PSP Overview – Parkinson’s Australia (2002)

This is the description of progressive supranuclear palsy (PSP) written by Parkinson’s Australia in 2002.

Finally I’ve found a definition of pseudobulbar palsy:

“pseudobulbar palsy [nerve problems that affect the muscles of the palate, tongue and throat, causing problems with swallowing, gagging and speech];”

Overall, I think this resource is pretty good. It is published on an Australian health website called mydr.com.

Robin
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https://web.archive.org/web/20060427145721/http://www.mydr.com.au/default.asp?article=3307

PROGRESSIVE SUPRANUCLEAR PALSY
Parkinson’s Australia, 2002

Progressive Supranuclear Palsy, or Steele-Richardson-Olszewski’s Syndrome
Progressive Supranuclear Palsy (PSP), also known sometimes as the Mona Lisa disease, is a distinctive and not uncommon cerebral degenerative disease of late adulthood, the cause of which is unknown at present.

It was first observed by Dr J. Clifford Richardson during the 1950s in Toronto. He recognised a few patients who exhibited an unusual neurological syndrome of supranuclear ophthalmoplegia [nerve problems that affect the eye muscles], pseudobulbar palsy [nerve problems that affect the muscles of the palate, tongue and throat, causing problems with swallowing, gagging and speech], dystonia [problems with the muscles], rigidity of the limbs, dementia and other less typical symptoms, and invited Dr John Steele and Dr J. Olszewski to study the pathological changes in the central nervous system.

They found that neuronal cell loss, neurofibrillary degeneration and gliosis [conditions in which the nerve fibres in the brain can become dense and tangled] were extensive in the brain stem, diencephalic and cerebellar nuclei. Damage to pontomedullary reticular nuclei was also likely. Changes in the metabolism of glucose were found in the prefrontal, premotor ungulate cortex and thalamus parts of the brain.

Analysis of protein in the cerebrospinal fluid (CSF) may provide a useful tool in differential diagnosis (i.e. between cortico-basilar degeneration and supranuclear palsy).

Through considerable observations it has been found that the illness is not caused by noxious or toxic substances, is not restricted to a specific geographic or climatic region and is not related to race, occupation, social or economic circumstance or diet. There seems to be a familial background in some cases.

Diagnosis
Symptoms begin in the fifth to eighth decade (the range of age of onset of PSP is 45-73). Early indications are variable and indefinite, and include symptoms such as :

* unsteady gait;
* abrupt falls;
* slowness (akinesia and rigidity);
* cervical dystonia (problems with the neck muscles);
* blurred vision;
* difficulty maintaining eye contact;
* disturbed vertical gaze;
* altered speech;
* dysphagia [difficulty in swallowing];
* forgetfulness;
* short temper and irritability;
* abnormally aggressive behaviour and subtle changes in personality which may be noticed by family members;
* depression; and
* impaired concentration.

As the early symptoms are common to many cerebral illnesses (such as cerebellar degeneration or dementia), accurate diagnosis is difficult and often not possible until further symptoms develop; in fact, the disease is often initially mistaken for Parkinson’s.

As the disease progresses however, symptoms become more defined and characteristic of PSP, so that accurate diagnosis becomes easier. Progression is also more rapid than in Parkinson’s.

Definition
Owing to the difficulty previously experienced by doctors in diagnosing PSP, the following diagnostic criteria have recently been produced. PSP is a progressive disorder (familial examples are rare), beginning in middle or old age with supranuclear ophthalmoplegia including down gaze abnormalities and at least 2 or more of the following 5 cardinal features:

* axial dystonia and rigidity (a broad-based sailor’s walk);
* pseudobulbar palsy [nerve problems that affect the muscles of the palate, tongue and throat, causing problems with swallowing, gagging and speech];
* bradykinesia [slowness of movement] and rigidity;
* frontal lobe signs; and
* postural instability with falls backwards.

