CADASIL

CADASIL is a rare, inherited type of vascular disease (a disease of the blood vessels such as arteries and veins) that can cause dementia. CADASIL stands for ‘Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy’.

CADASIL is caused by a faulty gene. If a person has one parent who carries the faulty gene they have a 1 in 2 chance of developing CADASIL. People who are worried that they may have inherited the faulty gene can have genetic counselling and testing. They can ask their GP to refer them to these services. For more information see Genetics of dementia.

A person who has CADASIL is likely to have a series of mild strokes that can damage their brain tissue, particularly the ‘white matter’ that connects different parts of their brain together.

CADASIL can cause a range of symptoms that develop slowly over many years. These include problems with memory and thinking as well as physical and emotional problems. They often start when a person is between about 30 and 50. Common symptoms include:

• migraines
• slurred speech
• weakness down one side of the body.

Women who have CADASIL may first notice symptoms when they become pregnant.

Around 2 in 3 people who have CADASIL will develop dementia at some point in their lives. The age that this happens can vary a lot from person to person. The symptoms of dementia are usually very similar to the symptoms of more common types of vascular dementia, particularly subcortical vascular dementia. For more information see Vascular dementia: what is it, and what causes it? and The brain and dementia.

Symptoms related to dementia include thinking more slowly, and problems with:

• paying attention
• planning
• organising
• reasoning
• problem-solving
• decision-making.

CADASIL may also affect the person’s memory and their ability to work out how the things they see are arranged in space (visuospatial skills). They may have emotional changes that begin as apathy where the person has a general lack of motivation. They may also feel anxious and depressed.

If a person experiences these symptoms they can have a range of interviews, tests and scans to see what is causing the problems. People with CADASIL are likely to have a family history of strokes and dementia. They may also have a particular pattern of damage to their brain that shows up on a brain scan. The diagnosis will then be confirmed by a genetic test for the condition.

There is currently no cure for CADASIL. Some people can successfully treat some of their symptoms, such as migraines. They are usually advised to take medication, such as drugs to manage high blood pressure and cholesterol levels, and to make changes to their lifestyle to prevent them having more strokes in the future. There is not enough evidence from clinical trials to suggest that people with CADASIL should take drugs used for other types of dementia to treat their dementia symptoms.

CADASIL Support UK has a website with information about CADASIL and links to more support. The Cambridge Stroke website also has more information. See Rarer types of dementia - other resources.