Reviving My Writing

Thanks for hanging in there with me.  I have been somewhat overwhelmed with both good, err, great things in our family and other physical and brain related things that have been challenging.  I plan to get my cyber pen warmed up and start to express my thoughts and communicate with you, my readers, friends and family that faithfully stand with us. 

Please watch for my next expression of questions, hope, faith and wonder at the surprises of this life we all live.  Regards,  Dan

"Looking Back", After 6 Years, I Would Have DBS Again-- "The Dance"

When I consider the alternatives, and the gains that resulted, I would do it all again.  Deep Brain Stimulation Surgery was risky and you could argue, ill-advised.  Let me rephrase.  It was generally thought to be not recommended for a Parkinson's Plus Syndrome, such as Multiple System Atrophy.  However, I had the best neurosurgeon on earth.  I really believe that.  I trusted him and understood his ability and experience level.  He explained the risk of a bleed and possible death, but also told me that I had the chance of extending my life and giving myself a better quality of life to share with my most important and priority above all priorities-- my wonderful wife, Karrie, my fantastic sons, their wonderful spouses and these beautiful grandkids!  Wow, am I a wealthy man in all the right ways-- the people in my life.  I am a simple man with simple values.  My brain disease has changed all of the superficial and practical abilities and access to my career and mobility.  I miss driving, working, leading, teaching and organizing people.  However, what really counts is the quality of life.

Deep brain stimulation surgery put implants in my brain and chest, along with cables in my neck which enabled me to move bigger and keep moving better than I would have, for a much longer time.  I have already gotten my money's worth.  It has helped me six years and it didn't do what the naysayers said it would-- it didn't kill me in a year, as had occurred in the case of two individuals I read about who had Parkinson's Plus Syndrome and died within the first year of surgery.  I learned while earning my Educational Doctorate that statistical significance goes up with the number of subjects and the controlling of variables.  This was too small a sample size and over generalized, as far as I can determine.

My surgeon knows the brain and knew that I had an unusual case with a great deal of abnormal involuntary movement issues.  This surgery didn't cure my disease.  I still have a degenerative process progressing, and now have more specifics about my diagnosis.  I have Corticobasal Syndrome and Multiple System Atrophy. I am confident in my movement disorder specialist who has done such a thorough job of assessing my symptoms and provided the best possible treatment. What a great Doc!  Also, going back, I originally had such a wonderful neurologist who took care of me for 10 year and recommended that I be evaluated for DBS.  My PC physician is wonderful and manages my case and all the variables.  The specialists I have that treat me for swallowing, speech, cardiac, urinary and digestive troubles all play a big roll.

I am blessed.  I am here and my family surrounds me.  We have a great church, and loving friends all over.  I would do it again.  As Garth Brooks says in The Dance,

And now I'm glad I didn't know
The way it all would end the way it all would go
Our lives are better left to chance I could have missed the pain
But I'd of had to miss the dance
Yes my life is better left to chance
I could have missed the pain but I'd of had to miss the dance.

-- Patient-Online

Strange Days: Adjusting to Decline with Family Support

What with both Multiple System Atrophy and Corticobasal Syndrome symptoms occurring simultaneously, I am learning to adjust to the the changes.  I find a need for scooting around in my transport wheelchair more these days.  I just take the footrests off of the chair and I can move around the house pretty well.  The great thing about the smaller transfer chair is it does not have the large wheels so it is narrower and fits through hall ways and doorways entering the restroom and bedrooms.  I can spend hours in this transport chair and it is quite comfortable.

I want you to know that I have closed my Facebook account because I don't want to support a company that is not forthcoming about sharing our personal information with business partners, let alone political campaigns.  (I changed my mind about this and reversed the process of closing the account).

Nevertheless, since I won't be on social media, I will be writing more often on this blog to communicate with you that stay in touch with me.

I am having a difficult time understanding why I have the cognitive behavioral dysfunctions that accompany CBS/FTD.  Corticobasal Syndrome is a part of the broader neurodegenerative disease known as Frontotemporal Degeneration.  I take Namenda to slow the progression of dementia that is associated with CBS.

