Category Archives: MSA

Concomitant PSP and MSA (4 Cases)

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This recently-published article is about one case of concomitant PSP and MSA in the Queen Square Brain Bank in the UK. (That’s one out of 120 cases of path-confirmed PSP.) And the article mentions that there are three other (independent) cases. One of these three is a Mayo Jax case; it was one case out of 290 path-confirmed PSP cases. So, that answers the question as to whether this is even possible!

Here are the highlights:

* “The apparent rarity of cases of coexistent PSP and MSA initially suggests they might be due to mere coincidence, rather than the result of any biological overlap.”

* “Grading of PSP tau pathology provided a score of 6, consistent with moderately severe tau pathology typical of Richardson’s syndrome [29].” [Robin’s note: this indicates that the RS form of PSP has more severe tau pathology than the other common form, PSP-P.]

* “Nevertheless, taking into consideration the rarity of MSA and PSP, in addition to the small number of cases that come to autopsy, the findings of 4 cases reported to-date is higher than expected.”

* “Whether these cases represent a pre-clinical stage of MSA, or simply a biological coincidence is yet unknown. The occurrence of pre-symptomatic MSA should be confirmed in prospective studies in which detailed neurological evaluation has been performed in the final months of life.”

* “Clinically our patient fulfilled the NINDS-SPSP operational criteria for the diagnosis of probable PSP and the only feature that might have suggested MSA was the urinary incontinence which started 20 years before death and resolved. The lack of overt cerebellar features of MSA is consistent with mild olivo-ponto-cerebellar pathology, whereas the prevailing parkinsonian syndrome is in line with more severe striato-nigral-degeneration, or the baseline PSP pathology.”

* “The rarity of pathologically proven cases of combined PSP and MSA does not provide sufficient evidence to confirm a common pathogenic mechanism for both diseases, but our findings suggest that systematic screening for PSP and MSA changes in atypical parkinsonian patients and control brain bank donors is warranted.”

The abstract follows.

Robin

Neuroscience Letters. 2009 Oct 14. [Epub ahead of print]

Concomitant progressive supranuclear palsy and multiple system atrophy: More than a simple twist of fate?

Silveira-Moriyama L, González AM, O’Sullivan S, Williams DR, Massey L, Parkkinen L, Ahmed Z, de Silva R, Chacón JR, Revesz T, Lees AJ, Holton JL.
Reta Lila Weston Institute for Neurological Studies, UCL Institute of Neurology, London, UK.

Progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) are both rare neurodegenerative diseases.

In the Queen Square Brain Bank, from 2001 to 2008, we received 120 cases of pathologically confirmed PSP and 36 of MSA, and one had concomitant PSP and MSA pathology.

The clinical symptoms in this case were compatible with PSP and did not predict the dual pathology.

The growing number of collective case reports, including the one reported here, might suggest an increased prevalence of concomitant PSP and MSA than what would be expected by chance.

PubMed ID#: 19835934

Concommitant PSP and MSA (4 cases worldwide)

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This recently-published paper addresses the question of whether PSP and MSA can co-exist.  The answer is “yes” but it’s quite rare.

There is one case of concomitant PSP and MSA in the Queen Square Brain Bank in the UK.  (That’s one out of 120 cases of path-confirmed PSP.)  And the article mentions that there are three other (independent) cases.  One of these three is a Mayo Jax case; it was one case out of 290 path-confirmed PSP cases.  So, that answers the question as to whether this is even possible!

Here are the most interesting excerpts:

“The apparent rarity of cases of coexistent PSP and MSA initially suggests they might be due to mere coincidence, rather than the result of any biological overlap.”

“Grading of PSP tau pathology provided a score of 6, consistent with moderately severe tau pathology typical of Richardson’s syndrome [29].”  [Robin’s note:  this indicates that the RS form of PSP has more severe tau pathology than the other common form, PSP-P.]

“Nevertheless, taking into consideration the rarity of MSA and PSP, in addition to the small number of cases that come to autopsy, the findings of 4 cases reported to-date is higher than expected.”

“Whether these cases represent a pre-clinical stage of MSA, or simply a biological coincidence is yet unknown. The occurrence of pre-symptomatic MSA should be confirmed in prospective studies in which detailed neurological evaluation has been performed in the final months of life.”

“Clinically our patient fulfilled the NINDS-SPSP operational criteria for the diagnosis of probable PSP and the only feature that might have suggested MSA was the urinary incontinence which started 20 years before death and resolved. The lack of overt cerebellar features of MSA is consistent with mild olivo-ponto-cerebellar pathology, whereas the prevailing parkinsonian syndrome is in line with more severe striato-nigral-degeneration, or the baseline PSP pathology.”

“The rarity of pathologically proven cases of combined PSP and MSA does not provide sufficient evidence to confirm a common pathogenic mechanism for both diseases, but our findings suggest that systematic screening for PSP and MSA changes in atypical parkinsonian patients and control brain bank donors is warranted.”

