MRI and MRS in PSP, MSA-P, PD (Brazilian study)

This small Brazilian study looked at 11 patients with PSP, 7 patients with MSA-P, 12 patients with PD, and 10 controls. Everyone was studied with magnetic resonance imaging (MRI) and spectroscopy by MRI (MRS).

The authors concluded:
“(1) Patients with PSP and MSA-P presented increased motor and cognitive impairment in the scales used, correlating with decrease in NAA/Cr in lentiform nucleus and NAA/Cho in midbrain in the PSP group;
(2) Cerebral and cerebellar atrophy were more prevalent and severe in PSP and MSA-P groups;
(3) Linear hypersignal in the lateral portion of the putamen, hypersignal in midbrain and in pons, all suggest the diagnosis of PSP or MSA-P;
(4) Midbrain or pons atrophy suggests atypical parkinsonism, the former PSP, and the latter MSA-P;
(5) Comparing the two methods, MRI and MRS, the former had better applicability.”

The abstract and lots of excerpts follow. The English-language article is available for free online here: –> PDF form … so&tlng=en –> HTML form

For me, the most interesting parts of this article were the three figures with captions, indicating what percentage of the patient groups had particular MRI or MRS findings, including the hot cross bun sign in MSA. (You’ll have to go online to see the figures.) And the Discussion section was worthwhile reading.


Arqivos de Neuropsiquiatria. 2009 Mar;67(1):1-6.

Neuroimaging in Parkinsonism: a study with magnetic resonance and spectroscopy as tools in the differential diagnosis.

Vasconcellos LF, Novis SA, Moreira DM, Rosso AL, Leite AC.
Hospital dos Servidores do Estado, Rio de Janeiro, RJ, Brazil.

The differential diagnosis of Parkinsonism based on clinical features, sometimes may be difficult. Diagnostic tests in these cases might be useful, especially magnetic resonance imaging, a noninvasive exam, not as expensive as positron emission tomography, and provides a good basis for anatomical analysis. The magnetic resonance spectroscopy analyzes cerebral metabolism, yielding inconsistent results in parkinsonian disorders.

We selected 40 individuals for magnetic resonance imaging and spectroscopy analysis, 12 with Parkinson’s disease, 11 with progressive supranuclear palsy, 7 with multiple system atrophy (parkinsonian type), and 10 individuals without any psychiatric or neurological disorders (controls). Clinical scales included Hoenh and Yahr, unified Parkinson’s disease rating scale and mini mental status examination.

The results showed that patients with Parkinson’s disease and controls presented the same aspects on neuroimaging, with few or absence of abnormalities, and supranuclear progressive palsy and multiple system atrophy showed abnormalities, some of which statistically significant. Thus, magnetic resonance imaging and spectroscopy could be useful as a tool in differential diagnosis of Parkinsonism.

PubMed ID#: 19330200

Here are excerpts from the full article:

“Magnetic resonance imaging (MRI) and spectroscopy by MRI (MRS) are noninvasive tools helping the physician to establish a more accurate diagnosis. MRI offers an adequate analysis of abnormalities in the basal nuclei, midbrain, pons, medulla, and cerebellum, which are impaired in atypical” parkinsonism.

We designed a prospective, case-control, double-blind, 24 months study. The MRI was performed in a GE machine, 1.5 Tesla Sigma Horizon model, the sequences analyzed were T 1, T 2, flair, diffusion, axial-oblique in T 2 in Fast Spin-Echo (FSE) and Proton Density (PD) and T 2 in Spin-Echo (SE). In addition to 5 mm slices, we included 3 mm slices in the lentiform nucleus. The MRS was single voxel (8 cc), PRESS technique (TR/TE=1500/50) bilaterally in lentiform nucleus, midbrain, white matter of frontal lobe and hippocampus.” …

“Forty individuals were included in this study (age range: 50 to 85 years), 30 with Parkinsonian syndrome and 10 without any neurological or psychiatric disorders.”

“All individuals were examined by the same neurologist, and 26 patients met the criteria for probable Parkinson’s disease (PD) [n=10], (Gelb et al.), progressive supranuclear palsy (PSP) [n=10], (Tolosa et al.), and multiple system atrophy-parkinsonian type (MSA-P) [n=6], (Gilman et al.). For clinical assessment, the scales adopted were Hoehn-Yahr stage, unified Parkinson’s disease rating scale (UPDRS) Part III and mini-mental status examination (MMSE). The patients performed the Tilt Table test for evaluation of dysautonomia.”

