“It’s all in your head” is a film featuring a couple of people with MSA and a neurologist from Wayne State. The film is about the struggle for diagnosis. It has been accepted into this year’s Neuro Film Fest. The winning film will be announced in April. See:
Robin
This sweet article was published last week on the website of ITV, a news station in the UK. It’s about Paul Norman, a former teacher with multiple system atrophy (MSA), giving “one last incredible lecture” and his son making a film about his dad. The lecture is at the end of this article. If you go to the webpage, you can view “One Last Lecture” plus some photos of Paul Norman and his family.
Teacher who lost the ability to walk, talk and care for himself gives one last incredible lecture
ITV REPORT
15 March 2017 at 3:31pm
Multiple system atrophy results in parts of the brain and spinal cord gradually becoming more damaged over time. For more information click here. (http://www.nhs.uk/conditions/multiple-system-atrophy/Pages/Introduction.aspx)
A teacher who lost the ability to walk, talk and care for himself gave one last incredible lecture. Paul Norman, 58, was diagnosed with Multiple System Atrophy in 2012 forcing him to retire from his job as an English teacher.
His son Will, 22, began filming his every day life, eventually taking him back to Billericay School, Essex, where he taught English for 15 years.
Paul delivered an inspiring final assembly to a packed hall of his former students, written on his iPad and played out on a speaker.
He told his audience of his devastating struggle with his condition, even making them laugh with jokes about his “Stephen Hawking” voice.
He passed away ten months later, in April last year, before film and English student Will had finished the documentary ‘One Last Lecture’, featuring the scene.
Much to Will’s regret, his father never got to see the completed film, which
has been watched thousands of times since it was published online last month.
During the emotional lecture, he tells the students:
“I’m doing this partly to educate you about disability. People have a habit of assuming that because the body doesn’t function properly the brain doesn’t either. But in reality the vast majority of disabled people function just the same mentally with all of the same feelings and emotional needs as everyone else.”
“It is frustrating of course being trapped inside a body that doesn’t work and frustrating for the people that care for me. The other day I was moving my hand to scratch my nose and my carer thought I was signalling for my glasses.”
“And recently my mum asked me what I would like to eat from the fridge. I asked for tiramisu and she thought I said tin of soup. It’s very confusing.”
– PAUL NORMAN
And leaving them with some important life lessons, he adds:
“Teaching is really all about learning, and I have not stopped learning due to my disability. In fact some of the things I have learnt I will share with you, because being disabled as I am enables me to gain insight into what really matters in life.”
“When I see the atrocities that people commit I wonder how far human beings can have yet to evolve. War is pointless. There are many good things about this world and most people are good.”
“They should look at the positive side of people rather than the faults. Health is more important than money and the world is still a beautiful place.”
“Love is important and making this film has certainly brought me closer to my son William. Life is a challenge and one should strive to be a better person.”
“Although I am not religious I do believe that you should always treat others as you wish to be treated yourself.”
– PAUL NORMAN
Will started making the film in July 2014 after he visited his father from Billericay, Essex, and found he was wheelchair bound.
He choked on a piece of rice, forcing Will to perform the Heimlich manoeuvre to save his life, and he went back to his university that night and “broke down”.
University of Sussex student Will explained:
“That’s when I decided to start filming, just to capture memories and record how he is now, to hold on to the past.”
– WILL NORMAN
The pair grew close and for the first time Will saw the reality of his dad’s every day struggles. They gathered an assembly of former students and staff, and wrote the speech together.
Final year student Will added:
“It gave him and myself something that we could work on together.”
“He always liked to help me with my homework, and now we had a project to work on together.”
– WILL NORMAN
He completed filming in the summer of 2015, before Paul passed away in April last year, and he completed the movie in mid-January this year.
“One of the most depressing elements of the whole process really is dad never got to see the full finished film. That’s my fault really.”
“He was always asking me when it was going to be ready, but I was always striving for perfection, trying not to let him down with it. There is a feeling of emptiness that lots of people have seen it but he never got to see it.”
“He never got to be the movie star that he wanted to be.”
– WILL NORMAN
Here is the full text of Paul Norman’s lecture.
“Hello everyone. It’s good to see you all.
When William first suggested this to me I was a bit apprehensive because of the changes in me and looking at me is scary.
