Symptoms of clinical intolerance during an acute levodopa challenge in relation to MSA

In this interesting research out of Argentina, the authors conclude that “symptoms of clinical intolerance during an acute levodopa challenge do not appear to be useful in the diagnosis of” multiple system atrophy (MSA).  The idea was to give patients levodopa (one brand name is Sinemet) to learn if they could differentiate between those with MSA and those with Parkinson’s Disease.  “Clinical intolerance” means nausea, vomiting, hypotension, and profuse perspiration.

The abstract is copied below.



Int J Neurosci. 2009;119(12):2257-61.

Does clinical intolerance to a diagnostic acute levodopa challenge differentiate multiple system atrophy from pd?

Estévez S, Perez-Lloret S, Merello M.
Movement Disorders Section, Raúl Carrea Institute for Neurological Research (FLENI), Buenos Aires, Argentina.

BACKGROUND: The diagnosis of multiple system atrophy (MSA) remains challenging.

OBJECTIVE: To determine if the occurrence of symptoms of clinical intolerance such as nausea, vomiting, hypotension, and profuse perspiration during a standard acute levodopa challenge may be a useful marker of MSA.

METHODS: A total of 507 dopaminergic acute challenge tests performed for different purposes in the last 10 years in a movement disorders clinic were reviewed, identifying patients who manifested symptoms of clinical intolerance during test performance. Only those tests completed for diagnostic purposes were included and these were matched by the presence or absence of response to levodopa, sex, and age, with a group of patients undergoing acute challenge without any symptoms of clinical intolerance. Presumptive diagnosis for each patient was performed by means of accepted clinical criteria after a significant follow-up period. Only patients with a final diagnosis of Parkinson’s disease (PD) or MSA were analyzed.

RESULTS: Twenty-three out of the 507 patients (women: 50%) presented symptoms of clinical intolerance and received a final diagnosis of PD or MSA, and underwent further analysis. Four out the 23 patients with intolerance (17%) and one out the 16 patients from the control group (6%) were diagnosed as having MSA (Chi-square = 1.05, p = .3). Overall sensitivity and specificity of the presence of clinical intolerance to predict diagnosis of MSA were 80% (95%IC: 45%-100%) and 44% (95%CI: 27%-61%) respectively.

CONCLUSIONS: Symptoms of clinical intolerance during an acute levodopa challenge do not appear to be useful in the diagnosis of MSA.

PubMed ID #: 19916854  (see for this abstract only)

PSP Research Update – Notes from Golbe Webinar (11/19/09)

I don’t know how many listened to tonight’s CurePSP webinar. Though it had been advertised as “PSP, CBD and MSA – Research for Dummies,” Dr. Golbe’s title slide made clear he was talking only about PSP. There was significant overlap with the last webinar (with Dr. Bordelon and Dr. Schellenberg) but I didn’t mind. I always like listening to Dr. Golbe because he is one of the top PSP experts in the world. Further, his explanations of complex topics are very clear.

I also appreciated the fact that all the CurePSP messages (from various staff members and Board members) were gone, and that the webinar was not pre-recorded. And I liked how the questions were handled this time around. (I do wish that we could improve the editing or even consider eliminating some of the duplicate questions that get asked at each webinar.)

I was rather busy typing notes and didn’t get a chance to ask any questions myself. And, when tonight’s moderator Kate was speaking, I couldn’t hear her so I missed a few of those questions.

Here are my notes from Dr. Golbe’s presentation, with a few comments of my own. (I’ve added headings.)



Topic – PSP Research for Dummies

Presenter – Lawrence Golbe, MD
Prof of Neurology, UMDNJ-Robert Wood Johnson Medical School, New Brunswick, NJ
Dir of Research and Clinical Affairs, CurePSP

The “for dummies” books are written for intelligent people. That’s how the talk is geared.

Most research in PSP is pretty molecular.


