Corticobasal Syndrome (CBS) is not Parkinson’s Disease. It is a Prime of Life Brain Disease. Corticobasal Syndrome (CBS) is the clinical diagnosis of Corticobasal Degeneration (CBD), which is finalized at autopsy. CBS has no known cause, treatment, or cure. It affects nerve cells that control walking, balance, mobility, vision, speech and swallowing. It is terminal and the life expectancy is 6-8 years. Don is in year six and time is of the essence.
Corticobasal Syndrome (CBS) Definition (Click Here)
Corticobasal Syndrome (CBS) is a progressive brain disease with no known cause or cure. It affects areas of the brain controlling limb movement, speech, and other movement functions.
CBD is rare, affecting an estimated 2,000-3,000 people in the United States, of whom only 500-700 are diagnosed. Symptoms begin, on average, when an individual is in the early 60's but may start as early as in the 40's. These include:
· Stiffness, shakiness, jerkiness, slowness, and clumsiness in either the upper or lower extremities
· Difficulty with speech generation (aphasia)
· Difficulty with swallowing (dysphasia)
· Difficulty with articulation (dispraxia)
· Difficulty controlling the muscles of the face and mouth (dysarthria)
· Walking and balance difficulty
· Asymmetric onset of symptoms (occurring on one side of the body first then gradually moving to the other side)
· Memory or behavior problems
CBD is rare, affecting an estimated 2,000-3,000 people in the United States, of whom only 500-700 are diagnosed. Symptoms begin, on average, when an individual is in the early 60's but may start as early as in the 40's. These include:
· Stiffness, shakiness, jerkiness, slowness, and clumsiness in either the upper or lower extremities
· Difficulty with speech generation (aphasia)
· Difficulty with swallowing (dysphasia)
· Difficulty with articulation (dispraxia)
· Difficulty controlling the muscles of the face and mouth (dysarthria)
· Walking and balance difficulty
· Asymmetric onset of symptoms (occurring on one side of the body first then gradually moving to the other side)
· Memory or behavior problems
Corticobasal Syndrome (CBS) Description (Click Here)
Corticobasal Syndrome is considered a tauopathy. The neurodegenerative symptoms are due to the abnormal misfolding and clumping of the tau protein within neurons, which causes progressive cell death. It is called a 4-repeat tauopathy.
The following statistics are for people in the United States and are essential to understanding what has happened in Don's case:
Alzheimer's disease affects about 5.8 million
Parkinson's affects about 1 million
Progressive Supranuclear Palsy (PSP) affects about 5 or 6 per 100,000
Corticobasal Syndrome (CBS) is very rare, with about five people per million. There has not been a proper study, but CBS is believed to affect about 10% of the rate of PSP.
Some of Alzheimer's causes are related to amyloid plaques and tau. Prior trials have focused on medication addressing the amyloid plaques with little success. The focus has now shifted to tau, but scientists need a disease that is a pure tauopathy.
The following statistics are for people in the United States and are essential to understanding what has happened in Don's case:
Alzheimer's disease affects about 5.8 million
Parkinson's affects about 1 million
Progressive Supranuclear Palsy (PSP) affects about 5 or 6 per 100,000
Corticobasal Syndrome (CBS) is very rare, with about five people per million. There has not been a proper study, but CBS is believed to affect about 10% of the rate of PSP.
Some of Alzheimer's causes are related to amyloid plaques and tau. Prior trials have focused on medication addressing the amyloid plaques with little success. The focus has now shifted to tau, but scientists need a disease that is a pure tauopathy.
There are billions of dollars to be made for a pharmaceutical company that offers treatment for Alzheimer's Disease. Biogen is an international biotechnology company that is interested in cashing in on this opportunity, but they need a way to test their medication. Progressive Supranuclear Palsy (PSP) is a tau disease with enough patients to form a clinical trial, which would allow scientists to study their treatments. Biogen enrolled over 450 PSP patients in their own trial called PASSPORT.
Don, Biogen, and Corticobasal Syndrome (CBS) (Click Here)
But where does Corticobasal Syndrome (CBS) fit?
CBS is also a tau disease, but it is much rarer than PSP. And, this is where our superhero, Dr. Boxer, enters. Dr. Boxer decided to put together a basket trial. Basket trials are used in the study of cancer but not in the study of neurodegenerative disorders. A basket trial is where you take a group of rare, but similar diseases and see if a medication can help.
Don was part of the TauBasket Trial that looked at four different but related, neurodegenerative disorders. The trial was to have eight patients from each disease for a total of 32 patients. They were only able to fill 22 slots. The trial was double-blind and placebo-controlled, which means neither the physicians nor the patients knew who was receiving the drug (both are blind), and that some patients would receive the drug, and some would receive nothing to check for the placebo effect.
