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Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy(CADASIL)is a recently described familial cerebrovascular disorder shown to map to chromosome 19q12.Familial hemiplegic migraine has also been shown in some families to map close to the CADASIL locus.The fully developed CADASIL phenotype consists of recurrent strokes developing in the fourth decade,progressing to a pseudobulbar palsy,spastic quadriparesis,and subcortical dementia.In an Irish family 15 members were fully investigated by magnetic resonance scanning;10 had typical magnetic resonance features of CADASIL.Five members of this family had familial hemiplegic migraine and 4 of these had magnetic resonance evidence of CADASIL.Two other members had migraine with an 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 this family was variable and age related and included familial hemiplegic migraine, migraine with and without aura,transient ischemic attacks,strokes,and spinal cord infarction.This family study increases our understanding of the spectrum of clinical manifestations of this underrecognized familial cerebrovascular disorder. |
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arteriopathy cerebral autosomal dominate arteriopathy with subcortical infarction and leukoencephalopathy cerebrovascular accident cerebrovascular accident,familial occurrence cerebrovascular accident,recurrent cerebrovascular accident,young adult chromosomal abnormality dementia dementia,subcortical electroencephalogram,pediatric patients familial familial hemiplegic migraine genetic neurologic disorders headache leukoencephalopathy migraine migraine,hemiplegic MRI MRI,abnormal pseudobulbar palsy quadriparesis spinal cord,infarction of
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