What does PSP stand for?
Progressive = tends to worsen with time
Supranuclear = not primarily in the “nuclei” (clusters of brain cells in the brain stem that control eye movement), but at higher (“supranuclear”) brain centers
Palsy = poor movement (in the case of the eyes)

Main features of PSP:
* balance: especially unheralded falls; 2/3 of people have this problem
* speech (“dysarthria”): these problems happen usually later in the disease; spastic speech; hypophonia can occur; ataxic speech – sounds like you are drunk
* swallowing: pneumonia is possible; the problem is with liquids (which is the hallmark of neurological disorders); gap in the seal between the throat and the windpipe
* slow movement (“bradykinesia”) and slow thought: parkinsonian symptom
* insufficient movement: person just sits there, without moving
* eye movement: sophisticated neurologist who knows how to look for this can detect this problem at even early stages; the issue is both up and down gaze, but especially down gaze
* “frontal” behavior: problems with prioritizing, abstraction, inhibiting behavior. With “frontal” behavior, people with PSP have serious complications given the balance problem and the swallowing problem. Those with PSP stuff their mouths. Those with PSP get up to

* usually the first symptom
* irregular gait. Often a drunken gait.

Bradykinesia (slow, insufficient movement):
* resembles Parkinson’s disease
* some of it is actually “rigidity” or muscle stiffness (face, neck)
* makes it difficult to compensate for balance problem

* ataxic: irregular bursts of speech
* spastic: strained quality
* hypophonic: low volume
* PSP usually has at least two of these three speech problems
* can have constant “growling.” “Frontal” behavior symptom.

* first and worse with thin liquids
* aspiration common
* large piece of food in windpipe is not usually the problem


Tau protein acts as scaffolding:
* Tau microtubules stabilize the structure of brain cells (and the axon) and serve as “railroad tracks.”
* In PSP, tau protein isn’t working right. The cell breaks down, and stops working properly.*
* Neurofibrillary tangles (NFTs): hallmark of PSP in the brain
* What’s the chicken and what’s the egg? Do the tangled clumps of tau proteins serve a useful purpose? Probably just getting rid of the tangles won’t solve PSP.


Is PSP inherited?
Very weakly hereditary: only about 1 in 100 patients has a relative with PSP

Intro to the H1 Haplotype:
H1 haplotype is the genetic marker for PSP. A haplotype is a string of genetic markers on a chromosome. In the case of PSP, this haplotype is on chromosome 17. This haplotype signals the presence of disease-causing mutation in or near that region.

H1 Haplotype (% of chromosomes in those with PSP): (Houlden et al, 2001)
H1: 94% of (chromosomes in those with) PSP; 92% of CBD; 77% of control
H2: 6% PSP; 8% CBD; 23% control
So…PSP has an over-representation of the H1 haplotype.
Could it be that the H2 haplotype is protective?

H1/H1 Genotype (% of individuals): (Houlden et al, 2001)
H1/H1: 88% (of individuals with) PSP, 84% CBD, 60% control
H1/H2: 12% PSP, 16% CBD, 34% control
H2/H2: 0 PSP, 0 CBD, 7% control
H1/H1 = getting an H1 haplotype from each parent

Tau alternative splicing: (Hardy)
There are four exons that have the code for microtubule binding.
In normal brains: 1:1 ratio of 3 repeat tau protein isoforms and 4 repeat tau protein isoforms
In PSP: 4 repeat tau is abnormally abundant

Tau isoforms expressed in NFTs in various disorders:
Mostly 4 repeat tau disorders: PSP, CBD, FTD with exon 10 mutations, Guadeloupean parkinsonism, AGD
Mostly 3 repeat tau disorders: Pick’s disease, myotonic dystrophy
3 and 4 tau equally: AD, FTD without exon 10 mutations, Parkinson-dementia complex of Guam, Postencephalitic parkinsonism

* the H1/H1 genotype is nearly necessary but far from sufficient for PSP to develop
* PSP has an excess of 4-repeat tau protein, but the significance of that remains unclear
* one chromosome 17 with the H2 haplotype reduces PSP risk
* both chromosome 17s with the H2 haplotype reduce PSP risk even more
* what “conditions” the genetic effect?


