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:
http://www.scielo.br/pdf/anp/v67n1/a02v67n1.pdf –> PDF form
http://www.scielo.br/scielo.php?script= … 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.

Robin

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.

“Method
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).” …

“DISCUSSION
…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 amazon.com
www.aftershockbook.com

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 marthastewart.com 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:
http://www.marthastewart.com/article/de … ewart-show
There’s a list of online resources available.

This page on Dr. Gruman’s website gives a great description of the book:
http://www.aftershockbook.com/book_inde … 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.

Robin

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: http://patienteducation.stanford.edu/programs/
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 http://www.aftershockbook.com/. 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 dosomething.org 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 taichiproductions.com) 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!
Robin

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):
http://www.bic.mni.mcgill.ca/users/gaby … 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.

Robin

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 pubmed.gov for abstract only)

Excerpts from
FRONTAL BEHAVIORAL INVENTORY (FBI)
(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 www.psp.org or www.pspinformation.com (Editor’s Note: This website no longer exists)

Robin
————————-

http://forum.psp.org/viewtopic.php?t=7882

PSP SYMPTOMS IN A NUTSHELL

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.

COMMON EARLY SYMPTOMS OF PSP
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.

LOSS OF BALANCE AND FALLING:
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.

DIFFICULTY SWALLOWING:
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.

VISUAL PROBLEMS:
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.

SPEECH:
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.

BRAIN FUNCTIONS:
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 www.psp.org or www.pspinformation.com (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

Atypical Parkinsonism – Breakout session notes (3/12/09)

On Thursday 3/12/09 at the Victory Summit (Symposium for People Living with Parkinson’s Disease) in San Jose, there was a one-hour breakout session on “Atypical Parkinson’s Disease.” There were two presenters:
* Grace Liang, MD, Neurologist/Movement Disorder Specialist, The Parkinson’s Institute
* Amy Manning-Bog, PhD, Researchers, The Parkinson’s Institute

In a one-hour period, I thought they gave a great summary of the clinical picture of the four atypical parkinsonism disorders, and of the basic research underway that pertains to these disorders with particular attention to MSA. The Q&A was especially good; there were many in the audience who grasped even the challenging research update. I shared the draft of my notes with Dr. Manning-Bog, and she kindly corrected them and expanded the explanation in a few places. Also, support group member Helen added many points I had missed. Here are our combined notes from the presentations and the Q&A…

Notes from Dr. Liang’s Presentation

In order to know what “atypical PD” is, we must first review what “typical PD” is. Typical PD:
* usually starts on one side
* usually has a resting tremor
* includes Lewy body pathology
* slow progression (several decades)
* responds to medications

Common parkinsonian motor signs include:
* bradykinesia (slowed movements)
* tremor
* rigidity
* balance difficulty/gait instability

Other motor signs of PD include:
* hypophonia
* freezing
* stooped posture
* decreased facial expression

There are many non-motor symptoms of PD:
* constipation
* sleep disorders
* depression

These additional non-motor symptoms are prominent in atypical PD:
* blood pressure fluctuations
* swallowing difficulties
* urinary symptoms
* change in thinking and memory abilities

Parkinsonism can include the following diagnoses:
* Drug-induced Parkinsonism: may be caused by dopamine blocking drugs (examples – Haldol, Reglan, Risperdal, Depakote); reversible to some extent; these medications should be avoided by those with PD or parkinsonism
* Vascular Parkinsonism: caused by loss of blood flow to certain regions of the brain; minimal response to medication

NPH (normal pressure hydrocephalus):
* symptoms include shuffling gait, urinary incontinence, and dementia
* very rare
* not easy to diagnose
* treated with surgery but not that the surgical results are variable

DLB (dementia with Lewy bodies):
* second most common dementia (after AD)
* early notable cognitive changes (can be before or after motor symptoms start; the “time course” determines whether we call this PDD or DLB)
* visual hallucinations are common and are made worse by medication
* fluctuating mental alertness
* delusions

DLB – treatment:
* there is a balance between motor and cognitive symptoms
* dementia drugs can be helpful (examples – Aricept, Exelon, Razadyne, Namenda)
* minimize factors that can increase confusion and hallucinations
* use antipsychotics sparingly for delusions and hallucinations. Note the FDA black-box warning on antipsychotics. The care team must balance the patient’s (and caregiver’s) quality of life with the risks of this type of medication.

