Cerebellar Cognitive Affective Syndrome

I have 'Cerebellar Cognitive Affective Syndrome' as symptoms attached to my diagnosed 'Cerebellar Ataxia'. I am willing to share my experiences with anyone who may also have this connective diagnosis alongside their Cerebellar Ataxia. I would like to point out that C.C.A.S should not be seen as being "All in the head" giving people the impression that nothing is wrong. A more appropriate description is its, "All in the brain". And believe me it's real. Very real. Thank you. Iain

[PatsyIpswich]

That sounds very interesting 🤔 I look forward to reading more from you 😀

[Hidden in reply to PatsyIpswich]

Thanks Patsy. Never spoken or known anyone with CCAS with ataxia

[Libra7]

This is something I have never heard of. What are the symptoms of CCAS?

[Hidden in reply to Libra7]

Hi Libra. My physical symptoms include: Balance and co-ordination, problems with swallowing/choking, digestion of foodstuffs and constipation. Cognitive and emotional symptoms include, word articulation ie language problems (Difficulty in putting sentences together properly and forgetting words in sentences), Difficulty in thought processes eg misunderstanding the meanings of what is being said to me, concentration lapses, unable to multi-task, can be extremely emotional at times (can burst out crying if something sad comes to mind or can get very frustrated or angry on mild problematical issues) These are my symptoms but can vary from person to person who also have Ataxia and CCAS.

[suzie44na in reply to Hidden]

I have most of those problems, but I have never heard of this before and no one has ever mentioned to me, specialists or doctors I mean. Very interesting, you are learning us something here, thanks.Suzie

[Hidden in reply to suzie44na]

Pasted a piece from Wikipedia below Susie. Unable to find anything less complicated. There was a very good explanation on the National Ataxia Foundation site but can't find it now. Been years since I've used it.

[coat2003 in reply to Hidden]

you sound like my bro who has ataxia....milder than me,i use a walker.

[Hidden in reply to coat2003]

CCAS isn't that common I don't think. Comes with the physical aspects of ataxia too. Coordination and balance being the main symptoms.

[Guardsman68]

Well fallguy you have hit the nail firmly on it’s head for me. I was diagnosed with CA seven months ago and I’ve been in neurology rehabilitation unit twice a week for physio and OT speech etc and I seen at CBT therapist two weeks ago fit the time after a four month wait and if I’m honest he was about as much use as an ashtray on a motorbike 🥴 he said to me I could also have FND?? But what you are describing is me every single day of the week to the point I’m now in a wheelchair if I have to walk any further the 4/5 meters. Thank you for putting this out on here I’m really interested to learn more.

Lee

[Hidden in reply to Guardsman68]

The information below might be useful Guardsman. Might be a bit complicated to understand but it was the best I could find.

[Hidden]

Found this information of CCAS on Wikipedia.

Cerebellar cognitive affective syndrome (CCAS), also called Schmahmann's syndrome[1] is a condition that follows from lesions (damage) to the cerebellum of the brain. It refers to a constellation of deficits in the cognitive domains of executive function, spatial cognition, language, and affect resulting from damage to the cerebellum.[2][3][4][5][6] Impairments of executive function include problems with planning, set-shifting, abstract reasoning, verbal fluency, and working memory, and there is often perseveration, distractibility and inattention. Language problems include dysprosodia, agrammatism and mild anomia. Deficits in spatial cognition produce visual–spatial disorganization and impaired visual–spatial memory. Personality changes manifest as blunting of affect or disinhibited and inappropriate behavior. These cognitive impairments result in an overall lowering of intellectual function.[2][4] CCAS challenges the traditional view of the cerebellum being responsible solely for regulation of motor functions. It is now thought that the cerebellum is responsible for monitoring both motor and nonmotor functions. The nonmotor deficits described in CCAS are believed to be caused by dysfunction in cerebellar connections to the cerebral cortex and limbic system.[2][6][7]

