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What is Atypical Parkinsonism?
MARCH 2016
By Joohi Jimenez-Shahed, MD
INSIDE THIS ISSUE:
Article: What is Atypical
Parkinsonism? continued
2-3
Griswold Cares
Foundation
3
Announcements
3
Marathon Wrap Up
4-5
HAPS Exercise & Support
Group Schedule
Insert
Article: Potential New
Drug Shows Promise for
Neuroprotection
6
Now Enrolling—PEP
6
Pancakes for Parkinson’s
6
Contributions & Tributes
7
Please check the
insert for changes
to HAPS groups
beginning this
month!
STAY CONNECTED...
Generally speaking, the term “atypical
parkinsonism” refers to a group of
conditions in which some signs or
symptoms that are associated with regular,
garden-variety Parkinson’s disease are
present, but in which there are also
features that are unusual for this
condition. It is these unusual features that
tend to predominate and create difficulties
with how patients respond to routine
treatments for Parkinson’s disease (PD).
Before digging deeper into “atypical
parkinsonism,” let’s review “typical” PD.
There are four major features of
“parkinsonism.” The term “parkinsonism”
is used to refer to a collection of
symptoms—from there we can consider
whether or not a person has PD or
something else. Parkinson’s disease is the
most common reason for parkinsonism.
Features of parkinsonism include tremor
(typically a resting tremor), rigidity
(stiffness of the muscles that occurs in a
specific pattern), bradykinesia (slowness
and irregularity of movements), and
postural instability (loss of balance on the
“pull test”). In the clinic, when we are
examining patients, we look for at least
two of these four symptoms in order to say
that parkinsonism is present. However, we
do not diagnose an individual with PD only
on the basis of these features. We often
like to know whether or not the symptoms
started on one side, how quickly they have
been progressing, whether they respond to
typical Parkinson’s medications, and
whether any “red flags” are present that
would indicate a
diagnosis
of
something other
than regular PD.
What are some
of the “red flags?”
The first of these “red flags” is the
presence of significant cognitive problems
that start before or soon after the onset of
any parkinsonism. This may include issues
with thinking, memory, orientation in time
or space, language, processing speed or
decision making. We are also particularly
concerned with the presence of any
hallucinations either before treatment
with Parkinson’s medications or very
prominent ones soon after starting
treatment. If these types of symptoms
exist, a person may actually be suffering
from Lewy body dementia rather than
regular PD. Some may ask why this
distinction is important. Patients with PD
may also experience cognitive difficulties,
but this is more likely a late complication
of the disorder. That means it is more likely
to occur several years after the onset of
parkinsonism. Patients with Lewy body
dementia tend to have a harder time
tolerating the medications that are used to
treat PD, due to a greater risk of
developing hallucinations. As a result, it
becomes difficult to balance the
medications that are needed to treat the
movement problems with medications that
are needed to treat the side effects.
Dementia medications and antipsychotics
Continued on page 2
are often necessary. On the other hand, some individuals with
Lewy body dementia may have more cognitive issues than
parkinsonism, and it is more imperative to address those
issues than focusing on improving problems with movement.
Another “red flag” that we want to look for is the early
presence of problems with balance and falling. Generally
speaking, patients with regular PD will start experiencing
these issues after they have developed obvious tremor,
rigidity or bradykinesia. If an individual reports that their
Parkinson’s journey began with significant problems with
walking, balance or falls, we would become concerned about
a condition called progressive supranuclear palsy (PSP). In
order to diagnose somebody with PSP, the patient should
have evidence of abnormalities with the way their eyes move.
This is sometimes very subtle, and patients may not even be
aware that these problems exist. Some complaints that
patients may experience include difficulty reading from line to
line, difficulty looking down when bringing food to their
mouth, or trouble turning just their eyes to look in different
directions. However, a good neurologic examination should
be able to identify these problems even if they are early and
subtle. If present in conjunction with a history of early falls
and parkinsonism, these findings would be enough to
diagnose a person with PSP. The importance of distinguishing
this condition is that patients with PSP generally do not
respond very well to levodopa, despite the fact that they can
have very significant gait and balance problems. This can
become very frustrating for patients and their caregivers
because patients are at high risk for injuries. In addition,
individuals with PSP may be more prone to mood changes,
certain cognitive problems, and swallowing problems. In
general, the progression of this condition is very different
from what would be expected with typical PD, and patients
can become disabled much more quickly.