MSA (multiple system atrophy):
* often called “Parkinson’s Plus”
* autonomic dysfunction (orthostatic hypotension or low blood pressure upon standing, urinary incontinence, erectile dysfunction)
* there is also a cerebellar form (where balance is a problem)

MSA – treatment:
* there is not usually a strong or prolonged response to PD medication
* to support blood pressure: increase fluid intake, increase salt intake, wear support stockings
* muscle exercises to improve tone and circulation (this can be something as simple as keeping the feet moving throughout the day)
* balance exercises
* evaluate whether there are sleep problems

PSP (progressive supranuclear palsy):
* named for eye movement problem: it’s hard to look up and down
* early balance problems (falls) and gait freezing
* swallowing difficulties
* speech changes
* impulsive behavior

PSP – treatment:
* PD meds may not improve symptoms (and can make balance worse)
* evaluate for speech and swallowing problems
* physical therapy and fall precautions
* dystonia and contractures might be treatable with botox injections

CBD (corticobasal degeneration):
* rare
* usually starts with loss of function of one limb
* apraxia (examples: forgotten how to put clothes on correctly; forgotten how to brush teeth correctly)
* dystonia (involuntary muscle contractions) may occur
* cognitive changes (decreased ability to interpret visual or sensory signs; language difficulty) (example of a sensory sign: the patient can’t tell if he/she is holding a dime or a nickel)

CBD – treatment:
* variable results
* might try dementia medication (Namenda or AChEIs such as Aricept, Exelon, and Razadyne)
* physical therapy is important
* speech therapy is important

“Helpers” or Important resources:
* grab bars
* walker (actually, people can become *more* mobile by using a walker)
* shower seat
* reacher (pick-up stick)
* special eating utensils with big grips and bendable metal
* gait belt
* thickener (example: ThickIt)

Fall prevention:
* remove throw rugs and clutter on floor
* don’t use a step stool
* put things at eye level rather than reaching up for things
* hold onto hand rails and don’t carry a load of things while using stairs

Key points:
* be observant
* control the environment
* protect your brain — reduce risk factors
* nutrition: consider vitamin B, fish oil, Mediterranean diet, CoQ10?, creatine. (Both CoQ10 and creatine are being studied in PD and atypical PD.)
* exercise, exercise, exercise. This is great for the mind, body, and spirit
* do something you enjoy each day

Organizational resources:
LBDA.org
curepsp.org
shy-drager.org
ppsg.org
wemove.org
caregiver.org
nfacares.org
pdtrials.org
atypical parkinsonism support group organized by Robin Riddle

Notes from Dr. Manning-Bog’s presentation:

Changes in brain tissue overlap between the atypical diseases:
* loss of dopaminergic neurons
* depletion
* presence of cytosolic inclusions

A “Lewy body inclusion” is an abnormal deposit of protein. Many proteins are included but it’s mostly alpha-synuclein.

Alpha-synucleinopathies (PD, MSA, LBD) can either be familial or sporadic.

There are modifiers:
* genetics
* environment
* aging

Alpha-synuclein comprises up to 2% of total brain protein in normal brains.

Alpha-synuclein can self-aggregate or bind itself to form multimers of the protein and even alpha-synuclein fibrils.

