Clinical Rating Scale for PSP

Some of you might remember that there was a Neurology ’06 article on “Measuring quality of life in PSP: the PSP-QoL.”  The UK/US authors of that article used a 45-item PSP Quality of Life scale (PSP-QoL) measuring the physical (22 items) and mental (23 items) health impact of PSP.  (That 45-item scale is not part of that article and I haven’t been able to obtain it…yet.)  I emailed you excerpts of the article just recently (June ’07).

I learned about a year ago that Dr. Lawrence Golbe, a US expert in PSP, had developed a clinical rating scale for PSP.  An AZ-based caregiver noted that her mother’s neurologist was using the scale, and that it had both Dr. Golbe’s name and the Society for PSP’s name at the top.  Dr. Golbe is the chair of the Scientific Advisory Board for CurePSP, the new name for the Society.  I got a copy of the rating scale but had no idea how to use it.

Then, in April ’07 I saw a PubMed abstract of an article written by Dr. Golbe and another researcher on his 28-item rating scale.  The article, in the journal Brain, contains the rating scale, instructions on how to complete it, and probability figures associated with survival times based on the rating.

Dr. Golbe believes the scale has prognostic value — that is, it can be used to determine likely survival time.  It is not a diagnostic scale; it can’t be used to determine if someone has PSP.

What follows is the abstract from the Golbe article in Brain ’07.


Editor’s Update:  The full article is now available online at no charge.


Brain. 2007 Jun;130(Pt 6):1552-65. Epub 2007 Apr 2.

A clinical rating scale for progressive supranuclear palsy.

Golbe LI, Ohman-Strickland PA.
Neurology, Robert Wood Johnson Medical School, University of Medicine and Dentistry of New Jersey, Brunswick, NJ.

We devised a Progressive Supranuclear Palsy (PSP) Rating Scale comprising 28 items in six categories: daily activities (by history), behaviour, bulbar, ocular motor, limb motor and gait/midline. Scores range from 0 to 100, each item graded 0-2 (six items) or 0-4 (22 items). Inter-rater reliability is good, with intra-class correlation coefficient for the overall scale of 0.86 (95% CI 0.65-0.98). A single examiner applied the PSPRS at every visit for 162 patients. Mean rate of progression was 11.3 (+/-11.0) points per year. Neither onset age nor gender correlated well with rate of progression. Median actuarially corrected survival was 7.3 years. The PSPRS score was a good independent predictor of subsequent survival (P < 0.0001). For example, for patients with scores from 40 to 49, 3-year survival was 41.9% (95% CI 31.0-56.6) but 4-year survival was only 17.9% (95% CI 10.2-31.5). For those patients, likelihood or retaining some gait function was 51.7% (40.0-66.9) at 1 year but only 6.5% (1.8-23.5) at 3 years. We conclude that the PSPRS is a practical measure that is sensitive to disease progression and could be useful as a dependent variable in observational or interventional trials and as an indicator of prognosis in clinical practice.

(PSPRS = PSP Rating Scale)

PubMed ID#: 17405767   (see for abstract)

“Dance of Loss” (article by LBD caregiver)

The July/August 2006 issue of Neurology Now contains a sweet article from Ginnie Burkholder, who cares for her husband with Lewy Body Dementia (LBD).  You can find the short article by going to and then doing a search on Burkholder.  I’ve copied the text below.

Neurology Now, a relatively new online publication of the American Academy of Neurology, is available free.  This magazine is written for patients, families, and caregivers of persons with neurological disorders — from migraine headaches to Alzheimer’s Disease.  This bi-monthly magazine focuses on positive messages regarding wellness and prevention, current research, clinical trials, and patient stories that emphasize coping.


Dance of Loss: Longing for the life shared before a husband’s dementia
Ginnie Horst Burkholder
Neurology Now
July/August 2006

It is a May morning as I listen to Anne Murray sing “Could I have this dance for the rest of my life?” I once knew that sentiment for my husband. I miss the vibrant person who invited that feeling, and I miss the dance.