“Results …
Dysautonomia was documented in 20% of PD and 100% of MSA-P.

In the motor scales (UPDRS and Hoehn and Yahr), the results showed higher scores in PSP and MSA-P than in PD. There was statistical significance in PD versus MSA-P, and a trend to statistical significance in PD and PSP.

Patients with PSP presented lower scores in MMSE, followed by MSA-P and PD, and there was statistical significance in the three groups comparing to controls (Table 1).

Image variables demonstrated cerebral atrophy in all cases of PSP and MSA-P, having statistical significance in PD versus PSP, PD versus MSA-P, controls versus PSP, and controls versus MSA-P. Cerebellar atrophy was more common in MSA-P and PSP, with statistical significance in PD versus MSA-P, controls versus PSP and controls versus MSA-P. We observed a higher prevalence of white matter alterations in atypical [parkinsonism] with no statistical significance. Signal change in the lentiform nucleus was observed more commonly in MSA-P and PSP, but no statistical significance was documented (Figs 1­3).”

Fig 1. Hyposignal in the lentiform nucleus (found in 67% of MSA-P group), and hypersignal in the pons (found in 33% of the MSA-P group) and the midbrain on T2, flair or DP sequences (found in 70% of PSP group).

Fig 2. Posterolateral linear hypersignal in the lentiform nucleus with asymmetric symptoms, T2 sequence (found in 50% in MSA-P group).

Fig 3. Transverse signal (“hot cross bun sign”) in the pons, T2 sequence (found in 33% of MSA-P group).” …

…Parkinsonian signs may be seen in different medical conditions, having variable course, treatment and prognosis so it is important to determine an accurate diagnosis as soon as possible. Based only in clinical data, especially in the early stages of the disease, physicians may not establish a correct diagnosis.

… One study conducted in a movement disorders specialized center, showed that the positive predictive value of PD was 98.6%, and to atypical parkinsonism 71.4%, confirming that the diagnosis of atypical [parkinsonism], even in specialized centers, is sometimes difficult to establish.

… We included the three [parkinsonism syndromes] that most frequently lead to misdiagnosis: PD, MSA-P, and PSP, all compared to control group. …

We used three clinical scales: motor part of UPDRS, Hoehn and Yahr and MMSE. These scales showed increased motor impairment (higher scores in UPDRS and Hoehn-Yahr) in the MSA-P, followed by PSP, and increased cognitive impairment (lower scores of MMSE) in PSP, followed by MSA-P. We did not observe a correlation between the duration of the symptoms with MRS abnormalities, but with the clinical diagnosis of patient.

MRI variables demonstrated that some are helpful to differentiated [parkinsonism] syndromes, as the presence of cerebral and cerebellar atrophy and signal enhancement of some encephalic structures (lentiform nucleus, midbrain and pons), more common in atypical [parkinsonism].

The decreased signal enhancement in the lentiform nucleus may be observed in normal aging, so in our study we only considered it as ‘abnormal’ if the hypointensity was moderate to severe. Our data showed that moderate to severe decrease hypointensity in lentiform nucleus was observed more frequently in MSA and PSP, with no difference between PD and control groups and when it was associated with posterolateral linear hypersignal in putamen, suggested the diagnosis of atypical [parkinsonism] (more frequently in MSA group).

The most useful measurement of encephalic diameter in our study was the midbrain, as it had been shown by Warmutth et al. Values below 15 mm in the midbrain suggested PSP or MSA-P, with lower values seen in PSP.

Some values of MRS had statistical significance, the most useful were from the lentiform nucleus, hippocampus, and midbrain, depending on the diagnosis, indicating a severe neuronal impairment (neuronal death). There are few studies in which the brainstem is evaluated by MRS, due to technical difficulties (bone proximity). In our study we demonstrated that it is feasible, but we had to repeat the exam, in some cases several times, to achieve a consistent chart. The study done by Watanabe et al. demonstrated the usefulness of MRS of the pons in MSA patients. As the midbrain is the most affected area in PSP, we analyzed it by MRS. We have found NAA/Cho decrease in midbrain of PSP group with statistical significance, indicating neuronal loss.

