Larry,

  I hope this helps to explain PD+ syndromes.

Deborah aka Tenacity
_________________________________________________

Imitators of Parkinson’s Disease
A pamphlet prepared by
David E. Riley, M.D.
Director
Movement Disorders Center

Mt. Sinai Medical Center
Cleveland, Ohio


Imitators of Parkinson’s Disease

Parkinson’s disease (PD) is a common nervous system disorder which usually
affects people around the age of 60. It causes tremor, muscle rigidity,
problems with posture and balance, and difficulty with performing voluntary
movements. This last feature (akinesia) causes the greatest problems for
most people with PD. Voluntary movements are reduced in speed and size.
Consequently, patients tend to move slowly, their stride and handwriting get
smaller and they don’t blink as often,
or swing their arms when walking.

Related symptoms include a softer voice, a lack of facial expression (the
‘masked face’), and difficulty turning over in bed, or getting out of chairs
or a car. PD typically starts on one side of the body, and remains worse on
that side as it gradually progresses. PD involves the loss of a specific
group of nerve
cells, known as the substantia nigra, which make and release a chemical
called dopamine. We usually treat PD by giving people medications that make
up for this decrease in dopamine.

Neurologist now recognize that a number of other, less common, diseases
cause many of the same problems as PD. Usually we distinguish a PD imitator
by additional features that are not found in PD (hence the name
‘Parkinson’s plus’ or ‘a-typical parkinsonian’ syndromes. However, these
differences are not always obvious, especially at the beginning.

As a result, some patients with other diseases look like they have PD at the
outset, and may do so for years. The lack of specific laboratory tests
compounds the problem, because there is no way to prove whether someone has
PD or not. Often, another disease is only suspected when a
person does not improve with treatment for PD. Recent autopsy studies show
that up to 1 of 4 people thought to have PD during life, actually have a
different disease!

One of the most important considerations in people with symptoms that
suggest PD is that some drugs can produce all the typical characteristics of
PD as a side effect. In particular, many drugs used to treat psychiatric
problems, and related drugs for stomach problems,
including metoclopramide (Reglan), can do this because they block dopamine,
mimicking the chemical effect of PD. It is crucial that a doctor be told
about all medications that a person is taking, or has
used within the last year. Another condition, which is often mistaken for PD
is essential tremor. Essential tremor (ET) usually affects both sides from
the start and only causes tremor, not the rigidity or akinesia seen in PD.
Shaking of the head is a sign of ET, not PD.

ET is usually inherited, and many people thought to have hereditary PD
actually have ET. Some people with early PD are thought, by family or
friends to have had a stroke. In truth, strokes rarely imitate PD, and
then, mainly by causing a shuffling gait.

Many other conditions may mimic PD in certain respects, but there are three
diseases which are most likely to be mistaken for true PD. They differ from
one another in important ways, but they also have features in common.

For example, they were all first described in the 1960’s,
and have names that are barely pronounceable. They are progressive
supranuclear palsy, multiple system atrophy and cortical-basal ganglionic
degeneration.

How do these imitators resemble PD?

They copy PD’s tendency to affect people in the second half of their life,
and to cause a gradual disappearance (degeneration) of specific groups of
nerve cells that are part of the motor system. In fact, all three can
involve the substantia nigra, the same part of the motor
system damaged in PD, which is why they produce many similar symptoms and
findings on the neurologic examination. They also share the tendency to
leave the rest of the brain intact, so that non-motor functions of the
nervous system, such as language, are usually
undisturbed. We do not know what triggers any of these degenerative diseases
of the brain, including PD.

The specific features that these diseases have in common are the major
features of PD: akinesia (difficulty initiating and performing movements,
and movements are reduced in speed and size), rigidity (muscle stiffness),
tremor (shaking) and disequilibrium (unsteadiness,
falling). Together we know them as parkinsonism. PD can be considered but
one cause of parkinsonism which is distinguished by special characteristics
with respect to the people it affects, the way it evolves, the additional
symptoms it causes, it’s response to medication and the damage it does to
the central nervous system.


How do they differ from PD?

Imitators of PD are less likely to cause tremor, especially the rest tremor
so common in PD. All three of these diseases lead to a loss of balance and
falling early in their course, whereas falls only occur
after many years of PD. Perhaps the most significant way in which they
differ from PD is that their response to medications which promote dopamine
within the brain, especially levodopa, is not nearly as pronounced or
sustained as in PD.