I do my exercises with the sun on my back, sitting in front of an east window. Finished, I stare at the wallpaper. I am back in time when it was hung by my husband, who always said he could do anything. The match is off on this wall. Was this the beginning of this cannibalistic disease or just one of those mistakes anyone can make?

I look at two more walls. One matches perfectly. Another waited years for the papering job to be completed. He had lost interest. Or was it the disease again? I am flooded with memories of life before the onset of this progressive dementia caused by brain abnormalities called Lewy bodies. I marvel at all we managed to enjoy before his diagnosis nine years ago at the age of 51.

People say you have your good memories. It is true. I remember that once he knew my birthday, knew I didn’t want sugar on my cereal, knew I needed a backrub, tossed me a wink. But the longing for what was eats at me, and I cannot stay there. The memories are stark reminders of what should have been. Like a bird with nowhere to land, my thoughts avoid the past and the certain deterioration in the future and, instead, alight unwillingly in what we have, the present.

Nelson still remembers my name, but he doesn’t remember me. Usually. Last night, he couldn’t say my name. He resorted to Amy’s mom. I hurt for him, provided my name, and said, “I’m here. What do you need?”

I’m calm, compassionate, but inside, like a leaky faucet, sadness drips from a never-ending spigot of loss.

The loss and longing are everywhere. It is there at lunch in an innocent conversation with my sister about water softeners when I ask how much salt they go through. She doesn’t know; her husband takes care of that. The longing for someone to be the live-in handler of such responsibilities explodes like a fire burst inside of me. I cannot escape that I alone am the manager for this household.

Another lunch, and another sister is having her neck rubbed by her husband. She says it feels so good she doesn’t want to move. I feel the stiffness in my own neck and shoulders, and know the futile longing for Nelson to be able to do what he once would have done willingly and easily. Lewy body disease has made his motions stiff and wooden.

Reminders are all around me. Couples our age are traveling. We take turns driving. It is like the carrot that should have been ours has gone to everyone else.

This morning I say to him, “Eat something and take your pills.”

“You played a trick on me,” he answers. “There is nothing in there.”

I tilt the pills from the container into his hand and assure him there is. I no longer bother to wonder why or how he can do what he does.

His offering of love to me is the morning kiss goodbye at the beginning of the day as he goes out the door to his adult daycare or the things he made as he comes back home later that afternoon. There is a snowman made from fuzzy little puffballs, a small painted wooden horse, or a card with some painting on it. He brings them proudly and we put them on display.

I long for him to anticipate my need and bring me unexpected love offerings. But the blinds this disease has pulled over his vibrancy are opened for only brief moments. “You are my queen for the day,” he says, as I tuck him in for the night. In those moments, I know his love remains even though the dance is gone, replaced by wooden feet, confusion, this always unpredictable disease, and so much longing for what could have been.

16 autopsied cases of PSP (Canadian research ’02)

I’ve been looking around for journal articles that review autopsied cases of PSP and relate these back to the clinical symptoms seen.  This 2002 article doesn’t have a large sample — only 16 cases.  In half of these cases, PSP was never diagnosed while the patient was alive.  All 16 cases were seen by one neurologist between 1969 and 2000 at a Canadian university.  (Incredibly, 30% of all patients seen at this movement disorders clinic in Canada agree to autopsy!)

This was the most interesting info in the article:

“Those with incorrect final diagnosis were mean 64.4 years old at onset (vs. 63 years (for those clinically diagnosed with PSP)), and had longer survival (mean, 9.8 vs. 7.5) than those diagnosed clinically.  The development of falls, evolution to H&Y stage >= 3 disability, bulbar symptoms, and cognitive decline were also delayed in the undiagnosed group.  Nearly two-thirds of those not diagnosed as PSP benefited from LD (levodopa) alone, but none of those diagnosed as PSP during life improved.”