Alpha-synuclein aggregation (or clumping) could be due to:
* increased expression
* decreased degradation (ie, the cell doesn’t get rid of the protein)
* exposure to toxicants that can stimulate alpha-synuclein to bind itself

In the last few years, a transgenic mouse model was developed for MSA (by Eliezer Masliah’s group at UC San Diego). This is an exciting tool because researchers can isolate and manipulate the alpha-synuclein gene! Now that Dr. Masliah has generated these MSA mice, there is a tool that we can use to study mechanisms to disaggregate and clear alpha-synuclein from cells (i.e. neurons in PD and oligodendroglia in MSA). We can use this tool to test therapeutics in a pre-clinical environment.

Images were shown of alpha-synuclein fibrils in test tubes. This research was done at UC Santa Cruz. This research is promising. Researchers could disintegrate the fibrils (although not always completely) and may leave some toxic synuclein species.

Eliezer Masliah’s group at UC San Diego tried to target the aggregation of synuclein and increase the degradation of synuclein by giving the drug rifampicin to MSA mice. This partially worked. Results were promising — less cell death and alpha-synuclein deposition were apparent in mice treated with rifampicin.

Dr. Manning-Bog’s research approach is to deliver alpha-synuclein to where it needs to go. The goal is to restore alpha-synuclein trafficking via the lipid raft.

If researchers find a mechanism that prevents alpha-synuclein build-up in cells, these agents can be tested in animal models of other diseases that involve alpha-synuclein (including the MSA model or a mouse model of alpha-syn build-up in neurons).

>From this standpoint, any work that is done on these aspects of alpha-synuclein is important to any PD form that has alpha-synuclein pathology. (For example, the alpha-synuclein trafficking arm of my research program, for example, is relevant to any PD form with alpha-synuclein pathology.)

[I asked Dr. Manning-Bog why she talked about the MSA mouse model during her presentation. She replied: “While putting together my talk for the symposium, I asked Dr. Brandabur what types of Atypical PD were most commonly treated at the Institute. She informed me that MSA is one of the more predominant Atypical PD forms of the Institute’s patient population. For this reason, I focused on the research of other labs, because it would be so directly relevant to many patients. Patients with atypical PD are starved for information, so I thought that I should try to tailor the presentation to their interests.”]

Dr. Manning-Bog is also at step one of some tau research. (She has an intern working on this.) She’s seen toxic tau protein changes in DJ1 transgenic mice. This may be a good model for researching Parkinson’s Disease Dementia. This is a first step.

[Robin’s note: PD, DLB and MSA are disorders of alpha-synuclein. They are called alpha-synucleinopathies. Any of the alpha-synuclein research should help these disorders. Of these, only PD and DLB are Lewy body diseases. AD, PSP and CBD are disorders of tau. They are called tauopathies. Any of the tau research should help these disorders. Because DLB typically appears with Alzheimer’s pathology – the so-called “Lewy body variant of Alzheimer’s disease” – tau research may help the DLB community as well.]

Notes from the Q&A:

Q: Can you have the other symptoms of PSP without having the eye movement problem?
A by Dr. Liang: Yes.

Q: What’s the story about statins?
A by Dr. Manning-Bog: Statins have been studied in a few models and seem to be neuroprotective. (See note 1 below.)
A by Dr. Liang: Statins can effect muscle tissue. The atypical PD disorders are neurological disorders, not muscular disorders, so statins are not implicated in the decline seen in these disorders.

Q: When do you get Lewy bodies?
A by Dr. Manning-Bog: Lewy bodies develop throughout the brain predominantly in disease conditions.
A by Dr. Liang: But it could be that Lewy bodies are part of normal aging. We don’t know.
A by Dr. Manning-Bog: We don’t know yet if Lewy bodies are toxic. Currently, we don’t think they cause the disease. They may serve to sequester toxic synuclein. It may be that when synuclein is not sequestered into a Lewy body, it’s toxic.
A by Dr. Manning-Bog: In PD, Lewy bodies start in the brain stem and progress to midbrain, then cortex. In DLB, there are Lewy bodies in the brain’s cortex.

Q: Can imaging see Lewy bodies?
A: No, Lewy bodies are only detectable upon autopsy.

