Progressive supranuclear palsy (PSP) - also known as
Steele-Richardson-Olszewski syndrome - is a rare degenerative
neurological disease - only
about 4-5 percent as common as Parkinson's disease. The peak age
for contracting this disease is in the mid-60's and it appears to
affect men more often than women.
PSP is characterized by symptoms similar to Parkinson's disease such
as unsteady gait, unexplained falling, stiff movements, and mild dementia plus other
symptoms not normally a part of Parkinson's disease icluding
problems with the voluntary movement of the eyes.
PSP is a disorder that is often misdiagnosed. Some of the
symptoms used to identify the disease, including the eye movement
problems, do not appear until the patient is several years into
the progression of the disease. For this reason, PSP is often
diagnosed as some other illness (such as Parkinson's or
Alzheimer's) in its earlier stages.
History of Progressive Supranuclear Palsy (PSP)
PSP 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.
The results of this study were published in a medical journal
in 1964. This resulted in the disease also being known as
Steele-Richardson-Olszewksi syndrome which is the term still used
in much of the world outside the United States.
PSP wasn't a new disease, it was just so rare that no one had determined
it was it's own condition and not a variation of Parkinson's disease.
The researchers 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.
Many parts of the brain are affected including the brainstem,
the basal ganglia, and the cerebellum. The brainstem is located
at the top of the spinal cord. It controls the most basic
functions needed for survival--the involuntary (unwilled)
movements such as breathing, blood pressure, and heart rate. The
brainstem has three parts: the medulla oblongata, the pons, and
the midbrain. The parts affected by PSP are the pons, which
controls facial nerves and the muscles that turn the eye outward,
and the midbrain, the visual center. The basal ganglia are
islands of nerve cells located deep within the brain. They are
involved in the initiation of voluntary (willed) movement and
control of emotion. Damage to the basal ganglia causes muscle
stiffness (spasticity) and tremors. The cerebellum is located at
the base of the skull. It controls balance and muscle
coordination.
What are the First Symptoms of PSP?
The first symptoms usually begin between ages 50 and 70
(peaking in the early sixties), with a range from the early
forties to the late eighties and is more common in men than in
women.
The most common first symptom usually is loss of balance while
walking or experiencing several unexplained falls over a year.
Patients may develop some stiffness and walking awkwardness and
be incorrectly diagnosed as having Parkinson's Disease.
Patients however rarely develop the resting tremor and stooped
posture characteristic of Parkinsonism. Falling can also cause
broken bones and fractures that are difficult to treat.
Other common early symptoms may be changes in personality and
forgetfulness. This 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. They would be more correctly diagnosed as being
symptoms of patient apathy.
Less common early symptoms are trouble with eyesight, slurring
of speech, difficuly driving a car and having unexplained
accidents.
The Development of Additional Problems and Symptoms
As it can happen with Multiple Sclerosis and other diseases,
PSP causes loss of the myelin sheath (the covering of the nerve
cell that speeds nerve impulse conduction) in some nerves and
destruction of the entire nerve in other areas. There is a palsy
or paralysis, that slowly gets worse as the disease
progresses.
Additional symptoms develop over time due to the increased
damage to brain tissue caused by the disease. It is this
development of new symptoms that further defines the condition
and often leads to a change in diagnosis from some other disease
to PSP.
Gaze palsy (vision)
This presense of this symptom (technically known as
ophthalmoparesis and uncommon with Parkinson's and
Alzheimer's diseases) along with the development of walking
difficulties are used as primary indications in the diagnosis of
PSP. In most cases the walking problems occur first followed in 3
to 5 years by the problems with the eyes.
Vision is controlled by groups of cells called nuclei
in the brainstem. In PSP, the nuclei continue to function, but
the mechanisms that control the nuclei are destroyed. The term
supranuclear means that the damage is done above
(supra) the nuclei.
With gaze palsy there is an impaired ability to voluntarily
move the eyes up or down. The effect is so noticible that PSP is
often referred to as the 'can't look up and can't
look down' disease. The upper eyelids may also be pulled
back, the eyebrows raised, and the brow wrinkled, causing a
typical wide-eyed stare.
This can interfere with eating. Patients can't see that
they are dropping food when they eat. They may esperience difficulty
with descending a flight of
stairs, among other things. This problem is not usually as vexing
for the patient and family as the inability to maintain eye
contact or to coordinate eye movements while reading, but is much
easier for the doctor to detect.
