Gastrointestinal and Urinary Dysfunction

This post is of interest to those dealing with gastrointestinal problems, urinary dysfunction, problems with saliva, dysphagia, etc.

This article on gastrointestinal and urinary dysfunction in PD was published today in PDF News.  PDF = Parkinson’s Disease Foundation.  The author is Dr. Ron Pfeiffer, the same neurologist who spoke on non-motor symptoms at last year’s PD Symposium in the Bay Area.

Here’s a  link to the article online:

Gastrointestinal and Urinary Dysfunction in PD
By Dr. Ronald Pfeiffer
PDF News, Spring 2007

I’ve copied a few excerpts below on dysphagia and stomach problems.



Excerpts from:

Gastrointestinal and Urinary Dysfunction in PD
By Dr. Ronald Pfeiffer
PDF News, Spring 2007

Difficulty swallowing, or dysphagia, is a very common problem in Parkinson’s.  At least 50 percent (some studies even suggest over 80 percent) of people with PD experience difficulty in swallowing, and an even greater percentage show abnormalities on x-ray tests of swallowing.

Difficulty swallowing is usually due to the lack of coordination among the many muscles in the mouth and throat that must work together in perfect precision to produce normal swallowing.  When food gets stuck in the mouth, the person may have to try several times to complete a swallow.  The muscles in the back of the throat — and in the esophagus — may also lose coordination, and individuals who have difficulty swallowing are at increased risk for food or liquid to get into the windpipe.  From there, it can get into the lungs (called aspiration), which can result in pneumonia.

Although treatment of dysphagia can be difficult, speech/swallowing therapists can instruct patients on swallowing techniques and on designing changes in food consistency that reduce the risk of aspiration.  Some improvement in coordination of the muscles used in swallowing may be achieved through adjustments in PD medications.  Only very rarely is it necessary to place a feeding tube.

Stomach problems
Impaired ability to empty the contents of the stomach, called gastroparesis, is another potential gastrointestinal complication of PD.  This may produce a bloated sensation and cause people to feel full even though they have eaten very little.  Sometimes nausea may develop.

Failure of the stomach to empty in a timely fashion may also impair or delay the effectiveness of PD medications, especially levodopa, since levodopa is absorbed from the small intestine and cannot get to its destination if it is trapped in the stomach.
Treatment of gastroparesis in Parkinson’s has not been extensively studied.  Domperidone is an effective medication, but unfortunately it is not available in the US.

Treatment routes that bypass the stomach, such as transdermal drug delivery by skin patch, may become available in the near future.  Another potential treatment under investigation involves a form of levodopa designed to be delivered directly into the small intestine via a feeding tube.

Progression of dysarthria + dysphagia in DLB, CBD, MSA, and PSP

This will be of interest to anyone dealing with dysarthria (speech problems) and dysphagia (swallowing problems), which, according to the article should be everyone within one year of disease onset.  (I thought dysarthria meant slurred speech.  But, according to this article, it can also mean hypophonic speech or monotonic speech.)

This article is about dysarthria and dysphagia in autopsy-confirmed cases of DLB, CBD, MSA and PSP, all APDs (Atypical Parkinsonian Disorders).  PD cases are part of the study as well.

Here’s the citation and abstract:

Archives of Neurology. 2001 Feb;58(2):259-64.  

Progression of dysarthria and dysphagia in postmortem-confirmed parkinsonian disorders.

Muller J, Wenning GK, Verny M, McKee A, Chaudhuri KR, Jellinger K, Poewe W, Litvan I.

BACKGROUND: Dysarthria and dysphagia are known to occur in parkinsonian syndromes such as Parkinson disease (PD), dementia with Lewy bodies (DLB), corticobasal degeneration (CBD), multiple system atrophy (MSA), and progressive supranuclear palsy (PSP). Differences in the evolution of these symptoms have not been studied systematically in postmortem-confirmed cases.

OBJECTIVE: To study differences in the evolution of dysarthria and dysphagia in postmortem-confirmed parkinsonian disorders.

PATIENTS AND METHODS: Eighty-three pathologically confirmed cases (PD, n = 17; MSA, n = 15; DLB, n = 14; PSP, n = 24; and CBD, n = 13) formed the basis for a multicenter clinicopathological study organized by the National Institute of Neurological Disorders and Stroke, Bethesda, Md. Cases with enough clinicopathological documentation for the purpose of the study were selected from research and neuropathological files of 7 medical centers in 4 countries (Austria, France, England, and the United States).

RESULTS: Median dysarthria latencies were short in PSP and MSA (24 months each), intermediate in CBD and DLB (40 and 42 months), and long in PD (84 months). Median dysphagia latencies were intermediate in PSP (42 months), DLB (43 months), CBD (64 months), and MSA (67 months), and long in PD (130 months). Dysarthria or dysphagia within 1 year of disease onset was a distinguishing feature for atypical parkinsonian disorders (APDs) (specificity, 100%) but failed to further distinguish among the APDs. Survival time after onset of a complaint of dysphagia was similar in PD, MSA, and PSP (15 to 24 months, P =.7) and latency to a complaint of dysphagia was highly correlated with total survival time (rho = 0.88; P<.001) in all disorders.

CONCLUSIONS: Latency to onset of dysarthria and dysphagia clearly differentiated PD from the APDs, but did not help distinguish different APDs. Survival after onset of dysphagia was similarly poor among all parkinsonian disorders. Evaluation and adequate treatment of patients with PD who complain of dysphagia might prevent or delay complications such as aspiration pneumonia, which in turn may improve quality of life and increase survival time.

PubMed ID#: 11176964  (see for this abstract)


These results were the most interesting (and depressing):

“Dysarthria or dysphagia within 1 year of disease onset was a distinguishing feature for atypical parkinsonian disorders (APDs).”

