“Tools for Meal Time” – good article on dysphagia

The Fall 2008 CurePSP Magazine (psp.org) has a good article on swallowing problems that may be of interest to everyone — not just those dealing with PSP (progressive supranuclear palsy).

I’ve copied the full text below.  At some point, the newsletter will be available on psp.org; I received it in the mail this week.  You can sign up online at psp.org to receive a copy of future newsletters in the mail.

Robin

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Tools for Meal Time
Laura Purcell Verdun, MA, CCC
Speech-Language Pathologist
Otolaryngology Associates, PC
Fairfax, VA

CurePSP Magazine
Fall 2008 Issue

Difficulty swallowing, or dysphagia, is commonly associated with many neurodegenerative diseases, specifically PSP or CBD.  In fact, difficulty swallowing may be one of the early symptoms of this disorder.  Given that complications related to difficulty swallowing are one of the most common causes of mortality, attention needs to be directed towards optimizing the ease and safety of swallowing.

What changes in eating and swallowing should we look for?

There are actually fairly typical behavioral changes with eating in PSP and CBD.  These often include difficulty looking down at the plate, mouth stuffing and rapid drinking, difficulty with self-feeding because of tremor or stiffness, restricted head and neck posture, and occasional difficulty opening the mouth.  These behaviors can make it more difficult to swallow and often contribute to decompensation of the swallowing mechanism resulting in coughing and choking.  Caregivers need to look out for these behaviors, because the person with PSP or CBD often is not aware of these changes.

What are some tools that we can use to help make meal times more successful?

1.  Use a suction machine, toothette swab (a pink or green sponge on a lollipop-type stick), or mouth rinse prior to meals to clear out secretions which may interfere with ease of swallowing.

2.  A firm chair with arms to support sitting upright for proper swallowing alignment is most ideal.

3.  Keep the plate in the line of vision, by placing the plate on top of a book or something similar, or substituting reading glasses for bifocals.

4.  Try experimenting with different plates and utensils.  Plates with higher edges and bottom grips enhance independence with eating.  Downsize eating utensils to limit how much food is being put in per mouthful.

5.  Experiment with different cups and glasses.  Some are just easier to drink out of than others, depending on a handle or even the thickness and shape of the lip.  Straws are generally not preferred because it results in acceleration of the liquid into the throat before it’s ready.

6.  Use a blender or food processor.  The goal is not to eliminate foods necessarily, but consider how they could be prepared difficultly to enhance ease and safety of swallowing.  Multiple consistency items such as fruit cocktail and broth based soups generally should be avoided, so blend them.

7.  A teaspoon can be used to restrict the amount of liquid placed in the mouth for each swallow.

Though clearly not a comprehensive list, here are some specific products that may be of benefit at meal times.  Look for other products available on these web sites as well:

Flexi-Cut Cup allows for drinking without extending the head and neck backwards, 3 sizes available (800/225-2610, www.alimed.com).

Provale Cup restricts the volume of liquid allowed per swallow (800/225-2610, www.alimed.com).

Maroon Spoons have a narrow shallow bowl to restrict how much food is placed on the spoon and in the mouth, come in 2 sizes (800/897-3202, www.proedinc.com). [Robin’s note:  that’s the correct website!  You can also find these spoons at AliMed.]

Less Mess Spoon is designed with holes to keep food on the spoon, or drain away liquid from a multiple consistency food item (800/257-5376, www.theraproducts.com).

Scooper Plate with Non-Skid Base has a high curve to help scoop food onto a utensil (913/390-0247, www.bindependent.com).

Skidtrol Non-Skid Bowl is a melamine bowl with non-skid base (972/628-7600, www.maddak.com).

Are there any cookbooks that may give us some ideas regarding meal preparation for people with trouble swallowing?

Achilles E and Levin T.  The Dysphagia Cookbook.  2003.  Cumberland House Publishing.

MEALS for Easy Swallowing.  2005.  Muscular Dystrophy Association Publications.  [Robin’s note:  the correct link to this book is www.als-mda.org/publications/meals/ The full contents of this book, including the recipes, are available online.]

Weihofen D, Robbins J, Sullivan P.  Easy-to-Swallow Easy-to-Chew Cookbook.  2002.  John Wiley & Sons, Inc.

Wilson JR and Piper MA.  I Can’t Chew Cookbook: Delicious Soft Diet Recipes for People with Chewing, Swallowing, and Dry-Mouth Disorders.  2003.  Hunter House, Inc.

Woodruff S and Gilbert-Henderson L.  Soft Foods for Easier Eating Cookbook: Recipes for People who have Chewing and Swallowing Difficulties.  2007.  Square One Publishers.

If you have any concerns regarding swallowing, be sure to discuss this further with your neurologist and speech pathologist.

 

Brain-Computer Interface for Communication (60 Minutes10/31)

A local support group member sent me this link to an amazing “60 Minutes” segment from last Sunday called “Brain Power.” A man with ALS (diagnosed in ’99) and a woman who had a stroke — both of whom are “locked-in” (fully functioning minds locked in disabled bodies) — are shown using their thoughts to communicate and use a computer. The man, Dr. Scott Mackler*, is a neuroscientist who is still able to run a research lab at the University of Pennsylvania. The woman also uses her mind to operate a wheelchair. It’s incredible.

