Cognitive development is no rmal in one, and the other case appears to be developing normally at 3 months of age. One has, in addition, a wide spinal canal on MRI of th e spine indicating some degree of dural ectasia. This report brings th e total number of children reported with this syndrome to six from fou r families; three of these families are Arab. This indicates that the gene for this syndrome is probably commoner in Arabs than in other pop ulations.