Category Archives: LBD

“The Big Four” dementias – AD, LBD, FTD, and Vascular

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There’s a wonderful article in the November/December 2009 issue of Neurology Now magazine.  It features Jerome and Renata Rafferty; Jerome had Lewy Body Dementia and Renata was his caregiver.

The “Other” Dementias (featuring Lewy Body Dementia story)

A companion article is titled “The Big Four.”  It gives short descriptions of four types of dementia – Alzheimer’s, Lewy Body Dementia, Frontotemporal Dementia, and Vascular Dementia.  The article notes that there are over 100 types of dementia.

Below the full text of the article, and a link to it online. You can also download the PDF of the article.

Robin
————————————

journals.lww.com/neurologynow/Fulltext/2009/05060/The__Other__Dementias.14.aspx –> HTML version

The Big Four
Neurology Now
November/December 2009 – Volume 5 – Issue 6 – p 26-27,31-34

More than 100 types of dementia have been found, but four of them account for nearly 98 percent of all cases of dementia in the United States.

ALZHEIMER’S DISEASE (AD)

DESCRIPTION: People with AD develop memory problems, often followed by confusion, apathy, depression, emotional volatility, and other problems.

CAUSE: People with AD develop two types of dysfunctional protein in the hippocampus, the part of the brain essential for creating new memories. Tau protein accumulates within neurons in that region, while clumps of amyloid protein develop between neurons in that region. Some researchers, however, suspect that the toxic proteins may be the result of the disease rather than the cause.

TYPICAL CASE: The first symptom of AD almost always involves memory problems, such as forgetting familiar names and misplacing items. As the disease progresses people may have trouble finding their way home or keeping up with routine obligations such as doctor appointments, paying bills, and preparing meals. Later stages may affect the frontal lobes, resulting in erratic emotions, loss of normal inhibition, and hallucinations.

TREATMENT: Since AD results in decreased levels of acetylcholine, a neurotransmitter essential for memory and learning, drugs that boost acetylcholine, such as donepezil and memantine, often help, at least for a while. Other treatments are available for specific symptoms such as depression, hallucinations, and movement disorders, but nothing seems to slow development of the disease.

ON THE HORIZON: Several drugs and vaccines designed to inhibit the production of toxic tau and amyloid protein, or remove it once it appears, are in development. However, people who have tried the drug experimentally failed to improve significantly, even though protein levels declined, sometimes dramatically.

LEWY BODY DEMENTIA (LBD)

DESCRIPTION: Like Alzheimer’s, LBD produces cognitive decline, but with three additional traits. Instead of declining continuously, people with LBD tend to fluctuate in terms of attention, alertness, ability to speak coherently, and other symptoms. They also tend to have visual hallucinations, often benign. Finally, they tend to develop symptoms of Parkinson’s disease, including rigidity, tremor, and slowness of movement.

CAUSE: A type of protein known as alpha-synuclein clumps into Lewy bodies, which appear inside of cells, or neurons. Lewy bodies may result from the inability of the cell to break down and recycle alpha-synuclein efficiently. As the protein accumulates, it sticks together, as though the cell is trying to gather its own debris to keep it out of the way.

TYPICAL CASE: People with LBD often act out violent dreams that involve being pursued or attacked. They may develop benign hallucinations involving, for example, children or animals running around the house. Attention and concentration may fluctuate, and patients may start to have trouble with visual-spatial abilities-they may misjudge the height of a step or miss a cup when they reach for it. Some people with LBD experience an overwhelming urge to sleep during the day. Their movements also may become rigid and slow, like the symptoms of Parkinson’s disease, and they may develop problems with memory, judgment, and mood, like the symptoms of AD.

TREATMENT: No treatment specifically for LBD exists. However, since LBD affects nearly every neurochemical system in the brain, specific aspects of the disease can be treated. Memory problems can be treated with donepezil and other drugs for AD. Movement disorders may respond to L-dopa and other medications for Parkinson’s disease. Modafinil may alleviate daytime sleepiness.

ON THE HORIZON: No drug yet exists that affects the synuclein protein, although some drugs exist for daytime sleepiness, and another, which resembles methylfenidate, is in development.