“Median dysarthria latencies were short in PSP and MSA (24 months each), intermediate in CBD and DLB (40 and 42 months), and long in PD (84 months).”

“Median dysphagia latencies were intermediate in PSP (42 months), DLB (43 months), CBD (64 months), and MSA (67 months), and long in PD (130 months).”

“Survival time after onset of a complaint of dysphagia was similar in PD, MSA, and PSP (15 to 24 months).”

On this last point, here’s the actual chart on this from the article:

Survival Time After Onset of Dysphagia, months
PD        24 (2-61)
CBD     49 (25-89…..including a single patient with dysphagia but without dysarthria)
DLB     10 (3-17)
MSA     15 (6-68)
PSP       18 (6-96)

Based on this, I don’t understand why DLB’s short survival time isn’t highlighted.

Here are some excerpts from the article’s Comment section:

“(Early) dysarthria and perceived swallowing dysfunction are not features of PD.”

“[Dysarthria] as a presenting symptom has been described in clinical series of CBD, MSA, and PSP.  In PD and DLB, hypphonic/monotonous speech represented the most frequent type of dysarthria, whereas imprecise or slurred articulation predominated in CBD, MSA, and PSP.”

“In a clinical study of CBD, Rinne et al described dysarthria as one of the initial symptoms in 11% of the patients, which is close to our findings.  At follow-up, on average 5.2 years, dysarthria was diagnosed in 70% of the patients…  According to our findings, dysarthria occurred in almost every patient with CBD.”

“In agreement with our results, Quinn described the speech of patients with MSA as more severely affected than that of patients with PD, with slurring dysarthria, as well as the low volume and monotone of parkinsonism.”

“In both PSP and MSA, progressive dysarthria is believed to represent a manifestation of brainstem and cerebellar involvement.  In fact, PET studies revealed marked hypometabolism in the cerebellum and brainstem of patients with MSA, which correlated with dysarthria.”

“In our study, dysphagia was associated with concomitant dysarthria in all parkinsonian patients except one.  This sequence of dysphagia following dysarthria has also been reported in clinical studies of PD, MSA, and PSP.”

“…Golbe et al reported dysphagia after a median of 1 year after the onset of dysarthria in PSP.”

In all these disorders, “bronchopneumonia has been reported as a leading cause of death, which may be subsequent to silent aspiration resulting from dysphagia.”

“Most of our patients with MSA and PSP complained of a swallowing dysfunction, in contrast to patients with PD, CBD, and DLB…  Impaired lingual proprioception is hypothesized to contribute to the unawareness of swallowing difficulties in PD and might in part explain significantly longer latencies to dysphagia in our PD cases.  In contrast, patients with PSP were reported to be keenly aware of swallowing problems, including those with cognitive impairment.”

“(The) similarly short remaining survival time in PD and PSP after the onset of perceived dysphagia suggests that this symptom represents a reliable marker for the onset of functionally relevant swallowing abnormalities in both disorders.”

“Our findings of increased latency to dysarthria and dysphagia and similar time interval from onset of dysphagia to death in patients with PD compared with patients with APDs suggest that extrastriatal and nondopaminergic lesions represent an important factor for the development of dysarthria and dysphagia.  Indeed, Bonnet et al reported that dysarthria, gait, and postural stability had a decreased levodopa response in patients with long-standing PD who still benefited from the levodopa effects on tremor, rigidity, and akinesia.  Whereas the APDs are characterized by multiple system neuronal degenerations, in PD disease progression is determined by a progressive dopaminergic deficit arising from the selective neuronal degeneration of the substantia nigra pars compacta.”


(Full) Transcript from 3/28/07 LBDA Online Chat w/Mass Genl Neurologist

Here’s the transcript from the LBDA’s live online chat with Stephen Gomperts, MD, PhD.  He’s a dementia specialist at Mass General.



Live Chat Event
with Dr. Stephen Gomperts, MD
March 28, 2007
Hosted by Lewy Body Dementia Association (

Victoria Ruff (Moderator)
Welcome to this evening’s LBDA online chat event. Our guest is Stephen N.
Gomperts, MD, PhD. He is the Assistant in Neurology at the Memory and
Movement Disorder Unit (MMDU) at Massachusetts General Hospital and
Research Fellow at Picower Institute for Learning & Memory at Massachusetts
Institute of Technology.

The MMDU is an integral part of the Massachusetts Alzheimer’s Disease
Research Center and is a part of Udall Parkinson’s Disease Research Center for
Excellence. Dr. Gomperts’ clinical and clinical research focus is on the cognitive
and behavioral problems that arise in neurodegenerative disorders, with
particular emphasis on dementia with Lewy bodies and Parkinson’s Disease. His
basic science focus is on the role of the dopamine system in learning and

Dr. Gomperts
Thank you for the opportunity to meet with you. I have to preface my response
to these questions by saying that without actually evaluating your loved ones and
speaking with each of you in detail, i.e. without knowing much more than we can
cover in a chat session, I cannot provide specific medical advice for a given
individual patient. I will therefore attempt to answer your questions in generic

I also need to preface by stating that I have no personal benefit in any of the
recommendations that I make below (e.g. medications), and my recommendations
are only recommendations.

Victoria Ruff (Moderator)
As a reminder, the questions that were emailed to the Victoria Ruff, Moderator
will be posed first. Should time permit, the board will be open for additional
questions to Dr. Gomperts.

Please note that some questions that were emailed can’t be answered completely
since Dr. Gomperts doesn’t know your loved one’s history, examination and

Another reminder: The information presented during the Online Chat is
for informational and educational purposes only and is not intended to
substitute the professional medical advice or treatment
recommendations provided by your loved one’s doctor.

Without further ado, let’s get to the questions.

1. Question from Norma: A person who has been off of Reminyl and Namenda
for 4 months and who, in the last 2 months has declined significantly (loss of
weight, ability to walk or stand, and whose cognition has changed) could Exelon
or Aricept help a little now? She’s 84 years old.