Based on our data we concluded that:
(1) Patients with PSP and MSA-P presented increased motor and cognitive impairment in the scales used, correlating with decrease in NAA/Cr in lentiform nucleus and NAA/Cho in midbrain in the PSP group;
(2) Cerebral and cerebellar atrophy were more prevalent and severe in PSP and MSA-P groups;
(3) Linear hypersignal in the lateral portion of the putamen, hypersignal in midbrain and in pons, all suggest the diagnosis of PSP or MSA-P;
(4) Midbrain or pons atrophy suggests atypical parkinsonism, the former PSP, and the latter MSA-P;
(5) Comparing the two methods, MRI and MRS, the former had better applicability.

Our study showed that anatomical analysis through MRI and MRS of some areas could be useful in the differential diagnosis of PD and atypical [parkinsonism], helping physicians to establish a more accurate diagnosis of [parkinsonian syndromes].”

Book recommendation: “AfterShock”

This book recommendation came around again to me recently and I thought I’d pass along the title here. The recommended book is:
“AfterShock: What To Do When The Doctor Gives You – Or Someone You Love – a Devastating Diagnosis”
by Jessie Gruman, PhD
$12 new or $1 used on

On Friday, someone on an online MSA support group noted that Dr. Gruman was on the Martha Stewart show on Friday 3/27. I dug around on but couldn’t find any segments from that Friday show available online. (Maybe the segment with Dr. Gruman will be posted later.) The Martha Stewart website did have some additional info on “Dealing with a Devastating Medical Diagnosis”:

“What would you do if you were diagnosed with a life-threatening illness? How would you handle this news? While shock, fear, and even hysteria might be normal reactions, it’s helpful to have a guide for what’s often a very tumultuous road ahead.

When you’re given the news that you have cancer, HIV, or another serious diagnosis, it may feel as if your world has shattered and all of your plans for the future have vanished in a flash. You feel fear, despair, anger, sadness — often all at once. It’s understandable; a serious diagnosis is a crisis, and you should treat it as one. Don’t force yourself to go to work or make big decisions while you’re really upset. Give yourself time to pull it together: Spend time with loved ones; don’t forget to eat; nap if you can; cry if you feel like it. There are no rewards for being tough. It’s a tribute to human resilience that as you learn more and adjust to the shock, you’ll find you regain some focus and are able to take the important next steps.

Finding a good doctor is really important — begin by looking for a specialist who has extensive experience treating the exact disease you have. Finding that person can be a puzzle. There are many referral sources, and none of them will tell you everything you need to know. The tried and true way is to ask a physician you know and like to refer you to another physician that he or she has worked with before. …

Often, friends, family members, and co-workers don’t know how to respond. They should begin by acknowledging the difficult situation. People say they’re uncomfortable raising the topic with someone who is sick and that they don’t want to remind the sick person of it or make them cry, but saying nothing is far more damaging. Say this: “I hear you’ve had some bad news. I’m so very sorry. I hope everything goes OK.” It means so much.

Also, don’t talk about a friend or family member’s illness without his or her permission, even to other family members. Ask what information can be shared and with whom. And then listen — many people with a serious illness swing between hope and fear.

When it comes to health care, you have to force yourself to act like a consumer. Things have changed a lot in health care in the past decade. Advances in surgery and drugs and diagnostics mean it’s now possible to live long and well with diseases that were a death sentence as recently as 10 years ago. But we will only benefit from these advances if we are involved. We have to decide which doctors to visit, get the tests, take the pills, and seek help when we can’t manage on our own. Patients have a critical role in the success of our health care.”

That’s from: … ewart-show
There’s a list of online resources available.

This page on Dr. Gruman’s website gives a great description of the book: … troduction

Below is an email I sent out in June ’07 to the local support group in which a social worker recommended “AfterShock” and gave some additional ideas for self management of long-term conditions.


Date: Mon, 18 Jun 2007 17:34:47 -0700
To: [email protected]
From: Robin Riddle <[email protected]>
Subject: “Self Mgmt of Long-term Conditions” (Meeting Notes)

I attended the Palo Alto Parkinson’s Disease support group meeting last week where I picked up one book suggestion and a few general suggestions that I thought I’d pass along. The speaker was Kate Lorig, Patient Education at Stanford Hospital. The topic was Self Management of Long Term Conditions.

