Of the approximately 50 cases worldwide, the majority were found in Japan, with a few cases in China, Spain, Portugal, and Romania. CARASIL appears to affect males more often than females. A ratio of 7.5 males to 1 female was observed in Japan.
Cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy
Hereditary cystatin C amyloid angiopathy
Walker–Warburg syndrome
Hereditary diffuse leukoencephalopathy with spheroids
Cerebral amyloid angiopathy
Familial British dementia
Moyamoya disease
Subacute sclerosing panencephalitis