Mitochondrial Genetics in PSP:
* mitochondrion = energy factories of cells
* make energy from oxygen and sugar
* have their own genes kept apart from the cell’s main set of genes
* mitochondrial genes do not work properly in PSP, PD, AD, etc.
* it’s very hard to study mitochondrial genes. Is the function genetic or toxic?


Cause of PSP: Environment?
* Not well-studied at all
* Dr. Litvan is studying this now
* Those with PSP are less well-educated. May mean more industrial exposure or exposure to toxins? (Dr. Golbe published a study about 20 years ago. A French study just published last month found the same thing: less well-educated.)
* Not a consistent finding: more farming. May mean more exposure to pesticides, fresh fruits/vegetables.
* Environmental toxins can damage mitochondria.

[Robin’s note: You can find the abstract of the French study posted here — — or look it up on using PubMed ID#19864660.]


Cause of PSP: Diet?
* The just-published French study didn’t find anything diet-related.
* Guadeloupe cluster of PSP-like illness. Those with PSP-like disease on Guadeoupe have consumed more sweetsop and soursop fruit. This fruit has been found to harbor toxins that act on the mitochondria. (Caparros-Lefebvre et al, Lancet 1999) Mouse experiments are currently underway. Maybe we have something in our diet with a similar toxin? (You might see this fruit in a Jamaican restaurant.)
* Guam cluster of PSP-like illness. Dietary theory now discounted. Genetic theory being re-examined. Mainly one ethnic group on Guam who get this disease. The disease is dying out.


New, exciting developments in genetics of PSP:
* hereditary FTD-17 as a human genetic “model” of PSP. Mouse, fruit fly, zebrafish genetic models. These animals are given FTD-17. Convenient to study drugs in such animals.
* whole genome analysis produced four new (previously unsuspected) genetic factors (plus the tau gene) implicated in PSP. We are awaiting confirmation. If confirmed, the research should be published in early 2010. This analysis will suggest new biochemical pathways and drug targets.


Medications for PSP:
* levodopa/carbidopa: might help
* amantadine: might help, even if
* antidepressants: can help with depression and behavioral abnormalities
* sedatives: can help with behavioral abnormalities
* bladder medications: useful
* constipation medications: useful

Constipation can be a side effect of amantadine and antidepressants.

Treatment of Dysarthria and Dysphagia:
* swallowing evaluation (especially a modified barium swallow study – an xray movie of swallowing) is helpful. This evaluation may recommend: thicken thin liquids; avoid tough, dry food; etc.
* speech therapy rarely helpful but worth a try
* amplifying devices often useful
* hand signs
* if patient can direct gaze downward, these might be useful: pointing boards and electronic typing devices. Stroke and brain injury rehab centers have experience with these boards and devices.


Nypta (medication):
* GSK3 beta inhibitor (prevents phosphorylation of tau protein)
* trial being organized; it will start in March 2010 in the US
* Noscira is manufacturer
* may slow progression and help symptoms

[Robin’s note: As reported a couple of weeks ago by Dr. Bordelon, UCLA is one of the trial sites for the Nypta trial.]

Davunetide (medication):
* trial being planned
* Allon is manufacturer
* administered by nasal spray
* may slow progression and help symptoms

[Robin’s note: UCSF is the lead site for this trial, whenever it gets kicked off.]

DBS (deep brain stimulation) of the PPN (pedunculopontine nucleus) for PSP: (Stefani, et al, Brain 2007)
* electrodes (stiff wire) are passed into the PPN of the brain
* Univ of Toronto is experimenting with this
* preliminary results are favorable but we’ll just have to see
* he thinks this may be unwieldy but since it’s one of the few treatments available, people may give it a try
* this will mainly help the balance problem

[Robin’s note: You can find general info on this CurePSP-funded study here —]


QUESTIONS AND ANSWERS (all answers were given by Dr. Golbe)
[Robin’s note: I’ve edited the questions, grouped together similar items, and added headings]

Question: Are there any clinical trials going on?