I view Dr. David Williams’ research from Queen Square Brain Bank in the UK to be a directly connected to this Canadian research.  Dr. Williams has found that there are two main clinical types of PSP — cases of Richardson’s syndrome (RS) made up 54% of all cases, and were characterized by the early onset of postural instability and falls, supranuclear vertical gaze palsy and cognitive dysfunction; cases of PSP-parkinsonism (PSP-P) made up 32% of all cases, and were characterized by asymmetric onset, tremor, a moderate initial therapeutic response to levodopa and were frequently confused with Parkinson’s disease.

Another piece of info from the Canadian research that I found distressing was:  “Our data indicate that accurate clinical diagnosis of PSP is most likely within 5 years of onset.”  That’s a long, frustrating wait.

When the Canadian research abstract says “Mean duration at PSP diagnosis was 4.8 (range, 2-11) years,” I take this to mean that the symptoms of PSP began 4.8 years before a PSP diagnosis was rendered.  So I believe that the other 8 who were never given a PSP diagnosis during life were not part of this calculation of “mean duration at PSP diagnosis.”

Note that the PSP diagnostic criteria only became set in 1996.  (These are the NINDS-SPSP criteria referred to in the excerpts.  The authors are not fans of the ’96 criteria.)  Despite the application of different diagnostic criteria, the diagnostic accuracy would not have improved, the researchers said.

The abstract follows.


Movement Disorders 2002 Nov;17(6):1255-64.

Progressive supranuclear palsy diagnosis and confounding features: report on 16 autopsied cases.

Birdi S, Rajput AH, Fenton M, Donat JR, Rozdilsky B, Robinson C, Macaulay R, George D.
Division of Neurology, University of Saskatchewan, Saskatoon, Saskatchewan, Canada.

We evaluated 16 (15 men, 1 woman) autopsy-verified progressive supranuclear palsy (PSP) cases during 31 years (1969-2000) for clinical diagnosis and the course of the disease. The onset was gait difficulty or postural instability in 9 (56.3%), general motor slowing in 3 (18.8%), and tremor in 2. One case had onset with cognitive decline and 1 as hemidystonia. Four cases had supranuclear ophthalmoplegia (SNO) at the first assessment and were diagnosed as PSP. By last assessment, PSP diagnosis was made in 4 additional cases, but in 8 (50%) who never manifested ophthalmoplegia (mean 9.8 years after onset), PSP diagnosis was not made. Other manifestations included bulbar symptoms in 13 (81.3%), and cognitive impairment in 10 (62.5%) during the course of illness.

Fifteen cases received levodopa, amantadine, anticholinergics, dopamine agonists, and selegiline in different combinations with symptomatic benefit in 9 of 15 (60%). Five had some improvement on levodopa alone and 3 showed more improvement when a dopamine agonist was added to levodopa. In general, the benefit was minimal and occurred only early in the course of illness.

The mean age at onset was 63.7 (range, 53-85) years. Mean duration at PSP diagnosis was 4.8 (range, 2-11) years. Mean survival was 8.6 (range, 3-24) years and mean age at death was 72.3 (range, 60-89) years. When the different diagnostic criteria recommended in the literature were used, the accuracy of clinical diagnosis did not improve substantially.

PubMed ID#: 12465065   (see for abstract)


Rarest type of PSP- Pure akinesia with gait freezing

This is a follow on to Dr. David Williams’ research at the Queen Square Brain Bank in London on PSP.  He argues that there are three different types of PSP.  The least common type he calls “pure akinesia with gait freezing” (PAGF).  He recently published a paper on PAGF.  The abstract follows.


Movement Disorders. 2007 Aug 21; [Epub ahead of print]

Pure akinesia with gait freezing: A third clinical phenotype of progressive supranuclear palsy.

Williams DR, Holton JL, Strand K, Revesz T, Lees AJ.
Faculty of Medicine (Neurosciences), Monash University (Alfred Hospital Campus), Melbourne, Australia.