If I see people slipping away I will understand, however if I see anybody on their phones they will be confiscated.
The last time most of you saw me I was a different person. I now have Multiple System Atrophy, or MSA for short.
It affects every part of me and I mean every part, except my brain.
The worst things are not being able to talk or walk anymore and having to rely completely on other people.
So seeing all of you is very emotional. I cried enough last Christmas when I saw the wonderful video which some of you were in.
I’m doing this partly to educate you about disability.
People have a habit of assuming that because the body doesn’t function properly the brain doesn’t either.
But in reality the vast majority of disabled people function just the same mentally with all of the same feelings and emotional needs as everyone else.
It is frustrating of course being trapped inside a body that doesn’t work and frustrating for the people that care for me.
The other day I was moving my hand to scratch my nose and my carer thought I was signalling for my glasses.
And recently my mum asked me what I would like to eat from the fridge. I asked for tiramisu and she thought I said tin of soup. It’s very confusing.
I have friends and family who visit me regularly and have stood by me.
I also have a wonderful team of carers who go beyond the call of duty to help me and I can’t praise them enough.
I certainly couldn’t do that job if I was able to. They get paid little by society and they do 12 hour shifts.
Imagine 12 hours with me. It’s enough to drive anyone balmy.
I’m not saying they are balmy by the way.
Honestly, I feel like Stephen Hawking – except I have a better voice.
As you can see my sense of humour has not gone, in fact it helps me see the funny side even the moments when it is most difficult.
In fact inappropriate laughter is one feature of MSA, so I’ll laugh when it is most serious. That’s my excuse anyway.
For example I was in a church not long ago and I suddenly started laughing for no reason.
This would have been even more embarrassing had the congregation not known.
It was my uncle Bob’s funeral.
Teaching is really all about learning, and I have not stopped learning due to my disability.
In fact some of the things I have learnt I will share with you, because being disabled as I am enables me to gain insight into what really matters in life.
When I see the atrocities that people commit I wonder how far human beings can have yet to evolve.
War is pointless.
There are many good things about this world and most people are good.
They should look at the positive side of people rather than the faults.
Health is more important than money and the world is still a beautiful place.
Love is important and making this film has certainly brought me closer to my son William.
Life is a challenge and one should strive to be a better person.
Although I am not religious I do believe that you should always treat others as you wish to be treated yourself.
Now enough of this before I turn into John Lennon.
Although I can’t talk any more, I can answer you as long as the answer is yes or no.
If for example you ask if it’s nice to see you I can go like that [thumbs up].
Conversely, if you ask me if sitting in a chair all day is much fun, I will go like this [thumbs down].
So if I chat to you later I’m afraid we”ll have to play by those rules, and whatever you do, please don’t ask me two questions at once.
Finally, thank you all for coming. It means a lot to me. I’m sorry it’s a bit short, but at least it’s sweet.”
– PAUL NORMAN
You might like to know about these “disease maps”:
PSP
www.diseasemaps.org/progressive-supranuclear-palsy/
MSA
www.diseasemaps.org/multiple-systems-atrophy/
You can use the map to “connect with people who share your condition and help each other.” The maps are for those with chronic and rare diseases worldwide.
Robin
My longtime friend Dan Brooks in Riverside reactivated his blog “We Will Go On” in 2016 and moved it to wewillgoon.com. Dan’s blog has the tagline:
Parkinsonism: Hard to Diagnose. Harder to live with.
A blog by a patient with Parkinsonism Plus Syndrome.
In 2006, Dan was diagnosed with multiple system atrophy (MSA), corticobasal syndrome (CBS), and progressive supranuclear palsy (PSP). As time went on, MSA became the most likely. It seems that CBS has come back into the picture, however.
Dan recently posted about the confusion over whether he has Parkinson’s Disease, CBS, and MSA. I’ve copied his post below.
Robin
————————-
www.wewillgoon.com/2017/03/confusion-over-parkinsons-contrasted.html
Confusion Over Parkinson’s Contrasted with CBS and MSA
by Dan Brooks
Saturday, March 4, 2017
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
“Supranuclear gaze palsy” (SGP) refers to impairment of horizontal gaze and/or vertical gaze. This symptom denotes “dysfunction in the connections responsible for conducting voluntary gaze commands to the brainstem gaze centers.”