Q: What’s the prevalence of dementia in these disorders?
A by Dr. Liang: The studies vary between 30% and 80% of those with PD getting dementia. It depends on what tests are given to research participants and the definition of “dementia.”

[Answer by Robin:
DLB: 100% of those with DLB have dementia; “progressive dementia” is a “central feature” of DLB (according to the diagnostic criteria)
MSA: none of those with MSA have dementia; according to the diagnostic criteria, dementia is an exclusionary criterion for MSA
PSP: according to the latest clinicopathological correlations, 54% to 62% of those with PSP had dementia as a primary symptom
CBD: there have been no studies on this; my impression is that the percentage for CBD is roughly the same as for PSP; however, it seems from Dr. Liang’s presentation that the percentage may be higher]

Q: What about stem cell research?
A by Dr. Manning-Bog: In research using iPS (induced pluripotent stem cells) to create dopmainergic neurons, the yield is only two percent! This means that iPS isn’t an efficient means of treatment currently. But iPS offers a great way to study the disease mechanism. Before any iPS cells are transplanted into patients, we need to study teratomas, the tumors that can grow.

Q: Are you hopeful about the research?
A by Dr. Manning-Bog: Yes, I’m hopeful!

Note 1:
Dr. Manning-Bog provided two references on statin studies. You can look up the abstracts on pubmed.gov by using the PubMed ID#.

1. The 3-hydroxy-3-methylglutaryl-CoA reductase inhibitor lovastatin reduces severity of L-DOPA-induced abnormal involuntary movements in experimental Parkinson’s disease.
Schuster S, Nadjar A, Guo JT, Li Q, Ittrich C, Hengerer B, Bezard E.
J Neurosci. 2008 Apr 23;28(17):4311-6.
PubMed ID#: 18434508

2. Simvastatin is associated with a reduced incidence of dementia and Parkinson’s disease.
Wolozin B, Wang SW, Li NC, Lee A, Lee TA, Kazis LE.
BMC Med. 2007 Jul 19;5:20.
PubMed ID#: 17640385

New book by Parkinson’s Plus Patient Dan Brooks

Dan Brooks started a blog about his life with Parkinson’s Plus in December 2006. (Ru posted about this on The Back Porch.) The blog — at http://wewillgoon.blogspot.com/ — has grown into a book, which has just been published! The book, “I Will Go On: Living with a Movement Disorder,” is available online through Amazon* for $16. Here’s some info from amazon.com about the book:

“Dan was a 50-year-old husband, father and district-level administrator in a public school system, when he first noticed pronounced tremors, speech difficulties and walking problems developing. In this book, Daniel chronicles his life with a Parkinson’s Plus syndrome and explains how he dealt with the neurological decline that resulted. Read a user-friendly, patient’s explanation of the defining symptoms of these atypical Parkinsonism disorders and find out how this neurodegenerative disease progressed in Dan’s case. This book addresses the many facets of neurodegenerative diseases, while primarily focusing on Atypical Parkinsonian disorders, namely PSP, MSA, and CBD. Parkinson’s Disease is also discussed in depth. Dan tells a compelling and inspirational story of how he maintained his faith in God, while courageously facing life with a movement disorder.”

Dan’s goal is to encourage and inspire patients or caregivers as they face the challenges posed by neurodegenerative illness. He wrote to me: “Sometimes this medical condition is overwhelming, as you know, but I have adjusted and learned to accept what is happening. There is still a lot of life to live and I am very energized right now by this book project. … I am very pleased to have finished [the book]. My hope is that patients and caregivers will appreciate the patient’s viewpoint from which I write. … If I help one person with this book, it will have been worth the effort.”

You can read an overview about the book and learn more about Dan at his blog http://wewillgoon.blogspot.com/ I’m sure you will agree that he’s a remarkable person! Many of us count ourselves lucky that our paths have crossed with his.