Here’s some general information on Stanford’s workshops and programs to help people live with long-term health problems:
Some programs are meeting-based and others internet-based.

This particular support group is mixed with caregivers and those with Parkinson’s Disease. The speaker asked those with PD “what are the biggest challenges of living with PD?” and, for the caregivers, “what are the biggest challenges of living with someone who has PD?” Then the group voted on the top challenges, which were:

* living with ever-diminishing hopes
* sense of loss
* comparing how things used to be (“the good ole days”) to how things are now
* balance/coordination/muscle weakness

On the psychological issues of ever-diminishing hopes and a sense of loss, the speaker said that once you have a chronic condition, death is a reality. She highly recommends a new book called “After Shock” by Dr. Jessie Gruman. (You can find info on this book at You might listen to some interviews with Dr. Gruman before purchasing the book.) None of your family and friends know how to deal with the “new you.”

The speaker recommends planning at least one thing every day that the patient and caregiver can still enjoy — such as having an ice cream cone, watching the news, or travelling. She thinks it would be best to have several small things every day planned.

She emphasized that it’s the patient’s job to tell other people exactly what they can do to help. Again, she thinks that having something small planned is worthwhile, such as telling someone “you can invite me to a movie once a month.”

The speaker believes that doing volunteer work is useful: “helping others will help you.” A meeting attendee recommended the website for volunteer activities of any time duration.

The speaker says that most people with chronic conditions and most people caregiving for those with chronic conditions are depressed — some sub-clinically (ie, not needing therapy) and some clinically. She recommends that everyone have a specific thing to do or think about if they perceive they are having negative thoughts. A specific thing to do might include going to the movies, exercising, or baking. A specific thing to think about might include polar bears or penguins. (These were her examples!)

The speaker addressed the challenge of balance/coordination/muscle weakness by saying that these issues can be addressed through exercise. She recommended that everyone with a chronic health problem get exercise that doesn’t hurt. In particular she likes tai chi, and recommends the tai chi tapes of Paul Lam (see and Jon Kabat-Zinn. JKZ also has mindfulness training tapes.

If you fall, don’t ask someone to lift you as they probably don’t know what they are doing and will hurt themselves or you. Instruct any helper to get others to help, particularly others who are trained in helping get people up off the floor.

She said that everyone using a cane, walker, or wheelchair needed lessons on using these tools properly.

That’s it!

Behavioral disturbances in CBD and PSP

Italian researchers gave 68 CBD patients and 57 PSP patients a test called the “Frontal Behavioral Inventory.” The “most frequent behavioral abnormalities present in both groups (>25%) were aspontaneity and logopenia.” [Logopenia = decreased speech output. Here’s a description from eMedicine: “Spontaneous speech can be sparse yet fluent in character, with preserved grammar (logopenia).]

Comparing the behavioral profiles of CBDers and PSPers, the researchers found that:
* apathy was more frequent in PSP;
* alien hand/apraxia was more frequent in CBD;
* “Aphasia (27.9%) and irritability (35.3%) were more frequent in CBDS compared to PSP, even if not statistically different.”

We’ll have to get the full article to understand why the researchers say that their study “further confirms the usefulness of the FBI scale.”

The abstract notes that scores on the FBI were relatively low in both patient groups. Low scores mean the behavior never occurs or is mild.

You can find a copy of this 24-page battery here (and I’ve copied the questions beneath the abstract below): … rs/FBI.pdf
It was developed by Canadian cognitive neurologist Andrew Kertesz, MD. Dr. Kertesz is an expert on the various types of frontotemporal dementias. He includes PSP and CBS as FTDs. He wrote a very good book called “The Banana Lady” that provides stories of various patients with FTDbv, PSP, and CBS.


International Psychogeriatrics. 2009 Mar 27:1-6. [Epub ahead of print]

Pattern of behavioral disturbances in corticobasal degeneration syndrome and progressive supranuclear palsy.

Borroni B, Alberici A, Agosti C, Cosseddu M, Padovani A.
Department of Neurology, University of Brescia, Italy.