Dr. Gomperts
Again, without evaluating your loved one to exclude reversible causes of decline
(including, for example, electrolyte imbalance, thyroid disorder, medication side
effects, etc), I cannot comprehensively speak to your particular problem. So I will
speak generally. Unfortunately, as I believe you may know, we presently have no
medicine that treats the underlying cause of dementia with Lewy bodies (DLB) or
Parkinson Disease dementia (PDD) (I will refer to both together as Lewy body
dementia (LBD) from here on). Instead, we have in our arsenal medicines that
treat the symptoms of the disease. But the disease progresses relentlessly while
we treat the symptoms. Many medical scientists are working on this problem,
with a goal of stabilizing or even reversing the disease, but we’re not there yet.

Many patients respond (to varying degree) to acetycholine esterase inhibitors
(the class of medicines that include Reminyl, Exelon, and Aricept). While some
patients can have a remarkably robust response, it can be subtle at best in other
patients. Although there are fewer studies out there on Namenda (memantine),
similar variability of response across people is likely to be true as well.

Because patients with DLB tend to get worse over time, these medications may
only lead to a temporary stabilization in symptoms (or even a decline in the rate
of progression). But that’s still a worthy goal.

The lack of a robust response is one reason why these medications are
discontinued. Another would be a side effect. Without knowing why Reminyl and
Namenda were discontinued for your family member, it’s a little hard for me to
answer your question.

Broadly speaking, yes, it would be reasonable to try other agents. These agents
are generally well tolerated, and if one causes a side effect, there is still a
reasonable chance that another will not.

Victoria Ruff (Moderator)
2. Question from Sue: My spouse becomes anxious when left alone in a room. He
knows where I am yet, he will shadow me around the house or constantly call for
me to come back into his sight or escalate his agitation to draw me off task and
back to him. I’ve given him Klonopin – 0.5 or 1.0 mg but it seems to increase
confusion. How can I decrease his anxiety when I need to move about the house?

Question from Phyllis: Are there any safe medications for anxiety or irritability
for LBD patients since they seem to be so sensitive to medications in general?

Dr. Gomperts
Medicines like Klonopin (the benzodiazepine class) often cause confusion in the
elderly as well as in patients with dementia. In my opinion, they are better left out
of a medication regimen for a patient with LBD. Many patients with LBD are
exquisitely sensitive to medications. That makes medical treatment a real

On the whole, I advocate for minimizing and streamlining the medication regimen;
stripping away every unnecessary agent. That reduces the probability of
medication side effects and medication interactions.

When a medication is required, my recommendation is to start low (dose) and
go slow (titrate up slowly as necessary). Non-medication approaches are certainly
worthwhile, and include reassurance and occupying him with something to do
while you leave the room.

Victoria Ruff (Moderator)
3. Question from Donna: What should caregivers do when loved ones won’t

Question from Bobbi: Are any meds being investigated to alleviate sleeplessness
in LBD patients?

Question from Marcia: SLEEP how do you get them to sleep, so that we
caregivers can get some sleep, too.

Dr. Gomperts
Let me finish a quick thought about anxiety.

As I stated above, I try to avoid prescribing medications to DLB patients unless
absolutely necessary. When necessary, however, there are some agents that can
help. One group that should not be overlooked is antidepressants (like the SSRIs
or NSRIs). Each is a little different, and some, like Effexor, seem to have some
anti-anxiety properties.

Antipsychotics like Clozaril (clozapine) or Seroquel (quetiapine) can also help, but
I would reserve their use for severe cases.

As you may know, there’s currently a hot debate about the safety of that class of
agents, so to use one of them in a given patient’s circumstances, their potential
benefits have to outweigh their risks.

Another good (and hard) question. Sleep problems are common in LBD. Patients
often sleep during the day (many for more than 2 hours total), and that can lead
to nighttime wakefulness. This can even lead to full-blown sleep-wake reversal.

This can be hard to treat. One thing to do is to try to minimize daytime naps, as
possible, to help the patient return to a nighttime sleep pattern. It is also
important to have your loved one’s doctor review his medications, to check
whether some of his night-time dosed medications may in fact be activating him,
and if some of his morning/day time medications may be contributing to daytime
somnolence. If that’s the case, it may be possible to move the activating
medications to morning, and the sleep-enhancing medications to night.

Another approach would be to substitute in an otherwise equivalent medication
for another to provide more morning activation or more nighttime sleepiness.
Sleep medications like benzodiazepines can cause confusion, and should be

An antidepressant that provides a little sleepiness, however, may be worthwhile.

Victoria Ruff (Moderator)
4. Question from Sue: My husband quickly becomes agitated then displays
generalized anger when ever I get a phone call lasting more than a minute or two.
What is causing this? A need to control the caregiver?

Question from Bobbi: Are any meds being investigated to alleviate agitation in
LBD patients?

Question from Phyllis: Do LBD patients usually get as aggressive as the disease

Question from Marcia: AGITATION and/or AGGRESSION: What’s the best
medication to control this?

Dr. Gomperts
The systems that hold emotions in check and inhibit us from “behaving badly” can
progressively deteriorate in LBD. As a result, some but not all patients develop
progressive agitation or aggression as the disease proceeds.

It is not clear why Sue’s husband is acting the way he is. There are many
possibilities. As you know him well (and I do not know him at all), your
interpretation may well be better than mine.

Here are a few of the many possibilities: Certainly it could be a demand for your
undivided attention.

Perhaps your speech or interactions provide him with something that he likes.
Perhaps you occupy him, and when unoccupied, he transitions into agitation.

It is worth keeping in mind that an unusual amount of agitation for a patient with
DLB (i.e., a change from baseline) may reflect a problem (like pain or discomfort)
that the patient is unable to articulate.

