PURPOSE:

Neuropsychological profiles of four patients with subcortical band heterotopia (SBH) are presented to delineate further the phenotype of this disorder.

METHODS:

Standardized, norm-referenced measures of cognitive functioning, including intelligence, processing speed, attention, language, visuomotor skills, memory, and fine motor ability were administered to four patients with magnetic resonance imaging evidence of SBH.

RESULTS:

Despite intellectual impairment and other severe cognitive deficits, all four patients displayed relatively intact episodic memory.

CONCLUSIONS:

This selective sparing of memory functions has not been previously reported in individuals with SBH and suggests that doublecortin does not play a role in the development of memory systems in the mesial temporal region, which tend to be spared in SBH.