Background: A careful characterization of behavioral abnormalities in corticobasal degeneration syndrome (CBDS) and progressive supranuclear palsy (PSP) by reliable tools is still lacking. Literature data provided evidence of the usefulness of the Frontal Behavioral Inventory (FBI) to operationalize such disturbances, particularly in the frontotemporal lobar degeneration spectrum. The study aimed to evaluate the frequency and pattern of presentation of behavioral disturbances in a large sample of CBDS and PSP patients by FBI.

Methods: Sixty-eight CBDS and 57 PSP patients entered the study and underwent a standardized clinical and neuropsychological battery, and a structural brain imaging study. Behavioral disturbances were carefully analyzed by FBI.

Results: FBI scores were relatively low in both groups, being 6.7 +/- 8.2 and 5.6 +/- 6.1 in CBDS and PSP, respectively. Comparison of the behavioral profile between CBDS and PSP patients showed significant differences in apathy were more frequent in the latter (57.9% vs. 33.8%, P = 0.007), and the presence of alien hand/apraxia more frequent in the former group 39.7% vs. 10.5%, P = 0.001).

Apathy correlated neither with age nor with motor disturbances as measured by UPDRS-III.

Overall, the most frequent behavioral abnormalities present in both groups (>25%) were aspontaneity and logopenia.

Aphasia (27.9%) and irritability (35.3%) were more frequent in CBDS compared to PSP, even if not statistically different.

Discussion: The present study has provided measures of behavioral disturbances in a population of PSP and CBDS patients, and further confirms the usefulness of the FBI scale.

PubMed ID#: 19323870 (see for abstract only)

Excerpts from
(c) Andrew Kertesz

0 (none / never)
1 (mild / occasional)
2 (moderate / often)
3 (severe / most of the time)

Negative Behavior Score: Total of 1 ­ 12: _____
Disinhibition Score: Total of 13-24 : _____
Total Score: _____

Negative Behavior Questions:
1. Apathy: Has s/he lost interest in friends or daily activities or is s/he interested in seeing people or doing things?
2. Aspontaneity: Does s/he start things on his/her own, or does s/he have to be asked?
3. Indifference, Emotional Flatness: Does s/he respond to occasions of joy or sadness as much as ever, or has s/he lost emotional responsiveness?
4. Inflexibility: Can s/he change his/her mind with reason or does s/he appear stubborn or rigid in thinking lately?
5. Personal Neglect: Does s/he take as much care of his/her personal hygiene and appearance as usual, or does s/he neglect to wash or change his/her underwear?
6. Disorganization: Can s/he plan and organize complex activity or is s/he easily distractible, impersistent, or unable to complete a job?
7. Inattention: Does s/he pay attention to what is going on or does s/he seem to lose track or not follow at all?
8. Loss of Insight: Is s/he aware of any problems or changes in behavior, or does s/he seem unaware of them or deny them when discussed?
9. Logopenia: Is s/he as talkative as before or has the amount of speech significantly decreased?
10. Semantic Dementia: Does s/he ask what words mean, has trouble comprehending words, and/or objects, or does s/he know the meaning of words?
11. Aphasia and Verbal Apraxia: Does s/he make language or pronunciation errors or has s/he developed stuttering or repeating sounds recently?
12. Alien Hand and/or Apraxia: Has s/he developed clumsiness, stiff hand, inability to use utensils or appliances, or does a hand interfere with the other, or behaves as if it did not belong, or can s/he use both hands as before?

Negative Behavior Score: Total of 1 ­ 12: _____

Disinhibition Questions:
13. Perseverations, Obsessions: Does s/he repeat or perseverate actions or remarks? Are there any obsessive routines or behaviors, or has s/he always been a creature of habit?
14. Irritability: Has s/he been irritable, short-tempered, or is s/he reacting to stress or frustration as s/he always had?
15. Excessive Jocularity: Has s/he been making jokes excessively or offensively or at the wrong time, or has s/he always had a jocular manner or a quirky sense of humor?
16. Impulsivity/Poor Judgment: Has s/he been using good judgment in decisions, spending or driving, or has s/he acted impulsively, irresponsibly, neglectfully or in poor judgment?
17. Hoarding: Has s/he started to hoard objects or money excessively or has her/his saving habits remained unchanged?
18. Inappropriateness: Has s/he kept social rules or has s/he said or done things outside what are acceptable? Has s/he been rude, or childish?
19. Restlessness/Roaming: Has s/he been pacing, walking, driving excessively or is the activity level normal?
20. Aggression: Has s/he shown aggression, or shouted at anyone or hurt anyone physically or is there no change in this respect?
21. Hyperorality: Has s/he been drinking or eating excessively anything in sight, or developing food fads, or even putting objects in his/her mouth, or has s/he always had a large appetite?
22. Hypersexuality: Has sexual behavior been unusual or excessive? This could include remarks or undressing, or is there no change in this respect?
23. Utilization Behavior: Does s/he seem to need to touch, feel, examine, or pick up objects within reach and sight, or can s/he keep his/her hands to him/herself?
24. Incontinence: Has s/he wet or soiled his or herself or does s/he have problems that can be explained by urinary infection or childbirth/prostate?