Karrie is so helpful and understanding.  We are focusing on family and friends.  We appreciate the support of our adult kids, their wonderful spouses and their beautiful children.  It is a tight group!

The local Parkinson's Support group is a great place to share with patients and caregivers.  Online, we stay in touch through CurePSP "go to meeting" groups.

And, of course, our Church is a great support and a caring family.

This is Multiple System Atrophy Awareness month and we encourage you to get involved.  Thanks for stopping by! -- Patient-Online

Corticobasal Syndrome Explained in Simple Terms

2 Corinthians 12:9 New International Version (NIV)

⁹ But he said to me, “My grace is sufficient for you, for my power is made perfect in weakness.” Therefore I will boast all the more gladly about my weaknesses, so that Christ’s power may rest on me.

I have Corticobasal Syndrome or C.B.S.  Formerly, this would have been named Corticobasal Ganglionic Degeneration or C.B.G.D.  Today, Corticobasal Syndrome is the handle given to this illness during the life of the patient because it has to be confirmed pathologically (with an autopsy of the brain tissue) after the passing of the patient.  All Parkinson's Plus Syndromes, Multiple System Atrophy,  Progressive Supranuclear Palsy, Lewy Body Dementia and Corticobasal Syndrome are not diagnosed, absolutely, until a brain study can be completed.  The diagnosis is called "probable" or "possible" during the life of the patient.

So, after 11 years of treatment for Parkinson's Plus-- thought to be probably Multiple System Atrophy (MSA), it has been determined that I do have M.S.A. and also Corticobasal Syndrome.  The reasons are that I have the movement disorder and cognitive symptoms of C.B.S., but I also have the neurologically caused autonomic failure of M.S.A.  I have explained this autonomic failure on this blog before and you can refer back to it in my pages above, the links to previous posts to the right or by reading my book, I Will Go On: Living with a Movement Disorder.  In this post, I will now explain the basics of Coriticobasal Syndrome.


Overview of Corticobasal Syndrome

Corticobasal Syndrome is a movement disorder that is similar to Parkinson's Disease, though faster progressing and includes additional symptomatic issues that are more complicated earlier in the disease process than in Parkinson's Disease.  Parkinson's Disease is similar because it has symptoms known as parkinsonism, which include: 1) tremor, 2)balance problems, 3)stiffness and 4)slowness -- all caused by a degeneration of brain neurons.   Because Coriticobasal Syndrome also involves the Basalganglia (movement control center of the brain and is also involved in Parkinson's) some of the symptoms overlap, thus it is a Parkinson's Plus syndrome. 

That is where the similarity stops.  I do not have Parkinson's and I never have.  From the beginning, my neurologist at the time said, "This is so much more than Parkinson's Disease."

It was thought to be either Progressive Supranuclear Palsy or Shy Drager (an earlier name given to the condition now referred to as Multiple System Atrophy).  Progressive Supranuclear Palsy is a close cousin to Coricobasal Syndrome, so we have been in the right arena over this past decade.  The treatment and care given to me were helpful and would have been the same with either designation.  I would have had the Brain Surgery I had in July 2012 and it would have been considered helpful and effective in giving me better movement and more ability to walk with assistance, rather than being in a wheelchair most of the time, as I had been prior to the Deep Brain Stimulation Surgery.

After brain surgery to implant stimulators in two places in my head, with a brain pacemaker in my chest, I was able to resume playing in the church band for several years along with my son, Mark, shown here on the keys. 

What is Corticobasal Syndrome?

Corticobasal Syndrome is a movement disorder that includes walking trouble, tremors/jerky movements, eye control movement impairment, swallowing difficulty, rigidity and balance trouble.  It also causes Cognitive Changes that affect behavior, speech, mood problems and difficulty planning and concentrating.  It is a form of dementia called, Frontotemporal Dementia, but it is not the same disease as other forms of dementia, such as Alzheimer's Disease (A.D.).  It should not be confused with A.D.