Answer: Besides the trials just mentioned, there are others that have been going on for some time.

One trial going on now of CoQ110 at Lahey Clinic in MA. There are other US sites where this trial is being conducted. Contact the CurePSP office to be referred to the Lahey Clinic researcher.

[Robin’s note: this is the first I heard that a CoQ10 trial is being conducted in other places in the US outside Lahey Clinic. I might’ve misunderstood this.]

The lithium trial is still going on but it has stopped recruiting. Most patients had severe side effects.

Dr. Litvan’s epidemiological study is ongoing. It’s not interventional.

Question: Does CoQ10 help any and what is it used for?

Answer: The German trial of CoQ10 (published in mid-2008) did show a benefit even though the treatment was only 6 weeks. The benefit seen was the amount of energy available in the brain was increased, as noted by a special scan. This needs to be confirmed.

[Robin’s note: You can find the abstract of the German study posted here — — or look it up on using PubMed ID#18464278.]

CoQ10 helps with overall brain energy and metabolism. It doesn’t really help symptoms.

Question: What evidence is there on the use of lithium to slow the progression of PSP and what are the associated risks? Does lithium work in the long term?

Answer: Unfortunately, lithium was not well-tolerated by the PSP population so we don’t know if it slows progression. There were many side effects: dizziness, nausea, etc. Lithium is not a realistic treatment for PSP.

Question: Has anyone looked at diet and micro-nutrients to reduce PSP symptoms? I am thinking of antioxidants.

Answer: Antioxidants have been tried in PSP without success.

Question: Are there any other possible treatments to slow the progression of PSP?

Answer: Nypta and Davunetide will be studied in 2010.

Question: Is there anyone who treats PSP with cord blood stem cells?

Answer: No.

We need to consider the goal of stem cell treatment. In PSP, many areas of the brain are affected (with many types of brain cells) so cell replacement therapy isn’t practical. Another issue is how can you get the replacement cells to form proper connections with other cells?

But maybe stem cells can be drug delivery vehicles: program a stem cell to make a drug. No one has gotten this far yet.

Question: Is there good news on the horizon?

Yes. In the past couple of years, there have been a lot of treatment trials in PSP, sponsored by drug companies and the NIH.

Question: What’s the difference between ubiquinone and ubiquinol?

Answer: I don’t know. Ubiquinone is CoQ10.

Question: [Robin’s note: Sorry but I didn’t hear the question. I think it was about the experimental drug Rember.]

Answer: Experts don’t think this works in Alzheimer’s Disease, and it hasn’t been tested for PSP.

[Robin’s note: If the question was about Rember, note that the manufacturer, TauRX in Singapore, has not been willing to supply this medication to be tested in PSP, despite the efforts of US-based researchers to initiate a trial.]

Question: When will we see some concrete results from the PSP genetics study, and what kinds of follow-up studies are anticipated?

Answer: We’ll see the replication study results in December or January. Assuming the findings are confirmed, a paper will be published shortly thereafter.

There will be lots of follow-up studies: animal models utilizing the genes; how proteins are behaving and what they are interacting with; drug treatment screening. A “whole new world of research” will be opened up.

Question: What is it so important to confirm four new unsuspected genes?

Answer: This could help with new treatment.

Question: Are genetic screenings available to check for one’s chances of getting PSP?

Answer: No. Even checking for the presence of the H1 haplotype is not that useful.

Question: How long does it take for PSP to develop? How long prior to diagnosis has the disease been in place?

Answer: We don’t know for sure. The theory with most neurodegenerative diseases is that they’ve been going on for 5-10 years prior to symptoms appearing.

Question: Why is there such a difference in the survival times of PSP patients?

Answer: Actually, among PSP patients there is less variance in survival times than we see with PD. This may depend on one’s underlying health — how quickly you can fight off pneumonia and how quickly you can heal from injuries. May depend on how many genetic abnormalities you have.