FRONTOTEMPORAL DEMENTIA (FTD)

DESCRIPTION: FTD includes several disorders that cause the frontal lobes behind the forehead, and the temporal lobes at the sides of the brain, to atrophy and shrink. Patients either develop speech difficulties, known as aphasia, or they display inappropriate social behavior. Aphasia may involve halting, effortful speech with the patient struggling to produce the right word. Behavioral changes may involve indifference to the concerns of others. Some patients developing FTD may start shoplifting or become attracted to shiny objects or fire.

CAUSE: In FTD, a protein known as TDP-43 accumulates within cells at the front of the brain. In one form of FTD known as Pick’s disease, tau protein, found in the hippocampus of people with AD, accumulates within cells in the frontal lobes.

TYPICAL CASE: A person developing FTD generally exhibits personality or mood changes. An outgoing person may become withdrawn and depressed, while an introverted person may become loud and outgoing. Socially inappropriate behavior may also become more common. Later, FTD patients may develop speech difficulties as they lose the ability to recall the meaning of words, or they may start to speak with great fluency while making no sense.

TREATMENT: Only symptomatic treatments are available with medications developed for other disorders, such as psychiatric medications for behavioral problems or mood disorders. There are no treatments for language problems.

ON THE HORIZON: Methylene blue, a drug in development for AD, inhibits the aggregation of tau protein, so it may help patients with Pick’s disease. Another tau aggregation inhibitor known as AL-108, or davunetide, is in clinical trials, and may soon become the first tau-active drug available in the U.S. TDP-43, the offending protein in other forms of FTD, was discovered only three years ago, leaving little time for the development of effective treatments.

VASCULAR DEMENTIA

DESCRIPTION: Since this dementia results from several small strokes, and strokes can affect any part of the brain, the symptoms of vascular can vary widely. However, they usually include declines in problem-solving ability, memory, and socially appropriate behavior.

CAUSE: Vascular dementia is believed to result from damage to brain cells caused by lack of oxygen when the blood supply is cut during a series of mild strokes. However, one study of 1,000 brains from demented patients who had died found only six that had pure vascular dementia, with the slow progression typical of the disorder. The rest also had another form of dementia.

TYPICAL CASE: To be diagnosed with vascular dementia, a patient must show evidence of a stroke in a location that could affect cognition, and cognitive problems must develop within three to six months of the stroke. A patient who meets these criteria may develop memory problems and have trouble speaking coherently or understanding the speech of others. They may also develop motor difficulties that prevent them from dressing themselves.

TREATMENT: The first goal is to reduce stroke risk by improving cardiovascular health. Statins may be prescribed to lower cholesterol, anti-hypertensives to lower blood pressure, and omega-3 pills to improve triglyceride levels. Low-dose aspirin may be prescribed to inhibit the clotting of the blood, and patients may be urged to give up smoking and drinking and reduce stress.

ON THE HORIZON: Damage from strokes cannot be reversed, but the brain can compensate for some deficits. Physical therapy designed to stimulate brain plasticity may provide some help.

Copyright © 2009, AAN Enterprises, Inc.

“Cognitive Dysfunction in Parkinson’s” – Dr. Marsh presentation

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Geriatric psychiatrist Laura Marsh, MD, gave a wonderful presentation at last
week’s APDA/NPF Young Onset Parkinson’s Conference on the topic of cognitive
dysfunction in Parkinson’s Disease.  The presentation is likely of interest
to those in our Lewy Body Dementia group as well.

Dr. Marsh reviews the prevalence, pathophysiology, clinical characteristics,
and treatment of cognitive dysfunction in PD. She also describes much of the
research into cognitive issues and PD. (Dr. Marsh is the author of much of the
research!)

The conference was recorded.  You can find the video/audio recording here:

event.netbriefings.com/event/youngparkinsons/Archives/jointconf1009/Viewer/wmpviewerP004c.html

You can find a PDF of Dr. Marsh’s slides here:

event.netbriefings.com/event/youngparkinsons/Live/jointconf1009/materials/youngparkinsonsyou002-04.pdf

I hope you enjoy the presentation as much as I did!

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.

“Botulinum Toxin in Parkinsonism”

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(As this article was mentioned by Dan Brooks in tonight’s webinar, I figured I had better post it here!)

