Cathepsin A–related arteriopathy with strokes and leukoencephalopathy (CARASAL) is a recently identified cause of adult-onset cerebral leukodystrophy due to CTSA gene mutations described in 3 Dutch and British families.1,2 The clinical phenotype of CARASAL continues to be defined. The gene discussed is CTSA; the disease is Leukoencephalopathy.