Disinhibition Score: Total of 13-24

“PSP Symptoms in a Nutshell”

PSP folks –

Someone named Brenda (“cruzgal”) on the PSP Forum posted* today this document she recently created to give to her mother-in-law’s hired caregivers and facility staff. It describes “PSP symptoms in a nutshell.”

As Brenda’s mother-in-law doesn’t have the dementia form of PSP, Brenda’s document speaks of dementia as not existing in PSP. Current research shows that 54-62% of those with PSP *do* have dementia; this is considered the classic form of PSP that was described by Richardson and Steele. So, I think this is a great document to you if you are not dealing with the dementia form of PSP (the non-dementia form is called PSP-Parkinsonism), or a great basis for a document that can be revised if you are dealing with the dementia form (the dementia form is called Richardson’s Syndrome).

I’ve copied the full text below.

Here’s one note from Brenda:

Italicized portions of this document are directly quoted from and additional information is available at or (Editor’s Note: This website no longer exists)



PSP (Progressive Supranuclear Palsy) is a neuro-degenerative brain disease that has no known cause, treatment or cure. It affects the frontal lobe of the brain and the nerve cells that control walking, balance, mobility, vision, speech, and swallowing. Five to six people per 100,000 will develop PSP with approximately 20,000 known cases in the U.S. PSP displays a wide range of symptoms which progressively worsen with time. Symptoms may occur at various stages of the disease and vary widely with each individual patient and with the specific type of PSP the patient has. Typical lifespan after onset of symptoms is 6-10 years, with a reported range of 2-17 years. Cause of death in patients with diagnosed PSP is usually a result of aspiration pneumonia, infections, or injuries resulting from a fall.

The most common first symptom is loss of balance while walking. This may take the form of unexplained falls or of a stiffness and awkwardness in the walk that can resemble Parkinson’s disease. Other common early symptoms are forgetfulness and changes in personality. The latter can take the form of a loss of interest in ordinary pleasurable activities or increased irritability and cantankerousness. These mental changes are often misinterpreted as depression or even as senility. Less common early symptoms include trouble with eyesight, slurring of speech and mild shaking of the hands. Difficulty driving a car, with several accidents or near misses, is common early in the course of PSP. The exact reason for this problem is not clear.

Some other symptoms that may occur at some point in the disease are fatigue, incontinence, rigidity of the muscles, a softening of the voice, a variance in body temperature and an inability to write clearly. In some cases the patient may exhibit episodes of inhibition and inappropriate behavior. PSP can only be confirmed post-mortem, but once a clinical diagnosis of probable PSP has been made, the patient and family may realize in retrospect that some of the problems the patient had been having for quite a long while were attributable to PSP.

Because of similarities in some of the symptoms, the patient with PSP is often mis-diagnosed as having Alzheimer’s Disease or Parkinson’s Disease or some other neurological disease. PSP is classified, in fact, as a Parkinson’s-Plus disease although there are distinct differences in PSP and Parkinson’s.

PSP patients are prone to losing their balance and should never be allowed to stand or walk without assistance. Broken bones resulting from a fall complicates the life and care of a patient with PSP so extreme caution should be exercised to prevent falls. A walker is not advised for the patient with PSP unless someone is close behind the patient AT ALL TIMES. Since those with PSP usually fall backwards, a walker could possibly cause further injury in a fall.