Other features which may be present include:
* resting tremor;
* chorea [loss of function of the neurones in the brain];
* dystonia [muscle problems] of the limbs and face;
* cerebellar ataxia [loss of muscle co-ordination caused by disorders in the brain];
* muscle wasting, fasciculation [brief, irregular twitching of muscles that can be seen under the skin] and weakness;
* dysphagia and dyspraxia [difficulty and pain when swallowing];
* respiratory dyskinesias (inspiratory gasps);
* depression;
* sleep disturbances;
* schizophreniform psychoses;
* echolalia and palilalia [involuntary repetition of the same word or phrase when speaking];
* myoclonus [muscle spasms or twitching];
* perceptive deafness;
* other ocular abnormalities such as slowness of blink or difficulty opening the eyes; and
* emotional incontinence.

Symptoms

Gaze palsy
This is the most evident symptom of this condition, along with walking difficulties. This is so apparent that PSP is often referred to as the ‘can’t look up and can’t look down’ syndrome. This symptom, technically known as ophthalmoparesis, is first noticed as a slowness and limited vertical movement of the eyes. By contrast, full reflex eye movement is possible with passive head movement. This symptom progresses through a loss of eye reflex to a complete failure to control eye movements up or down, with some loss of control over horizontal movements, making focusing a problem. Driving and reading also become difficult.

Facial stiffness
The face becomes stiff, immobile and furrowed. Facial and jaw jerks are exaggerated, though more often than not the mouth gapes open, and drooling is common. These symptoms are also signs of pseudobulbar palsy.

Gait and muscles
The head is usually hyperextended and the neck becomes stiff and extended and will resist forward and backward movement, making going up and down stairs difficult. Rigidity and bradykinesia of the limbs develop slowly. The combination of these symptoms (inability to look down, rigidity and stiffness) causes an increasing awkwardness, disturbance of gait and hesitancy. Unsteadiness and falls are common problems (often the first symptoms) and the person can often totter backwards and fall without knowing why. Walking becomes more and more hesitant and awkward as the tendency to fall backwards continues. The cause of this phenomenon is unknown and is often mistaken for the gait disturbance typical of early Parkinson’s.

Swallowing problems
There are speech and swallowing difficulties, with repetitive swallowing of saliva, explosive coughing and heightened palate and throat reflexes.

Common signs that a person is having difficulty swallowing might include:
* frequent coughing while eating or drinking;
* multiple swallows on a single mouthful;
* the sensation of food ‘sticking’ in the back of the throat;
* weight loss; and
* chest congestion after eating or drinking.

Mental changes
Mental changes are often limited to personality alteration and forgetfulness. However, more recent studies have shown dementia is more common than first thought. A study done in 1986 found that while cognitive impairment did not parallel motor impairment it did correlate with visual impairment. There is evidence of some impairment of judgement and loss of abstract thinking.

Late stages
In the late stages of PSP, the eyes are fixed centrally, and reflex movement may be totally absent. Bradykinesia is prominent and the person assumes a rigid and double hemiplegic-type posture. That is, the body becomes totally rigid and unable to be moved voluntarily. There is particular difficulty with trunk movements when turning from side to side and sitting up. Because of these symptoms and the inability to control one’s movements, the person becomes immobile and bedridden. In extreme cases of poor swallowing a tube may be inserted into the stomach for feeding.

The average length of illness, from diagnosis to death, is 5 to 6 years, with the range being 2 to 11 years. Men seem to be more affected by the disease than women. There are an estimated 20,000 cases in the USA, 6,000 in the UK and up to 1,500 in Australia. Misdiagnosis is frequent and it is most commonly mistaken for Parkinson’s, although it is only about 3 per cent as common.