Question: How long will I live after diagnosis?

Answer: No way to know for sure. Seems to be related to the skill and dedication of the caregiver.

Average survival after the first symptom appears is 7-8 years. Some people do better than this.

[Robin’s note: There’s no research evidence to support Dr. Golbe’s assertion that caregivers can influence survival time. It largely depends on what type of PSP you have, your gender, your age at onset, and the interval between disease onset and reaching the first clinical milestone. See, in particular, the “Clinical Outcomes” paper on this topic. At present, it is available for free here —]

Question: How can I tell what stage I’m in?

Answer: See the PSP Rating Scale on It is a prediction of future survival. There is a margin of error.

[Robin’s note: Dr. Golbe authored the rating scale several years ago. You can find it here —

See this post about an article on interpreting the scale —]

Question: Is the eye movement problem = downbeat nystagmus?

Answer: [Sorry, I didn’t hear the answer but I’m pretty sure he said “no.” I don’t think there is such a thing as “downbeat nystagmus.” There is downward gaze palsy in PSP and nystagmus.]

Question: Is the eye palsy always downwards?

Answer: No, it can start out upwards. Upward gaze palsy is common among the elderly.

Question: What physiologically causes the stiffness in the neck? Any treatment for this?

Answer: There is no research on this. There’s not even an informal recommendation on this.

Botox can be used for neck stiffness. Botox must be injected cautiously.

Question: Can hunching over occur in PSP?

Answer: Usually there is a very erect posture or a backwards posture. But the majority of PSP patients hunch forward and even sideways (“scoliosis”).

Question: Do you find that a low dose of Prozac can help the spastic speech and dysphagia in PSP?

Answer: I haven’t heard of this.

Question: We are using Seroquel for restlessness and Ambien for sleep. Should my loved one be sleeping/resting more? Are there better choices of drugs?

Answer: Sleep problems in PSP can worsen. Talk to your MD about medications.

Question: What’s the key difference between PSP and CBD?

Answer: PSP – mostly a disorder of balance (worst problem); symmetric

CBD – balance is not that big of a problem; mostly a problem of the use of limbs; very asymmetric (one side affected much worse than the other side; eventually both sides are affected); apraxia (limb loses the ability to perform a task that has been practiced in the past, such as saluting); issue with perceiving spatial concepts (eg, tracing a number on the palm)

Question: What is the relationship between alpha-synuclein and tau, and the effect on them by hsp70?

Answer: Alpha-synuclein is associated with Parkinson’s Disease. AS is to PD as tau is to PSP. Complicated: it was recently discovered that in PD there can be a problem with tau. And the H1 haplotype is more common in PD than in normal controls.

hsp70 (heat shock protein 70) is a chaperone protein. They regulate the activity of other proteins. hsp70 help blobs of protein from forming. He can’t speak to the relevance of hsp70 as a treatment in PSP.

[Robin’s note: You can read about the University of South Florida study in mice here —]

Question: My husband has been diagnosed with MSA. But PSP now sounds like a possibility. Should we spend more money/effort to study neurodegenerative disorders in general or does specificity help?

Answer: Lots of research is applicable to all neurodegenerative diseases.

Question: Is there a connection between PSP and MS (multiple sclerosis)?

Answer: No. MS is an immune disorder. This doesn’t happen in PSP. MS is a disease of the cells that insulate the axons. MS isn’t primarily a disease of brain cell breakdown.

Question: I had a DNA test done and was diagnosed with SCA3. Is it possible that I could also have other forms of a movement disorder? Should I get tested for all disorders?

Answer: SCA3 is a completely different disease.

Question: Is cerebellar ataxia a type of PSP? Is PSP a type of Parkinson’s? Is SCA a type of PSP?

Answer: No (to all three questions).

Cerebellar ataxia is a collection of symptoms related to the cerebellum.