Eventually ,patients loose the ability to look up and down at all,
and usually about a year later, the ability to look from side-to-side is
also lost.
The reduction in the ability to control vertical eye
movement is usually the first clue to the doctor that the
diagnosis is PSP. The diagnosis is often changed from Parkinson's Disease
to PSP at this time.
Other conditions, particularly Parkinson's disease and
normal aging, can also sometimes cause difficulty moving the eyes up.
However, PSP is nearly unique in also causing problems moving the
eyes down.
Because the main difficulty with the eyes is in aiming them
properly, reading often becomes difficult or impossible. The
patient finds it difficult to shift down to the beginning of the
next line automatically after reaching the end of the first
line.
This is 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.
Another common visual problem is an inability to maintain eye
contact during conversation. This can give the mistaken
impression that the patient is senile, hostile, or uninterested.
The same eye movement problem can create the symptom of
"tunnel vision" and can interfere with driving a
car.
With PSP reflex or unwilled eye movements remain normal. Thus,
when the patient's head is tilted upwards, the eyes move to
look down. These reflex movements remain normal until late in the
course of the disease.
Other Eye Problems
A different 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 is 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 can itself become annoying.
As it can happen with Multiple Sclerosis and other diseases,
PSP causes loss of the myelin sheath (the covering of the nerve
cell that speeds nerve impulse conduction) in some nerves and
destruction of the entire nerve in other areas.
What Causes PSP?
PSP is caused by (or perhaps causes) damage to nerve
cells in a portion of the brain called the "midbrain"
These cells are involved in eye-movements and balance. The
cause of the degeneration of these cells is unknown.
The damage results in progressive lack of coordination,
stiffness of the neck and trunk, eating and breathing problems,
balance control, unexpected and uncontrollable falling,
difficulties with the ability to control eye movement, slow
physical movement, cognitive dysfunction, and difficulty with
walking and muscle coordination.
People with PSP also have deposits in brain tissues that
resemble the deposits found in Alzheimer's disease.
There is atrophy (loss of tissue) in most areas of the brain.
We don't know what causes PSP. That said, there does seem
to be risk factors leading to the development of the disease that
are similar to the recognized risk factors for other neurological
diseases such as Parkinson's and Alzheimer's
diseases.
Through considerable observations it has been found that the
illness is not caused by any single preventable cause. It 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. Most
people who develop PSP come from families with no history of the
disease, so it does not seem to be inherited, except in certain
rare instances. It is not passed on to children.
Diagnosis
PSP is a clinical diagnosis, meaning there is no "test"
for PSP. It is normally diagnosed by a neurologist who looks at the
symptoms and compares them with some source of information intended
to narrow the possible choices as much as possible.
Diagnosis is always an "educated" guess and can be
incorrect. The diagnosis can also change as symptoms appear or
change during the progression of the disease.
Owing to the difficulty previously experienced by doctors in
diagnosing PSP, the following diagnostic criteria have recently
been produced. PSP is a progressive disorder 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
How is PSP Different from Parkinson's Disease?
PSP is often mistaken for Parkinson's disease, which is
also associated with stiffness, frequent falls, slurred speech,
difficulty swallowing, and decreased spontaneous movement. The
facial expression in Parkinson's, however, is blank or
mask-like, whereas in PSP it is a grimace and wide-eyed stare.
PSP does not normally cause the uncontrolled shaking (tremor) in muscles
at rest that is associated with Parkinson's disease. Posture
is stooped in Parkinson's disease, but erect in PSP. Speech
is of low volume in both diseases, but is more slurred and
irregular in rhythm in PSP.
Patients with PSP develop abnormal vertical eye movements.
A few other neurological diseases share this symptom so presence of
this symptom cannot be used as a guarantee the patient has PSP
rather than some other disease. As an example Gaucher's type-III,
also causes a progressive supranuclear palsy, but it begins
horizontally. Other disorders that may be mistaken for
PSP include corticobasal degeneration, Pick's Disease,
Multi-system Atrophy (MSA) and Diffuse Lewy Body disease.
When compared with the symptoms of parkinsonism, patients with
PSP usually have gait instability, absence of tremor, little or
no response to levadopa and dopamine agonists and develop the
vision problems normally associated with the disease's
progression.
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).