Check out the abstract or full paper for more.  The abstract follows.

Robin

————————————

Neuroscience Letters. 2009 Oct 14. [Epub ahead of print]

Concomitant progressive supranuclear palsy and multiple system atrophy: More than a simple twist of fate?

Silveira-Moriyama L, González AM, O’Sullivan S, Williams DR, Massey L, Parkkinen L, Ahmed Z, de Silva R, Chacón JR, Revesz T, Lees AJ, Holton JL.

Reta Lila Weston Institute for Neurological Studies, UCL Institute of Neurology, London, UK.

Progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) are both rare neurodegenerative diseases.

 

In the Queen Square Brain Bank, from 2001 to 2008, we received 120 cases of pathologically confirmed PSP and 36 of MSA, and one had concomitant PSP and MSA pathology.

The clinical symptoms in this case were compatible with PSP and did not predict the dual pathology.

The growing number of collective case reports, including the one reported here, might suggest an increased prevalence of concomitant PSP and MSA than what would be expected by chance.

PubMed ID#: 19835934

 

“Clinical outcomes” paper – full paper available

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Earlier in the year we posted about the O’Sullivan “Clinical outcomes” paper on PSP and MSA.  This is one of the most important papers published to date on these two disorders.

The UK researchers analyze the clinical records (to determine clinical milestones and survival time) of 110 pathologically-confirmed PSP cases and 83 path-confirmed MSA cases (all from Queen Square Brain Bank).

This paper is now available online for free.  I’ve noticed that articles don’t always remain free, so if you want a copy I suggest you save it to your hard drive now.

Here’s a link to the article:

Brain. 2008 Apr 2
Clinical outcomes of progressive supranuclear palsy and multiple system atrophy.
O’Sullivan SS, Massey LA, Williams DR, Silveira-Moriyama L, Kempster PA, Holton JL, Revesz T, Lees AJ.

http://brain.oxfordjournals.org/cgi/content/full/131/5/1362 –> HTML version

http://brain.oxfordjournals.org/cgi/reprint/131/5/1362 –>
PDF version

Update:  local support group member Ted has explained some of the statistics in this paper.  See our post here about the key statistics.

Robin

Ataxia, OPCA, and MSA-C Webinar with Susan Perlman, MD – Notes

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Tonight’s webinar with UCLA neurologist Dr. Susan Perlman was terrific!

Lily, in our local support group, said that Dr. Perlman provided the first diagnosis of MSA to Lily’s mother.

Dr. Perlman’s powers of explanation are impressive.  She seems very interested in educating patients.  She thinks patients must be aggressive about getting education and information from their MDs.  She had interesting things to say about stem cell therapy in China.  My notes from the webinar are below.  If you attended, please add to or correct anything I’ve said below.

Robin

——————–

Robin’s Notes from

Susan Perlman, MD, UCLA Ataxia Clinic Director
Topic:  Ataxia, OPCA, and MSA-C
September 17, 2009

Ataxia is a lack of coordination of muscle movements

It is caused by problems in the brain or nerves

Problems in the balance center of the brain (cerebellum) –> “cerebellar ataxia”

Other types:  sensory ataxia, vestibular ataxia

The causes are different of the different types of ataxia

Tonight we are talking about cerebellar ataxia

Image of the brain found here:
content.answers.com/main/content/img/McGrawHill/Encyclopedia/images/CE093200FG0010.gif

It’s important to know the brain areas and be able to speak with your MD about them.

Types of cerebellar ataxia include:
* Pure cerebellar ataxia
* Cerebellar ataxia with problems in the brainstem = OPCA (olivo ponto cerebellar atrophy)
* Cerebellar ataxia with problems in the spinal cord = spinocerebellar ataxia
* Cerebellar ataxia with problems that look like Parkinson’s = MSA or PSP (also called Parkinson’s Plus syndromes)
* Cerebellar ataxia with problems that look like Parkinson’s and have memory problems = PSP

A neurological exam will tell you what type you have.  This is important because the causes are different, and the treatment may be different.  Some causes are genetic (inherited), some are not.

MDs usually refer to cerebellar ataxia and OPCA as the same thing.

Two types of MSA:  MSA-C (cerebellar) and MSA-P (parkinsonism)

PSP and CBD have problems of dementia (memory and thinking).

Educational organizations:
National Ataxia Foundation – ataxia.org
Friedreich’s Ataxia Research Alliance – curefa.org
Cooperative Ataxia Group – cooperative-ataxia-group.org
WeMove – wemove.org
CurePSP – curepsp.org

The UCLA Ataxia Clinic has seen 1565 patients in the past 10 years.  The clinic evaluates about 150 new patients each year.  40% of cases have either FA or a known genetic (inherited) ataxia.  So 60% of cases have an unknown gene or non-genetic cause.  (24% have pure cerebellar ataxia.  The next largest group is OPCA.  The next largest group is MSA.  The next largest group is PSP.)  The Ataxia Clinic has a DNA bank and works with two tissue banks.

