Age at diagnosis

Hi, I was diagnosed in July 2014 age 54 after 3 years of tests, I am “retired” from work, I couldn’t cope with it, high stress and I got muddled and couldn’t logically pursue an issue, word blindness, slurred speech handwriting illegible and unsteady, need a tripod stance to feel safe. I wonder what lies ahead…?

Dear Runner, I was diagnosed with Sporadic Cerebellar Ataxia (idiopathic/unknown cause) eleven years ago, at age 49, although I had very small symptoms starting about eight years before diagnosis. I'm 60 years young now and my ataxia symptoms have progressed. For instance, about four years ago I fell, injuring my back. Therefore, I started using a cane when I leave my home, although I'm still able to walk very carefully in my home without it. I havwe symptoms 24/7...,gait/balance, dexterity (writing/printing/fine motor skills), speech (slurred), swallowing (sometimes coughing/choking) and vision (blurred, especially left eye or when tired). I've had three MRI's (one at diagnosis and every five years thereafter) and the atrophy in my cerebellum is a little bit worse each time. My neurologist says that symptoms can be worse without seeing additional atropy though. I was initially diagnosed through a process of elimination (with blood tests, a nerve/ muscle test, as well as visual tests and, of course, an MRI. The team of neurologists were looking for a brain tumor. stroke, MS, Myothenia Gravis, Parkinsons Disease, etc. Although difficult, I believe heavily in exercising for strength and balance, as well as eating as healthy as possible. ;o)

I received my official diagnosis last year.

I knew even when I was a child that something was wrong. Tripping, falling, couldn't run, dropping things, be walking perfectly straight then start to veer off in another direction when I wasn't meaning to, etc. This was around 6 yrs old when I realized this, and it would go through spurts of being better, and then spurts of being worse. My family is ate up with hereditary Ataxia ( SCA1) , some were in wheelchairs , some were just " clumsy" as my family always said . I was called a Klutz from my very first memory. Doctors who saw me all my life, with bad back issues, said nothing was wrong with me ( but yet at 13 I was in the hospital all the time from then on, with back problems caused by falling or whatnot.. but , nothing was wrong with my head just my back.. uh huh ok )

As time progressed my father got more and more wobbly and needed a cane. Would just drop to the ground for no reason,.. his driving was atrocious , etc. As he progressed with " the Kirkland curse " as they called it, I had a back surgery in 08. Something went wrong as I was under for 12 hours and no one would tell me why 2 orthos and 2 neuros were working on me . Yes I knew what they were doing was bad, but they had figured 4 hours in the operating room. When I woke up, I noticed they did not let me get out of the bed for 5 days and only turned me to change the dressing. I figured it was just because my surgery was so " complicated " so I went with it.

After I got out of the hospital, and then out of the wheelchair, I noticed I had to use my cane ALL the time instead of just when my back hurt. That my balance was WAY off and I couldn't even do physical therapy after surgery because it was so bad.

A few years later my father was diagnosed with SCA 1 after his brother had been diagnosed . I had a feeling that was what I was getting. After years went by, I pretty much knew that was what it was. When I went to get tested for SCA1 finally about two years ago, I finally figured out what had happened to ramp mine up so fast......I received my back surgery report... during the back surgery, they had cut me bad and I had a really wicked cranial spinal fluid leak they couldn't get under control for a while. It undoubtedly leaked out quite a bit of my spinal and cranial fluid that surrounds the cerebellum , which in turn jump kicked my SCA1.

So, I knew at 6 yrs old something was wrong, at 46 I was officially diagnosed , and now I'm 48. No wheelchair yet, but I do need a scooter or buggy ( cart ) if walking far. I can walk in the house unaided if my back isn't "out" .

I was 5 when I knew something was wrong with me, at 37 I started having very noticable problems, at 38 I was diagnosed, Im now 39.