Multiple strokes or abnormal accumulations of fluid within the
skull (hydrocephalus) can also cause balance problems similar to
PSP. Magnetic resonance imaging (MRI) scans of the brain may be
needed to rule out these conditions. In advanced cases, MRI shows
characteristic abnormalities in the brainstem described as
"mouse ears."
Symptoms
Symptoms may include:
Motor/Physical
Unsteady gait
Loss of balance
Abrupt falls
Deteriorating handwriting
Slow to perform daily activities
Fatigue
Slurred speech
Loss of strength
Problems with neck muscles and stiffness
Dizziness
Coughing when eating
Difficulty swallowing (Dysphagia)
Back or neck pain or injury
Muscle cramps or spasms
Shakes, tremors or jerks
Abnormal hand posture
Abnormal leg posture
Loss of consciousness
Vision
Can't move his/her eyes down
Difficulty maintaining eye contact
Difficulty reading
Blurred vision
Double vision
Personality/Emotion
Changes in personality
Altered speech
Depression
Losing control of emotions
Short temper and irritability
Lack of emotions
Excessive anger
Impaired concentration
Cognitive
Difficulty concentrating
Forgetfulness
Word finding difficulty
Failing memory
Other
Changed sleeping patterns
Difficulty in sleeping
Bowel/bladder function
Eating too quickly
Eating too much at once
Ringing or loss of hearing
Loss of sensation anywhere
Tingling or burning
More Information Concerning Symptoms
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 (Dysphagia)
Swallowing tough foods or thin liquids can become difficult
because of throat muscle weakness or incoordination. This tends
to occur later than the walking, visual, and speech problems, but
can become very troublesome if the patient tends to choke on
food. Unlike the other difficulties in PSP, this one can
sometimes pose a danger for the patient - the danger of food
going down the wrong pipe into the breathing passages, termed
"aspiration." Usually, difficulty managing thin liquids
precedes difficulty with solid food. This is because in PSP, the
swallowing muscles have difficulty creating a watertight seal
separating the path to the stomach from the path to the lungs.
The same is true for the swallowing difficulty of many
neurological diseases. For non-neurologic conditions such as
stricture of the esophagus, however, difficulties start with
solid foods.
Repeated, minor, often unnoticed episodes of small amounts of
food and drink dripping into the lungs can cause pneumonia.
Often, it is not apparent to the physician or family that the PSP
patient's pneumonia is in fact the result of subtle
aspiration. But "aspiration pneumonia," in fact, is the
most common cause of death in PSP.
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. As time goes on however most patients develop a
mild or moderate degree of mental impairment or dementia.
In PSP, the dementia, if it does occur, does not feature the
memory problem that is so apparent in Alzheimer's disease.
Rather, the dementia of PSP is characterized by slowed thought
and difficulty combining different ideas into a new idea or plan.
These mental functions are performed mostly by the front part of
the brain (the "frontal lobes"). In Alzheimer's, on
the other hand, the problem is mostly in the part of the brain
just above the ears (the "temporal lobes"), where
memory functions are concentrated.
Alzheimer's disease also includes either difficulty with
language (such as trouble recalling correct names of common
objects) or difficulty finding one's way around a previous
familiar environment. Fortunately, these symptoms almost never
occur in PSP.
Slowing of thought can cause major problems for people with
PSP by making it difficult to partake in conversation. A question
may be answered with great accuracy and detail, but with a delay
of several minutes.
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.
Patient Care and Treatment
PSP cannot presently be cured. A true cure would require the
ability to regenerate, restore and renew the brain tissue and
neurotransmitter connections destroyed or damaged by the disease.
There is hope that future medical progress will lead to the
ability to control and slow the presently relentless aggressive
progression of the disease and by doing this extend the life of
its victims.
People with PSP usually can remain at home with their families
during its early stages. As the disease progresses they may require
more care than the family can provide and access to a skilled
nursing facility may be necessary. Falling is always a danger
when the patient is able to still get around physically so
their activities require constant close supervision. The patient
should be checked often by their medical team to monitor the
effects of any treatment and make changes if needed.
Individuals with PSP should remain physically, mentally, and socially
active as long as they are able.
Daily physical exercise helps maximize body and mind functions and
helps to maintain a healthy weight.
The individual should engage in as much mental activity as he
or she can handle. Puzzles, games, reading, and safe hobbies and
crafts are good choices. These activities should ideally be
interactive.