This article on the use of Botox in parkinsonism (PD and atypical parkinsonism) is in the Spring ’09 issue of the APDA (American Parkinson Disease Association) quarterly newsletter. Cervical dystonia of PSP and limb dystonia of atypical parkinsonism disorders are specifically mentioned.

I’ve distributed the newsletter at recent support group meetings because it has several articles of interest. Here’s the full article and a link to the newsletter.

http://www.apdaparkinson.org/data/NewsL … 202009.pdf –>
article starts on page 1

Botulinum Toxin In Parkinsonism

By Alan Freeman, MD
Associate Professor of Neurology
Emory University School of Medicine, Atlanta, Ga.

Botulinum toxin (BTX) has been successfully used for hyperkinectic movement disorders for more than 20 years. Botulinum neurotoxin is produced by the anaerobic bacillus Clostridium botulinum. There are seven botulinum stereotypes labeled A-G. Two forms of BTX A and B have been approved for clinical use. BTX A is available worldwide as Botox® (Allergan), in Germany as Xeomin® (Merz pharma) and elsewhere as Dysport® (Ipsen). Botox is the only form of BTX-A currently available in the USA, although other forms may be available for therapeutic use next year. A formulation of BTX B is also available in the USA as Myobloc® and in Europe as NeuroBloc® (Solstice). BTX Types C and F have also been used in humans, but only on an experimental basis. The US Food and Drug Administration (FDA) has approved Botox for the treatment of blepharospasm (forced eyelid contractions), cranial nerve seven disorders (hemifacial spasm), cervical dystonia (involuntary neck spasm), and hyperhydrosis (sweating) and Myobloc for the treatment of cervical dystonia.

Other uses of BTX are being investigated, but none has been approved by the FDA. Some of these include spasticity, headache, sialorrhea (drooling), tremor, overactive bladder, limb dystonia, and tics.

BTX works by inhibiting acetylcholine release at the neuromuscular junction and salivary/sweat glands. This causes temporary paralysis of the muscles and a decrease in secretion of the glands. The duration of effect varies but is usually 10-20 weeks (for involuntary movements and excess salivation).

Doses of BTX are dispensed in units. The units of each form of BTX are not clinically equivalent; therefore, the different formulations are not interchangeable. In addition, each formulation has different diffusion and side effect profiles.

Botulinum toxins may be used in idiopathic Parkinson’s disease (PD) as well as atypical parkinsonian syndromes, as progressive supranuclear palsy. Patients who develop blepharospasm, from either the primary illness or medications, can be successfully treated with BTX and are typically injected with small doses of toxin approximately every three
months.

Drooling can be a major problem with advanced PD. Both Botox and Myobloc have been successfully used in this condition. Myobloc may have an advantage, with its side effect of dry mouth, seen when used to treat cervical dystonia. We were recently involved in a Myobloc sialorrhea study for PD, and preliminary results look very promising.

Botulinum toxin can also be used in cervical dystonia in patients with progressive supranuclear palsy, and in limb dystonia with PD and parkinsonian syndromes. This includes the “dystonic clenched fist” in the upper extremity and foot inversion or toe flexion/extension in the lower extremities.

Once the FDA approves new uses for BTX, it will be much easier to get reimbursement from Medicare and insurance companies. Hopefully it will be in the near future.

Note: This article was adapted from one published in the APDA Atlanta, Ga I&R Center newsletter, Winter/Spring 2009. [Robin’s note: I couldn’t find this APDA Atlanta newsletter on the apdaatlanta.org website.]

Caregiving with Love through Neurodegenerative Disease – Webinar Notes

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Today, CurePSP (psp.org) hosted a webinar on caregiving with Janet Edmunson. It was titled “Caregiving with Love through a Neurodegenerative Disease.”

The presenter was Janet Edmunson.  Janet’s book is called “Finding Meaning with Charles” and is about her caregiving experience for her husband Charles.  Her website — findingmeaningwithcharles.com — is worth checking out.  She visited the Bay Area a year ago, and met with some of our support group members and at various other events.

