Introduction

Cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CARASIL) is a rare hereditary disease characterised by ischaemic cerebral white matter lesions, spondylosis and alopecia.1 2 Like cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), CARASIL is caused by abnormalities of a single gene. The causative gene has been recently identified as HTRA1.3 The white matter lesions, which result from non-hypertensive small-vessel arteriopathy, appear in early adulthood,1 3 but their association with neurological manifestations is poorly understood.