SIGNS & SYMPTOMS

Signs and symptomsCerebellar cognitive affective syndrome (CCAS), also called Schmahmann's syndrome[1] is a condition that follows from lesions (damage) to the cerebellum of the brain. It refers to a constellation of deficits in the cognitive domains of executive function, spatial cognition, language, and affect resulting from damage to the cerebellum.[2][3][4][5][6] Impairments of executive function include problems with planning, set-shifting, abstract reasoning, verbal fluency, and working memory, and there is often perseveration, distractibility and inattention. Language problems include dysprosodia, agrammatism and mild anomia. Deficits in spatial cognition produce visual–spatial disorganization and impaired visual–spatial memory. Personality changes manifest as blunting of affect or disinhibited and inappropriate behavior. These cognitive impairments result in an overall lowering of intellectual function.[2][4] CCAS challenges the traditional view of the cerebellum being responsible solely for regulation of motor functions. It is now thought that the cerebellum is responsible for monitoring both motor and nonmotor functions. The nonmotor deficits described in CCAS are believed to be caused by dysfunction in cerebellar connections to the cerebral cortex and limbic system.[2][6][7]

[Guardsman68 in reply to Hidden]

Thank you fallguy for sharing this information it has shed some light on the symptoms I’m having. I have a brain lesion in the CPA first discovered in 2015 a size of 2.5cmBut I was not informed of this just told I had an hemiplegic migraine? And told to take aspirin. I had been suffering brutal headaches/ tinnitus/ gradually my hearing was going all on the left side balance issues/ I couldn’t judge distance which was not good with my occupation. Fast forward to 2021 I’d been admitted to A&E after a head injury and concussion. I was falling over quite a lot just losing balance.

MRI reports I had a 4.5 cm lesion so it’s grown 2cm so I wasn’t happy as I have had this and been suffering for years as it’s a symptomatic brain lesion. I was told it was inoperable as it had attached itself to a few cranial nerves so over the years the cerebellum had some degeneration which I was told I had idiopathic cerebella ataxia but I asked why that diagnosis as I had the brain lesion. The six neurologist I seen while in hospital all agreed but I’m seeing a neuro surgeon on Tuesday which is a bit of a turn around. The CCSA as you have described is most of how I feel every day. I’m feeling quite frustrated with the neurological team as they just flob you off with so much BS.

Apologies for long reply

Keep well

Lee

[Hidden in reply to Guardsman68]

No need for apologies Guardsman. Your story is interesting and I hope your appointment with Neuro surgeon goes well. My diagnosis is late onset Idiopathic Cerebellar Ataxia. Experienced symptoms for years before my diagnosis in 2010. Wobblybee's link below explains cognition and emotion in CCAS a bit better. I think it's easier to understand.

[Guardsman68 in reply to Hidden]

Thanks fallguy I appreciate itI will take a look at wobblybees link as she always puts some really helpful advice on here. Stay safe and keep well

[Jenandbeth in reply to Hidden]

Yep Beth has all of above, and whilst I had been told it was all associated with her condition and accepted that to be true, it has never been labelled to the best of my knowledge. I shall enquire at her next neurology appointment. Thanks for sharing.

[Jenandbeth in reply to Jenandbeth]

Beth had an ADHD diagnosis that was treated throughout school and I knew to be associated.

[Hidden in reply to Jenandbeth]

That's a really good idea to bring it to the attention of Beth's Neurologist. CCAS should be discussed more widely to get the message across to others. Good luck.

[wobblybee]

🙂 This can help us understand how emotion can be affected..

ataxia.org/wp-content/uploa...

[Hidden in reply to wobblybee]

That's the link I was trying to find wobblybee. Thanks for that. This explains cognition and emotion in a better context and how CCAS can arise as a result of cerebellar damage. Hope it's of help to others.

[Jenandbeth in reply to wobblybee]

This is a really helpful reminder to me as Beth had a really awful weekend managing her emotions and it was unbelievably stressful! Thank you for timely share.

[chelpet]

I can relate to everything written in this thread!

[suzie44na in reply to chelpet]

I know me too.

[coat2003]

GOOD LUCK WHATEVER YOU DECIDE TO DO!monika x

[ninotchka]

I find all this info truly fascinating, as I have been diagnosed with only "Late Onset Cerebellar Ataxia" or, LOCA myself.

[Hidden in reply to ninotchka]

The more people who get to know about CCAS in some ataxias and how it affects behaviour and emotions the better. Keep well.

[ninotchka]

Thank you! Believe me, I query my neurologist all the time, send her links to articles and research, etc, but I feel like I know more than she does! I've been blood-tested for all types of ataxia and nothing ever shows up.🤬 I do feel as though I get worse every day and am turning into a sad person. Supplements, PT, Pilates, good nutrition is keeping me moving but not optimistic.