PET Scan and Corticobasal Degeneration

I haven't been a regular blog writer this summer, to be sure!  I guess the teacher in me still sees the natural need for a break?  Sounds like a very nicely worded excuse!

Anyway, I was a principal and district office administrator, so I had a fairly conventional vacation schedule (very little :) the last 20 years of my career as a manager.

On the brain disease front, I am still working with our MDS (movement disorder specialist, neurologist) at the medical clinic to get a very specific diagnosis.  I continue to be considered, as I have been for 11 years, as having Parkinson's Plus, with MSA, PSP and CBD all showing symptoms in my case.  (Multiple System Atrophy, Progressive Supranuclear Palsy and Corticobasal Ganglionic Degeneration).

CBD is often referred to as CBS (Corticobasal Syndrome) during life and confirmed as a CBD after death.   CBS is under the umbrella of Parkinson's Plus, and more specifically, one of the Fronto Temporal Dementia's (FTD).  There are several categories under FTD, including PSP, CBS and Primary Progressive Aphasia (predominantly a severe language issue).

In my book, I WILL GO ON: LIVING WITH A MOVEMENT DISORDER, I discussed CBD, MSA and PSP, knowing that I had the potential for being defined has having one of the three.  I didn't know that 8 years after publishing that book that my diagnosis would be possibly redefined-- well that isn't the best way to express it-- further defined. My neurologist way back when said it was "so much more than Parkinson's Disease," and that was in 2006.  He thought that Shy Drager or PSP were likely, and later landed on Shy Drager and more specifically, Striatonigral Degeneration.  Then, in 2012, I had brain surgery and Deep Brain Stimulators were inserted in my brain to block faulty signals causing walking and shaking/jerking issues.

I have had a lot of benefit from the DBS system I have in my brain in two areas and also the generator place in my chest, connected to cables that send the electrical stimulation to the movement center of my brain.  Not many on this earth with Parkinson's Plus have had DBS, so I feel pretty special!

Now, with an outstanding Movement Disorder Specialist working with us, I have had a DAT scan which had very noticeable results confirming that I have a Parkinson's Plus Syndrome.  Now, our MDS  has ordered a PET, which will use radioactive isotope to track metabolism in my brain to determine the type of protein damage I have.  The proteins in MSA (alpha synuclein)  and PSP or CBD (tau) clump (MSA) or tangle (PSP, CBD).  The aggregation of these proteins causes brain degeneration and is incurable and irreversible.  Attempts are being made through research to develop methods that are hoped to recognize these degenerative diseases early and possibly arrest them in their progression, or even just slow them down.  Cures are a long way off, but we are not out of the fight because we care about future generations who could develop the same type of conditions. 

What I have is not genetic and is not communicable.  It is a disease that begins spontaneously or sporadically.  It is possibly caused by toxins in the environment, including workplace or dwelling.  The domino effect of the proteins folding brings about the loss of neurons that provide for the function of the body: everything from blood pressure regulation, breathing control, swallowing, eliminating, eye movements and gross motor function, to name several but not all of the disabling affects. 

I have lost abilities, and will continue to decline, but I am still here and loving this life!  There is so much going on and I have so much hope and enthusiasm for my family, faith and music.  I have so much I want to accomplish each and every day. 

I will post after the PET Scan to share what might be learned about the effects these degenerative processes have brought. I end with a scripture reference that once was the motto of my Christian band I played in with John Moffett and Dale Samuelson, "Changing Heart":

2 Corinthians 4:16-18 New International Version (NIV). Therefore we do not lose heart. Though outwardly we are wasting away, yet inwardly we are being renewed day by day. For our light and momentary troubles are achieving for us an eternal glory that far outweighs them all. 
 -- Patient-Online

Time to Let it Sink in: Coriticobasal Syndrome

I took some time.  All of April to be exact, and then some.  Here it is May and I am thawing out my writing impetus after 5 weeks of writer's block.  After getting a new (November 2016) probable diagnosis of a disorder called Corticobasal Syndrome, a member of the Fronto Temporal Disorder family, I ran out of words to say, or write.