PD imitators affect parts of the brain’s motor system other than the
substantia nigra, and sometimes other systems as well. This is why they
cause additional symptoms and signs not found in PD, which is how we
usually recognize them. We are still learning how to do this accurately and
reliably.


Why is it important?

It is important for physicians to be able to sort out those people affected
by PD imitators from those with true PD. As of the writing, there is no
specific treatment for the diseases to be discussed below.
However, we are at a relatively early stage of understanding these
illnesses, and there is a great deal of research activity in this field. If
any useful remedy is going to be developed, it’s effectiveness can be
properly determined only when we are reasonably
sure the people trying it have the disease in question. As always in
medicine, a correct diagnosis allows people to avoid repeated consultations,
unnecessary laboratory tests and fruitless treatments.

It is equally important that we not enroll patients who have other types of
parkinsonism into research studies on PD, because the information learned
from them may produce misleading conclusions about PD. For example, one of
the first patients who underwent a fetal
substantia nigra transplant did not respond, suggesting the operation might
have a limited value. When the brain was examined at autopsy, however, what
was found was not PD at all, but striatonigral
degeneration (see below)

PROGRESSIVE SUPRANUCLEAR PALSY

This disease is called Steele-Richardson-Olszewski syndrome in Europe, after
the 3 Toronto physicians who first recognized and reported it in 1964. The
name ‘progressive supranuclear palsy’ (PSP) refers to a
characteristic disorder of eye movements which results in difficulty looking
downward or upward. This frequently causes problems with reading, walking
down stairs, or eating. Trouble with opening or
closing the eyes is also common. The eye movement disorder is the most
distinctive feature of PSP, but is not always present early on. The most
common initial symptom is loss of balance and falling. Other early
signs of PSP include a strained or soft (often whispered) voice, slurred
speech, impaired swallowing, personality changes and forgetfulness.

PSP causes more problems than PD because it damages not only general motor
centers but also directly involves areas in the brainstem (the part
connecting the brain to the spinal cord) that control eye movement,
swallowing, and the other functions that are impaired as noted above.
PSP tends to involve both sides of the body equally, unlike PD.

PSP tends to affect a slightly older age group than PD and men somewhat more
than women. Approximately 5% of people with parkinsonism with no obvious
cause will turn out to have PSP. If you would like more information, there
is a well-organized national foundation devoted to
PSP:

The Society for Progressive Supranuclear Palsy, Inc.
Johns Hopkins Hospital
5065 Outpatient Center
601 N. Caroline Street
Baltimore, Md 21287


MULTIPLE SYSTEM ATROPHY

The term multiple system atrophy (MSA) encompasses three variations once
thought to be separate diseases: striatonigral degeneration (SND),
olivopontocerebellar atrophy (OPCA), and Shy-Drager disease (SDD) or
syndrome (SDS). SND produces mainly or only parkinsonism, and principally
causes trouble in the form of difficulty starting or slowness in performing
voluntary movements. This form of MSA is often
only distinguished from PD by the lack of benefit that levodopa brings.

The brain areas most affected by SND are the substantia nigra and it’s
target center, hence the close resmblance of SND to PD. In SDS, there is
parkinsonism like SND along with dysfunction of the autonomic nervous
system, which governs the nervous systemís operations not
normally under conscious control such as regulation of blood pressure, heart
rate, bladder function, intestinal function, sexual function, and sweating.
Problems stemming from this aspect of MSA include lightheadedness (due to
low blood pressure), urinary retention,
incontinence and impotence. Autonomic symptoms often help to distinguish
people with MSA from those with PD.

Although it is never easy to discuss personal bodily functions, problems of
this type are vital clues to a correct diagnosis. Degeneration of the
cerebellum and
brainstem due to OPCA leads to a form of incoordination known as ataxia, as
well as speech and eye movement disturbances. Any form of MSA can also cause
spasticity.

These three variations of MSA can occur in any combination. MSA tends to
start slightly younger than PD. Of the three major imitators, it is usually
the most difficult to distinguish from PD because people with
MSA mimic PD most closely, and many show at least partial improvement from
levodopa. This gives a false sense of security that patients do have PD,
since we know that almost nothing else responds consistently to
levodopa. Our ability to diagnose MSA may turn out to be the most common of
these 3 PD imitators.

