Cognition (thinking skills)

 

Due to the nature and location of the disease process, an individual may experience difficulties in their thinking skills. Thinking skills are often called cognition or cognitive function. Difficulties with cognitive functioning usually start when people with CADASIL are in their 40’s or 50’s, but they can occur earlier or later.

 

The cognitive difficulties tend to follow after people have TIA’s or Strokes, although they are also known to occur before such events. The difficulties are usually subtle and may only be noticed by close friends or relatives. However, as the disease progresses the cognitive difficulties also become worse.

 

The main difficulties people may notice include:

• difficulties with maintaining attention
• difficulties with remembering things (particularly new things that one has learnt including names of people, appointments, etc.)
• problems with the speed and coordination of all of the thinking skills
• difficulties with planning and organising activities
• problems with monitoring behaviour
• difficulties with reasoning and problem solving

Most of these thinking skills are referred to as ‘Executive Functioning’.

 

As the disease progresses, difficulties are also seen in:

• language skills (for example, finding the right words when expressing yourself), and
• visual and spatial skills (for example, how the brain makes sense of things that it sees in the environment)

The frequency and severity of the cognitive difficulties is variable in different people and also within different members of a given family. This could be due to the variable location of damage to the brain in different people. If you have noticed difficulties with any of your thinking skills, it is important to talk to your doctor about this. If appropriate, you may be referred to a Clinical Neuropsychologist who can assess the extent of the difficulties and offer some advice about how such problems can be managed.

 

If you are referred to a Clinical Neuropsychologist, you will be asked to carry out some paper and pencil tasks which are designed to assess the different cognitive skills. This is called a Neurocognitive Assessment or Neuropsychological Assessment. If this is applicable to you, you will be given more information about this assessment when an appointment is arranged for you.

 

Currently there is no cure for cognitive problems in CADASIL. However, research evidence shows that the best way to manage cognitive difficulties is through the use of compensatory strategies (for example, using a dairy to record appointments if you are having difficulty with your memory). A Clinical Neuropsychologist can give you some advice about which strategies to use.

 

Alexa MacDonald, a Cambridge Neuropsychologist, gave a talk on managing cognitive symptoms at our 2019 CADASIL Conference. Her slides can be found here.