Management

Medication
Most people with PSP are resistant to dopaminergic medications, probably because they have lesions in non dopaminergic neurotransmitter systems more profound than those in Parkinson’s. Though limb rigidity and bradykinesia and balance improve in patients treated with levodopa, ophthalmoplegia and axial dystonia are unaffected. Other treatments which seem to show some effect are tricyclic antidepressants (e.g. amitryptiline), bromocriptine, pergolide, idazoxan and lisuride. Medication needs to be individualised and doctors should work with the patient to establish a suitable drug regimen. Surgery has been tried without success.

Eating
* Check that the person is sitting up as straight as possible.
* Minimise distractions such as TV.
* Concentrate on the task, considering speed and quantity, as well as how well the person is chewing.
* A teaspoonful of food at a time is ample.
* Allow enough time for the person to eat so that they don’t feel rushed.
* Be sure they have completed the swallow before offering another spoonful.

Walking
* An aid to walking may need to be weighted in front.
* Raising heels of shoes may reduce the tendency to tip backwards.
* Rails in the bathroom may be helpful.
* Remove low objects to prevent the patient falling over them.
* Prismatic glasses have been of limited assistance.

Other exercises
* Exercises can help retain some joint mobility.
* Breathing exercises may be needed to prevent complications of bed rest.

Carers and coping strategies
* It is important to understand changes that are part of the disease process and are therefore out of voluntary control.
* Fluctuation in function and personality are to be expected.
* Lists can help with memory loss.
* Include the individual in all discussion and normal decision-making.
* Carers should not blame themselves but blame the disease.
* If irritability is a problem, distraction is better than argument.
* Reserving time for carers’ own interests is important.

Consultants
Physiotherapists, occupational therapists, speech pathologists, psychologists and social workers all have important roles in assisting in the management of PSP.

myDr, 2002. Reproduced with kind permission from Parkinson’s Australia.
Reviewed : 19/7/2002

“What Caregivers Need to Know”

“What Caregivers Need to Know,” an article in today’s “Parade Magazine,” offers advice on how caregivers can cope physically and psychologically. The article was written by Claire Berman. She is also the author of the book “Caring for Yourself While Caring for Your Aging Parents: How To Help, How To Survive.”

Here’s an excerpt:

“‘In caring for others, are caregivers neglecting the most basic self-care?’ asks NAC President Gail Hunt. ‘Are they getting their flu shots? Are they getting their mammograms? Are they getting their Pap tests? Or are they saying, ‘I don’t have time for that because I’m so immersed in caregiving’? The message has got to be: If you want to do this kind of caregiving, you need to be sure you get the most basic preventive health care for yourself.'”

Ms. Berman offers these coping strategies:

* Learn about the illness.
* Ask for help — and be specific.
* Maintain contact with friends.
* Seek assistance within your community.
* Keep the focus clear.
* Find support in a group.
* Enjoy life outside of the caretaker role.
* Appreciate the good moments.

Here’s a link to the article:

https://web.archive.org/web/20090628153244/http://www.parade.com/articles/editions/2005/edition_10-09-2005/featured_0

What Caregivers Need To Know
By Claire Berman
Parade Magazine
October 9, 2005

And copied below is a short excerpt on coping with the emotional issues raised by caregiving.

Robin

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How Do I Deal With My Feelings?

Studies show that many people are better able to cope with the practical challenges of caregiving than with the emotional issues. Among those issues:

“I feel guilty.” Guilt is the most pervasive emotion of the caregiver—as much for the adult child who lives with a housebound parent as for the one who tries to manage things from a home across the country. We all feel we should be doing more, acting more loving, being more tolerant.

“I feel angry.” There is anger that a loved one should be so ill and needy, and resentment that our own lives have been turned around or that other family members aren’t pitching in. A conversation among siblings might solve the problem, but the angry caregiver thinks: “I shouldn’t have to tell them—can’t they see I’m exhausted?”

“I’m afraid.” We fear the progress of the disease and the difficulties yet to come. Often, we fear that we may not be up to the tasks required of us.

“I miss him.” It’s heart-wrenching to accept the waning powers of a loved one who now requires assistance just to make it safely through a day. We mourn the loss of the person we once knew.