The clinical syndrome of pure akinesia has most often been associated with progressive supranuclear palsy (PSP) and is characterized by difficulty initiating gait and “freezing” during walking, writing and speaking. Similar syndromes have been described under the rubrics of primary progressive freezing gait and primary gait ignition failure. We investigated the specificity of the clinical syndrome of pure akinesia with gait freezing (PAGF) for PSP-tau pathology. Among 749 patients archived at the QSBB, only 7 fulfilled proposed diagnostic criteria of: gradual onset of freezing of gait or speech; absent limb rigidity and tremor; no sustained response to levodopa; and no dementia or ophthalmoplegia in the first 5 years of disease. In these cases detailed pathological examination was performed. PSP was the pathological diagnosis in six patients, and Parkinson’s disease (PD) in the seventh. As defined, this syndrome had a positive predictive value of 86% for PSP-tau pathology. In the cases with PSP there were no additional features of coexistent vascular or PD and the median PSP-tau score was 3, reflecting relative mild tau load. The clinical syndrome of PAGF appears to have a high specificity for PSP-tau pathology. This relatively uncommon presentation of PSP-tau pathology has less severe tau accumulation than in the more common, “classic” PSP clinical phenotype: Richardson’s disease.

PubMed ID#: 17712855


First Steps after a Dementia Diagnosis

Hurley Elder Care Law offices are based in Atlanta. In the August 2007 issue of their publication “The Elder Issue,” they offer some guidelines for families to follow once a family member has been diagnosed with Alzheimer’s or any dementia type.

Some of the steps families should follow include:

* Organize a family meeting
* Assess your loved one’s abilities
* Learn about Alzheimer’s disease
* Find a good healthcare provider
* Make long term plans
* Create a support network
* Investigate resources for local support

Here’s a copy of the law firm’s advice. Just replace “Alzheimer’s Disease” with whatever disorder of interest to you as I think the guidelines apply to us all.



Family Caregivers’ Guide to First Steps after a Diagnosis of Alzheimer’s Disease
Hurley Elder Care Law
The Elder Issue
August 2007

Family situations vary tremendously. Sometimes all adult children and the spouse of the person with AD are in agreement as to the next steps to take, but possibilities for family disagreements are many. The cooperation of the person with AD is very important. Sometimes the person with AD is willing to stop driving, sign all of the important legal documents, and accept the care that he or she may need. But often there is resistance to making changes by the person with dementia.

There are concrete steps that family members can take to make the journey smoother. Some of the steps that you can take as a family member of the person with dementia are:

FAMILY MEETING: Arrange for a regular family meeting to discuss all of the issues related to the diagnosis. Discuss the diagnosis with everyone in the family including family members in other cities or states. Talk about what needs to be done now and in the future. Although the responsibilities of various family members will differ, everyone needs to know what is happening. It is a good idea to have one person who will speak for the family on issues related to health care and the same person or a different person who will speak for the family on financial issues. After the family decides the correct person for each responsibility, formal power of attorney forms can be signed. For example, some family members may live in the same city as the person with dementia. It would make logical sense to designate one of those family members with power of attorney for health care since they can go to the doctor’s office with the person with dementia.

FAMILY MEMBER WITH AD: Make a realistic assessment of the abilities of the person with the disease. The family members cannot rely solely on what the person says she or he can do. These actions must be observed first hand. The idea is to give the person with the disease as much as they can reasonably do for themselves, while not making unrealistic demands. Driving, making financial decisions, staying alone, and using the kitchen safely are all examples of issues that need to be examined on a regular basis. As an example, a person with dementia may think that it is still safe to drive the car alone even though she or he may have gotten lost recently.