Robin

* Link to amazon.com for “I Will Go On: Living with a Movement Disorder”
http://www.amazon.com/Will-Go-Living-Mo … 597&sr=8-1

FDA warning – transdermal patches with metallic backings

This advisory came out today. I have confirmed with Novartis that the Exelon patch does not contain a metallic backing. (Exelon is a dementia medication.) I don’t know about Fentanyl or scopolamine. Maybe someone else can find out??

http://www.fda.gov/cder/drug/advisory/t … lpatch.htm

FDA Public Health Advisory
Risk of Burns during MRI Scans from Transdermal Drug Patches with Metallic Backings
Dated 3/5/09

The FDA has been made aware of information about certain transdermal patches (medicated patches applied to the skin) that contain aluminum or other metals in the backing of the patches. Patches that contain metal can overheat during an MRI scan and cause skin burns in the immediate area of the patch.

Transdermal patches slowly deliver medicines through the skin. Some patches contain metal in the layer of the patch that is not in contact with the skin (the backing). The metal in the backing of these patches may not be visible. The labeling for most of the medicated patches that contain metal in the backing provides a warning to patients about the risk of burns if the patch is not removed before an MRI scan. However, not all transdermal patches that contain metal have this warning for patients in the labeling.

FDA is in the process of reviewing the labeling and composition of all medicated patches to ensure that those made with materials containing metal provide a warning about the risk of burns to patients who wear the patches during an MRI scan.

Until this review is complete, FDA recommends that healthcare professionals referring patients to have an MRI scan identify those patients who are wearing a patch before the patients have the MRI scan. The healthcare professional should advise these patients about the procedures for removing and disposing of the patch before the MRI scan, and replacing the patch after the MRI scan. MRI facilities should follow published safe practice recommendations concerning patients who are wearing patches.1,2

Until this safety issue is resolved, FDA recommends that patients who use medicated patches (including nicotine patches) do the following:

* Tell the doctor referring you for an MRI scan that you are using a patch and why you are using it (such as, for pain, smoking cessation, hormones)

* Ask your doctor for guidance about removing and disposing of the patch before having an MRI scan and replacing it after the procedure.

* Tell the MRI facility that you are using a patch. You should do this when making your appointment and during the health history questions you are asked when you arrive for your appointment.

The FDA urges health care professionals and patients to report possible cases of skin burns while wearing patches during an MRI to the FDA through the MedWatch program by phone (1-800-FDA-1088) or by the Internet at http://www.fda.gov/medwatch/index.html.

(1) Kanal, et. al, “ACR Guidance Document for Safe MR Practices: 2007,” AJR 2007; 188:1­27.
(2) Guidelines for Screening Patients For MR Procedures and Individuals for the MR Environment, Institute for Magnetic Resonance Safety, Education, and Research, www.imrser.org, 2009.

Five types of PSP and diagnostic challenges

This is an interesting article by perhaps the top two PSP researchers in the world — Lees of the old guard and Williams of the new guard.

When looking at the brains of those with a pathological diagnosis of progressive supranuclear palsy (PSP), there are some differences:
(a) in the severity of pathology in the brain,
(b) in the distribution of pathology in the brain, and
(c) clinical features

Presumably three of the disease “subgroups” the authors refer to include: Richardson’s Syndrome (described elsewhere as “classic PSP”), PSP-Parkinsonism, and primary progressive freezing gait (PPFG). I’m unclear if “pure akinesia with gait freezing” (PAGF) is another PSP subgroup or not. (Or if it’s the new name for PPFG. So many acronyms!)

This review article addresses these differences.  You’ll have to check out the full article to see all the differences.  In this post, I’m only sharing the differences in “clinical features” or symptoms.