The link is: (you may have to cut and paste to get the link to fit on one line)
http://www.cbsnews.com/stories/2008/10/ … 0940.shtml

Robin

* Someone on an MSA-related discussion group posted these two links for previous media coverage of Dr. Mackler:

http://www.alsphiladelphia.org/NetCommu … Doc?id=198

http://www.philadelphiaweekly.com/view.php?id=9839

NPH Commonly Misdiagnosed as PD, Atypical Parkinsonism or AD

Good Morning America (on ABC) had a segment this Thursday on NPH (normal pressure hydrocephalus). (Janet Edmunson alerted me to this segment.) Common symptoms in NPH are gait problems (shuffling), urinary incontinence, memory problems, and slowed thinking. Some of these symptoms can also be found in the four atypical parkinsonism disorders. I have occasionally read about people being diagnosed with PSP, CBD, MSA, or LBD initially, and then MDs figure out it is actually NPH. (I’ve probably read about more cases that go the other way: initial diagnosis of NPH, and a later diagnosis of an atypical parkinsonism disorder.) According to this GMA segment, NPH can be misdiagnosed as Alzheimer’s or Parkinson’s Disease. An MRI is critical in diagnosing NPH. This is one of the few dementia-causing conditions that is treatable.

Here’s a link to the video of the GMA segment:
http://abcnews.go.com/Video/playerIndex?id=6196343
(You’ll have to watch a short commercial beforehand. And the volume is set *very* high for both the ad and the segment; you can turn it down.)

At the bottom of this post is a transcript of the Good Morning America segment. Here’s a link to the transcript:
http://abcnews.go.com/GMA/OnCall/story? … 680&page=1

Finally, you can locate info on NPH here:
http://www.lifenph.com/
http://www.emedicine.com/neuro/topic277.htm

Is It Really Alzheimer’s?
NPH Commonly Is Misdiagnosed as Alzheimer’s Disease
By JUJU CHANG, THEA TRACHTENBERG and IMAEYEN IBANGA
Good Morning America (ABC News)

Nov. 6, 2008 —

When 74-year-old Phil Myers was barely able to walk and his memory seemed to be going quickly, his wife, Shirley Myers, was terrified. She watched as the man she loved and was married to for 54 years slipped away, with thoughts of the two children they’d raised clearly in her mind.

“At first I was scared,” Shirley Myers said. “He took care of his family. He was just a family man. That was it. And he did so much for me.”

Is It Alzheimer’s?
“The doctors kept saying, ‘Oh, he’s OK. He’s OK,’ but then things kept getting worse — like dragging his feet. And then he started kind of forgetting where he was at,” Shirley Myers said.

The first diagnosis doctors gave Phil Myers was devastating; they believed it was a form of Alzheimer’s.

“When they told me it was Alzheimer’s, I couldn’t believe it because he was only 70 years old and it just didn’t seem like it was his time yet,” Shirley Myers said.

So the two went to see neurologist Mark Luciano from the Cleveland Clinic. He suspected it wasn’t Alzheimer’s at all, but rather something called normal pressure hydrocephalus, or NPH.

What Is NPH?
According to “Good Morning America” medical editor Dr. Tim Johnson, NPH can begin at age 55 and no one knows why.

The condition occurs when cerebral spinal fluid accumulates in the brain. The extra fluid pushes the brain against the nerves that affect memory, walking and balance, and bladder control.

“Hydrocephalis just means water inside the brain. It’s when water builds up and starts pressing on the brain itself,” Luciano said.

“The symptoms come on very gradually and they can be very subtle,” he said. “A gait problem, a problem with your bladder control, a problem with your memory, those are all very common symptoms in our elderly population.”

Other symptoms include problems with thinking, a slowing down of the thought process. Since the symptoms are so common, the condition is often misdiagnosed.

That happens in part because the warning signs come on very gradually and they can be very subtle, Johnson said. Also, in the elderly population, loss of memory, walking slowly, bladder control problems are all very common problems, so it looks like many other diseases, such as Parkinson’s, Alzheimer’s or dementia, he added.

“It is one of the only treatable forms of dementia or memory loss. It can be not only treated, but in many cases reversed to a great extent just by removing the fluid,” Luciano said.

An MRI of the brain will show the enlarged ventricles and is a way for doctors to properly diagnose the condition, Johnson said.

Treatment of NPH
Treatment for NPH is a relatively simple operation.

“When they told us it was — it would be treatable, it was like a miracle,” Shirley Myers said. “We all felt like it’s a miracle.”

A shunt is implanted in the brain and leads the excess fluid away from the brain and into the abdomen.

“Quite honestly, we weren’t sure that fluid removal was going to help him,” Luciano said.

Four months ago, Phil Myers had the operation to implant the shunt, four years after his symptoms first appeared.