This is very interesting -- having to wait to get diagnosed, I mean...I'm new to the group, but I just wanted to share a bit of my story here....My first trip to a local neurologist took place when I was 29 -- reason being for slurred speech and occasional leg muscle shakes. After my examination, he agreed that yes, something was brewing -- but that I should wait it out for a few years, since the atrophy on the MRI (if I had a brain-related disorder, such as an hereditary ataxia -- since other family members of mine had similar problems) wouldn't show up until I was older. Did anyone else hear of such a thing?? (NOTE: Needless to say, he was right! I waited until I was 37.)

Dear Access

I think, snice there is nothing to be done, they delay,delay,delay.I think "the wait" is due to knowledge they would prefer not to share or lack of knowledge. There's an element od disinterest too.
access said:

This is very interesting -- having to wait to get diagnosed, I mean...I'm new to the group, but I just wanted to share a bit of my story here....My first trip to a local neurologist took place when I was 29 -- reason being for slurred speech and occasional leg muscle shakes. After my examination, he agreed that yes, something was brewing -- but that I should wait it out for a few years, since the atrophy on the MRI (if I had a brain-related disorder, such as an hereditary ataxia -- since other family members of mine had similar problems) wouldn't show up until I was older. Did anyone else hear of such a thing?? (NOTE: Needless to say, he was right! I waited until I was 37.)

Good point, Neta!..., ;o)

Yes, most definitely! But I'm still wondering if cerebellar atrophy cannot be seen until you're older?? My guess is no, since I've read stories about infants having it -- but then again, I guess it depends on the severity of the disorder??

Ataxia seems to be a bit of a mystery, as not that much is known about it..., ;o)

Right "Access" and "Rose"

All I know is that I was a perfectly healthy person until this weird illness or condition came upon me in about 2007 in the form of slight, ever so slight, swaying. It was (then) so minimal, it took me 2 years to go to a dr. .Maybe there were slight indications even before that. Nobody in my family had/has this unless someone is lying. Now that I think of it, there's alot of autism in my joint families also diabetes-- I dont know if this is related. My cousin in NYC, a young neuro, said "Dont worry. You wont feel its effects but in 500 years a descendant of yours may develop it younger and stronger". How wrong he was!!!

Next,t I went to a neuro at Columbia-Presbyterian hospital in NYC, who said its aikn to getting eyeglasses. I was formally diagnosed in 2010 by yet another neuro with "sensory ataxia" whatever that is. An investigative neuro told me its ataxia with no reason. Finally, the following year, 2011, I got this new diagnosis from a very senior neuro. He said its CA induced by autoimmunity. Now, I am wondering if he said this just to "satisfy" the hot-shot dr who runs a fancy autoimmune clinic, who lavished praise on him (the neuro) . I am saying all this to illustrate, the lack of clarity in this field,

Neta, our stories are very similar except I don't have an autoimmune condition. I have no idea why I have ataxia, as I was very athletic and active before this. I first noticed something very minor, but that was about eight years before my formal diagnosis. I was a big down-hill skier and had gone out West (I live in the US) with my two children, my son's friend and my sister and her husband. I kept falling on the hill, which was very unusual for me. My sister commented and I just chalked it up as having a bad day. Eventually (years), I started having problems swallowing. I thought it was all in my head, as I thought there could'nt possibly be anything wrong with me. I had always been so healthy! A year passed and my knees started to shake going downstairs so I finally went to my doctor. He referred me to a neurologist and the rest is history (that was twelve years ago). My ataxia ,has progressed, as I now use a cane when I leave my home. Like you, no one in my family, as far back as we know, had/has ataxia except me! I have no idea why...., ;o)

  1. (No idea why there.are numbers
  2. Great, (I say that sarcastically). Seriously I remember your story from past posts. This is such a shock for me. Undoubtedly 4 u 2. My older sister and brother (not to mention our parents--- in their 90's!!!!-- are all fine! his is so frustrating!!!