These are the notes I took during the webinar.
The notes are divided into these sections:

* Intro/Timeline – this is of general interest as it’s short

* Symptoms – these are likely of most interest to those dealing with PSP and CBD but those dealing with MSA may find some relevance

* Affirmations – these are of general interest to those dealing with PSP, CBD, MSA, and LBD

* Questions and Answers –  these are likely of most interest to those dealing with PSP and CBD but those dealing with MSA may find some relevance.  (There was one question on LBD.)

* CurePSP+ Overview – this will only be of interest to those dealing with PSP, CBD, and perhaps MSA

Robin

—————————–

Robin’s Note on

Webinar Presenter – Janet Edmunson
Chair of the Board, CurePSP
Author of “Finding Meaning with Charles”

INTRO/TIMELINE

Her late husband Charles developed his own mission statement and read it every day:  “I will make a significant different in the world by bringing grace with integrity into the lives of others.  Therefore, through a disciplined focus, I will apply my life to bringing peace for people who are in turmoil…”

Charles wrote a book called “Paradoxes of Leadership” in the last years of his life.

Charles developed had his first symptoms in 1995 at the age of 45.  First symptoms are trouble with stairs.

1997 they saw Dr. Litvan at NIH.  She suggested CBD.

1998 they saw a group of MDS at Boston Teaching Hospital.  Diagnosis was PSP.

2000:  Charles fought brain disorder for 5 years.  Upon autopsy, it was confirmed that he CBD.

SYMPTOMS

Symptom:  eye movement problems
No peripheral vision or depth perception
Syncopated, double vision, no focus
Problem driving (didn’t know where he was in space)
Reading problems (tracking)

Symptom:  fine motor movements
Buttoning buttons, tying ties
Writing (squiggly and small)
Typing
These are called “cortico-sensory problems.”  They are classic symptoms for CBD.

Symptom:  rigidity and unsteady gait
Extremely stiff  (brain caused frozen back muscles to contract at same time)
Neck hyper-extended
Lost ability to run
Lost ability to walk, eventually

Symptom:  not many falls
Falls when jogging
This is a bit different with PSP where falls can be frequent

Other symptoms:
Impulsivity
Stiff, unsteady gait
Out of bed
Symptoms started out one-sided  (typical with CBD)
Apraxia – couldn’t do purposeful movements, such as clapping hands
Essential tremor – especially when reaching
Alien limb – foot didn’t feel connected to body and arm would float up  (typical with CBD)
Bladder incontinence started early on.  Charles might’ve gotten to the restroom in time but couldn’t get his trousers down in time.

Symptom:  speech
Slowed
Quiet
Slurred, jumbled, though Charles didn’t think it was slurred
Just gave up

Symptom:  swallowing
Straws, sippy cups
Thickening liquids
Pills in pudding and apple sauce
Chopped food into small pieces
He’d “pass out” while chewing.  After awhile, he’d start chewing again.  Happened frequently.  Seizures?
Based on a swallow study, some of the food was being pocketed.  They would tell Charles to swallow a second time.
She recommends a swallow test

Symptoms:  cognitive
Word find
Slowed thought-processing  (this requires patience from caregivers)
Perseveration, where Charles would put the same word (“process”) in every sentence.  (To resolve this, they would have to get Charles off track.)
Yes/No backwards
Math

Symptoms:  behavioral
Depression.  No antidepressants helped.  Finally, an atypical antipsychotic called Seroquel helped with the depression and sleep issues.
Sleep issues.  Charles would wake up every hour!
Obsessions with time and Beatles music
Inappropriate behaviors

Symptoms:  ADLs
Toileting.  Charles sat for 30 minutes on the toilet.
Constipation.  Lactulose and a suppository helped.
Showering.  She recommends a shower chair with a sliding seat.
Washing and combing hair
Brush teeth.  Very hard because Charles would clamp down on the tooth brush.  They got a bite block from the dentist.
Eating.  Charles wanted to eat on his own.  It was a mess with food everywhere.  He eventually had to accept help.
Transfers from the bed, to a recliner, and to a wheelchair.  They were taught how to do transfers by an OT.  They used a Hoyer lift later on (with hospice).

