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Tuesday, January 19, 2010
Defining the terms: What exactly does spinocerebellar ataxia mean?
The spinocerebellum is an area of the brain which is connected to the spinal cord and controls a person's motor abilities.

This is an image of the cerebellum:



Fig. 1: An image of the cerebellum

Source: http://depts.washington.edu/neurogen/downloads/ataxia.pdf

As you can see, the cerebellum is located at the back of the brain, so it's not very likely that it will be damaged. In the case that it is damaged (for instance, through a brain injury or stroke), a person will feel uncoordinated and awkward because the cerebellum controls balance and the coordination of movements.

Ataxia is a symptom where a patient is unable to control his or her movements in a normal and comfortable manner. For instance, they might find it hard to balance when standing up or move their hands in a steadily.

Another name for this disease is "spinocerebellar degeneration" because it is triggered by the degeneration of the brain.

Now compare these the two images below:



Fig. 2: Two MRI scans. The cerebellum of the brain on the left shows signs of degeneration while the cerebellum on the right is healthy.

Source: http://depts.washington.edu/neurogen/downloads/ataxia.pdf

And here are two other interesting images to take a look at:



Fig. 3 - MRI scan shows the degeneration of the cerebellum

Source: http://www.marmaramedicaljournal.org/pdf/pdf_MMJ_399.pdf



Fig. 4 - MRI scan shows the degeneration of the cerebellum

Source: http://www.marmaramedicaljournal.org/pdf/pdf_MMJ_399.pdf

In the MRI or CT scans (these are both brain scans, like the image above) of many spinocerebellar ataxia patients, there is a indication that their cerebellum is shrinking and/ or breaking down.

Of course, the extent to which the cerebellum has deteriorated depends on the onset of the disease. SPA (Spinocerebellar ataxia - I will be using this short form a lot) is a slow and progressive disease, which means that the symptoms will worsen over a long period of time. However, there are many types of SPA, some which can progress faster than others.

This disease rarely appears in children under 18, with the majority of patients being well into their 20s, 30s, and 40s.

In the next post, I'll explain some of the symptoms of SCA.