LEARNING ABOUT AD: Learn all that you can about the disease. Read books, search the Internet, talk with other family caregivers like yourself, and talk to knowledgeable health care providers. One good place to start is the Alzheimer’s Association at and 1-800-272-3900. A very useful book is “The 36 Hour Day: A Family Guide for Persons with Alzheimer’s Disease, Related Dementing Illnesses, and Memory Loss in Late Life” by Nancy Mace & Peter Rabins.

MEDICAL CARE: Find a health care provider with whom you can work. It may be the physician who diagnosed the disease, or it could be a research physician at Wesley Woods of Emory University or another research program. The person you work with should understand the progression of the disease and know the latest information on medications to treat the symptoms. It is very important that someone goes to each physician appointment with the individual suffering from Alzheimer’s disease in order to better understand the disease and the treatment. This is actually true for most people since they cannot remember everything that the doctor told them five minutes later, regardless of whether they have memory impairment or not.

LIFE CARE PLANNING. Make certain that the necessary legal and financial plans are in place. The most important thing that people should consider is how to find, get and pay for good long term care. This includes the need for an evaluation of the assets available, who needs to have access to those assets and what are the alternative means of financing long term care. From the legal document perspective, a review of or putting into place a Durable Power of Attorney, Health Care Power of Attorney and a Will and/or Trust is very important while the individual still has sufficient capacity to make such decisions.

EMOTIONAL SUPPORT: Set up a support system for yourself. Who in your family or among your circle of friends would be the most supportive of you and your family? Work with them in finding the help you need. You may just need to have someone listen to you and provide some relief from care giving. You may want to join a support group sponsored by the Alzheimer’s Association to work through the feelings that you have and to get ideas about how to best care for your family member with AD. Remember that if you burn out as a caregiver, you are no good to anyone, including yourself.

SAFE RETURN: At a minimum, register the person with Alzheimer’s disease in Safe Return. This is a program of the Alzheimer’s Association that consists of a national registry and an identification bracelet. The cost for Safe Return is $40.00 for the first year and $20 for each year your relative is in the program. With changing technology, there are more and more options available for people to locate a lost or wandering loved one.

RESOURCES TO HELP: Find out about services available in Georgia to assist a person with Alzheimer’s disease. Develop a list of places to contact including adult day, home care agencies, and long term care facilities. Know the services available in your community so you can access these organizations and services as you need them.

CAREGIVER SUPPORT: Take care of yourself. You need to think of yourself as a long-distance runner, not as a sprinter. Pace yourself. Prepare for the long haul. This is not a disease that develops or progresses quickly. Learn to recognize your stress risks and find ways to relieve them. Accept help. Your life and the life of the person with the disease depend upon you caring for yourself.

DIFFICULT SITUATIONS: Difficult situations can easily develop related to family members with Alzheimer’s disease. One example is when the spouse or adult children will not take the keys away from the person with AD, nor will they admit that there are safety issues involved because they themselves are in denial. People in certain stages of the disease cannot make rational decisions. Remember that and take action to protect not only your family member, but the public at large. Another frequently occurring situation happens when one spouse has Alzheimer’s disease and the other spouse has physical health problems. In this circumstance, the husband and wife need different kinds of care and may not be able to stay in their home indefinitely. It pays to be prepared for this eventuality.

HELPING CHILDREN AND TEENS. If you are an adult daughter or son caring for a parent with Alzheimer’s disease, it is likely that you have young children or teenagers still living in the home. Children often experience a wide range of emotions when a parent or grandparent has AD. Younger children may be fearful that they will get the disease or that they did something to cause it. Teenagers may become resentful if they must take on more responsibilities or feel embarrassed that their parent or grandparent is “different.” It is important to find out what the emotional needs of your children are and try to meet them.

The diagnosis of Alzheimer’s disease or any related dementia can be very threatening news, but when a spouse and/or adult children are willing to deal with the illness in a systematic manner, family conflicts can be minimized and the quality of life of the person with the disease can be maximized. The most important thing to do is to take action and take action quickly. The sooner that action is taken, the more options there are available.