What could’ve caused this?  We don’t know.
Family history?  Dad had PD, Mom had AD.
Chemical exposure?
Heart disease or treatments?
Very low-fat diet?  (You need fat for your brain function.)
Diet sodas that used sacchrine?
Gum disease?
Tick bites?  Charles was never tested for Lyme Disease.
Sleep issues?  Charles had sleep apnea.
Mobile phone use?
Virus?

She encourages those with PSP (who can still talk or communicate) to enroll in Dr. Litvan’s PSP study.  See pspstudy.com.

AFFIRMATIONS

“When life kicks you, let it kick you forward.”  (Message from someone with breast cancer.)

Affirmations:  positive messages; uplift; inspire

Caregiving Affirmation #1:  hold on to your passions because they are the essence of who you are
Examples:  hobbies, being with friends, genealogy

Caregiving Affirmation #2:  I can make it through the difficult transitions of this disease
First hard transition for Charles was not driving
Another hard transition for Charles was not walking and using a wheelchair
Another hard transition for Charles was accepting an aide

She suggests that family members allow others to help:  don’t try to do all the caregiving yourselves!
Make a list of tasks that you’d like to have done.  Whenever someone says “is there anything I can do for you?,” take out the list and say “can you do this?”
See caringbridge.org to allow family and friends to help

Caregiving Affirmation #3:  explore life’s adventures together to store up fond memories that will sustain you
Example:  celebrate your anniversary every month
Example:  dinner with friends at your house, with Subway sandwiches, paper plates, and paper cups

Caregiving Affirmation #4:  I will give myself credit for staying strong despite being pushed to my limits

Caregiving Affirmation #5:  I will give myself grace when I occasionally blow it

Caregiving Affirmation #6:  I will expect that some people will find it difficult to visit my loved one.  That’s OK — it doesn’t mean that they’ve stopped caring.
Consider encouraging visitors to come over in pairs so that they can talk and your loved one can listen in

Caregiving Affirmation #7:  I will remember that the difficult personality changes are not my loved one — they are the disease

Caregiving Affirmation #8:  I will consider hospice sooner versus later; the support will bring me relief

Caregiving Affirmation #9:  It’s perfectly normal to grieve even before my loved one dies
You grieve with each loss.
Janet bought a grief book even before her husband Charles died.

Caregiving Affirmation #10:  Look for the gifts that only this type of tragedy can afford
Caregivers can gain patience
Caregivers can make new friends at support groups
One gift – the ability to say “goodbye”

Last thought from Charles’s boss:  “May this [disease] make you better, not bitter.”

QUESTIONS AND ANSWERS  (answers are by Janet Edmunson, unless indicated)

Q:  Why does it take so long to get a diagnosis?

A:  To get it right, probably isn’t as important as we think it is.  For the person with the disease, getting a diagnosis is very important.

It takes time for enough symptoms to appear so that a diagnosis can be made.

Many people start with a primary care physician, who has probably never seen another person with these diseases.  Even many neurologists haven’t seen people with this disorder!

Get a DVD from CurePSP to give to your MD!  [You can find it here:  https://www.psp.org/forms/reqmater.php]

Q:  Where can I find an online group of caregivers?

A:  There’s the PSP Forum at forum.psp.org.

There are a few Yahoo!Groups available:  ShyDrager, PSPinformation, cbgd_support.  Look in the health section on Yahoo!Groups.

[Robin’s note:  There are four Yahoo!Groups that deal with MSA:

“ShyDrager” –
http://health.groups.yahoo.com/group/shydrager/

“Multiple System Atrophy” –
http://health.groups.yahoo.com/group/multiplesystematrophy/join

“idcircle” –
http://health.groups.yahoo.com/group/idcircle/join

“ChristianMSAGroup” –
http://health.groups.yahoo.com/group/christianMSAgroup/join ]

Q:  How did you remain so positive?  I don’t think it is reasonable that someone can remain as upbeat as you did during your caring for Charles.

A:  Part of it is Janet’s optimistic nature.  What’s the alternative?  See “Learned Optimism” by Martin Seligman.  He says that optimism energizes.

Q:  What did the brain autopsy show?

A:  The pathologist said that the diagnosis was hard for her to finalize.  It looked like both PSP and CBD, but more like CBD.  Charles also said hardening of the blood vessels in the brain.