Questions our patients ask:
* what do I have?
* what is the cause?
* are my children at risk?
* can it be cured?
* will it get worse?
* how bad will it get?  how soon?
* is there any research?

Patients with ataxia coming on after 50 years of age might be misdiagnosed and in reality have an atypical parkinsonian disorder or Parkinson’s Plus.

Diagnosis can be made by
* symptoms, signs, rate of progression
* lab tests (MRI may be helpful)
* response to treatment

Symptoms that may occur in late-onset ataxia – usually from the cerebellum:
* staggering, wide-based walk (which means there is a risk of falling)
* incoordination of fine hand movements (which can include tremors with hand use)
* slurred or uneven speech
* choking
* jumpy, double vision, eye “tremors” (nystagmus)
* mild bladder control problems

Symptoms from the brainstem (midbrain, pons, medulla)
* reduced or slowed eye movement
* dizziness
* speech difficulties
* swallowing difficulties; risk of choking
* tightness of speech or breathing (called stridor)
* neck weakness
* spasticity of arms and legs

“Hot cross bun” sign – often appears on an MRI in MSA
Increased white shadows

In PSP on an MRI scan, the midbrain is atrophied

Symptoms from the basal ganglia (Parkinson’s symptoms):
* stiffness or rigidity of muscles
* freezing or slowed movements
* soft speech

Autonomic symptoms from the autonomic nervous system:
* orthostatic hypotension (significant fall in BP when standing), causing dizziness, blurred vision, lightheadedness
* trouble emptying urine in bladder
* constipation
* male sexual dysfunction

Other symptoms that may sometime come on before the ataxia or years after the ataxia comes on:
* bladder urgency, often at night
* breathing problems at night, sleep apnea
* parasomnias, eg sleep talking, leg movements during sleep, vivid dreams, nightmares
* fatigue, slowed thinking, dementia
* depression or anxiety
* weight loss, bedsores, infection.  These complications can lead to death.

In AD (Alzheimer’s Disease), there is atrophy in the front of the brain (in the cerebral cortex).

In CBD, there is atrophy in the back of the brain.

Rate of progression of symptoms can help determine the cause or uncover complications:
* Acute/rapid (overnight) – traumatic, vascular, metabolic/toxic, infectious, inflammatory causes
* Sub-acute (weeks or months) – post-traumatic, metabolic/toxic, infectious, neoplastic
* Slowly progressive (months to years) – atypical parkinsonism disorders fall into this category of progression

Everyone deserves testing for:
* which part of the brain is involved (MRI)
* medical factors (prior illnesses, toxic exposures, thyroid disease)
* immune or paraneoplastic
* possible genetic disease (SCA6 and FXTAS screening should always be given)

Why is there no family history of ataxia?
* no one asked
* information is unavailable (such as through adoption, loss of contact, non-cooperation, paternity issues)
* prior generations may have died before showing symptoms
* maybe it really is non-genetics
* a genetic counselor can help determine possible causes

Approach to late-onset predominantly cerebellar syndromes:
* use the history and neurological exam, imaging or other diagnostic tests
* detailed family and environmental history
* rule out genetic causes
* work with MD to minimize risks

Differential diagnosis:  late-onset ataxia vs. Parkinson’s Plus
Late onset ataxia:  slow progression
OPCA: slow progression
FXTAS: slow progression;
MSA-C: moderate progression (less than 10 years to wheelchair, especially if they have symptoms of PD)
MSA-P: moderate
PSP: moderate
CBD: moderate
PD:  slow progression

Many times an MRI can can help diagnose these disorders

Environmental causes – pesticides, chemicals, vascular (stroke)

MSA and PSP have a possible response to levodopa.  CBD has a poor response to levodopa.

MSA – most common form of Parkinson’s Plus or atypical PD
* all forms of MSA have symptoms of ataxia, Parkinson’s, and autonomic failure
* 80% start with parkinsonian symptoms, 20% start with cerebellar ataxia

25% of patients with sporadic cerebellar ataxia go on to develop MSA, within 5 years, especially if over 50 years.

The term OPCA has been used interchangeably for MSA-C but it is not the most accurate term.

18F-fluorodopa PET scan can be helpful in separating MSA for pure cerebellar ataxia.

When is MSA not the likely diagnosis:
* onset after age 75
* family history of ataxia or Parkinson’s
* classic pill-rolling rest tremor
* chorea (involuntary twitches)
* slowed or limited eye movement
* dementia

Treatment of MSA:
* medications used to treat the symptoms become less effective as the disease progresses
* PT and OT always help
* levodopa and dopamine agonists are somewhat helpful for the slowness and stiffness of MSA but they may make low BP worse.
* OH can be treated with medications
* incontinence may be treated with bladder medication or catheterization
* constipation may be improved with dietary fiber or laxatives
* male impotence can be treated
* speech therapy may be able to help swallowing
* sleep disturbances may respond to medication or breathing devices

Once the causes are known, disease-modifying treatment and ultimately a cure will be possible

Neural replacement therapy (stem cell therapy and growth factor therapy) is under development

Active research study in South Korea on stem cell therapy for MSA:  NCT00911365

She does NOT recommend any other stem cell programs at this time

Another useful website:  hopes.stanford.edu/treatmts/index/trhome.html
Designed for Huntington’s Disease but has very useful info about medications and nutritional supplements

ClinicalTrials.gov studies on MSA:
* stem cell trial in South Korea
* rasagiline trial
* L-DOPS augmentation
* others

Questions and Answers (by Dr. Perlman, unless indicated):
[Robin’s note:  I’ve grouped the questions by topic — ataxia, OPCA, SCA, MSA-C, treatment, caregiving and support, and education.]