The authors describe five clinical subgroups or types of PSP:

#1- Richardson’s syndrome (“classic PSP”)

Symptoms include:  lurching gait; postural instability; unexplained backwards falls; personality change; cognitive decline; slowing of vertical saccadic eye movements (“an early telltale sign”); eyelid abnormalities; severely impaired spontaneous blink rate; slow, slurred, growling speech; swallowing difficulties; overactivity of the frontalis; surprised, worried facial appearance; muscle tone can be normal; acoustic startle response absent in most patients; auditory blink reflex absent in most patients.

“The median survival in the original series was 5 years from disease onset; in larger, more recent studies, disease durations to death of 5 to 8 years have been reported.”

#2- PSP-Parkinsonism (PSP-P)

Symptoms include:  limb bradykinesia; limb rigidity is more common and severe than in patients with Richardson’s syndrome; jerky postural tremor; 4-6 Hz rest tremor; asymmetry of limb signs in some cases; axial rigidity; “moderate or good improvement in bradykinesia and rigidity” following levodopa therapy, “although the response is rarely excellent”; acoustic startle response absent in most patients; auditory blink reflex preserved in all patients.

Those with PSP-P are “commonly misdiagnosed with Parkinson’s disease.”

“Falls and cognitive dysfunction occur later in PSP-P than they do in Richardson’s syndrome and, perhaps as a consequence, the time for disease duration to death is about 3 years longer in PSP-P.”

PSP-P compared to other disorders:

(a) “PSP-P and Richardson’s syndrome can be distinguished by their different clinical pictures in the first 2 years; however, there is clinical overlap, and after 6 years of follow up the clinical phenomenology might become similar.”

(b) auditory blink reflex “was absent in most patients with Richardson’s syndrome but was preserved in all patients with PSP-P”

(c) “[We] emphasize the difficulty in separating these [PSP-P] patients from those with [PD]. Early pointers that might help a clinical diagnosis of PSP-P could include rapid progression, prominent axial symptomatology, or a poor response to levodopa…”

“In a few patients, a purely parkinsonian syndrome predominates until death, and abnormalities of eye movement or other characteristics of Richardson’s syndrome might never appear. A sustained response to levodopa and drug-induced choreic dyskinesias with a long duration of disease seem to characterise these patients.”

The prevalence of the PSP-P type seems to be between 8% and 32% of those with PSP pathology.

#3 – PSP-Pure akinesia with gait freezing (PSP-PAGF)

Symptoms include:  “progressive onset of gait disturbance with start hesitation and subsequent freezing of gait, speech, or writing”; “without rigidity, tremor, dementia, or eye movement abnormality during the first 5 years of the disease”; no benefit to levodopa therapy; acoustic startle response present in all patients; auditory blink reflex preserved in all patients.

PAGF was “first described in 1974 in two patients who developed freezing of gait, writing, and speech, with paradoxical kinesia. At presentation, these patients were cognitively intact, had no abnormalities of eye movement, and, as is the case in many patients, there was a long disease duration without the development of other parkinsonian features.”

“The median duration of disease was 11 years.”

Fewer than 1% have this type of PSP.

#4- PSP-Corticobasal syndrome (PSP-CBS)

Symptoms include:  asymmetric limb dystonia; apraxia; alien limb. “An increase in latency to initiate saccadic eye movements, which leads eventually to compensatory head tilts, is the most common eye movement abnormality and is typically more pronounced on the side on which the apraxia predominates. The distinction between this and the typical slowness of saccadic eye movements in Richardson’s syndrome can be difficult to make early in the course of the disease.”

“Most patients with PSP-CBS eventually develop postural instability but this occurs much later in Richardson’s syndrome.”

“Pathological series have indicated that only 50% of patients with CBS have pathology that is typical of corticobasal degeneration… Cerebrovascular disease, Alzheimer’s disease, and progressive supranuclear pathology account for most of the other patients.”

“PSP-CBS seems to be a rare presentation of PSP-tau pathology; only five patients from a pathological series of 160 patients with PSP was identified with asymmetric limb dystonia, apraxia, and alien limb phenomena.”