The third “red flag” that is often assessed in the clinic is the
presence of significant problems with certain automatic body
functions. There is a dedicated part of everyone’s nervous
system that controls things like blood pressure, bladder
function, bowel movements, sweating, and sexual function.
Collectively, this is termed the “autonomic nervous system.”
Patients who have problems with their autonomic nervous
system are said to have “dysautonomia.” Patients with PD are
likely to have some mild issues in any or all of these areas.
Although they may progress in severity as the PD progresses,
they often won’t become a major part of the management
strategy until later. If an individual experiences very obvious
problems with bladder control (e.g., having full-blown
incontinence), or with low blood pressure after standing to
the point of nearly or actually passing out (something called
“orthostatic hypotension”) early in the course of their
parkinsonism, we become concerned about a different
2 | HOUSTON AREA PARKINSON SOCIETY
condition called multiple systems atrophy (MSA). MSA is
important to recognize because in some cases, it can really
look very similar to regular PD, and patients may react very
similarly to Parkinson’s medications. In other cases, patients
may have or develop major problems with balance, called
“ataxia.” In either case, the difficulty that patients with MSA
experience with their blood pressure or bladder can very
significantly impact their quality of life, beyond any problems
they may have with mobility. Sometimes these symptoms
become the focus of management rather than the movement
problems. Similar to PSP, disability in MSA can progress much
more rapidly and differently than in regular PD, and the
mobility problems may become less responsive to levodopa
over time. Patients with MSA may have additional problems
with their breathing, their voice, or their swallowing that may
require additional measures for treatment.
The fourth “red flag” is the presence of something called
“apraxia.” This term refers to a problem with the messages
from the brain to initiate a movement getting disrupted. The
person knows what they want to do, but cannot get their limb
or other body part to do it. For some people with regular PD,
it may seem that they have this problem, because it is hard
get their limb to react as quickly as they would like. However
with true apraxia, the signals do not get there, and it is either
very effortful or sometimes impossible for patients to execute
a command to that limb. As a result, it may look like patients
are “ignoring” their arm, or that they stop using it as much as
the other side. When apraxia involves the legs, patients have
difficulty initiating gait, often worse on one side compared to
the other. Although this may seem similar to gait freezing, it is
not overcome with tricks or visual cues, like freezing can be.
Patients with apraxia in combination with their parkinsonism
most likely have a condition called corticobasal degeneration
(CBD). CBD is also important to recognize because once again,
patients do not have that robust response to levodopa
compared to patients with regular PD. Their symptoms
progress much more rapidly, and they may develop particular
problems with stiffness or spasticity of the affected side that
are very different from the rigidity of parkinsonism. In
addition, patients may develop mood issues, language
dysfunction or cognitive problems that further contribute to
disability.
The last “red flag” is a known history of exposure to
medications that can block the effects of dopamine. These are
often medications that are used to treat psychiatric
symptoms, such as antipsychotics, or used to treat certain
gastrointestinal disturbances such as nausea or reflux. When
people are exposed to these types of medications, they may
develop parkinsonism while receiving the medication. If the
offending medication is stopped, it may take several months
for the side effects to resolve. In this case, a person is said to
have “drug-induced parkinsonism.” Anybody who has
experienced persistent parkinsonism beyond six months of
stopping a medication like this should be checked for
consideration of underlying PD.
In reality, there are several other things that we do consider
beyond the “red flags” mentioned above, which, if present,
may point to other conditions that mimic PD. Some of the
conditions mentioned here, and others, may also have telltale
signs that can be seen on brain imaging tests, such as an MRI.
It is always important to inform your treating physician about
all of the symptoms that you are experiencing or request an
evaluation with a specialist. Features of particular concern
include a rapid progression, or insufficient response to
adequate doses of levodopa.