These feelings are normal. The key is to acknowledge them and recognize that they are universal. Most important, don’t feel ashamed of your emotions. It might help to discuss them with a trusted friend.

Disclose early, find roses, and hope in Parkinson’s (2005 article)

I’ve been reading about the importance of hope, and ran across this article from The Washington Post, published last week. It’s certainly worth reading. The author, Daniel Stark, has written extensively about his battle with Parkinson’s Disease (PD). Though it’s about PD, I think the principles apply to any neurodegenerative disorder.

Here’s a link:

www.washingtonpost.com/wp-dyn/content/article/2005/06/20/AR2005062001057.html

Living Large With Parkinson’s
It’s A Messy Path Ahead, the Author Finds — But Better With a Map
By Daniel Stark
Special to The Washington Post
Tuesday, June 21, 2005; Page HE01

Robin

PSP Stages/Phases, by two caregivers

This August 8, 2000 document on the phases/stages of progressive supranuclear palsy (PSP) was written by two caregivers who were members of the now-defunct Johns Hopkins PSP Listserv. Many neurological diseases, such as Alzheimer’s and Parkinson’s, have “stages.” So this was an attempt by two PSP caregivers to have the same guide posts for the PSP community.

The guide is copied below. (I’ve fixed a few typos.)

Robin

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www.pspinformation.com/disease/psp/stages.shtml
[Editor’s note, 2013: This website is no longer active]

Caregiver’s Guide to the Phases/Stages of Progressive Supranuclear Palsy
Compiled by Patricia Lake of Texas and Mary Holeman of Indiana
August 8, 2000

In 1999 several members of the Johns Hopkins PSP Listserv decided to pool their collective wisdom to try to see if they couldn’t categorize the symptoms associated with the normal progression of progressive supranuclear palsy enabling the defining of phases or stages of the disease.

This is the result of these efforts. It was originally posted as a message on the PSPInformation mailing list. Visitors to the PSPInformation website often tried to find the information on the website and couldn’t – so, “by popular demand” we are also publishing it here. PSPInformation.com does not claim any credit nor does it assume any responsibility for the statements made in this article. It is solely the viewpoint of the writers.

This categorizing of the symptoms of progressive supranuclear palsy is not meant to be scientific. If you are caring for someone with PSP you should realize the disease follows its own “timetable”. The progress of the disease may be different in your case. The purpose of the article is to serve as a generalized caregiver’s guideline to what to expect as the disease progresses.

We must also note that progressive supranuclear palsy is often misdiagnosed because the diagnosis is based on symptoms. Many symptoms can apply to more than one of the many diseases and conditions that are very similar and determining their real association can be very difficult if not impossible. Most patients may also be on medication and very susceptible to possible medication caused side-effects. Thus, they may develop symptoms that may be thought to be from progressive supranuclear palsy but are not.

The Phases/Stages of PSP

Phase 1 – Deterioration of handwriting and difficulty writing. Speech problems, difficulty being understood by others, slurring, etc. Coordination problems leading to unexpected falls and stumbling; change in walking rhythms/patterns. Vision problems; some complain of ‘blurred’ vision; some try prisms, but examinations by eye Dr. generally will only find normal vision physically. Lethargy, apathy, no desire to do anything; changes in sleep patterns. Cognitive problems; decrease of sound judgement, decrease in modesty; increase in impatience and irritability.

