Primary Autoimmune Cerebellar Ataxia (PACA)

[wobblybee]

Diagnosing and treating primary autoimmune cerebellar ataxia (PACA)

Many people with ataxia are not given a specific diagnosis for the cause of their ataxia. This is known as idiopathic ataxia.

In two new publications, Professor Hadjivassiliou (below) at the Sheffield Ataxia Centre, and his colleagues, describe a type of ataxia that could

be responsible for a number of idiopathic ataxia cases. They explain how neurologists can make this diagnosis and how this type of ataxia can be treated.

Immune-mediated cerebellar ataxias are caused when the body launches

an unnecessary immune reaction against the cerebellum (the balance centre) causing damage, resulting in ataxia. Gluten ataxia is an example

of an immune-mediated cerebellar ataxia. In patients with gluten sensitivity, the body recognises gluten as harmful and produces antibodies against it. These antibodies travel to the brain and attack cells of the cerebellum, which results in ataxia. In this case, gluten is known as the ‘trigger’. However, in some cases of immune-mediated ataxias, the trigger is unknown, which is when the condition is called primary autoimmune cerebellar ataxia (or PACA).

DIAGNOSING PACA

There is no specific test that will definitively diagnose PACA. However, an International Task Force on immune-mediated cerebellar ataxias, of which Prof Hadjivassiliou is the lead, have written a comprehensive list of clues that neurologists should look for when considering the possibility that a patient has PACA. A diagnosis of PACA can be made if certain criteria outlined in this document are fulfilled, and if an experienced neurologist or ataxia specialist has ruled out other possible causes (such as genetic ataxia).

A TREATABLE FORM OF ATAXIA

In the second article, the team from the Sheffield Ataxia Centre describe

a possible treatment for PACA. They treated 22 PACA patients with an immunosuppressive drug called Mycophenolate, which reduces the immune response that causes the ataxia. This treatment is expected to prevent further damage to the cerebellum and salvage any sick cells. Their results using brain scans and ataxia rating scales showed that those receiving treatment improved or stabilised, and those who did not got progressively worse.

Prof Hadjivassiliou said: “Identifying patients with PACA is incredibly important to allow them to receive the appropriate treatment, and early diagnosis could prevent permanent neurological damage.”

If you have been diagnosed with idiopathic ataxia and would like to explore the possibility of PACA, we recommend you speak to your neurologist about these new publications. If you do not see a neurologist regularly, email [e-mail]. uk for details of Specialist Ataxia Centres.

Copied from ataxia.org.uk

[rideabike]

That might be helpful to me as I am yet considered idiopathic. Thank you!

[crazycat18]

As I received a ideopathic diagnosis over 10 year's ago and had little to no new information since then this gives me some information that is new to me and is worth looking into. Thanks for taking your time to pass on new information we didn't see ourselves. Thanks again.

[cocoa]

I have idiopathic cerebellar ataxia.

I requested this test the last time I had a consultation with my neurologist. The reults were negative but it is good to know that there are still avenues to explore

[suzie44na]

I see him but I don’t have a definite diagnosis . Did not get to see him in December because of lockdown, now having to wait until next December.

[wobblybee in reply to suzie44na]

I haven’t had face to face for 18mths, all my appointments have been via telephone. Hopefully things will improve as the current situation eases. Would you be willing to try the immunosuppressive if it was offered 🤔

[suzie44na in reply to wobblybee]

I don’t really know what it is.

[wobblybee in reply to suzie44na]

The bodies of those with autoimmune conditions are attacked by their own immune systems, which can cause inflammation and tissue, organ, and joint damage, along with overgrowth of cells. Those conditions are varied and include diseases like rheumatoid arthritis, lupus, inflammatory bowel disease (IBD), and psoriasis, among others. Suppressing the immune system in these cases can reduce these symptoms for many people with autoimmune disorders.

Copied from health.com

[suzie44na in reply to wobblybee]

Thank you for explaining. We do have a lot of autoimmune conditions in our families, a lot of my cousins have them.

[Klutzy]

If I am reading the information correctly, is this a blanket term for those whose ataxia is from thyroid, gluten or other triggers? It's encouragiing to know those types are treatable.