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Neta, our stories are very similar except I don't have an autoimmune condition. I have no idea why I have ataxia, as I was very athletic and active before this. I first noticed something very minor, but that was about eight years before my formal diagnosis. I was a big down-hill skier and had gone out West (I live in the US) with my two children, my son's friend and my sister and her husband. I kept falling on the hill, which was very unusual for me. My sister commented and I just chalked it up as having a bad day. Eventually (years), I started having problems swallowing. I thought it was all in my head, as I thought there could'nt possibly be anything wrong with me. I had always been so healthy! A year passed and my knees started to shake going downstairs so I finally went to my doctor. He referred me to a neurologist and the rest is history (that was twelve years ago). My ataxia ,has progressed, as I now use a cane when I leave my home. Like you, no one in my family, as far back as we know, had/has ataxia except me! I have no idea why...., ;o)

Thanks for sharing part of your stories, Neta and Rose. I'm sorry that this is what it is, for all of us...

I was diagnosed 26 or so years ago. I fell off a shovel and down a hill when I was planting bulbs. The Dr. had me take an MRI and sent me to a Neurologist. He looked at the MRI and told me all the cells in my cerebellum were dead or dying. He gave me tests and since no one in my family had Ataxia, he said I had Ideopathic Cerebellum Ataxia. Since then things have progressed. The last year I have been on a walker, with all the speech, eye, gait, balance problem’s getting worse. I have been exercising my legs so as not to bee in a wheelchair. When I was diagnosed the Neurologist said I would be in a wheelchair in 20 years, so I know it is coming.

My Mom always said I was clumsy. I couldn’t walk a log l, like other kids, I couldn’t play hop scotch cause I would loose my balance. There are so many things I look back on now, that my Ataxia started back then, but no one had heard of Ataxia back then. Am just now at 73 learning more of what I was diagnosed with 26 years ago.

Dear Jubie, I, like you, was diagnosed with Sporadic Cerebellar Ataxia twelve years ago (idiopathic or unknown cause, symptoms 24/7, progressive), although I had very minor symptoms starting about eight years before diagnosis. I’ve used a cane when I leave my home for about six years now, although I’m still able to walk, extremely carefullly in my home without it (I should use it in my home as i’ve taken some nasty falls over the years. I use an Access Active Rollater for pleasure walking outside. I’m also trying to keep my leg muscles strong in hopes of delaying having to use a wheelchair. I have no idea why I have this, as no one in my family, as far back as we know, has/had ataxia, except me! I’m 62 years young now and try to keep a positive attitude, although at times it’s difficult! My best to you…,;o)

Yes Rose we are about the same. It is so nice to talk to other people in the same situation. I live with my neice, her husband , and my brother- in-law since my husband died last year. We had been married 46 years so was a change. A change for them because they had never been around someone with Ataxia. We live in Port Orchard, Washington. I have a electric wheelchair so I went for a short ride in it today. The weather is finally clearing up.

Nice to hear from you, Jubie! I live in the State of Michigan. So sorry about the loss of your husband! 46 years is a long time to be married. How good that you are able to live with family though! My first husband died in 1996 when our two children were 12 (daughter) and 14 (son). I remarried my first husband’s best friend (it’s a looong story…,ha!) ten years ago. My children are now 34 (son) and 32 (daughter). My son is married and has three children ages 6, 3 1/2 and 1 1/2, two boys and a girl, my three precious grandchildren! My daughter is getting married in September. I also have two lovely step-daughters, 30 and 28. The older one got married in April. My family doesn’t know anyone else with ataxia either, just me. Hard to explain it, so I don’t even try anymore. Yes, it’s nice to talk to someone in the same situation, as they understand ataxia! My email address is ■■■■■■■■■■■■■■■■■■■■■■■■ if you ever want to talk, as I’m a great listener…,ha! Hugs, Rose ;o)

I was stunned on being told my SCA was genetic, like many other people, my MRI showed atrophy but no-one in my family has any history of Ataxia.

It turned out that the only way I could have a connection to a specific mutant gene, and develop SCA, was if both my parents had passed it on. They were both carriers, and unaffected themselves. My brother is unaffected but could be a carrier.

If either one of them had married someone else, and that person didn’t carry the mutant gene, any offspring would have been Ataxia free.

It’s not likely that my children will experience SCA but they could be carriers.

This particular genetic type is called Recessive. The specific mutant gene is Syne1.

I was 42 when I was diagnosed with MJD(Machado Joseph Disease) or SCA3. I am now 44 turning 45 in Dec.