2020..Possible treatment for Idiopathic Cerebellar Ataxia

[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 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 (which can be accessed here and here). If you do not see a neurologist regularly see details of Specialist Ataxia Centres.

Posted on 25/09/20

Copied from ataxia.org.uk

[Hidden]

All that is obvious really especially to someone who never went to school but has done a few first aid courses.

All this brain stuff is far too complicated for my 3 brain cells which is already overloaded with easier things like quantum physics, bio chemistry and how the universe was created.

Call me easily pleased but I’m happy if I wake up and my heart

Is still beating Lol.

Thank you for the info.

You take care my friend.

Who cares wins.

[wobblybee in reply to Hidden]

😂 I know what you mean, it can be a minefield trying to interpret medical jargon, on the whole this is not bad. It does sound promising for a specific group of people.

Stay safe 🙂

[cocoa in reply to wobblybee]

I am hoping something may come of this because I am in that category.

My next consultation at my local hospital, is due to be done by phone.

I intend to write to my consultant's secretary beforehand with all the information.

[wobblybee in reply to cocoa]

🙂I already know of someone who has been contacted as a viable participant. I’m wondering if I may be suitable myself, I’ll mention it in an upcoming telephone appointment 🙂

Notice in the post it mentions ‘new publications’. For some reason the link to these failed but you can view them by logging onto the original at ataxia.org.uk and searching for research news.

[Hidden]

What does immune-mediated mean wobblybee? I have idiopathic Cerebellar Ataxia. Just don't understand the immune -mediated bit. Does autoimmune also mean for example someone who may have little or no control over bowel or bladder movements? I'm confused 😕 sorry.

[wobblybee in reply to Hidden]

🙂please don’t apologise.. this is something best answered by Harriet. But I found this link, unfortunately ‘medical jargon’..

Immune-mediated diseases are conditions which result from abnormal activity of the body's immune system. The immune system may over-react or start attacking the body. Autoimmune diseases are a subset of immune-mediated diseases.29 Jul 2011

longdom.org › open-access

[Hidden in reply to wobblybee]

Thanks. I looked at the link/site and tapped on the immunology section. Understand slightly better now 👍

[penelope2]

Hi wobblybee

Thank you for that great info.

Out of desperation I have asked my neurologist's Secretary to print off these research papers so he can read them before my next phone consult.

There can be no excuse for not keeping up with new research when so much is at stake for us ataxia sufferers.

To take control I am 6 weeks into being gluten free and have noticed some small subtle changes.

When asked if i could talk to a dietician, my gp said no, so I have had to research gluten free myself.

Coeliac UK is a good website and lots of info there. It talks about periphery neurotherapy which I think I have.

Auto-immune ataxia needs to be brought into the limelight as it may be treatable.

[PW18]

Is the first key symptom the speed of onset? If your Ataxia came on slowly then it sounds as if PACA is unlikely?

[wobblybee]

Copied from ncbi.nlm.nih.gov Immune Mediated Cerebellar Ataxias

Genetic ataxias tend to progress slowly (usually over many years) with often a poorly defined temporal onset uncommon. Most IMCAs are of acute (days) or subacute (weeks or months) onset. Progression as a rule is faster in immune than in genetic ataxias, and immune ataxias follow a progressive course when untreated.