See info on brain donation here:  http://psp.org/page/braindonation

Talk about brain donation now!  Be sure that all the paperwork is handled about 2 months before your loved one dies.

CurePSP pays for most of the charges associated with brain donation.  Families often have to pay a local pathologist for the extraction.  CurePSP can reimburse families for part of these costs.  [Robin’s note:  this reimbursement is available to PSP, CBD, and MSA families only.]

A by John Burhoe (CurePSP Outreach Committee Chair):  Lou’s brain was donated.  Hospice did a lot of groundwork on the brain donation arrangements.

Q:  How did you balance working fulltime and caring for Charles?

A:  Janet had a team of aides.  Because she couldn’t afford 7×24 aides, she had some volunteer aides.  Hospice provided some aides.

A by John Burhoe:  John was able to work at home.

Q:  Where can I find the latest information on PSP?

A:  See curepsp.org.  In particular, find the Guide or Guidebook.  This can be downloaded for free or it costs $10.  [See:  https://www.psp.org/forms/reqmater.php]

Also get on the CurePSP newsletter mailing list.  It comes out quarterly.  This will keep you up to date on research.

Tune in to the frequent webinars, organized by Larry Schenker.

Also see shy-drager.org (for MSA), lbda.org (for LBD), ftd-picks.org (for CBD).

Call Vera James with the Shy Drager Support Group (MSA).

Call any of the “communicators” listed.  It doesn’t matter if they are in a different state than the state you live in.

A by John Burhoe:  The website is being updated and will be launched at the end of the year (November) or early next year.  All these webinars will be available on curepsp.org.

All of the support groups are listed by state.  And there are “communicators” as well, listed by state.

Other sources of support:  physical therapy facilities, your church, hospice, friends.

Q:  How do you discuss poor judgments with a parent who is no longer independent but still believes she can take care of herself?

A:  Get the MD involved in explaining to the patient what is needed.

Q:  Can you describe the speech exercises you did with Charles?

A:  Her sister developed the LSVT for PDers.  She instructed Charles to keep saying “aaaaaahhhhh” for as long and as loud as he could.  Also, she instructed Janet to give a word to Charles and then Charles had to come up with a sentence using that word.

She recommends finding LSVT-trained speech therapists for those with PSP, CBD, and MSA.  See:  http://www.lsvtglobal.com/  They also have LOUD training.

A by John Burhoe:  The swallow tests always came out clear though, at home, Lou was always gagging and choking.

Q:  How do we make the journey as easy and dignified as possible?

A:  Keep telling your loved one how much you love them.

Use utensils to let people keep eating with dignity.  Figure out how to use the bathroom with dignity.

A by John Burhoe:  Keep doing things!  He kept taking Lou to church every week.

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

A:  They may not, but we don’t know.  Treat them as if they do.

A by John Burhoe:  Hell yes.  Lou was right there, all the way through.  Treat them just as you treat everyone else.  Don’t let someone talk about your loved one in the third person (“does she want this?”).

Q:  What are the pros and cons of a feeding tube?

A:  One consideration is how much quality of life is there.  It seems appropriate if it’s very early in the disease course.

Be sure you discuss when the feeding tube should be removed or when tube feedings should be stopped.

A by John Burhoe:  There’s no right or wrong answer.  It’s a very personal decision.  It should be discussed early on so your loved one can be involved in the discussion.

They chose not to have a feeding tube because they were concerned it would extend life, and they were concerned it would be necessary to move Lou outside the home.

Q:  Can you speak about LBD?  It was my understanding that this webinar would be relevant to our situation (my wife has PDD/LBD) but you mostly spoke about PSP and CBD.

A:  The hard part is the dementia element to LBD.  Dementia gives caregiving more of an emotional component than a physical component.

We often confuse dementia with Alzheimer’s Disease.  They are different.

Behavioral things that can happen in LBD can be very disturbing to the caregiver.  Someone with LBD can accuse his/her spouse with having an affair.

Bringing humor to a the situation is so important!  (Husband with LBD:  you have other men in our bed at night!  Wife:  that’s why I got a big bed.]

Do not argue with hallucinations and delusions!

Q:  Can we get a copy of this slide presentation?

A:  Email Janet at [email protected] for a copy of these slides.