ATAXIA
Q:  Exactly what is ataxia?
A:  Thinning of nerve cells of the cerebellum are usually seen on an MRI.  There are many types of cerebellar ataxia (as we learned in the presentation).

Q:  Is ataxia hereditary?  Will my children get this?  I am age 70, and have just been diagnosed with ataxia.
A:  As this person is over 70, it’s unlikely that this is genetic.  It’s unlikely it will turn in to MSA.  She should have an MRI to look for signs of MSA.  She should have testing for the two genes discussed earlier.  Perhaps the cause of this person’s ataxia is nutritional imbalance, numbness of feet (causing imbalance), etc.

Q:  My husband was diagnosed with ataxia.  What type of ataxia is it?
A:  It’s important to speak with the MD as to what evidence he/she has of the diagnosis.  Even a simple brain MRI can help identify what type of ataxia it is.  Blood work is helpful to know about vitamin deficiencies, poisons, etc.  The patient must be proactive.  The patient must know the terminology.  The patient must actively pursue information and knowledge.  40% of people have genetic or inherited ataxias.

Q:  I saw a general neurologist and got an ataxia diagnosis.
A:  A general neurologist thinks of disorders associated with older people.  A general neurologist may never have seen anyone with ataxia.  A common misdiagnosis for someone over 50 or 60 is something other than ataxia.  A general neurologist may miss blood pressure problems.  If you have any question about your diagnosis, you should ask to be referred to an ataxia specialist or a movement disorder specialist.

The most common reasons for a misdiagnosis are:
* Inexperience on the part of the neurologist
* Tendency for the MD to think of the common things, and not the rarer things
* The MD not thinking of diagnostic tests that can be done

Q:  [didn’t catch it] A:  80% of what Dr. Perlman knows about ataxia she has learned from patients.

OPCA
Q:  Is all sporadic OPCA MSA-C?  If so, why is the term OPCA still used?
A:  The diagnosis OPCA came from looking at the brain on autopsies and on MRI scans.  Atrophy was seen in certain areas of the brain.  OPCA is basically an MRI diagnosis.  OPCA isn’t the final diagnosis.  25% of those people with symptoms of cerebellar ataxia will go on to develop MSA.  MSA-C is a separate condition.  There may be common causes.  The two terms are not interchangeable.

We don’t yet know how to identify these people in advance.  Part of the research at UCLA is trying to understand why the 25% go on to develop MSA and why the other 75% do not.

SCA
Q:  I have SCA6.  What treatment is there?  What about gabapentin?  What about creatine?
A:  There are many medications used to treat the symptoms of ataxia and eye tremors (nystagmus).  Gabapentin can help with nystagmus.  Creatine may protect nerves from deterioration.  Creatine is an anti-oxidant.

The Stanford HOPES website has a complete list of supplements.  Supplements or medications should be added one at a time.

Q:  What is the status of gene therapy trials with SCA?
A:  We are about 5 years away from human trials with gene therapy in SCA.  Two avenues:  blocking the bad gene and stimulating the good gene.  To block the bad gene, we are using RNAi (a technique).

We are about one year away from human trials for another technique (histone acetylase inhibitors or something like that).

Q:  What is the prognosis for SCA1?  My son saw improvement with Chinese stem cell therapy.
A:  SCA-type 1 slowly progresses with time.  Most people with the genetic ataxias will progress over 15-25 years until they are completely disabled.  The earlier a genetic ataxia comes on the more rapidly it progresses.   For example, someone who gets SCA-type 1 in their 20s, might be disabled in their 30s.

She’s seen several cases who have been to China for stem cell therapy.  The organization in China is not forthcoming.  They have published nothing in reputable journals.  They do not follow up with people so how can this organization know how these people are doing?  Nerves grow and become stronger slowly.  Don’t expect to see improvements in stem cell therapy for at least 6 months.  Any improvements before six months are not from the stem cell therapy.  The treatment in China includes other things such as acupuncture, Chinese herbs, etc.  The benefits from that other treatment should decline upon leaving China.  The Chinese organization does admit that those with genetic ataxias don’t derive as much benefit from the second and third rounds of stem cell treatment in China as they do from the first round.