The first symposium devoted exclusively to MSA took place in London,
England, in March 1997. Unfortunately, there is no national association for
MSA patients like there is for PSP, however, there is a support
group for people with Shy-Drager syndrome, with an emphasis on dealing with
the autonomic disturbances caused by MSA. They can be reached at
the following address and phone number:

Shy-Drager Syndrome Support Group
1607 Silver Ave. S.E.
Albuquerque, NM 87106
Tel: 800-SDS-4999


CORTICAL-BASAL GANGLIONIC DEGENERATION

This disease, known as corticobasal degeneration in Europe, was first
described in Boston in 1968. No further cases were reported until 1985, but
since that time cortical-basal ganglionic degeneration (CBGD) has
been recognized as another, though less common, imitator of PD. Like PSP, it
tends to occur in a slightly older population than PD. The name derives from
the fact that both the cerebral cortex (outer gray matter) and the basal
ganglia are involved in this disease.

The term ‘basal ganglia’ refers to clusters of nerve cells deep in the brain
that are components of the motor system. One of the basal ganglia is the
substantia nigra, the part of the brain that degenerates in PD. It is also
involved in CBGD, so patients have some symptoms and signs similar to those
with PD. However, the degeneration of additional parts of the brain causes
problems not usually encountered in PD, such as abnormal
limb postures (dystonia), loss of sensation of position or movement, arm or
leg jerking (myoclonus), and a peculiar disorder of voluntary movement
(apraxia) in which people’s ability to transform thoughts into
coordinated movements is impaired. Another striking manifestation of CBGD is
the alien limb phenomenon, where a person’s limb makes complex movements
without their knowledge or despite attempts to refrain from
doing so. CBGD shares with PD the tendency to involve just one side of the
body at the beginning, and remain worse on that side.

Because of a late start, comparatively less is known about CBGD than some
other PD imitators. However, we are starting to catch up. A symposium
devoted exclusively to CBGD was held in Washington, DC in October 1995.
There is no foundation yet established to serve the needs
of patients and families with CBGD.


CONCLUSION

People with a diagnosis of PD often come to see a movement disorders
specialist because they are not doing well. One of the most common reasons
is that they actually don’t have PD. Medicine is rather late in
coming to recognize and distinguish the principal imitators of PD. In part,
this is because of the nature of the nervous system.

Understanding of neruologic diseases often lags behind developments in other
medical fields because the brain is more complex than other organs, and we
have less access to it. For example, biopsy of the brain areas involved in
these diseases is usually out of the question because it is simply too
risky. Since there are no other tests available, we can’t be entirely
certain which of these diseases someone had until we
examine their brain after they die. Most of the major PD imitators can be
identified by proper autopsy studies, but during life, there is no
laboratory test that can conclusively distinguish between them.

What can you do?

Our most pressing need is simply more knowledge. Doctors don’t learn
anything new about the symptoms and other effects of a disease unless those
who live with it share their experiences with their physicians.

If you have any questions or make any new observations about your illness,
write them down so you will be sure to mention them at your next visit.
Learn as much as you can about your condition and stay informed.


What does the future hold?

Coping with PD imitators is often difficult. One of the drawbacks to our
relatively recent recognition of these diseases is the lack of knowledge
about how to treat them effectively. Consequently, PD imitators usually lead
to a disabled state more rapidly than PD itself,
where treatment can maintain a normal lifestyle for many years. Fatal
complications are rare in PD, but more frequent with it’s imitators.

However, the outlook is not entirely bleak. Many of the symptoms of PD
imitators can be treated individually, and various forms of supportive care
often help where medication is lacking. It is important to involve
physical therapists, occupational therapists, speech therapists and often
dietitians as soon as warranted.

There is clearly a great need for more research in this area. The good news
is that there is currently a tremendous amount of interest in PD imitators
among neurologists. It is hoped that this attention and
research activity will lead to meaningful improvements in our ability to
more accurately identify and help people with PD imitators in the near
future.

Acknowledgement:

I am indebted to Irene Litvan, M.D., Niall Quinn, M.D. and Steven Reich,

M.D. for their helpful comments.





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