FAMILIAL HEMIPLEGIC MIGRAINE AND AUTOSOMAL-DOMINANT ARTERIOPATHY WITHLEUKOENCEPHALOPATHY (CADASIL)

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a recently described familial cerebr ovascular disorder shown to map to chromosome 19q12. Familial hemipleg ic migraine has also been shown in some families to map close to the C ADASIL locus. The fully developed CADASIL phenotype consists of recurr ent strokes developing in the fourth decade, progressing to a pseudobu lbar palsy, spastic quadriparesis, and subcortical dementia. In an Iri sh family 15 members were fully investigated by magnetic resonance sca nning; 10 had typical magnetic resonance features of CADASIL. Five mem bers of this family had familial hemiplegic migraine and 4 of these ha d magnetic resonance evidence of CADASIL. Two other members had migrai ne with and without aura as a presenting clinical symptom of CADASIL. This disorder has been shown by linkage analysis to map to the CADASIL locus at chromosome 19. The phenotype at presentation of CADASIL in t his family was variable and age related and included familial hemipleg ic migraine, migraine with and without aura, transient ischemic attack s, strokes, and spinal cord infarction. This family study increases ou r understanding of the spectrum of clinical manifestations of this und errecognized familial cerebrovascular disorder.