What Causes Corticobasal Syndrome?

It is not clear what causes the degenerative process in the brain of individuals with C.B.S., but what we do know is that there is a specific protein in the brain known as Tau that breaks down creating tangles. These tangles destroy nerve pathways and inhibit the ability of the brain to communicate signals that help us move-- whether it be coordinating movements, walking, talking, grasping, swallowing or seeing. 


What is Alien Limb Syndrome?

One of the symptoms of C.B.S. is a phenomena called alien limb syndrome, where a hand, arm, or leg seems to have a mind of its own, assuming postures and movements that seem alien to the patient's awareness.  Also, there are twisted postures of hands, fingers, arms, neck and/or legs/feet.  These movements may also appear in a jerky unpredictable manner, while a form of tremor in some, it may be less regular and random in its form.  Parkinson's Disease typically, when tremor exists (not all Parkinson's patients have a tremors) it is a slow, regularly patterned tremor that occurs generally during rest.


What is a Taupathy?

The illnesses that are caused by the breakdown of the protein Tau, include: Alzheimer's Disease and Progressive Supranuclear Palsy, along with Corticobasal Syndrome.


How is it treated?

C.B.S.  does not have a cure, but there are a number of treatments that may help with symptom relief.  Some of the medicines used for Parkinson's are also given to those with C.B.S., which has been true in my case.  Sinemet is given to help some with rigidity and tremor.  I do receive some benefit from Sinemet.  Other treatments address the cognitive issues and can include medicines used to assist with memory.  Speech therapy can be used to assist with swallowing problems, which has been beneficial in my case.  Swallowing problems have to be combated because they can lead to serious choking and eventual pneumonia.  Falling and gait difficulty require physical therapy and assistive devices such as canes, walkers, wheelchairs and electric scooters.  I use all of the above.  Special lenses may be prescribed to help with the eye movement control problems.  These would be meant to help to prevent double vision.  This eye aiming and movement problem occurs in both Progressive Supranuclear Palsy and Corticobasal Syndrome.  These two syndromes are often confused with one another and a brain study post-mortem is the only way to distinguish which is the correct diagnosis.  Both will show Tau tangles in the brain tissue.


In Summary

Understand that my explanation is from previous study and is by no means a scientific description.  Rather, think of it as a start toward me helping my family and friends gain knowledge about that with which what I am struggling.  I see this as an opportunity to overcome these symptoms and disabling problems to whatever degree is possible through continuing effort and positive forward motion in life.  This is a daily decision and a better choice than sitting down and giving up.  Along with C.B.S., I am fighting the low body temperature regulation problems, the falling blood pressure (orthostatic hypotension), trouble with urination, digestive dysfunction, breathing difficulty, heartbeat irregularities-- all of which are caused by a breaking down of neurons from a different cause than C.B.S.  M.S.A. involves the folding of alpha synuclein protein, rather than Tau tangles.  It is being found that some patients have both proteins causing neurodegeneration.  Diseases aren't fair and don't "leave you alone" because you already struggle with another condition.  There is no rule than you can't get two neurodegenerative diseases. (This link will take you to an article that gives an example of how these two proteins misfolding can work together in the disease process: https://mayoclinic.pure.elsevier.com/en/publications/alpha-synuclein-and-tau-teammates-in-neurodegeneration

Thank you for taking time to read this explanation.  Hopefully, this will help to explain the differences that make my condition an exception to the more common brain illness known as Parkinson's Disease.  Henceforth, I will hope to take part in the effort to bring light to this serious condition known as Corticobasal Syndrome.  I am very thankful for the exceptional Movement Disorder Specialist I am treated by who investigated my brain disease very thoroughly and with all relevant technology and advanced diagnostic techniques.  Special thanks, as always, to my caregiving spouse, Karrie, who loves me and shows such grace and patience in dealing with all of the inabilities and changes that affect her nearly as much as me.  Also, I thank my sons, their spouses and our grandchildren, who are with me frequently, and are helpful and encouraging to me in making the most of every day.  -- Patient-Online