Planning for the End of Life: The Role of Hospice in PD Care

This new two-page Parkinson’s Disease Foundation publication was mentioned today during the “Care for the Caregiver” webinar. Though the title refers to PD and PDF is the publisher, I don’t think there’s anything here that is PD-specific. I especially liked the section on “How Should Hospice be Discussed with a Loved One?” though I thought it was too brief. I also liked the list of “Questions to Ask When Considering Hospice” but felt that some suggestions should’ve been provided for each question.

One note: it’s mentioned below that private health insurance may provide hospice benefits. Many insurance companies limit hospice benefits to a lifetime maximum of 6 months. One CBD family I know of in Arizona exhausted that benefit. Fortunately a non-profit hospice agency is providing free hospice care to this family. … ice_09.pdf

Planning for the End of Life: The Role of Hospice in Parkinson’s Disease Care
Parkinson’s Disease Foundation
Fact Sheet, 2009

As Parkinson’s disease (PD) progresses into the advanced stages, its symptoms can often become increasingly difficult to manage. The daily care needs for a person with PD may overwhelm the family caregiver’s physical, mental and emotional capabilities and require more help than the caregiver alone can provide.

In such situations, hospice can be an option. Although hospice is often associated with a terminal disease such as cancer, it is an option for individuals with chronic diseases, such as PD. Hospice can provide services and support with the goal of providing a quality, peaceful death while allowing the person with PD to stay in a familiar environment.

What is Hospice?
Hospice is a program of care designed to improve quality of life through pain relief and symptom management for individuals who are
facing the end of life. It can also provide valuable caregiver and family support with bereavement services for up to 11 months after the death of a loved one.

What Benefits Will Hospice Provide for a Person and Family Living with Parkinson’s Disease?

Hospice care is provided through home care agencies or in a facility (nursing home or hospice facility). Home hospice services are the most common type of hospice service used by a person with Parkinson’s and offer the opportunity for the individual to remain at home during his or her last days and months of life surrounded by friends and family.

Hospice care provides a person with PD, his or her caregiver and family with health care providers who have expertise in the complex dying process. The core home hospice staff is comprised of a physician, nurse, social worker and home health aides. Other personnel may include physical, occupational and speech therapists, and pastoral services.

Hospice may also provide durable medical equipment, medical supplies,medications and counseling. Other services may be provided depending on the needs of the person with PD and the structure of the home hospice agency.

When is the Right Time for Hospice?
Determining when the time is right to consider hospice services can be a difficult decision for the person with Parkinson’s, their family and health care providers. Parkinson’s, while a chronic and progressive disease, has a course that can be uncertain with no clear indication of the end of life. Yet, those with PD often have additional significant medical problems — such as advanced dementia,
recurrent pneumonia, weight loss, urinary incontinence, infections and pain — that could be better managed through hospice.

Current Medicare benefit guidelines ask health care providers to project that an individual has six months or less to live to enroll in
Medicare reimbursed hospice programs. However, many individuals live beyond six months while enrolled in hospice. At the end of the initial six-month period, the hospice agency will reevaluate the care plan and needs of the person with Parkinson’s and either reenroll the individual for an additional three months or discharge the individual from hospice. Patients are discharged from hospice if the individual improves and doesn’t meet the criteria any longer.

How Should Hospice be Discussed with a Loved One?
There are four parties involved in enrolling a person in hospice and managing end-of-life care: the family/caregivers, the person with Parkinson’s, the hospice agency and the health care provider.

Hospice often has negative associations for individuals and is thought to be a sign of “giving up” or accepting “no hope.” Overcoming these negative associations is an important first step in having the conversation about the benefits of end-of-life care in hospice. Discussing end-of-life issues is difficult and often avoided. As part of the care team, individuals with PD, caregivers, and health care providers can participate equally in ongoing discussions about planning for end-of-life care.