Phase 2 – Problems sitting down or getting up; cannot lower self into chair gently; just ‘plops’ down. Increased difficulty walking; begins using a cane for balance; will progress to a walker; increased number of falls. Stooped posture because of vision problems; can’t see downward easily. Problems opening or closing eyes; some patients get ‘dry eye’ because their eyes do not close all the way. Difficulty dressing; cannot do buttons or zippers; hands and fingers do not work as they used to. Almost impossible to write anything legibly. Eating problems; coughing and choking; loss of eating etiquette; fills mouth too full; lots of spills; begins wearing a bib to save clothes. Bathroom problems; difficulty voiding/unable to get to bathroom in time; constipation or diarrhea; may need help with personal hygiene. Needs help bathing; may need hand rails/bathing bench, etc. A mobile shower head is a good idea, if possible. Weakness or neglect on one side of body; one side more dominant; ie: drags left or right foot, etc. (ShyDrager Syndrome) Subject to infections; urinary tract, respiratory tract (pneumonia) etc. Alien hand; sometimes holds on to things and cannot let go or takes the hand a long time to release. Difficulty concentrating; sometimes seems ‘out of it’.

Phase 3 – Some obsessive-compulsive behavior: ie: fingers “pill rolling”, hands smoothing out imaginary wrinkles on table, etc. Increased irritability; increased impatience. May become incontinent of urine and bowel. Increased speech problems; often very difficult to understand; cannot articulate proper speech sounds. Increased eating problems; more coughing/choking. Increased cognitive problems; cannot follow stories on TV; cannot read much, due to vision; will watch game shows, some news. In Theater movies are not recommended, as some suffer from ‘sensory overload’; loud sounds and many colors, lots of movement on screen make patient ‘nervous’. sleeps much of the day, and all night, too. Instances of ‘restless leg’ syndrome. Limbs and neck may become rigid; may loose ability to support self on legs. Increased falls; some falls may be close to geing described as ‘seizures’; complete loss of control of arms and legs, with resultant fall. After fall, will sleep for an hour or so; may not always know whether is injured or not; may not ‘feel’ the injury. Increased coughing and choking; drooling becomes common; often does not close mouth; Infections may be more frequent. Requires much more help in dressing and with all activities of daily living. Does not speak much, but does enjoy seeing friends and relatives, even though patient may not respond much to them. May have pain in arms or legs; non-specific pain for no apparent reason; application of ‘heat’ rubs may help; Tylenol may also help.

Phase 4 – Unintelligible speech/mumbling; cannot say words; may go days with out saying anything. constant drooling; coughing and choking may become so severe that eating normally is impossible; Dr. may recommend feeding tube, which requires a surgical procedure to install. May have trouble opening mouth, even for meds. Increased incontinence/constipation problems. Loosing interest in daily activities; sleeps most of the time; uncomfortable sitting for any length of time; prefers bed. Cannot support self on legs; ‘spaghetti legs’; body rigid, especially neck area. Little eye movement; cannot ‘look’ at something; slow to focus on things in view. delusions, hallucinations at times; may be disoriented and not know where they are. Pain, but cannot identify the area. Withdrawn, but remains aware of people; cannot move on own; needs extensive help for all activities of daily living.

Note: These phases or categories often overlap and are not the same for all patients. Some may have two or three phase 1 problems and one phase 3 problem. Some may never have all of the problems, but most will need extensive help to live out their lives and will need to be made as comfortable as possible for the duration of their illness. It is wise to gain from the patient, specific information as to feeding tubes, restorative therapies, etc. before the disease is well advanced; a living will is a good idea, so that loved ones will know how the patient wishes to be treated, should life threatening acute problems occur. Long term care will probably be needed and plans should be made before the patient becomes unable to participate in the planning.

“Challenging the Odds: forget the prognosis”

Back in 2004 when my father was diagnosed with progressive supranuclear palsy, I joined some online support groups related to PSP.  I met an incredible woman named Aletta, who had been diagnosed in the mid-80s with multiple system atrophy (though she prefers the old term Shy Drager Syndrome).

Today, Aletta posted this wonderful article by Barry Bittman, MD on accepting the diagnosis (or getting a second opinion) but forgetting the prognosis.

Robin


Challenging the Odds: forget the prognosis
by Barry Bittman, MD
Emerge, March 1996

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 favor – Mind Over Matter!
Copyright 1998,1999 Barry Bittman, MD. All rights reserved.