Once those problems have been ruled out (or treated), and we are left with the
stereotyped agitation that can arise in LBD, again I think it is better to try nonmedication
based treatments first. If severe, however, the antipsychotics Seroquel
and clozapine are useful and can often help with agitation.

As I talked about above, their use has been implicated in shortening life span; it
was a small effect in one study (not seen in several other evaluations), and is a
hot topic of debate at the moment.

We do not know for certain that these medications are harmful in the long run,
but they may be. However, at some point agitation and aggression can get severe
enough to warrant their use.

That is a decision for you, your doctor, and your loved one to make. I should also
add that clozapine requires weekly blood tests to ensure against a serious but
very rare possible side effect called agranulocytosis.

Victoria Ruff (Moderator)
5. Question from Sue: In periods of delusional activity, is it best to orient the
patient back to reality?

Question from Donna: Is there a way to stop the hallucinations?

Question from Imogene: Does a person’s TV habits (i.e.; violent or love story
movies) have any bearing on the type of hallucination they may have?

Question from Marcia: HALLUCINATIONS: Again, what is the best medication,
to date, to control the hallucinations?

Dr. Gomperts
Some medications for the parkinsonism (tremor, rigidity, motor slowness,
impaired balance) of LBD can cause hallucinations. It would therefore be
important to minimize all medications that can cause hallucinations (while still
optimizing motor treatment).

Infections or electrolyte imbalance (and rarer problems like alcohol withdrawal
or toxic ingestions, etc) can also contribute and need to be ruled out. If
hallucinations persist, however, as they often do in LBD, then treatment with
medication needs to be considered.

The antipsychotics Seroquel and clozapine are effective medications for
hallucinations – and also for delusions. Clozapine may be a little better, yet
another contentious topic in the medical literature. I’ve talked about their risks

I strongly avoid all other antipsychotics, as they can produce severe side effects in
patients with LBD, including, notably, severe worsening of parkinsonism.

Some hallucinations are pleasant or neutral (like seeing family members in the
living room), while others are scary and upsetting and provoke agitation. I treat
the latter type, and leave the former type alone.

I am not aware of any studies looking at whether TV (or other behavioral) habits
could influence the nature of the hallucination experienced, and I would be
guessing if I tried to answer your question about TV.

Reorienting can help with modest hallucinations, for example in mild, early
disease, in large part because the patient retains insight into their hallucinations
and is able to understand and be reassured by the information that the
hallucination they are experiencing is not real.

That becomes much more difficult if the patient has significant cognitive
impairment. In a delusion, a patient has a false, fixed idea about something that is
not real. Persuasion in that setting is very unlikely to convince them otherwise.

Victoria Ruff (Moderator)
6. Question from Pat: I have a question about the hallucinations. My spouse has
them all of the time but only at our house both outside and inside. He has had
them for about a year, and they keep getting more severe. Is that a sign that he is
getting worse? He is on medication, but he still sees them.

Dr. Gomperts
The fact that his hallucinations are getting more severe suggests that your spouse
may be slowly getting worse, unless his hallucinations can be explained by his
taking increasing doses of Parkinson medications (like dopamine agonists) or
other medications that can cause hallucinations as a side effect.

Again, I do not aggressively treat hallucinations unless they cause trouble. It
sounds like they may be causing trouble for your husband, but that is unclear. Is
the medication that you refer to an antipsychotic agent (also known as a

If so, antipsychotic agents are more effective at higher doses, so there may still be
some room to go with his medication, though increases should be gradual and

If there is no benefit once a given medication dose is maximized, his doctor may
consider trying a different antipsychotic medication.

Victoria Ruff (Moderator)
7. Question from Anita: Many times our loved ones complain of perceived
medical problems or simply not feeling well in certain areas. For instance my
loved one constantly complains of something being wrong with his system
meaning he thinks he needs to go to the bathroom and can’t, etc, etc. It has
gotten so bad at times that I take him to the Doctor and they run expensive tests
only to find out that nothing is wrong. Is there any special things that we should
look for in our LO in order to determine if a further look is necessary? Are there
any real guidelines to go by for us to know if seeing the Doctor is in order
considering the constant complaints we get from our loved ones?

Dr. Gomperts
Many patients with LBD have trouble communicating, and that can lead to all
sorts of difficulty when they don’t feel well. As a result, it can be hard for his
doctor to identify whether there is a problem, and it can be hard for you as well.
Unfortunately, there is no single thing for you or his doctor to look for to know
that something is or is not wrong.

Certainly, a change from prior behavior/function raises concern, e.g. a new
complaint, or a more severe old complaint, or a change in behavior (e.g. suddenly
more quiet and subdued), or the obvious signs of true illness, like fever, cough,

However, it’s good to be vigilant. So if you are concerned, I would bring him in to
his doctor for an evaluation. Over time, with experience, I suspect that you will
develop a good sense about when he is ill and when he is not really ill. I wish it
were easier.

Victoria Ruff (Moderator)
8. Question from Imogene: Has anyone experienced a LO with a problem for
eleven years? (My husband couldn’t do math in 1995. He quit his job because of it.
He soon forgot all the phone numbers in his head, and messed up the checkbook.
In 2005, he was diagnosed with Lewy Body Dementia. He is still considered early
stage.) Did he actually have it that long?

Dr. Gomperts
Without seeing your husband in clinic, speaking with you both in detail to review
his history, examining him, and reviewing his evaluation, I cannot provide a firm
medical opinion about his diagnosis. The course (duration of disease) of DLB
does vary significantly across individuals.

That said, 12 years is an extraordinarily long time to carry early stage DLB, and it
is possible that he may not have DLB, or may not have DLB in isolation. (One can
develop new cognitive problems after many years of Parkinson Disease, and they
can progress over time, but that does not fit the history that you gave me.)