The CBS section of the TauBasket Trial was able to fill all eight slots. Six patients would receive the medication, and two would not. After six months, those that did not receive the drug would be granted access for six treatments. Don immediately began to see changes. Please see our timeline of videos. The changes were cumulative, and every month unlocked a surprise. Dr. Boxer thought there was merit in asking UCSF and Biogen to continue the trial, and they granted the extension. Don was fortunate to receive the medication for a full year, and then our world collapsed.
Remember, the pharmaceutical goal is a cure for Alzheimer's, and the pharmaceutical company is Biogen. The PASSPORT trial did not meet their end-point goals, and on December 13, 2019, that trial was canceled. Biogen also stopped all access to their medication, BIIB092, Gosuranemab, for the TauBasket Trial. Biogen refuses to acknowledge the TauBasket Trial because it was investigator sponsored, and in fact, a new patient had received her first medication on December 12, 2019.
Dr. Borderlon called Biogen on December 16, 2019, asking for continued access to the medication. They said they were not releasing any medicines to the clinical trial sites. Dr. Devaney also contacted them on December 19, 2019, and explained the success that Don was having with the medication, and they denied access because it wasn't their trial.
We have tried the following appeals to Biogen under The-Right-To-Try Act:
February 12, 2020, Dr. Borderlon sent an email at 1:34 PM, and at 2:05 PM, they denied.
February 19, 2020, I also sent an appeal, they denied.
February 25, 2020, Dr. Devaney sent an email; they denied it.
March 2, 2020, Dr. Borderlon examined Don, and she believes there has been a 10-15% decline since her examination on December 16, 2019.
April 23, 2020, Pat Bradley, Don's mother-in-law requested that they look at the evidence.
May 02, 2020, Monica Mann, PhD, Head of Global Pipeline-Worldwide Medical from Biogen, denied access based upon the Passport Study.
Don must be granted access to BIIB092, Gosuranemab, immediately.
CBS is also a tau disease, but it is much rarer than PSP. And, this is where our superhero, Dr. Boxer, enters. Dr. Boxer decided to put together a basket trial. Basket trials are used in the study of cancer but not in the study of neurodegenerative disorders. A basket trial is where you take a group of rare, but similar diseases and see if a medication can help.
Don was part of the TauBasket Trial that looked at four different but related, neurodegenerative disorders. The trial was to have eight patients from each disease for a total of 32 patients. They were only able to fill 22 slots. The trial was double-blind and placebo-controlled, which means neither the physicians nor the patients knew who was receiving the drug (both are blind), and that some patients would receive the drug, and some would receive nothing to check for the placebo effect.
The CBS section of the TauBasket Trial was able to fill all eight slots. Six patients would receive the medication, and two would not. After six months, those that did not receive the drug would be granted access for six treatments. Don immediately began to see changes. Please see our timeline of videos. The changes were cumulative, and every month unlocked a surprise. Dr. Boxer thought there was merit in asking UCSF and Biogen to continue the trial, and they granted the extension. Don was fortunate to receive the medication for a full year, and then our world collapsed.
Remember, the pharmaceutical goal is a cure for Alzheimer's, and the pharmaceutical company is Biogen. The PASSPORT trial did not meet their end-point goals, and on December 13, 2019, that trial was canceled. Biogen also stopped all access to their medication, BIIB092, Gosuranemab, for the TauBasket Trial. Biogen refuses to acknowledge the TauBasket Trial because it was investigator sponsored, and in fact, a new patient had received her first medication on December 12, 2019.
Dr. Borderlon called Biogen on December 16, 2019, asking for continued access to the medication. They said they were not releasing any medicines to the clinical trial sites. Dr. Devaney also contacted them on December 19, 2019, and explained the success that Don was having with the medication, and they denied access because it wasn't their trial.
We have tried the following appeals to Biogen under The-Right-To-Try Act:
February 12, 2020, Dr. Borderlon sent an email at 1:34 PM, and at 2:05 PM, they denied.
February 19, 2020, I also sent an appeal, they denied.
February 25, 2020, Dr. Devaney sent an email; they denied it.
March 2, 2020, Dr. Borderlon examined Don, and she believes there has been a 10-15% decline since her examination on December 16, 2019.
April 23, 2020, Pat Bradley, Don's mother-in-law requested that they look at the evidence.
May 02, 2020, Monica Mann, PhD, Head of Global Pipeline-Worldwide Medical from Biogen, denied access based upon the Passport Study.
Don must be granted access to BIIB092, Gosuranemab, immediately.
© COPYRIGHT 2020. All rights reserved. Unless attribution otherwise noted.
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THANK YOU“I would like to thank all of you from the bottom of my heart that took the time to read this site and those that took action concerning my situation with CBS. The clinical trial has helped my quality of life. My goal has always been to help science to help others with this terrible disease. God bless and thank you all.”
- Don Sladek |
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