Deborah ger

Greetings from Kernow (Cornwall UK) and many thanks for this exceedingly
valuable information.

lowena dhis  (joy to you)

Julie

----- Original Message -----
From: "Deborah Setzer" <[log in to unmask]>
To: <[log in to unmask]>
Sent: Tuesday, May 14, 2002 1:40 PM
Subject: Re: Degrees of PD ?


> 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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