First, of all, I am very grateful to our great doctors-- our primary care doctor at the clinic, the various specialists who treat me for everything from choking/swallowing difficulty to severe eye pain, and up to and including the outstanding Movement Disorder Specialist/Neurologist that is working so hard to  find answers to my rare Parkinsonian disorder.

For years, I wrote on this blog (since 2006 with some breaks) details of my experiences, feelings, faith struggles, spiritual plateaus, and thorough verbal descriptions that defined and categorized all of the various Parkinson's Plus syndromes that I am similar to, if not experiencing.

Let us not become weary in doing good, for at the proper time we will reap a harvest if we do not give up. -- Galatians 6:9

Now, after all these years, I am at peace.  I have for years been defined as having Parkinsonism and one of the Parkinson's Plus syndromes, most likely Shy Drager. Shy Drager is known as Multiple System Atrophy and I have the hallmark symptom, low blood pressure upon rising from bed or getting up from a sitting posture.  This causes blacking our, loss of oxygen to the head and can be an hours-long condition where my neck and shoulders ache, my arms and upper extremities get a numbness and lack of feeling, and my strength is very limited, causing great fatigue.  This blood pressure issue gives pause to my neurologist and is the reason why in addition to Coricobasal Syndrome (Alien hand and Eye control/focusing/tracking issues) and Parkinsonism (tremors, stiffness, loss of balance, and many other symptoms), I am considered to possibly still have MSA.

My behavior, emotions, thought changes-- an area so personal to me that I can't discuss yet-- means that I am being looked at for Fronto Temporal issues.  FTD is scary.  I don't know what to think about it.  If I am confusing you, just imagine how confused I am.

All I can say is, I am beginning to want to share about these things.  I respect my physicians and want to give them the space and freedom to consider all the avenues and pursue the best truth that describes the need and leads to the best possible treatments.  My Deep Brain Stimulator is working for me and keeps me walking-- at least enough to use my walker.

Today, Karrie is taking me on a very nice outing to Ikea.  That sounds like a lot of fun.  This will require my wheelchair and mean that I will be dodging nice people all day long. It will be great to be out and part of the world for a time.  Just enough time to let it sink in again-- I may be disabled, but I am not grounded.  Not completely.  I have Coricobasal Syndrome:  a loss of cells in my basal ganglia on both sides enough to show up in my DaT scan results and cause the loss of body control that has put me in a walker and stollen my career.

After all this time, I don't have as much to say.  However, I am beginning to want to share again.  I will give it a try, very soon.  Meanwhile, I've got so much to do. I'm a father, a husband, a grandpa and a guitar player!  Life is beautiful, man! Stay tuned-- Patient-Online

Confusion Over Parkinson's Contrasted with CBS and MSA

This is Multiple System Atrophy Awareness month!

Go to MSA Coalition Page for More Info

I have quite often discussed with some of you in the family, and various friends, about the difference between Parkinson's Disease and the Atypical Parkinsonian disorders.  I am going to give you a few points to chew on, knowing you are the best ambassadors we have for spreading the word about these rarer forms of Parkinsonism.

Parkinsonism is a condition in which signs and symptoms of Parkinson's appear in the patient's disease.  Even though the person may not have Parkinson's Disease, they have a brain disorder that causes similarly appearing symptoms, including tremors, balance problems, stiffness, walking difficulty and cognitive changes.

Parkinsonism appears with Parkinson's Plus syndromes such as Multiple System Atrophy, Corticobasal Syndrome and Progressive Supranuclear Palsy.  Since I was first diagnosed in 2006, the neurologist I saw for ten years thought that my condition was one of these three.  As time went on, Multiple System Atrophy became the most likely.  All three of these conditions are determined to be "probable" in life, and are confirmed after death through a brain tissue study.