Q:  I have SCA-type 1.  Is this the same as OPCA?
A:  SCA-type 1 is a genetic form of ataxia.  SCA-type 1 on an MRI shows thinning of the cerebellum.  This is different from OPCA.  OPCA is a general term.  OPCA can have multiple causes.

MSA-C
Q:  I have MSA-C.  What is the latest research being done to treat and cure this disorder?
A:  The oldest and largest research project to date is a natural history study being done in Europe and the US.  In the US, the leader is Dr. Sid Gilman at the University of Michigan.  The focus is finding the cause of MSA.  We must first know the cause before we can know the cure.

See clinicaltrials.gov for MSA trials.  The South Korean stem cell trial’s early results look very good.  Work with your treating neurologist to find a promising study.  Even if there’s no study near you, many of the drugs being studied in the trials can be tried off-label, under the direction of your physician.

Note that when you look on clinicaltrials.gov for multiple system atrophy, you will also see trials for multiple sclerosis (usually at the bottom of the list).

Q:  I have MSA-C.  What is 4-AP and will this help?
A:  4-AP is being developed by a company in Ireland for use in multiple sclerosis.  This drug may be helpful for nystagmus.  It is available now through compounding pharmacists (over the counter) but it should be used under an MD’s guidance.  A serious side effect is epileptic seizures.  This medication is similar to gapapentin.  Hopefully this will be available by prescription soon.  The compounded form available now may or may not be covered by insurance.

TREATMENT
Q:  Anything new about stem cell therapy?
A:  There is now active research in the US on stem cells.  We aren’t ready in the US for the sort of clinical trials that are going on in South Korea.  You shouldn’t pay $30K for unproven stem cell therapy.  Most funded research in the US allows patients to participate without charge.

She’s in favor of exploring alternative therapies — herbs, homeopathic therapies.  These should be monitored by an MD.  If in 6-12 months you don’t feel better from a therapy, you should discontinue and move on.

Q:  What medications are used to treat depression?
A:  Most anti-depressants affect the serotonin pathways.  Others affect the adrenaline pathways.  Lexapro is a very good drug but it doesn’t affect serotonin or adrenaline.  If Lexapro doesn’t work, she tries Wellbutrin, Cymbalta, etc.

Q:  I frequently undergo IVIG treatment and it seems to help my ataxia.
A:  IVIG is immunoglobulin given intravenously.  Perhaps this person’s ataxia is caused by the immune system.  When the immune system is not involved, IVIG should not help.  The immune system isn’t involved in a genetic form of ataxia.

Sometimes chemotherapy drugs are tried in those where the immune system is involved.

CAREGIVING AND SUPPORT
Q:  What can I do to help my mother accept her condition?
A:  Acceptance is a lot to ask of someone diagnosed with a neurodegenerative illness where the cause is unknown and there is no cure.  There are amazingly strong individuals who are able to accept the diagnosis and take advantage of research.  The encouragement of others with the same condition can be a strong motivation.

You have to encourage and support.  Start with little steps — try PT, try exercising together.

MDs shouldn’t say “there’s nothing can be done.”  There is a lot that can be done.

Q:  It’s expensive to hire caregivers for someone.  Is there help?
A:  In short, no.  Resources that are available include IHSS (in-home supportive services) in CA.  There are some respite care grants.  A few insurance companies are starting to cover nursing home care.  Hospice care can be applied for and provides various levels of care in the home.  It’s impossible to predict when someone is within six months of death.  Hospice care can be renewed.

With proper care, we can put people on plateaus for long periods of time.

EDUCATION
Q:  How do I get a copy of the book you wrote?
A:  The National Ataxia Foundation sponsored the development of a booklet on ataxia.  It is written for physicians about diagnosing ataxia.  Patients or family members will find it readable.  Or take the booklet to an MD, PT, or OT and ask for an explanation.

For webinar participants, the National Ataxia Foundation will reduce the price and offer free shipping of this booklet.  Send an email to Susan at the National Ataxia Foundation, [email protected], with “Dr. Perlman’s Book” in the subject line to take advantage of this offer.

Q:  Can I get a copy of this presentation?
A (by the moderator):  CurePSP is trying to make this presentation available.  Maybe it can be emailed out.  It will be available on the psp.org website later this year or early next year.

Q:  Next webinars?
A:  The next five will all be movement disorder specialists:
10/8 – Dr. Neal Hermanowicz, from UC Irvine – Fundamentals and diagnosis of PSP, CBD, and MSA
10/22 – Dr. Robert Hutchman – Interventions
11/5 – Dr. Yvette Bordelon – Latest research
11/19 – Dr. Lawrence Golbe – Research for dummies
12/3 – Dr. Panel of movement disorder specialists, led by Dr. Jerome Lisk

Making the Most of Life with a Parkinson-Plus Syndrome

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These are the notes I took during the webinar tonight with Dan Brooks, diagnosed with Parkinson’s Plus. Dan’s wife Karrie was also on hand to answer questions. I thought this webinar was wonderful.