Hopefully the CurePSP website will eventually include the recordings of these webinars.

Q:  Can PT help to slow these disorders?

A:  I’m not sure it can slow these disorders.  PT can give quality of life.

A by John Burhoe:  The biggest aspect of PT for Lou was the social interaction.

Q:  Does CurePSP have more information about what my husband with MSA will experience in the coming years?

A:  We are getting more info about MSA onto our website.  The Board just approved including this disorder in its mission.

See shy-drager.org.

A by John Burhoe:  Don’t forget google.com.

Q:  Does anyone have puffy cheeks?  I have PSP.  Puffy cheeks caused me to bite the inside of my mouth.  It affects my speech and chewing.

A:  Don’t know anything about this.

Q:  I have PSP.  Should I try to walk and stay active, even though I fall alot?

A:  We don’t want you to fall!  If you break a bone, this will bring you down a lot.  Let someone hold onto you or use a gait belt around you.  There are techniques you can learn from a PT on how to walk.

It’s important to get a 4-wheeled walker or a very good 3-wheeled walker.  It’s a great idea to get a walker with a basket and seat.  You might consider putting weights on the walker.  You might look at www.wheelchairs.com.

A by John Burhoe:  Put a fold-up wheelchair in the car so you can still get out!

A by Larry Schenker (webinar organizer):  You might consider a brace that comes down from the ceiling while you are on a treadmill.  Consider using a helmet.  Consider swimming.

Q:  My husband signed up for the PSP genetic/environmental risk factor study at Case Western Reserve University in Cleveland.  What other PSP studies are there?

A:  See pspstudy.com

There’s a CoQ10 study at Lahey Clinic.

There’s a lithium study funded by NIH.

You might be able to be seen at NIH for evaluation.

There’s a drug study coming up for NAP.  It is coordinated by Dr. Adam Boxer at UCSF.

Brain donation is an important means of research.  There are over 1000 PSP and CBD brains being looked at now in a genetics study.

Q:  How did John Burhoe travel despite his wife’s incontinence?

A by John Burhoe:  On airplanes, John could lift Lou onto the plane.  They sat next to the toilet.  This was never a problem.

A:  Charles used Dignity pads along with Depends.  This is an extra pad that goes inside the Depends.

Charles used a condom catheter on airplanes.

Q:  How can I get Janet’s book “Finding Meaning with Charles”?

A:  At a bookstore, on findingmeaningwithcharles.com, on Amazon.com, on bn.com.

Q:  Is there a doctor any place in the world that we might be able to visit to get a definitive diagnosis?  My wife’s neurologist can decide between MSA, CBD, or PSP.

A:  See a movement disorder specialist, not just a neurologist.

She recommends seeing Dr. Larry Golbe.  Or:  Dr. Ravi in Boston, Dr. Diana Apetauerova at Lahey Clinic in Boston, Dr. David Riley in Cleveland.

A by John Burhoe:  He found good MDs at Mass General and Scripps.

He recommends seeing someone at a major teaching hospital such as the University of Michigan, Duke, or UCLA.

CUREPSP+ OVERVIEW

CurePSP+ Vision Statement:  cure and prevent PSP, CBD, and related disorders

CurePSP: started in 1990; 30 members initially; now, the organization communicates to over 30K people a year and receives gifts from over 8K donors

Educational Mission:
PSP, CBD, MSA, AGD (Argyrophilic Grain Disease), Pallidal Degeneration

Research Mission:
PSP, CBD, AGD, Lytico-bodig (LyB), Guadelupean Tauopathy (GT)

Outreach and education:
Magazine
Guidebook and other materials
Website (videos, downloadable materials, Forum, resource)
Webinars

Research:  118 research projects since 1997; values at $8.2 million
Genetics Consortium:  funded primarily by Charles D. Peebler, Jr; 13 member scientics from US, UK, and Germany
Major Programs:  Pollin Fund for CBD; Troxel Brain Bank; General Investigator-Initiated Projects

Areas of Research:
tau genetics
non-tau based pathologics
non-tau based genetic studies
anatomic and histopathologic surveys
brain bank

www.curepsp.org
800/457-4777 US
866/457-4777 Canada