Victoria Ruff (Moderator)
9. Question from Claudia: Can the REM Sleep Disorder in LBD mean lack of all
dreaming, or is that an indication of another illness?

Dr. Gomperts
I am not sure that I fully understand your question. REM sleep behavioral
disorder (RBD) is an abnormality of REM sleep. In REM sleep, we dream but are
normally unable to move (in fact, we’re paralyzed). In REM sleep behavioral
disorder, the paralysis is lost, so patients act out there dreams. RBD is common
in LBD but is not always present.

RBD is also common in Parkinson Disease. (Many people wake up from sleep
unable to remember their dreams. That by itself is not concerning for an illness.)

Victoria Ruff (Moderator)
10. Question from Donna: I understand that everyone is different and reacts
differently to meds, but what would your first med of choice be for the following
LBD symptoms:

• Cognition/Dementia?
• Depression?
• Parkinsonism?
• Hallucinations?
• Anxiety?
• REM Sleep Disorder?
• Excessive Daytime Somnolence?
• Restless leg syndrome (RLS)?
• Orthostatic Hypotension?
• Insomnia?

Question from Bobbi: Are any meds being investigated devoted to LBD

Dr. Gomperts
This is a hard question, exactly because everyone is different, and because the
medical problems that they have and the other medications that they are on can
affect a doctor’s medication recommendations. However, you’ve asked me to
ignore the complicating factors.

I want to again stress that it’s better to minimize medications, rather than having
one medication for each of these problems and thereby creating a set-up for

My first choices are:

Cognition/dementia: Aricept, Exelon, or galantamine, as tolerated. (Also attempt
to discontinue all medications that can cloud thinking; some anticholinergic bladder
medications, for example, can cause significant cognitive problems.)

Parkinsonism: Sinemet

Hallucinations: Seroquel (although one can make a compelling case for Clozaril)

Anxiety: effexor/Seroquel

REM sleep behavioral disorder: This does not always warrant medication for
management. If the patient is injuring himself, however, that would certainly drive
me to recommend treatment. One option would be melatonin.

EDS: consider a more activating antidepressant and take it in the morning/move
sedating medications to night dosing.

Restless Legs Syndrome: Sinemet (can be slow release form if necessary)

Orthostatic hypotension: Ted hose and, if safe for the patient, liberal salt intake
(several medications can provide additional help if those maneuvers are

Insomnia: consider a more sedating antidepressant and take it at night, and move
all sedating medications to night.

That list covers much of the gamut of problems that patients with LBD get, but
does not cover so-called “cognitive fluctuations”. Unfortunately, we do not yet
have medications directed at the cognitive fluctuations that some patients with
LBD get.

Victoria Ruff (Moderator)
11. Question from Linda: What sort of pain medications are the best to use for
LBDers. I know that hospice tends to use morphine and I have seen that
morphine is on the bad drugs list. What are the alternatives?

Question from Bobbi: Are any meds being investigated to alleviate pain in LBD

Dr. Gomperts
LBD by itself does not cause pain. Pain when it arises has to be well treated. If
not the patient will be uncomfortable. Secondarily, pain may lead to significant
agitation. Pain treatment really depends on the problem being treated.

Acetaminophen (Tylenol) and ibuprofen (Motrin) are preferable for mild pain. For
more severe pain, Darvocet (Darvon) or Ultram may be preferable to oral
opiates. When an IV pain medication is required, the shorter acting the better (as
any side effects, such as confusion, may be short lasting).

The bottom line is that effective pain control is critical, and has to be balanced
against the need to maximize cognitive function. Unfortunately, the medication
sensitivity that is common in LBD can commonly lead to side effects such as
confusion. Each patient’s doctor should be happy to discuss medication options
with you.

Victoria Ruff (Moderator)
12. Question from Sue: Do you have any suggestions for brain exercises to slow
down the process of cognitive/ST memory decline in LBD patients?

Dr. Gomperts
Early evidence suggests that cognitive (and gentle physical) exercise may help in
Alzheimer’s disease. We do not know for sure that cognitive (and gentle physical)
exercise will slow down cognitive decline in LBD, but it’s a real possibility, and
cognitive exercises certainly won’t hurt.

I recommend doing anything that is fun for the patient to do, because that
increases compliance with the task. Secondarily, the more challenging the better.
The exercise to choose depends not just on the patient’s interests but also on
his/her level of cognitive function. I tell patients to do what you can. So, if you are
able and interested, play bridge or chess, do sudoku, do the New York Times
crossword, etc. If not, do something a little easier.

The idea is to keep the mind active. It probably does not have to be super heavy

Victoria Ruff (Moderator)
13. Question from Phyllis: Are there any definable stages in LBD as in

Question from Victoria Ruff, Moderator: I’ve read in researching online that
there were “six developmental stages” found during autopsy, have those “stages”
been transferred to note LBD stages that are found during clinical diagnosis and
can be shared with the caregivers?

Dr. Gomperts
Physicians currently use a variety of tools to help us with clinical staging of LBD.
Clinical stage can be defined along the axes of both dementia and parkinsonism,
and is a useful tool for doctors. Mild, moderate, and severe dementia (and mild
cognitive impairment) are all reasonable terms that doctors can quantify with
rating scales (like the CDR scale).

Several different rating scales exist. The motor impairments of parkinsonism can
also be rated, for example using the Hoehn and Yahr scale, which scores
involvement of a single side, involvement of both sides, the additional involvement
of the midline and balance, and severity of motor impairment thereafter.

Other rating scales exist, too (such as the UPDRS subscale III). One goal in using
scales such as these is to tailor treatment to a given dementia and motor stage.

The six neuropathological stages that have been described at autopsy by Braak
and Braak over the last few years describe the location of cells that contain Lewy
bodies in the brain of patients with Parkinson Disease (not LBD, per se). In stages
1 and 2, Lewy bodies are found only in the lowest part of the brain (medulla,
pons, anterior olfactory structures).