I’ve divided Dan’s presentation into several parts — Symptoms and Treatment, Four Disorders, and Making the Most of Life. After the presentation is the Q&A. I slightly changed the order of the questions and answers below. I could hear nothing that the webinar moderator said which meant that I couldn’t hear some of the questions. Many of the answers given can be taken more generally than one specific disorder.

Excluded in these notes are the introductory remarks made by the webinar moderator Larry Schenker, Janet’s Edmunson’s remarks about CurePSP’s vision and mission, and Kathleen Speca’s remarks about fundraising for CurePSP.

Here are my notes. If you took notes, please share!

INTRODUCTION: by Karrie Brooks

Dan’s PSP symptoms began in 2005

The Dan of today is very different to look at and to talk to then the Dan of 5 years ago. However, he is still the Dan Karrie loves and admires.

PRESENTATION: “Making the Most of Life with a Parkinson-Plus Syndrome” by Dan Brooks

Married almost 30 years
Three adult sons
Career included roles as principal and assistant superintendent in public schools
Guitarist, singer, songwriter
Karrie is 50. Dan is 54.

One goal of this webinar is to give encouragement and practical ideas for keeping your life moving forward, with purpose

SYMPTOMS AND TREATMENT

Dan’s diagnosis:
* extrapyramidal disorder
* involuntary movement disorder
* Parkinson-Plus syndrome

Dan’s symptoms:
* walking gait, coordination and balance issues
* swallowing difficulty
* speech troubles
* cognitive disorder
* eye movement problems
* trunk movement and head tremors
* arm, hand and leg tremors
* dystonic movements
* autonomic signs
* depression

June 2005: noticed a head tremor and hands trembling; stiffness in feet and ankles when rising from chair; eye pain; light sensitivity; sway and balance problems when standing

Fall 2005, Neurologist visit: MRI with no significant findings; blood tests; prescribed levodopa/carbidopa and eventually added Comtan; helped to calm head tremor and left arm trembling for up to two hours

February 2006: went out on disability – stopped working as administrator; walked with a cane; speech was worsening; eye movements – turning up (under lids) and crossed; trouble coordinating walking gait; slowed responses to questions; stopped driving because of balance, coordinator, and reflex issues in addition to tremors that ran through my whole body

More diagnostic tests: PET scan showed mild atrophy in left frontal lobe; spinal tap; neuropsychologist did cognitive testing revealed mild cognitive decline affecting short term memory and language, with a potential for dementia

May 2006: permanent disability; no more driving; Parkinson-Plus syndrome; possible PSP or Shy Drager (MSA)

Today: using a wheelchair more and more for trips to store; scooter for longer trips; walking is increasingly difficult; speech is soft, monotone at times; face masked and makes Dan look angry; cognitive difficulties; balance trouble; body temperature low; constipation; frequent urination urge; light headed upon standing

Current medications: carbo/levo 25/100; Cogentin 1mg 2/day; Azilect 1mg 1/day; Seroquel XR 150mg 1/day; Vesicare

FOUR DISORDERS

MSA subtypes: Shy Drager (autonomic difficulty, including orthostatic hypotension); SND or MSA-P (tremors, balance, rigidity, speech, parkinsonism); OPCA or MSA-C (balance, ataxia, eye movements, slurred speech)

PSP: loss of balance; eye movement problems; speech impairment; swallowing problems; depression

CBD: symptoms often appear on one side of body; alien limb; depression; emotional changes

LBD: symptoms similar to PD and AD; repeated visual hallucinations; varying levels of alertness; depression; delusional thoughts

[Robin’s note: I didn’t take down everything Dan said about the four atypicals. You can find his descriptions of these disorders on his blog — http://wewillgoon.blogspot.com/ Scroll down on the home page beyond the photo of Dan and his dog to a section titled “Atypical Parkinsonian Disorders (Parkinsonism Plus).”]

Parkinson-Plus syndromes are more similar than different. They are all movement disorders.

“I need not worry about a specific Parkinson-Plus syndrome name for the condition I face, but rather realize that, although frustrating, this lack of narrow definition” is not a problem for me.

Diagnosing the disease is like a flower: as it blossoms, doctors can identify the disease more distinctively.

MAKING THE MOST OF LIFE

Not all of Dan’s suggestions will be applicable to everyone.

Understanding and adjusting to the disorder:
* Read: they are many books and internet sites on PD and Parkinson’s Plus
* Make a list of questions between visits to the doctor
* Join online support groups
* Maintain your values and faith
* Get counseling and therapy. Very helpful in working through grief.