#5- Progressive non-fluent aphasia (PNFA)

Symptoms include:  non-fluent spontaneous speech, with hesitancy; agrammatism; “phonemic errors that require substantial effort in speech production.”

“In a small case series, five of seven patients who presented with PNFA and prominent early apraxia of speech had underlying PSP-tau pathology. The other two patients had Pick’s disease and corticobasal degeneration… The apparent specificity of prominent early apraxia of speech for tauopathies, particularly PSP-tau pathology, has [led] to the suggestion that this syndrome should be regarded as a clinical subtype of PSP. Patients who present with prominent early apraxia of speech do so at similar ages of onset or disease duration as patients with Richardson’s syndrome.”

Conclusions.  In the conclusion to the review article, the authors state:

“The early recognition of patients with Richardson’s syndrome, PSP-P, PSP-CBS, PAGF, or PSP-PNFA would be enhanced by biomarkers for tauopathies and clinical criteria with a high positive predictive value for Parkinson’s disease, which is the main differential diagnosis for these conditions and is 30 times more prevalent than PSP.”

And:

“Clinical features, such as visual hallucinations, drug-induced dyskinesias, hyposmia, and a prolonged sustained response to levodopa are uncommon in PSP, but have not yet been prospectively assessed in PSP-P, PAGF, or PSP-CBS.”

This sentence of the abstract is important, given that it is in conflict with the “typical”(?) patient’s desire to have a diagnosis:

“[For] patients for whom the diagnosis is unclear, clinicians must continue to describe accurately the clinical picture of each individual, rather than label them with inaccurate diagnostic categories, such as atypical parkinsonism or PSP mimics.”

Copied below is the abstract.

Robin


Lancet Neurology. 2009 Mar;8(3):270-9.

Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges.

Williams DR, Lees AJ.
Faculty of Medicine (Neurosciences), Monash University, Melbourne, Australia; Reta Lila Weston Institute of Neurological Studies, University College London, London, UK.

Progressive supranuclear palsy (PSP) is a clinical syndrome comprising supranuclear palsy, postural instability, and mild dementia. Neuropathologically, PSP is defined by the accumulation of neurofibrillary tangles. Since the first description of PSP in 1963, several distinct clinical syndromes have been described that are associated with PSP; this discovery challenges the traditional clinicopathological definition and complicates diagnosis in the absence of a reliable, disease-specific biomarker.

We review the emerging nosology in this field and contrast the clinical and pathological characteristics of the different disease subgroups. These new insights emphasise that the pathological events and processes that lead to the accumulation of phosphorylated tau protein in the brain are best considered as dynamic processes that can develop at different rates, leading to different clinical phenomena.

Moreover, for patients for whom the diagnosis is unclear, clinicians must continue to describe accurately the clinical picture of each individual, rather than label them with inaccurate diagnostic categories, such as atypical parkinsonism or PSP mimics. In this way, the development of the clinical features can be informative in assigning less common nosological categories that give clues to the underlying pathology and an understanding of the expected clinical course.

PubMed ID#: 19233037  (see pubmed.gov for this abstract)

 

Foster independence and offer respect (advice for caregivers)

Local support group member and volunteer Denise is reading the book How to Care for Aging Parents.  She is offering occasional reports on the highlights of the book.  She has read the second chapter, which is about the caregiving role.  Here are Denise’s take-aways.  Other than the first paragraph of Denise’s notes (which applies to adult children caregivers), all of the rest of the information applies to all caregivers.

Robin

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Denise’s Notes to

Chapter two – “Your Parent and You”
of the book “How to Care for Aging Parents”
by Virginia Morris and Robert Butler
2004

Chapter 2 acknowledges how common it is to still feel the push and pull of the parent/child relationship, no matter how old you are. It recommends hiring a good therapist to tackle those issues, but goes into some detail as to how to approach them on your own. At a minimum you must find ways to set aside your parent’s ability to push your buttons so you can do an effective job of caregiving with as little frustration as possible.

It also has some good words of advice for ALL caregivers.

