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CADASIL

What are the symptoms?

CADASIL is characterised by recurrent stroke most commonly first occurring in the 30's to 50's although it is now known that the disease can be very variable and in some people may not present until their 60's. A few individuals identified with CADASIL have remained well in their 70's. The type of stroke affecting people with CADASIL are lacunar strokes (literally meaning a small lake or hole in the brain). As these strokes are small, they tend to be fairly mild and individuals often recover well. The most common type of stroke is weakness affecting one side of the body. If recurrent strokes occur, this can lead to persistent disability which is most usually arm or leg weakness, or slurring of the speech.

Migraine is another common feature of the disease. This most commonly starts in the 20's but the onset is variable. Usually these are 'complex' migraines. This means that in addition to the headache there are short-lived neurological symptoms, most commonly, some disturbance of vision or numbness down one side of the body or speech disturbance.

Individuals with CADASIL can suffer from anxiety or depression. Depression is very frequent after any stroke and usually improves with time and treatment if necessary. However, occasionally, depression may occur before any other symptoms of CADASIL. Rarely, seizures (epilepsy) occurs as part of CADASIL. Over time, as the disease progresses, memory problems may occur and if these become severe, they are likely to occur in the 50's or 60's. An unusual feature is the onset of confusion and reduced consciousness over a period of hours or days, sometimes with fever and seizures; this often follows a migraine attack. It recovers completely over one to two weeks.

View Background Background  |  What are the causes? View What are the causes?

Medical text written March 2003 by Contact a Family. Last reviewed January 2008 by Professor H Markus, Professor of Neurology, St George's Hospital Medical School, London, UK.

 

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