In stages 3 and 4, they spread to involve the adjacent midbrain where the
dopamine cells reside (substantia nigra) and to other nuclei of the basal forebrain.
These stages are felt to be the likely point at which patients develop the motor
symptoms of parkinsonism.

In the final stages 5 and 6, the Lewy body pathology spreads to involve the
cerebral cortex (neocortex). One of the Braaks’ hypotheses is that the
involvement of cells in the cortex may cause the cognitive problems/dementia
that arise in dementia with Lewy bodies (DLB) and Parkinson disease dementia
(PDD). Many centers are exploring the hypothesis.

If Lewy bodies do prove to be the cause of the disease, then medicines that stop
them from forming (or spreading to neocortex) may treat the disease.

Victoria Ruff (Moderator)
14. Question from Bobbi: Why doesn’t the doctor who does diagnose LBD
inform the caregiver of the wide variety of LBD symptoms and perhaps how to
prepare for it?

Dr. Gomperts
I think that it would be reasonable and appropriate for doctors to inform patients
and their caregiver of the wide variety of LBD symptoms that can arise. However,
different problems can become primary in different patients with LBD, and can
manifest at different stages of disease, so it would be hard for your doctor to
cover every contingency in terms of preparation.

It is important that once a problem arises, your doctor can work with you and
your loved one swiftly to treat it.

Victoria Ruff (Moderator)
15. Question from Pat: My second question is about him perceiving me as
someone else exactly like me but negative, and he is difficult when he thinks I am
her. It happens like switching on and off a light switch. The switch happens, and I
can’t see any pattern for it. He sometimes feels he isn’t in his house and wants to
go home. Any suggestions for dealing with this?

Dr. Gomperts
These are common delusions, so common that they have names Capgras
syndrome for thinking that a loved one has been replaced by an imposter; and
reduplicative para-amnesia for thinking that his house has been replaced by an
imposter. Sometimes these problems are worse at certain times of day (for
example, late afternoon). They are not always easy to deal with.

Reassurance and patience are both worthwhile. An antipsychotic agent like
quetiapine (Seroquel) or clozapine (Clozaril) may help, and may be warranted if
the repercussions of the delusion are severe (e.g. if he gets very agitated).

Victoria Ruff (Moderator)
16. Question from Bobbi: Why is the diagnosis of LBD so elusive?

Dr. Gomperts
It is actually not that hard a clinical diagnosis to make, if there is evidence of
parkinsonism, hallucinations, or cognitive fluctuations, along with cognitive
decline. The diagnosis may appear elusive because (1) LBD is a relatively new
addition to clinical medicine, and (2) Alzheimer’s disease is much more common,
so that many problems that cause dementia are mistakenly attributed to
Alzheimer’s disease. The gold standard diagnosis, however, remains looking at the
brain at autopsy. We are working to improve the clinical diagnostic criteria.

Victoria Ruff (Moderator)
17. Question from Phyllis: Are heart problems and strokes more prevalent in
LBD patients?

Dr. Gomperts
No. Actually they are not.

Victoria Ruff (Moderator)
18. Question from Claudia: Why does my spouse seem to experience cognitive
difficult around me and no one else?

Dr. Gomperts
If I understand you correctly (and I may not), I suspect that you may simply be
more aware of your spouse’s cognitive problems than other people who do not
know him as well. If his problems are not too severe, then he may appear normal
to people who do not explore too deeply.

Victoria Ruff (Moderator)
19. Question from Victoria Ruff, Moderator: Are you researching and getting any
positive results with any type of dietary aides / nutritional supplements? For
example, we noticed positive results with Alpha Lipoic Acid added to my mom’s
cocktail of meds.

Dr. Gomperts
I personally am not. I recently did a search on Medline on this question, and did
not find a single study on the topic. Certainly more research needs to be done in
this area. Coenzyme Q10 is an antioxidant medicine that seems to help in ALS,
another neurodegenerative disease. There is no evidence to date on whether it
may help in LBD.

Victoria Ruff (Moderator)
20. Question from Imogene: In layman’s terms which Dementia of AD and LBD
has the tangled neurons, and which has cell death?

Dr. Gomperts
Alzheimer’s Disease has amyloid plaques (deposits of a protein called amyloidbeta
outside of and around brain cells) and accumulation of neurofibrillary tangles
(composed of a protein called tau) inside brain cells. Many brain cells die as the
disease proceeds, including the workhorse cells of the brain as well as cells that
make the chemical (neurotransmitter) acetylcholine.

In LBD, there is accumulation of Lewy bodies (spheres of proteins including
alpha-synuclein) inside brain cells. Some brain cells die as the disease proceeds,
most notably the cells that make “neuromodulators”, like dopamine and
acetylcholine, but there does not appear to be quite as much cell death as in
Alzheimer’s disease.

Victoria Ruff (Moderator)
21. Question from Bobbi: Why have so many doctors never even heard of LBD?

Dr. Gomperts
LBD has only recently begun to gather the attention it deserves as the second
most common dementia after Alzheimer’s disease. It is now getting better
publicized, and more physicians and scientists are studying it, so I think that
physicians are growingly increasingly aware of it. It takes time for information to
trickle out to the entire medical community.

Victoria Ruff (Moderator)
22. Question from Claudia: If there are no hallucinations, is it still LBD?

Dr. Gomperts
The core criteria for a clinical diagnosis of probably DLB are (a) dementia,
accompanied by (b) at least 2/3 of (1) spontaneous features of parkinsonism, (2)
recurrent visual hallucinations, and (3) cognitive fluctuations with marked
variation in level of alertness or attention. So, as you can see, you don’t actually
need to have hallucinations to have “probable DLB”.