As many of you know, SGP is a classic clinical feature of progressive supranuclear palsy (PSP). In fact, it is part of the diagnostic criteria for PSP. However, this symptom is not specific to PSP and can occur in many other neurological disorders, including parkinsonian conditions.
In this Washington University (St. Louis) study, researchers examined the clinical records of 221 parkinsonian patients who had visited the movement disorders clinic and who had donated their brains for research. [By the way, Brain Support Network has been responsible for over 650 brain donations — quite a bit more than the WashU brain bank.]
Of the 221 parkinsonian brains in their brain bank, 27 had supranuclear gaze palsy noted in the clinical records. The confirmed diagnoses of these 27 were:
* progressive supranuclear palsy (9),
* Parkinson’s Disease (10),
* multiple system atrophy (2),
* corticobasal degeneration (2),
* Creutzfeld-Jakob Disease (1), and
* Huntington Disease (1).
The researchers also looked at the 14 brains donated of those with PSP in their brain bank. Nine of the 14 had clinical evidence of SGP but five did not.
Curiously, their brain bank doesn’t have many dementia with Lewy bodies (DLB) cases because their brain bank has a bias towards movement disorders rather than dementia.
This paragraph about MSA is interesting:
“In a study of oculomotor function in MSA, Anderson and colleagues suggest that the presence of clinically slow saccades, or moderate-to-severe gaze restriction, implies a diagnosis other than MSA. In contrast, our data indicate that SGP can be seen in patients who have subsequent autopsy-confirmation of MSA at a frequency similar to that seen in PD. Cognitive impairment is an exclusion criterion for the diagnosis of multiple system atrophy (MSA), according to the second consensus statement. However, some patients with pathologically confirmed MSA have been reported to have dementia. Cykowski and colleagues have reported that the presence of Lewy body-like inclusions in neocortex in MSA, but not hippocampal alpha-synuclein pathology, was associated with cognitive impairment. We suggest that the association of SGP with MSA in some individuals provides further evidence for cortical pathology.”
The authors point out that other studies show that 90% of those with CBD develop SGP.
SGP is also reported in other disorders such as spinocerebellar degeneration, amyotrophic lateral sclerosis, Whipple disease, and Niemann-Pick disease type C.
I’ve copied the abstract below.
Robin
———————–
Parkinsonism Relat Disord. 2017 Feb 24. [Epub ahead of print]
Pathologic correlates of supranuclear gaze palsy with parkinsonism.
Martin WR, Hartlein J, Racette BA, Cairns N, Perlmutter JS.
Abstract
INTRODUCTION:
Supranuclear gaze palsy (SGP) is a classic clinical feature of progressive supranuclear palsy (PSP) but is not specific for this diagnosis and has been reported to occur in several other neurodegenerative parkinsonian conditions. Our objective was to evaluate the association between SGP and autopsy-proven diagnoses in a large population of patients with parkinsonism referred to a tertiary movement disorders clinic.
METHODS:
We reviewed clinical and autopsy data maintained in an electronic medical record from all patients seen in the Movement Disorders Clinic at Washington University, St. Louis between 1996 and 2015. All patients with parkinsonism from this population who had subsequent autopsy confirmation of diagnosis underwent further analysis.
RESULTS:
221 unique parkinsonian patients had autopsy-proven diagnoses, 27 of whom had SGP documented at some point during their illness. Major diagnoses associated with SGP were: PSP (9 patients), Parkinson disease (PD) (10 patients), multiple system atrophy (2 patients), corticobasal degeneration (2 patients), Creutzfeld-Jakob disease (1 patient) and Huntington disease (1 patient). In none of the diagnostic groups was the age of onset or disease duration significantly different between cases with SGP and those without SGP. In the PD patients, the UPDRS motor score differed significantly between groups (p = 0.01) with the PD/SGP patients having greater motor deficit than those without SGP.
CONCLUSION:
Although a common feature of PSP, SGP is not diagnostic for this condition and can be associated with other neurodegenerative causes of parkinsonism including PD.
Copyright © 2017 Elsevier Ltd. All rights reserved.
PMID: 28256434 (see pubmed.gov for this abstract only)