Although the possibility of “atypical parkinsonism” can be a
scary idea for patients who are previously diagnosed with
Parkinson’s disease, it is important to remember that these
Griswold Cares and
Gives Back
conditions are much less common than PD itself. Also,
patients with regular PD frequently have mild features of
these other conditions. It is a matter of degree and course of
time to decide if there is enough to be concerned about
atypical parkinsonism. A careful re-examination of the history,
progression, non-movement symptoms, imaging, and
response to medication should be able to provide
reassurance about whether or not atypical parkinsonism is
present.
Joohi Jimenez-Shahed, MD is Assistant Professor of Neurology,
Associate Director Neurology Residency Program and Director
Deep Brain Stimulation Program Baylor College of Medicine in
Houston, Texas and a member of the Parkinson's Disease Center
and Movement Disorders Clinic. She is board certified by the
American Board of Neurology and Psychiatry and specializes in the
care of patients with movement disorders. Dr. Jimenez-Shahed is
the recipient of numerous honors and awards. She is the author of
many publications and is an active member of the HAPS Medical
Advisory Board.
Houston Area Parkinson Society recently received a generous
contribution from The Griswold Cares Foundation, a private charity
established by Griswold Home Care to further the company’s belief that
care for the elderly and disabled should be available to all. The
Foundation was created in 2010 to further the company's collective
mission and values and to give back to the communities that they serve.
HAPS was one of the nonprofit organizations chosen by The Griswold
Cares Foundation to receive funding to build, strengthen and sustain
programs that enhance the quality of life for seniors and adults with
disabilities who choose to age in place, including those with limited
financial means.
Brenda Gross, Madison Garrison and Jim Vonderhaar of
the Griswold Home Care Houston area offices present
HAPS executive director Anne Thobae with a certificate
in recognition of its shared mission.
The involvement of area businesses contributing to an improved quality
of life in our community is invaluable to us all. We applaud Griswold
Home Care and the company’s Foundation for this partnership and its
outstanding support of HAPS efforts.
Several months ago, HAPS announced that the 2016 Annual Educational
Symposium would be held on Saturday, May 21, 2016. However, HAPS will
take a break from presenting this educational program this year and will focus
on other educational programs during the summer and fall months. The HAPS
Annual Educational Symposium will return in the spring of 2017 as we
recognize the 200th year since Parkinson's was first described.
Thank
You!
Many thanks to HAPS Medical Advisory Board member Mya Schiess, MD, Assistant
Professor
of
Neurology,
University
of
Texas
Movement
Disorders
&
Neurodegenerative Disease Program who led the February 13 th presentation of the
Newly Diagnosed Education Program. We appreciate your time and expertise.
MARCH 2016 HAPS HAPPENINGS | 3
Superheroes do it again...
This year’s Chevron Houston Marathon HAPS
Superhero Squad raised an impressive $111,200
and increased Parkinson’s awareness with a massive
group of nearly 300 runners and walkers
participating in race weekend. We thank our fearless
leaders Gary Brentlinger and Leon Keeble who
assembled an amazing group of heroes who joined
forces to make this a successful and fun event.
HAPS would also like to thank all of the walkers,
runners, fundraisers, volunteers, spectators and the nearly 800 donors (listed
below) who participated in the Marathon and helped make this a “super” year!
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4 | HOUSTON AREA PARKINSON SOCIETY
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Krista Villarreal
Barbara Virany
MARCH 2016 HAPS HAPPENINGS | 5
Potential New Drug Shows Promise for Neuroprotection
In tests on a mouse model of Parkinson’s disease (PD),
an experimental drug in the early stages of development
prevented damage to dopamine neurons by protecting
them from inflammation. The results appear in the
December 16 edition of the Journal of Neuroscience.
The immune system normally defends a person’s body
from infection and injury. But sometimes it unnecessarily
goes on high alert, which leads to inflammation. In PD,
inflammation may speed up the death of dopamine
neurons in the brain — the cells that help control the
body’s movements. Specifically, inflammation happens
when immune cells in the brain (microglia) become
activated, and other immune cells (T cells) attack
dopamine neurons.