Social interaction is stimulating and enjoyable for most people
with PSP. Most senior centers or community centers have scheduled
activities that are suitable for those with dementia.
A balanced diet that includes plenty of fruits and vegetables
will help maintain a healthy weight and prevent malnutrition and
constipation.
Medications
Drugs are sometimes given in an attempt to try to relieve or
modify the symptoms associated with the disease. None claim to
either cure or slow its progression, Thus, treatment with drugs
is usually disappointing and expermental.
Remember too that each drug has the capability of causing
serious side-effects either immediately or over the long-term
which in turn may cause the patient's condition to worsen
rather than improve.
Most persons with PSP are resistant to the dopaminergic
medications commonly used used to treat in Parkinson's
disease, such as levodopa (Sinemet). This may be because these
patients usually have more lesions in non-dopaminergic
neurotransmitter systems than they have those in the dopaminergic
areas of the brain normally affected by Parkinson's
disease.
Though limb rigidity, stiffness, slowness of movement and
balance may improve somewhat in patients treated with levodopa,
the eye movement problem and axial dystonia - both common with
PSP - are unaffected.
Anticholinergic medications, such as trihexyphenidyl (Artane),
which restore function to neurotransmitters, or tricyclic drugs,
such as amitriptyline (Elavil) may improve speech, walking, and
inappropriate emotional responses.
It must be remembered though that since it has been found the
lack of a neurotransmitter called acetycholine may also
contribute to the symptoms of PSP the use of any anticholinergic
medication may also tend to worsen symptoms long-term or increase
the speed of progression of this disease.
Medication, if provided, needs to be individualised. Doctors
should work with the patient and their caregiver to establish a
suitable drug regimen. It is a personal choice whether to provide
drugs to the patient at all since most seem to have either have
adverse side-effects or do not work at all. Remember too that as
we age the liver generally works slower and has a greater problem
getting drugs out of the body. Thus, a drug dosage that provides
no side-effect problems with a younger person may be too large
for an older person.
Other Treatments
Surgery - which has been useful to those diagnosed with
Parkinson's disease has been tried without success to treat
those diagnosed with PSP.
Botulinum toxin can be used to treat eyelid spasms and other
types of dystonia that sometimes occur in PSP.
Artificial tears, drops and ointments can help prevent the
drying out of the eyes that may occur from decreased blinking. It
is important to keep the eyes moist. Failing to do so may cause
physical damage to the surface of the eyeball. Ask your medical
advisor for product recommendations.
Speech therapy may help manage the swallowing and speech
difficulty in PSP. As the disease progresses, the difficulty in
swallowing may cause the patient to choke and get small amounts
of food in the lungs. This condition can cause aspiration
pneumonia. The patient may also lose too much weight. In these
cases, a feeding tube may be needed.
Additional Tips on how to Cope with the Disease
The PSP patient may need alternative communication devices,
such as pointing boards or computer-based systems. Books on tape
provide an alternative when reading is no longer possible.
The home environment should be modified to decrease potential
injury from falls. Caregivers can improve the safety of the
person with PSP by keeping pathways in the home clear of objects
such as toys, floor rugs, or low furniture that are difficult to
see without looking down. An occupational therapist can advise on
these and other home modifications to improve safety, comfort,
and usability of the home environment.
Walkers can be weighted in front, to prevent backward falls
and handrails can be installed in the bathroom.
Dry eyes from infrequent blinking can be treated with drops or
ointments.
Eating
When the patient is trying to eat:
If the patient cannot adequately feed themselves
handfeeding by the caregiver may be needed.
Make sure the person is sitting up as straight
as possible. It aids swallowing and tends to reduce the
possibility of aspiration and pneumonia. Try not to encourage
patient slumping.
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 or bars in the bathroom and around the
house 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.
Physiotherapists, occupational therapists, speech
pathologists, psychologists and social workers all have important
roles in assisting in the management of PSP.
Prognosis
PSP gets progressively worse but is not itself directly
life-threatening. The patient's condition gradually
deteriorates. After about seven years, balance problems and
stiffness make it nearly impossible for the patient to walk.
Persons with PSP become more and more immobile and unable to care
for themselves. Death is not caused by the PSP itself. It is
usually caused by pneumonia related to choking on secretions or
by starvation related to swallowing difficulty. It usually occurs
within 10 years, but if good general health and nutrition are
maintained, the patient may survive longer.