How Does One Pay For Hospice?
Private health insurance may provide hospice benefits. Medicare does provide hospice coverage. More information on Medicare hospice benefits can be found at

What is the Application Process for Home Hospice Services?
Physicians initiate the enrollment process for a person with PD by making a referral to a hospice agency. Families can have input in the selection of the hospice agency by getting recommendations from outside resources (i.e., support groups) or by researching local hospices. The National Association of Home Care and Hospice provides information on how to locate hospice services at

Once the doctor has sent a referral to the hospice agency, a hospice nurse will come to the home for an initial assessment to determine if the person with PD is eligible for hospice. If the individual qualifies, he or she will be evaluated for their specific needs,
types of services, frequency of care and equipment.

Is Hospice the Only Option?
Not all people with Parkinson’s will decide to enroll in a hospice program for end-of-life care. There are alternatives to hospice. Persons with PD and caregivers who are not ready for hospice services but who need assistance with care might consider respite services, nursing home placement or home health aides to assist with care.

Respite care is a temporary care service that provides patients with care based on his or her needs and allows a break for the caregiver. Nursing homes or assisted living facilities may offer respite services. Often a caregiver uses this time for his or her own health care or to visit family members or friends that live at a distance. Nursing home placement is an option for persons who need full-time care that the caregiver or family is not able to provide in the home.

Questions to Ask When Considering Hospice:
1. Does the person with PD understand his or her prognosis and health care needs?
2. Does the person with PD want to remain at home until the end of life?
3. Has the family, caregiver and person with PD discussed long-term care options?
4. Has the person’s health care provider been consulted in planning for end­of-life care?
5. Is there a living will and/or power of attorney in place for the person with PD?
6. If the person with PD is unable to communicate their wishes for end-of-life care, can someone represent their wishes for end-of-life care?
7. How much does the person with PD know about hospice and how does he or she feel about it?
8. Does the person with PD have insurance or is he or she Medicare-eligible for hospice care?
9. What other resources can be used to ease caregiver burden?

Eileen Hummel, R.N., B.S.N., and HeidiWatson, R.N., B.S.N., are Clinical Nurse Coordinators at the Philadelphia VA PADRECC (Parkinson’s Disease Research, Education and Clinical Center). This fact sheet has been reviewed by Lisette Bunting-Perry, M.Sc.N., R.N., Assistant Clinical Director, PADRECC and Barbara Habermann, Ph.D., R.N.,Associate Professor, Indiana University.

To find additional resources for managing advanced Parkinson’s disease, visit or order a copy of PDF’s Parkinson’s Disease Resource List at (800) 457-6676 or

If you have or believe you have Parkinson’s disease, then promptly consult a physician and follow your physician’s advice.
This publication is not a substitute for a physician’s diagnosis of Parkinson’s disease or for a physician’s prescription of drugs, treatment or operations for Parkinson’s disease.

Advance Directives – Link to Neurology Now Article and Robin’s Resources

The latest issue of Neurology Now magazine has a good article on advance directives.  If you are reading this email, you should have advance directives for yourself and any adult you may be caring for!

We are lucky in California that our state accepts the POLST form. This is the form that anyone with a neurodegenerative disorder should have filled out.  You can find info on the POLST form here:


POLST form for CA:
[Editor’s note:  the CA POLST form can be found at]

If you live in California and don’t have a neurodegenerative disorder, you should consider completing this form:

Info on the California Medical Association (CMA) Advance Health Care Directive Kit:

Another resource I like a lot, which is not mentioned in this article below, is:

Five Wishes from Aging with Dignity:  (cost is $5)

Many people (including myself) have completed the “Five Wishes” document, have added it to our living will, and have given it to our healthcare power of attorneys.

Here’s a link to the “Neurology Now” ( article on advance directives:

Get It in Writing!
Advance directives give you control over your health care.
by Lisa Phillips
Neurology Now, Volume 5(5), September/October 2009, p 35-36

Dealing with anosognosia (unawareness of decline) is a caregiver support forum for Alzheimer’s caregivers. They have an interesting article on their website about anosognosia, which is a lack of self-awareness about one’s decline or condition. The best part of the article are “examples of how to approach, interact and speak to someone who has anosognia.”