In addition, (for your information) while you need (a) and (b) for a clinical
diagnosis of probable LBD, you can call it “possible” DLB with only 1/3 of those
(b) criteria. And you can bump from “possible” to “probable” if the patient has (i)
neuroleptic sensitivity, (ii) REM sleep behavioral disorder, or (iii) evidence for loss
of dopamine transporter signal on a nuclear medicine scan of the dopamine
transporter. With these clinical criteria, we presently do a moderately good (but
not very good) job in identifying people whose brains (at autopsy) show the
characteristic changes that define LBD. That said, we are trying to come up with
better diagnostic techniques.

At present, the diagnosis can only be made certain by looking at the brain at

Another thing to keep in mind is that in DLB, cognitive problems occur before or
concomitant with parkinsonism. In contrast, in Parkinson Disease dementia,
Parkinson Disease comes first, and patients only develop cognitive problems

(We currently arbitrarily require a 1year interval from PD to onset of dementia
to call it PDD; if less than that, we call it DLB.) Clinicians are currently studying
how DLB and PDD are related.

Victoria Ruff (Moderator)
23. Question from Bobbi: What is being done to publicize LBD?

Dr. Gomperts
The LBD Association, which is hosting this chat forum, is a major resource for
publicizing LBD. You all have its contact information. The Alzheimer’s Disease
Association is another. Parkinson disease groups, such as the Parkinson Disease
Foundation, are another resource, although they tend to focus primarily on the
motor problems of PD.

This condition is gaining attention among doctors, especially those who see PD
and dementia patients. As a result, within the medical community, LBD is
increasingly studied, and scientific progress often heralds increased research
funding and therefore public awareness.

Victoria Ruff (Moderator)
24. Question from Claudia: Do the symptoms progress gradually or rapidly?

Dr. Gomperts
Progression of disease varies greatly across different people. Acetylcholine
esterase inhibitors like Aricept, Exelon, and Razadyne (and possibly the agent
memantine) can help many DLB patients’ symptoms (to varying degree), even
though they do not treat the underlying disease.

From onset of dementia, on average, patients tend to live less than a decade, but
there is a large amount of variability across patients, so that is a very rough guide.

Victoria Ruff (Moderator)
25. Question from Sandy: I would like to know what differences appear between
AD and LBD patients’ actions or reactions or abilities. Do LBD patients bounce
>from good days to bad days more often sometimes changing hour to hour?

Dr. Gomperts
The cognitive profiles of early AD and LBD vary, but as these diseases progress,
they can appear to converge. Loss of short-term memory is the key signature of
AD. There may (or may not) be trouble with word finding or name retrieval.
There may (or may not) be temporal disorientation (time, date) and/or spatial
disorientation (e.g. newly getting lost when driving).

With time, judgment and planning can get affected as well. In severe, late cases of
AD, there may be hallucinations or delusions. There are no motor changes of

Some patients with LBD present similarly to AD in terms of their cognitive
profile. Others have more initial trouble with attention and judgment and
planning (so called executive function). There may also be a marked problem with
visuospatial skill (more than AD for a given level of cognitive decline). In addition,
some patients with LBD (but not all) have “cognitive fluctuations”.

This term refers to changes in level of alertness that can occur over several
timescales, day-to-day, hour-to-hour, and sometimes even minute-to-minute.
What you describe is a typical example of these fluctuations. Cognitive
fluctuations are not typically seen in AD (though they can occur with metabolic
or infectious disturbances, like a urinary tract infection).

In addition, patients with LBD frequently develop hallucinations or delusions early
on in the course of disease, along with parkinsonism.

Victoria Ruff (Moderator)
Thank you Dr. Gomperts – before the floor opens… Angela Taylor (President,
LBDA Board of Directors) has something to say…

Angela Taylor
On behalf of the LBD Association, I’d like to thank Victoria Ruff for the initiative
to launch this event. She deserves the lion’s share of the credit for asking LBDA
to host the event.

I’d also like to thank Dr. Gomperts for helping us with this pilot chat event. Our
deepest appreciation for your time and knowledge!

Dr. Gomperts
Thank you for the opportunity to speak with you.

What do you think of the medicine Detrol to control frequency of urination?

Dr. Gomperts
As I wrote above, so called anticholinergic agents that are used for urinary
urgency can cause cognitive problems.

Detrol is one of these agents. If possible, non-anticholinergic agents would be
preferable from my perspective, to remove any cognitive side effects.

My husband age 71 was diagnosed at UBC in Vancouver in 2003 he has been on
Aricept since. He had a question about visual disturbance, loss of ability to read
which have become more troublesome. He has trouble recognizing a common
face across a room, getting lost in his room of off days.

Dr. Gomperts
If colourful could please clarify her question, I would be happy to address it. I do
not understand what you are asking.

How should visual testing be done for person with LBD? Are there any special

Dr. Gomperts
The major issue is how do you address a subjective complaint in a person with

It isn’t always easy. His neurologist can assess him for a brain basis for his visual

If he is unable to tolerate an ophthalmologic exam, and cannot articulate his
problems, then it may be difficult.

Can too much of a med like Seroquel cause patient to regress and loose a handle
on reality?

Dr. Gomperts
Too much Seroquel can certainly cause sleepiness and asthenia. I would not
expect it to cause confusion per se, if titrated up gradually and systematically as

Are there any antibiotics that are on the ‘bad list’?

Dr. Gomperts
I do not currently recall any antibiotics that are particularly notorious in DLB.

My husband’s neurologist has mentioned Donepezil as a possibility to mitigate
some of the non-cognitive issues. Are you familiar with this drug?

Dr. Gomperts
Donepezil is also known as Aricept. I discussed it above for treatment of the
cognitive problems that arise in LBD.

Studies have shown that Aricept and Exelon can both help.

Victoria Ruff (Moderator)
No more questions please…

Would you mind my emailing you the rest?

Dr. Gomperts
Not at all.

Victoria Ruff (Moderator)
Wonderful! THANK YOU Dr. Gomperts — very much appreciated!