Shoes with smooth soles are often better than rubber-soled athletic shoes. In many people with PSP, the gait disorder includes some element of “freezing,” a phenomenon that makes it difficult to lift a foot from the ground to initiate gait. Such people can fall if they move their body before the foot moves. In these cases, a smooth sole could make it easier to slide the first foot forward. Shoes with a lifted heel might also help prevent the backward fall.

While Physical Therapy has not been shown to IMPROVE the symptoms of PSP, it’s important for the patient to get as much exercise and to walk (with assistance) for as long as possible to avoid the muscle atrophy that occurs from lack of use. Extreme caution should be used to prevent the patient from falling and perhaps taking the one escorting him/her along on the fall.

The disease impairs the ability to swallow (dysphagia) and the greatest risk is with thin liquids which may be aspirated into the lungs. This can eventually cause aspiration pneumonia, the most common cause of death in patients with PSP. Tucking the chin when swallowing liquids may help prevent choking in the earlier stages of the disease and thickened liquids are recommended as the disease progresses. Pills may need to be crushed and placed in applesauce or similar food.

Patients with PSP are prone to “mouth stuffing” and “rapid drinking” which often causes them to choke. Small bites should be taken and each bite should be swallowed before taking another bite or drink. They may also get choked from improper chewing, from hard -to-chew foods such as steak, or from “mixed-textured” foods containing both liquid and solids. Foods such as vegetable soup, cold cereal and uncooked dairy products should be avoided. Patients may also choke on their own saliva. Carbonated beverages and sparkling water may assist in cutting the phlegm that accumulates in the throat. As the disease progresses, pureed food may become necessary and some patients may eventually require surgical insertion of a feeding tube (peg) although clinical studies have not shown that a feeding tube will completely eliminate the possibility of aspirated pneumonia.

In most cases, the visual problem is at least as important as the walking difficulty, though it does not appear, on average, until 3 to 5 years after the walking problem. Because the main difficulty with the eyes is in aiming them properly, reading often becomes difficult. The patient finds it hard to shift down to the beginning of the next line automatically after reaching the end of the first line. This is very different from just needing reading glasses. An eye doctor unfamiliar with PSP may be baffled by the patient’s complaint of being unable to read a newspaper despite normal ability to read the individual letters on an eye chart. Some patients have their mild cataracts extracted in a vain effort to relieve such a visual problem.
Yet another eye problem in PSP can be abnormal eyelid movement — either too much or too little. A few patients experience forceful involuntary closing of the eyes for a few seconds or minutes at a time, called blepharospasm. Others have difficulty opening the eyes, even though the lids seem to be relaxed, and will try to use the muscles of the forehead, or even the fingers, in an effort to open the eyelids (apraxia of lid opening). About 20 percent of patients with PSP eventually develop one of these problems. Others, on the contrary, have trouble closing the eyes and blink very little. While about 15 to 25 blinks per minute are normal, people with PSP blink, on average, only about 3 or 4 times per minute. This can allow the eyes to become irritated. They often react by producing extra tears, which, in itself, can become annoying.

The eyes often become glazed and wide-eyed, giving the patient a startled look or the appearance of “staring into space”. Involuntary closing of the eyes is also a frequent occurrence. It becomes increasingly difficult for the patient to read or to watch TV. Dinner plates may need to be placed on a “riser” in order to bring the plate into the patient’s line of sight.

A patient with PSP frequently repeats the same word or phases involuntarily (palilalia) and stuttering may also occur as well as “parroting” phrases or questions spoken by another person. It may take awhile for the patient to form a sentence, so it’s important to give him/her time to speak without interruption. An erroneous impression of senility or dementia can be created by the PSP patient’s combination of speech difficulty, slight forgetfulness, slow (albeit accurate) mental responses, personality change, apathy and poor eye contact during conversation.

The PSP patient usually remains alert to his or her surroundings and understands all that is going on around him/her. Memory is not affected as in Alzheimer’s patients, but may falter at times, especially when trying to speak and the right words won’t come. As the disease progresses it becomes quite difficult for the patient to have a “normal” conversation and great patience is required on the part of the listener.

Many PSP patients display signs of mild dementia, but in some cases the slowness of speech and difficulty communicating only gives the impression of dementia. Other patients, especially those with a specific type of PSP, develop actual and more severe dementia. The dementia of PSP is characterized by slowed thought and difficulty synthesizing several different ideas into a new idea or plan.