Even though I have  Parkinsonism it is not Parkinson's Disease in the simplest form because the disease process in my brain is more involved than in Parkinson's.  I have Corticobasal Syndrome (CBS), and it is uncertain if it would be alternatively considered Multiple System Atrophy (MSA).  These have overlapping symptoms and are best described as rarer forms of Parkinson's.

Has much changed?  Not really, except I have more clarity and certainty of the degenerative brain disease that has taken so much from our lives in the Brooks family.  We are fortunate to have this increased clarity because the neurologist I have been seeing of late is a Movement Disorder specialist, which is a doctor of Neurology who specializes in all things Parkinson's and Parkinson's-like.   She saw a clear indication in the results of my DaT Scan which demonstrated that I have Parkinson's Plus, not simply Parkinson's Disease.

Parkinson's Plus has long been the understanding of my condition.  I wrote about this in my book, I WILL GO ON: LIVING WITH A MOVEMENT DISORDER.  The confusion arises because the word "Parkinson's" appears in both descriptions of the diseases.  Technically, they are different in that Parkinson's Plus is a faster progressing disease and causes more disability sooner.

That is why I was unable to continue driving and had to retire at 51.  I have difficulty walking with coordination and I struggle with choking on food and liquids.  I also have digestive, urinary, heart rate, blood pressure regulation and body temperature issues.  These are not visible to friends and family so what appears to be a better day, could be a day I am having trouble with my blood pressure or having great difficulty coughing after drinking liquids.

I also have very abnormal horizontal eye movements which are caused by a loss of neurons in the area of the brain that controls eye movement.  At times I see double as a result. 

I am so glad that you are interested and are trying to grow in your knowledge.  MSA is a disease I have been identified with for 10 years or more.  I have CBS, but if it were to turn out to be MSA at some point, that would be a very similar prognosis. My greatest concerns are pneumonia, breathing constriction, and urinary infections.  I do not have idiopathic Parkinson's, but I do have a form of Parkinsonism, and much of the research being done for P.D. will have a benefit and weight heavily on the potential for discoveries that relate to PSP, MSA and CBS syndromes.  I will always identify with my fellow patients who have Parkinson's Disease, and the support groups for Parkinson's are virtually the only in person, brick and mortar groups we can attend anywhere near the Riverside, CA area. As always, thanks for reading! -- Patient-Online

DaT Scan Results on the Heels of the Loss of Our Dear Mother

It has been a trying couple of weeks, but the sun shines behind the clouds, and it is breaking through. Losing my mother, Marguerite, is devastating and creates a void no one can fill.  However, her passing also makes crystal clear the beautiful life she lived, her several talents and the way her life lives on through each of us who were her family and were close to her.  I feel her with me more than ever, in my personality, abilities, and zest for life.

While this great loss was impacting our family, Karrie and I were going through another battle.  We have been fighting my disease by seeing an excellent specialist, a Neurologist specializing in Movement Disorders at Loma Linda University Medical Center.  I won't list the physician's name to protect her professional privacy but I would shout it from the rooftops otherwise! A wonderful doctor, indeed!

Over the 5 or 6 visits we have had with her, we have had very thorough conversations and examinations as she gathered clinical information about my 11 year history with a parkinsonian syndrome.  She approached it using scientific methods, which included starting from scratch with our story and her physical tests.  She spent 2 hours with us initially and has had at least three other doctors examine me while in her offices, as well.  She has adjusted my medications and also my Deep Brain Stimulation system generator each time I have seen her over a 5 month period.

Her actions have included giving me a memory medication called Namenda and it has helped with clarity of thought and provided for more peaceful nights of rest.  Also, she has sent me to an Ear Nose and Throat specialist to study my speech delays and swallow/choking issues. He put a scope up and down through my nose to view my vocal chords with a camera.  My breathing is compromised by my vocal cords which tend to close part way as I breath, causing stridor.  I am now getting speech therapy to help with both speech and swallowing, and I am going to pay more careful attention to my breathing now and in the future.