Dr. Gomperts quit (leaves the chat room)

Victoria Ruff (Moderator)
Everybody — I’ll grab those questions not posed and email Dr. Gomperts & then
email you all the answers…

Questions that were emailed to Dr. Gomperts for further answers:

How do you feel about computerized bio-feedback units? Would it be more of a
hindrance than useful?

Dr. Gomperts
Bio-feedback tools vary widely. They can be used for many purposes, including
cognitive therapy and improving motor function. Some may be helpful in the right
setting, but they are largely still experimental tools and are not yet well-validated.
Your physician may be able to guide you in the utility of one in your specific
circumstances. A patient has to be able to understand and complete the task at
hand, so computer program associated therapy may be more helpful earlier in the
course of disease.

My husband was on Seroquel but was changed to Risperdal when the 325mg of
Seroquel seemed not be helping. I wonder if the Seroquel could or should be
started again at a higher dose and the Risperdal discontinued due to side effects?

Dr. Gomperts
Without evaluating your loved one, I do not have sufficient information to make
clinical recommendations. Speaking generally, Risperdal is more likely than
Seroquel to cause significant side effects in patients with LBD.

Are you familiar with the med Abilify? What do you think of it for
agitation/aggression in LBD patients?

Dr. Gomperts
Because Abilify is a relatively new neuroleptic (antipsychotic agent), we do not
have extensive experience with it yet. Its potential to worsen parkinsonism in
patients with LBD would lead me to use it only with extreme caution.

How does one access clinical trials?

Dr. Gomperts
The LBDA website has a very useful list of clinical trials and clinical trial resources
for you to explore (under Resources), including those recently approved by the
FDA. Other online resources exist as well. Your doctor may also know about or
be involved in relevant clinical trials. Patient information resources at academic
hospitals in your metropolitan area are an additional good place to ask.

Of the three currently marketed agents that have been studied in the NET-PD
trials (minocycline, 2400 mg/day CoQ-10, creatine 5 g bid), are there any that
you would feel were too risky to use off-label while more definitive trials for
efficacy are conducted?

Dr. Gomperts
Minocycline has known drug interactions and has been associated with a variety
of adverse reactions. It should be used cautiously, if at all, under the guidance of a
physician. Creatine and CoQ-10 appear to be less likely to cause problems, if
used in appropriate doses. All medications should be used under the guidance of
a physician.

Does heat intolerance occur with LBD?

Dr. Gomperts
I am not sure that I understand what you mean by heat intolerance. Heat
intolerance, per se, is not a classic symptom of LBD. Patients with LBD, however,
can be physically and cognitively frail, and thereby more susceptible to extremes
of temperature.

Could you comment on your use of psychostimulants in your clinical practice?

Dr. Gomperts
I use psychostimulants in a subset of my patients with neurological diseases, to
increase arousal. When prescribed, they need to be used cautiously and
monitored closely by a physician.

NH Bob
How can we find Doctor(s) that have an interest in treating patients with LBD?
Our challenge is to find a Doctor(s) who can suggest different medications to
alleviate the classic LBD symptoms. (PS from Moderator, Victoria Ruff — Dr.
Gomperts, NH Bob is in NH and his mother is in Boston — are you accepting
new patients? or someone on your LBD team accepting new patients? Thanks in

Dr. Gomperts
General neurologists (and General Practitioners and Internists) have the option
to refer to Dementia and Movement specialists to get additional input on LBD
management. A patient can always request a referral. Dementia doctors tend to
focus on the cognitive problems, and movement doctors on the motor
complaints, but that is not always the rule. For additional input on
LBD management in the New England area, one option (of several) would be
referral to the Massachusetts General Hospital Movement Disorder Unit and
Memory Disorder Unit. I am taking new patients in the Movement Unit and
would be happy to see your family member in clinic. If he is interested, you or he
can call (617) 726-5532 to make an appointment.

Date: Sun, 11 Mar 2007 20:25:47 -0700
To: [email protected]
From: Robin Riddle <[email protected]>
Subject: LBDA Online Chat w/Mass Genl Neurologist, 3/28, 4pmCA time

LBD folks –
This was posted today on LBDcaregivers (a Yahoo!Groups online forum).

Assistant in Neurology, Memory and Movement Disorder Unit (MMDU),
Massachusetts General Hospital
Research Fellow, Picower Institute for Learning & Memory,
Massachusetts Institute of Technology

Date: Wednesday, March 28, 2007
Time: 7:00 PM EST
Duration: 1 Hour

LBDA is proud to invite you to a live, real-time Question and Answer
(Q&A) session with Dr. Stephen N. Gomperts, an Assistant in Neurology
at the Memory and Movement Disorder Unit (MMDU) of Massachusetts
General Hospital. The MMDU is an integral part of the Massachusetts
Alzheimer’s Disease Research Center and is a Udall Parkinson’s
Disease Research Center for Excellence. Dr. Gomperts’ clinical and
clinical research focus is on the cognitive and behavioral problems
that arise in neurodegenerative disorders, with particular emphasis
on dementia with Lewy bodies and Parkinson’s Disease. His basic
science focus is on the role of the dopamine system in learning and

This live event will be held at LBDA’s website in an online chat
room. (For those who are unfamiliar with online chat, it is a virtual
method of communication in which all attendees log into an Internet
chat room and can view or participate in a text-based discussion.
There is no audio or video with an Online Chat and the only software
required is a web browser.)

To register for the event and to receive log-in instructions, email
the event Moderator, Victoria Ruff, octoryrose (no
spaces). Because this is our pilot for live chat events, we are
limiting attendees to 100 people this first time. The transcript of
the event will later be posted on the LBDA website as well.

The information presented during the Online Chat is for informational
and educational purposes only and is not intended to substitute the
professional medical advice or treatment recommendations provided by
your doctor.