Familial spastic paraplegia is described in four Cook Island children. Prior to the onset of the disease at age six or seven years, the children were apparently normal. Disease was characterised principally by progressive spastic paraplegia of the lower extremities, in addition to tremor and slow articulation. The disease in these children was probably due to the expression of an autosomal recessive gene. The disorder is of interest because the findings in these children appear to overlap the pure spastic and ataxic heredofamilial syndromes.
Adolescent Child