The decision she made to send me for a DaT scan was really key to breaking the log jam, to my eyes.    I had the scan the week after my Mother's services.  It was hard to be in the same facility in which she was last alive the week before, but the importance of this nuclear imaging test was worth this emotional reminder for Karrie and me.  I felt that Mom was present in my heart and mind while we were undergoing this DaT process.

A DaT is a study of the "Dopamine Transporter" to determine if neurons located in the Basal Ganglia responsible for the transport of dopamine have been lost.  I received an injection of Loflupane I 123 and this nuclear substance attaches itself to the remaining neuron cells.  During the scan, a Gama Camera detects this substance and shows a lighted area where the dopamine transport neurons remain intact.  If there is a loss or reduction in the normal shape, that of a semi-colon according to my Movement Disorder Doctor, then this indicates either Parkinson's or an Atypical Parkinsonian disorder.  Generally, the pattern would be lost unevenly on one side in Parkinson's and would show up with more uniformity on both sides in a Parkinson's Plus syndrome. My reference for this information about the DaT process is the following article found at this link: http://www.parkinson.org/find-help/blogs/whats-hot/april-2014 )

I have borrowed an example image to give the reader an idea of what it looks like in the normal versus the abnormal states of the basal ganglia:

Our Doctor studied my results and showed them to Karrie and me.  We were able to see examples which showed the dot of the semi colon without the comma.  The round portion is the Caudit and the tail, or comma, the Putamen.  In my images, there was little visibility of the Putamen.  The images showing the significantly reduced Putamen were very symmetrical.  The beauty of this is that our Doctor found that this scan affirmed (I didn't use the word confirmed for a reason, affirmed to me means "supported?") her clinical findings (observed phenomena), using the these results which were consisted with what she sees trending in my visits.  I do not have Parkinson's Disease.  I have a Parkinson's Plus condition which is most like Corticobasal Syndrome (CBS).   It is somewhat like MSA.

Corticobasal Syndrome is in the family of Frontotemporal Dementia.  There are four subtypes, with one being focused on a movement disorder that has some movement problems that appear similarly to Parkinson's, but also includes additional problems which makes the prognosis worse and the average time leading to death is typically shorter, on average.  Our Doctor continues to work on my case, but sees it as CBS, and within the FTD family.  Either CBS or MSA have to be confirmed through a study of brain tissue.   Until then, the parkinsonisms are diagnosed as a "probable" and confirmed post humorously if the patient and family choose that path.

You will hear me refer to my diagnosis as Corticobasal Syndrome for the foreseeable future.  I will not be referring to my condition as Parkinson's Disease, since my not having typical PD has been the consensus view of several doctors, including our long term 10 year (now retired) Neurologist and up to and including our Movement Disorder Specialist we have been seeing for 5 months.

As I began, I reflected on these challenges arising along with the changes that come from the passing from this life to the next of our Mother.  Mother was supportive and compassionate.  Her beautiful life puts this in perspective and gives me reason to be so thankful for each hour, day, week, month and year.  Like Mom, I will continue to push forward learning and living with all the love for family and those experiences that I can find within the confinement of my disabilities.  What a beautiful life we have been given, and losing my mother gives me even greater reason to give thanks to God for all that we have and enjoy.

Thanks for caring and for reading.  --Patient-Online

DAT Scan Procedure in Parkinson's Plus

I am so grateful that we were able to gain approval for a DAT scan that I underwent yesterday. After eleven years, I think we are closing in on the most specific information regarding the degenerative brain disease that has changed my life so drastically.

A DAT scan is a nuclear study that allows images that show the areas of my brain that are being affected and causing the progressive disabilities that I suffer. It is as high tech as is available today.  I had an IV injection with a low degree of radiation that would be viewed as it moves through my brain.

Next week I will return to the Movement Disorder Specialist and learn what the findings of this study conducted at Loma Linda University Hospital indicate.  I will then share here on my blog what I can on that front. Thanks for your interest! -- Patient-Online