Websites:
psp.org
pspinformation.com
cmdg.org/Parkinsonisms/parkinsonisms.htm (Canadian Movement Disorder Group)
psprecognition.com
wemove.org
parkinsonresearchfoundation.org
emedicine.medscape.com/article/1154074-overview (was topic596)
wewillgoon.blogspot.com

Books:
“The First Year – Parkinson’s Disease: An Essential Guide for the Newly-Diagnosed” by Jackie Hunt Christensen
CurePSP Guide
“Finding Meaning with Charles” by Janet Edmunson
“Lucky Man” by Michael J. Fox
“Saving Milly” by Morton Kondracke
“100 Questions and Answers About Parkinson Disease,” Abraham Lieberman
“To Love a Stranger” by Fran McMahon (2007)
“Awakenings” by Oliver Sacks

Living on with purpose:
* Participate
* Each day counts: Today is the greatest day
* Look up! Place your faith in a higher power or a spiritual concept that is meaningful for you
* Make peace with the past
* Set new goals
* Cultivate friends and take time to be with them
* Value and enjoy family
* Get a pet that you are able to take care of
* Do what you love
* Count your blessings each day
* Volunteer to help
* Get out into the community (stores, library, parks, events)
* Travel to a destination you have long-desired to see

How Dan has lived on:
* Began writing songs again
* Playing the guitar and singing are just the therapy Dan needs
* Started a blog called PD Plus Me
* Authored a book “I Will Go On: Living with a Movement Disorder”

Is it easy?
* Being diagnosed with a Parkinson-Plus syndrome is not easy. Dan has been greatly troubled at times.
* Support groups have been a Godsend to both Dan and Karrie

Support groups:
* There is sharing about medical challenges
* As humans, we do better when we are able to discuss our struggles with others
* Big impact: early-onset PD group
* Karrie and Dan help lead a local PD group
* There’s also an atypical parkinsonian disorder support group in their area

What it is all about: family

Info on Dan Brooks:
Dan’s email address: [email protected] (You can email him for a copy of his presentation.)
Dan’s blog: wewillgoon.blogspot.com
Dan’s CD, “I Will Go On,” is available on cdbaby.com
Dan’s book, “I Will Go On,” is available on amazon.com

QUESTIONS AND ANSWERS (Answers are by Dan Brooks or Karrie Brooks, unless indicated otherwise)

Question: What symptom did you first experience?

Answer by Dan: Head-nodding tremor. Sway/balance issue. Depression. Eye pain and light sensitivity. Cognitive problems. (He had problems in meetings.) Walking awkwardness. Frequent tripping. Hand tremor.

Question: Please tell the attendees about PatientsLikeMe.

Answer by Dan: On the website patientslikeme.com, patients and caregivers share info about medications and symptoms. This info can be shared with a medical care team.

Question: [sorry….I couldn’t hear it]

Answer by Dan: Dan feels he has a fine neurologist. Can’t overstate the value of working with a neurologist at a nearby university medical center.

Question: My spouse who has CBD cannot walk, talk or feed herself for the last 11 months. How can I make her quality of life better?

Answer by Karrie: Keep her connected to other people. Go outside or go to a supermarket — one place a week. Watch a movie at home. Have a special dessert. Invite friends over that she is comfortable with. Focus on the little things.

Question: My husband was 54 when he was diagnosed with PSP. Can you speak about the early progression rate?

Answer by Dan: Parkinson-Plus disorders have a faster progression than PD. Balance, eye, and swallowing problems can be quite severe early on. There’s no way to know how quickly your husband will progress. Important things in determining the progression: family support, exercise, attitude, spiritual outlook.

Question: My husband has a good bit of dementia along with PSP or CBD. How much can he truly understand of what is happening to him?

Answer by Dan: He cannot speculate how much the husband understands. The best approach is to assume that our presence (voice, laughter, singing, attention) brings happiness to our loved ones. It is best to assume the patient understands. Never talk about the person as if they are not there.

Question: Do PSP patients understand what is going on around them?

Answer by Dan: This is best addressed by a neurologist. It’s best to assume that the patient does understand. It’s not fair to make the assumption that the patient doesn’t understand. Always assume that something you do with a positive feeling will have a positive impact on the patient.

Question: My spouse has PSP. What do you think caused him to get the disease?

Answer by Dan: Each of us asks this question. He’s searched the web about this. Some possibilities: a virus that lies dormant in the body; random genetic mutations; exposure to a chemical in food, air, or water; cellular damage caused by free radicals.

This question begs another question: Why me? Dan believes in living his life to the fullest.

Answer by Janet Edmunson: Dr. Irene Litvan’s study is looking at the causes of PSP. See pspstudy.com. It’s important for everyone to participate!

Question: I have been diagnosed with PSP. Have you heard anything about a treatment for PSP in recent years?

Answer by Dan: There are virtually no medications well-suited to PSP. Medications are an individual-patient consideration. It’s best to speak with your MD. Medications for parkinsonism can be somewhat effective in Parkinson-Plus syndromes. Medications for depression can be effective.

Question: My wife has PSP. Please address botox for muscle relaxation.

Answer by Dan: Dr. Alan Freeman article on botox; it has been used to treat parkinsonism for a long time.

[Robin’s note: You can find an online copy of this article here: http://forum.psp.org/viewtopic.php?t=8135]

Question: My husband has been taking Sinemet for over three years and it doesn’t seem to be helping any. His problems are progressing. Is there any medication other than Sinemet that will work with a PSP patient?