To try to treat inflammation in PD, researchers at the
University of Nebraska Medical Center in Omaha,
NE, took advantage of a naturally occurring antiinflammatory molecule called vasoactive intestinal
peptide (VIP). They knew that VIP could not be used as
a PD therapy because it activates both good and bad
inflammatory responses and because it leaves the body
too quickly to be therapeutic. So they created molecules
similar to VIP but with improvements, and tested them in
mice with PD-like brain changes and symptoms.
Results



Of the molecules tested, one (LBT-3627) protected
up to 80 percent of dopamine neurons in the brains
of mice with PD-like brain changes. It also lasted
much longer than VIP in the body before being
broken down.
The drug LBT-3627 also decreased inflammation
caused by immune cells.
The same molecule LBT-3627 seemed to work by
targeting a receptor on T cells known as VPAC2, and
by binding to this receptor, it changed the behavior of
the T cells from inflammatory to protective.
What Does It Mean?
Therapies that provide neuroprotection — the ability to
slow or stop the loss of dopamine neurons in the brain,
which underlies PD are urgently needed. Recently, the
role of inflammation in PD has drawn interest in the
scientific community. If indeed there is an “exaggerated
response” of the immune system in PD (meaning it
overreacts), then therapies which modify the response
may help slow down PD. The results of this early-stage
study with laboratory mice suggest that LBT-3627, being
developed by Longevity Biotech, Inc., may hold promise
for providing neuroprotection by fighting inflammation in
the brain.
Continued on page 7
HAPS is currently enrolling individuals for the
upcoming Parkinson’s Enrichment Program (PEP)
session which begins in April!
This fun, five-week enrichment program is offered
quarterly to individuals with mild to moderate
Parkinson’s disease. The four-hour program is
presented at Memorial Drive Lutheran Church each
Friday for five consecutive weeks and has
components of exercise; recreation; socialization;
education; peer-led discussion; professionally
facilitated support; and lunchtime conversations.
Previous sessions have included virtual travel, art,
dance, photography and educational lectures.
Space is limited; registration is required.
Contact Celeste Harris, LMSW at
713-313-1706 or [email protected] to
register or for information about the
2016 PEP schedule.
6 | HOUSTON AREA PARKINSON SOCIETY
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However, questions remain. The role of the immune
system in PD is not very well understood. The role of
VIP, the molecule the novel drug imitates, is also not
completely known. Whether this molecule LBT3627 would be helpful in treating people with PD is
unknown. For instance, will it be able to cross the blood
brain barrier and allow for delivery as an injection or will
surgery need to be performed to allow direct delivery to
the brain? If further laboratory studies achieve similarly
successful results and are able to answer other
questions, a phase 1 clinical trial—a test for safety in a
small number of study participants—could launch as
soon as 2017.
Reference: Olson KE, Kosloski-Bilek LM, Anderson KM, Diggs
BJ, Clark BE, Gledhill Jr. JM, Shandler SJ, Mosley RL,
Gendelman HE. (2015). Selective VIP Receptor Agonists
Facilitate
Immune
Transformation
for
Dopaminergic
Neuroprotection
in
MPTP-Intoxicated
Mice.
J
Neuroscience 35(50): 16463-16478.
This article was originally published as part of "Parkinson's
Science News: What Does it Mean?" on the Parkinson’s
Disease Foundation (PDF) website on February 1, 2016. It
is reprinted, in its entirety, with permission from PDF. For other
science news, please visit www.pdf.org/science_news.
MARCH 2016 HAPS HAPPENINGS | 7
Board of Directors
Board of Advisors
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Mike Hendryx - Chair
Chris Brewster - Vice President/Treasurer
Jo Furr - Vice President/Secretary
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Ron Bernell
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Aubrey Calvin
Joiner Cartwright, Jr., PhD
Bob Casey, Jr.
Rich Clifford
Robert Cruikshank
Meredith Cullen
Joe Ahmad
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Medical Advisory Board
Richard K. Simpson, Jr., MD, PhD - Chair
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