Cognitive reasoning is affected and PSP patients may not be aware of what they should or should not do in their own best interest. They may “THINK” they can walk by themselves or that they don’t need help doing certain things even when there’s a risk involved. All precautions available should be used to protect the patient from their own poor judgment.

No treatment or cure is currently available for PSP, but medications may be available to treat some of the symptoms such as dry eyes or depression. A doctor familiar with this disease should be consulted for recommendations of these medications as PSP patients often experience adverse effects from certain drugs.

NOTE: Italicized portions of this document are directly quoted from and additional information is available at or (Editor’s Note: This website no longer exists)

March 2009
Information compiled and written by Brenda Cyrus, Fort Smith, AR
Daughter-in-law of PwPSP, Elizabeth, diagnosed Sept. 2008, symptoms since 2005.

Advice – Accept the diagnosis, forget the prognosis

Years ago, online friend Aletta, who has MSA, emailed me this piece of advice. We’ve circulated it around the local support group many times.

Challenging the Odds: forget the prognosis
by Barry Bittman, MD

Have you ever met anyone who was given 3 months to live 10 or more years ago, who is still alive today?

Have you ever known a person, who despite an immediately fatal prognosis, managed to beat the odds and survive for a certain occasion such as a child’s wedding?

Have you ever lost a grandparent who accurately predicted his/her death upon losing a soul mate?

Did you ever stop to consider if it is possible for a doctor to tell us how long we have to live?

If you’ve answered yes to any of these questions, read on. It’s a fact that many people are alive and thriving today who were told many years ago that they had only a short time to live. It’s also common knowledge that some people live just long enough to witness the birth of a new grandchild or to attend a graduation or wedding. And it doesn’t seem to surprise anyone when the death of one grandparent follows shortly after the other.

Yet, few of us understand how any doctor can make the statement, “You have 3 months to live.” I’ll let you in on something …. they can’t!

Actually, all that a physician can tell you is how long the average person with your condition typically survives. The problem here is with the words, “average” and “typically.” The doctor relies on statistical data based upon a bell-shaped curve that documents the range of survival for people who are suffering from a given disease. At the peak of the curve is the most common survival time experienced by the group under study. It comes as no surprise that everyone does not fit there, and often the range of possibilities is extensive. Some succumb earlier than expected, while others far exceed their prognosis.

Sometimes, however, I wonder if physicians really understand how their conveyed prognosis has the potential to become reality, not because of statistics, but rather as a result of its impact on the patient’s belief system. In essence, the doctor’s words become a self-fulfilling prophecy. Some people go home and get their things in order, while others go home and get their lives in order.

You’re probably asking yourself what is the difference. Frankly, the distinction is as wide as the Grand Canyon. The first group of patients returns home, announces the bad news, proceeds through the predictable stages of Kubler-Ross, (anger, denial, etc.) revamps their wills, tidies up their safe deposit boxes, lies down and dies on cue.

The second group, however, goes home and gets their lives in order. They maintain a fighting determination to complete unfinished business – to accomplish what they never have before. Remaining time is spent on what they have always hoped to do. A focus on surviving gives way to planting gardens, creating wildlife sanctuaries, teaching Sunday School, playing with grandchildren, volunteering time for others, and expressing their love. This group attends classes, reads enlightening books, becomes more spiritual, and sets out on a quest to discover meaning in their lives.

And then something extraordinary occurs – they flourish. Eating right, exercising, and taking care of one’s self comes naturally, and not as a way to prolong survival. Rather, self-care simply evolves as a logical means for enabling their mission in life.

It’s easy to pick such individuals out of a crowd. These “survivors” make the world a beautiful place, help others, and fulfill their dreams. They’re the ones who are living mindfully, appreciating every moment, and treasuring each experience with gusto and gratitude to our Creator. They are our best teachers and guides.

So where does this leave us when faced with a less than desirable prognosis? My recommendations are simple. Accept your diagnosis, or, if in doubt, get another opinion. But never accept your prognosis!

Know that all things are possible, and listen to your inner voice. Realize that living beyond a serious illness may not be in the cards for all of us no matter what we do. Yet always remember that it’s the way we live each day that makes the difference. Love life, realize your dreams, and tip the balance in your favor¾ Mind Over Matter!

copyright 1998,1999 Barry Bittman, MD all rights reserved