Answer by Dan: Some PD drugs may be appropriate or beneficial for PSP. Ask the MD about other possibilities. The MD is the only one who knows what is appropriate.

Question: I have participated in the PSP Genetics and Environmental Risk Factors Study at UCLA. What other PSP studies are underway?

Answer by Dan: See clinicaltrials.gov. There are several PSP studies listed there. CoQ10 is being looked at, for example.

Question: My sister has had PSP for about 4 years but was diagnosed just a year ago. She is barely audible but still very mobile and active. Her husband would like her to enroll in a sign language class. Is this a realistic option?

Answer by Dan: Ask the sister. This shouldn’t be forced on her. Anxiety and frustration are hard to ignore. Is it reasonable to expect the sister can learn another language?

Question: I have had PSP for about 3 years. It started with a fall. I first went to my primary care physician who tested me for a stroke. The results were negative. Then to a neurologist who again could not see anything wrong. Why do many primary care physicians and general neurologists find it so difficult to diagnose PSP?

Answer by Dan: Dan was also checked out for the possibility of a stroke.

Hopefully CurePSP is helping the medical community be more aware of PSP such that we won’t have to ask this question any more.

Question: My husband has PSP. When is it appropriate to tell a loved one that he should no longer drive a car?

Answer by Dan: Been there, done that. I love driving but that’s in the past. I miss driving but I don’t miss the thought that I could hurt someone. Talk to the doctor.

Question: I have PSP. I am trying to walk on a daily basis but I’m falling more and more. Should I continue to walk?

Answer by Dan: Keep walking but be safe. Use safety aids. Take short walks – 4 or 5 houses down the street.

Question: I am an only child, living 1K miles away from my mother. My mother is in an assisted living facility. She walks with a UStep walker. She has problems with speech, falling and swallowing. The doctor says she is depressed. She’s not interested in helping herself. Any suggestions on how to engage her?

Answer by Karrie: Many PSP patients have apathy. Fine line between pushing a loved one and letting a loved one make his/her own decisions. It’s tough when you have to start making decisions for a loved one.

When giving choices to a loved one, give them two choices. Keep things simple.

Question: My father has LBD with movement problems. How does LBD differ from PSP?

Answer by Dan: Both may appear to be PD initially. Both have parkinsonism symptoms. LBD is related to Lewy bodies; these are protein deposits. PSP is related to tau protein. In LBD, the dominant issue is progressive dementia. In PSP, the more severe problems are with balance and eye movement.

Question: My husband has MSA and LBD, and even though he is in the last stages, I am still looking for answers and information. Where do you suggest I look?

Answer by Dan: Answers are best found with the experts — the neurologists and movement disorder specialists. Try online support groups, especially the Yahoo!Groups. There are lots of websites with good info.

Question: My spouse has MSA. Is there any recent research into immuniglobulin and/or stem cells?

Answer by Dan: As a layperson, he doesn’t know much about it. See lancet.com for lots of good research.

Question: I have been diagnosed with PD. Can you explain how some people with PD diagnoses can actually have Parkinsons Plus disorders?

Answer by Dan: 15-25% of those diagnosed with PD actually have Parkinsons-Plus. Good website – emedicine.com – for a comparison of PD and Parkinsons-Plus syndromes.

Question: My husband has PSP. Where can I find an online group for caregivers?

Answer by Dan: Look into:
PSP Forum: forum.psp.org
PSPinformation Yahoo!Group
The online-based support groups being organized by Larry Schenker

Question: How does Karrie take care of herself (as a caregiver)?

Answer by Karrie: It is easy to get overwhelmed. You have to be there for yourself first, and then your husband. Life as a caregiver is tiring physically, mentally, and emotionally. It’s important to stay fit. Important to have something to look forward to each day, such as an afternoon TV show, a book, a puzzle. Isolation can be a real enemy. It’s important to get out of the house alone every once in awhile. It’s important to talk to others about problems, and to laugh with others. She saw a professional counselor. When Dan lost his insurance, the counselor cut her hourly rate so that Karrie could continue with counseling. Karrie has to work at this every single day.

Question: How do you, Dan, remain so positive? I don’t think it is reasonable that someone remain as upbeat as you.

Answer by Dan: I’ve always been optimistic and positive. I have a great family that keeps me positive. Faith, family, music, writing – these things keep me going.

Each day starts with two questions: What do I want to accomplish today? Who do I need to get in touch with?

Life is precious. You must make a conscious decision to embrace it. I can be down at times. But this very moment is beautiful. Be present for the people who love you.

Question: Who is speaking at the next webinar?

Answer by Karrie: Dr. Neal Hermanowitz will present at the next webinar 10/8/09.

Question: How can I see the Janet Edmunson webinar that I missed?

Answer by Janet Edmunson: Anyone can email Janet ([email protected]) to get a copy of her presentation.

Answer by Karrie: These webinars will not be on psp.com until perhaps the end of the year.