The first is to foster independence wherever possible. It is difficult to watch a loved one struggle with tasks that were once easy for them. Sometimes we worry about symptoms or frailty contributing to an injury, but we all need exercise. Any amount of movement helps maintain flexibility, balance, even good bowel health. Doing for oneself is also good for the brain. Maintaining one’s identity and dignity can help ward off depression and, possibly, the onset of dementia. It actually takes more patience, time and effort for you, as a caretaker, if the person is slow or messy about some tasks, but it does worlds of good if you are both willing.

Try not to be overprotective of the person you care for. If you feel they are taking excessive risks, start by expressing your concerns rather than bossing around a grown adult. Perhaps your elderly mother doesn’t know breaking a hip can be fatal. Offer solutions to reduce the risk and still allow the activity to continue. If you’re worried she isn’t steady enough on the stairs, suggest moving her bedroom downstairs to avoid them all together. Remember, you only have so much influence dealing with a competent adult. We all have the right to make risky decisions. We do it every day when we get in the car, smoke a cigarette, even eat fatty foods. Some people would rather die living fully than live longer, just like the saying:

*** Life is not a journey to the grave with the intention of arriving safely in a pretty, well preserved body, but rather to skid in broadside, thoroughly used up, totally worn out, and loudly proclaiming, “Wow! What a ride!!!” ***

There are times when it is appropriate to step in and forbid someone from exercising their independence. If the safety and/or health of themselves and/or those around them are seriously threatened you have an obligation to intervene. If you find medication is being misused, you should take steps to dispense them properly. You may find it necessary to pay bills to prevent utilities being shut off or turn off the gas when someone is incompetent to use the stove. Many of us have had to take away car keys. Its not easy. Enlist the assistance of a doctor, if you are unable to manage it on your own. They can report to the DMV, which will suspend or revoke the license, depending on the doctor’s statement.

If you find yourself in doubt about where to draw the line; whether to step in and, if so, how; seek advice from someone with experience. Since this book is all about helping aging parents, it suggests asking a Geriatric Care Manager, Hospital Social Worker or Geriatric Department, an Area Agency on Aging, or Adult Protective Services, which is part of the Department of Social Services.

The second bit of good advice for all caregivers is a big reminder to offer respect. Remember, you may be in their shoes one day. Some suggestions to that end are:

– If the person you’re caring for is inactive, take the time to ask their opinion on various topics to keep them involved in daily life around them. If they’re up to it, read the newspaper, magazines or pick out a recipe together. Don’t forget to listen to their comments and answers.

– If they are unable to speak, assume he or she understands. Explain what’s going on and reassure that his or her needs and preferences are being considered at every point. This is where having those conversations ahead of time come in handy.

– Never speak about someone as if they aren’t present, when they are.

– Don’t be afraid to ask that other caregivers do the same. Correct them if they are behaving in a disrespectful way or doing anything you feel the patient would feel uncomfortable with, if they could speak for themselves.

Keep in mind that IF you’re dealing with someone who is exceptionally difficult you could be dealing with a bigger problem. For instance:

– Unremitting orneriness, social withdrawal, or refusal to eat can be signs of depression. Seek medical advice.

– Complaining, calling constantly, or becoming sick at just the wrong moment (like when you’re about to leave on vacation) can be a way to control you. Get a reliable backup caregiver so you have respite.

– Between 2-5% of elderly people living in the community (not long term care facilities) are excessively suspicious or paranoid. Seek a psychiatrist with elder experience. Treatment is usually carefully balanced medication.

– At least 5% of people age 65 and older suffer hypochondria or undifferentiated somatoform disorder. In the former, a person is obsessed with the idea they have a serious illness. In the latter, a person complains about symptoms (fatigue, loss of appetite, abdominal pain) that have no medical basis. Psychotherapy doesn’t help either of these conditions. Your best bet is a good, firm doctor to sort out the real medical issues and be firm about the imaginary ones.

-Denise