Here’s the summary from the article: “The person who has anosognosia is unaware of deficits or the progressive decline in abilities to manage tasks and self-care. The person with anosognosia is not in denial; they have limited awareness or are unaware of the decline. When people with anosognosia confabulate, they believe what they are saying; they are not lying. Their remarks should be treated with respect, followed by a smooth transition to whatever tasks or activities need to occur next. Regular help for the home and family, planning ahead and working with a positive, partnership approach will help with the long-term, daily care management.”

Here’s a link to the full article and some additional excerpts. … gnosia.pdf

“A lack of awareness of impairment, not knowing that a deficit or illness exists, in memory or other function is called anosognosia. The term anosognosia refers to brain cell changes that lead to a lack of self-awareness. … The impairment may be in memory, other thinking skills, emotion, or movement.”

“Anosognosia differs from denial. Denial is a strategy used to reject something that a person wants to ignore, partially avoid, or reject outright because it is too difficult or causes too much stress. The person may minimize a problem or accept part of the truth, for example, the person may accept the fact of being chronically ill but want to avoid dealing with it by not taking medicine. Sometimes a person is in denial in order to avoid taking any responsibility for an issue or situation. Anosognosia is not denial.”

“Anosognosia may occur in different progressive memory disorders. Often the progressive dementia (sometimes referred to as a progressive memory disorder) is of the Alzheimer’s disease type, sometimes it fits into the category of Lewy body disease or a frontal-temporal lobar degeneration.”

“Interaction Tips
Providing regular assistance with daily chores, transportation, and personal care and restricting unsafe activities are important. For example, someone may need to make sure that meals are readily available, that spoiled food is discarded, and that alcoholic beverages are not accessible. The controls for operating the stove and water heater should be inaccessible. Someone should be responsible for setting the home thermostat at an appropriate temperature and then locking the thermostat so that the person who is not accurately interpreting body temperature cannot reset the room temperature at too high or too low. Soiled clothing should be laundered immediately or kept unavailable (out of sight ­ out of mind) until the clothing is clean.”

“The Checklist for Family Matters, located at is a useful tool to help families with planning for long-term care management. Regular respite for the family caregiver(s) is essential!”

“Examples of how to approach, interact and speak to someone who has anosognosia:

1. Down-size and decrease unnecessary chores and responsibilities.

Use a positive approach, such as, “It is time to plan ahead about moving to a retirement community where there are kind people and some of your friends so you have more time to do what you like, such as read and go for a walk every morning.”

Don’t use a negative approach, such as, “This house and yard are too much work for all of us. It is hard for you to take care of the house, the yard, and yourself. You need to move to a place where people are always around to help you.”

2. Partner with the person.

Use a positive approach, such as, “Let’s work together on the front porch, then go out for a nice dinner.”

Don’t use a negative approach, such as, “You really need to clean up that mess of old magazines, newspapers and piles of trash on the front porch.”

3. Focus on the person’s concern and subtly include your concern.

Use a positive approach, such as, “When you take this multi-vitamin, how about taking these “brain-vitamins” that the doctor prescribed to keep your memory strong?”

Don’t use a negative approach, such as, “The doctor prescribed these pills and you have to take them every morning.”

4. A gentle, positive voice should be part of a positive empathic approach.

Use a positive approach, such as, “To keep up with these bills, we should work as a team. I will come over on Saturday mornings with your favorite breakfast and we will write out the checks together. After you sign the checks, we will put them in their envelopes and take them to the mailbox.”

Don’t use a negative approach, such as, “You have to pay these bills on time. The utility companies have sent notices threatening to shut off the gas and electricity. I’ll handle the bills from now on.”

5. Provide available assistance and a structured schedule of tasks including personal care, activities including chores and leisure
activities, and “down-time” including a favorite activity or no activity.

Use a positive approach, such as, “After we walk the dog, we will finish the laundry and then sit down for some of that applesauce I cooked this morning.”

Don’t use a negative approach, such as, “There is so much to do? What do you want to do this morning? We have to walk the dog, finish the laundry, and clean the kitchen. The work really piles up fast around here.”