Now the man, who once had tremendous trouble walking, travels with much more ease.

“It was quite, quite nice to be able to do the things you want to do,” he said.

Phil Myers remains in recovery and Luciano said it can take up to a full year to see all the effects of the procedure.

“The improvement that we see now, we hope, that he has for many years,” Luciano said.

“He still has a ways to go on some issues, but his walking is terrific. And that’s what the doctor was looking for I think. And his balance has improved,” Shirley Myers said.

Click here to get more information about NPH: http://www.lifenph.com/

Diagnostic criteria for MSA (“second consensus statement”)

International experts on multiple system atrophy (MSA) have revised the diagnostic criteria.  The new criteria are called the “second consensus statement.”

You can find the second consensus statement online at no charge:

www.ncbi.nlm.nih.gov/pmc/articles/PMC2676993/

This paper includes the diagnostic criteria for MSA-P and MSA-C.  (Note that MSA-A no longer exists.)  The “P” stands for parkinsonism while the “C” stands for cerebellar.

One important table in the statement is table 4, which lists symptoms that do NOT support a diagnosis of MSA.  This list includes:

* classic pill-rolling rest tremor
* clinically significant neuropathy
* hallucinations not induced by drugs
* onset after age 75 years
* family history of ataxia or parkinsonism
* dementia (on DSM-IV)
* white matter lesions suggesting multiple sclerosis

Also, interestingly, the criteria for the diagnosis of probable MSA mentions “urinary incontinence.”  That symptom is described as “inability to control the release of urine from the bladder.”  In contrast, the criteria for the diagnosis of possible MSA mentions several possible urinary problems, including “otherwise unexplained urinary urgency, frequency or incomplete bladder emptying.”

The criteria for the diagnosis of probable MSA requires that there be a certain decrease in blood pressure:  “orthostatic decrease of blood pressure within 3 [minutes] of standing by at least 30 mm Hg systolic or 15 mm Hg diastolic.”  If that level of decrease isn’t seen, then the person might be diagnosed as having possible MSA.

I believe that those diagnosed with MSA should understand on what basis they’ve been given that diagnosis, and this helps to accomplish that understanding.

Emotional and Cognitive Aspects of PD

The Parkinson’s Disease Foundation (PDF) hosted a symposia on Parkinson’s on July 18, 2008.  The overall topic is “Mind, Mood and Body: Understanding Nonmotor Symptoms of PD.”  Here’s a link to the archived recording of the symposia:

event.netbriefings.com/event/pdf/Archives/nonmotor/register.html

The second speaker, Dr. Matthew Menza, gave a good presentation on the topic of “Emotional and Cognitive Aspects of Parkinson’s Disease.”

Even though this presentation was focused on PD, some references are made to LBD.  And, of course, there are emotional and cognitive aspects to all of the atypical parkinsonism disorders.

The most important thing I got from Dr. Menza’s presentation is that SSRIs should *not* be considered as the first line treatment in dealing with depression in PD.  A recent study showed that an old antidepressant, nortriptyline (Pamelor), performed better than Paxil, an SSRI.  Other drugs that are similar to nortriptylnie are Cymbalta and Effexor.

Of the atypical parkinsonism disorders, the only antidepressant that has been studied is Elavil (amitriptyline) in PSP.  Otherwise, I’m not aware of any studies.  You might take this recent PD research to your MD to find out if his/her recommendation would change based upon the new info.

Dr. Menza spent some amount of time on antipsychotics.  He explained that there was good data to recommend Clozaril and that in some studies Seroquel performed no better than the placebo.

He did talk about Lewy Body Dementia a couple of times, but I didn’t think his description was very good.  (Dr. Menza is a neurologist and psychiatrist.)

These are my notes from his presentation and his answers to the questions directed to him.  Of course it’s much better to watch the video yourself.

Robin


Robin’s notes from:

Emotional and Cognitive Aspects of Parkinson’s Disease
Matthew Menza, M.D., Prof. of Psychiatry and Neurology, Robert Wood Johnson Medical School

Non-motor symptoms of PD include:
* sleep disturbances, fatigue, and excessive daytime sleepiness
* depression
* drug reactions, including psychosis and impulsivity
* mild cognitive impairment to dementia

The non-motor symptoms have become the focus of research because they are so important to how patients feel day to day.

Depression:  about 40% have some.  It has a major impact on quality of life and functioning (faster progression of motor symptoms; greater decline in cognitive skills; greater decline in ability to care for self).  Depression often precedes the PD diagnosis.

Symptoms of depression include:  sadness, lack of appetite, sleep problems, lack of interest and motivation, fatigue, crying spells, etc.  Some of these symptoms are seen in PD without depression.  (6:22)

Depression is very inter-related with anxiety (worrying about things in an excessive way).  Anxiety is a common symptom in PD.

What causes depression in PD?  Probably a mix of neurochemical changes in the brain that accompany PD and the stress of living with an illness.

We encourage people to get involved in support groups.  There’s a lot of knowledge in SGs about handling day to day problems.  SG members may have common wisdom that MDs may not know about.

Try relaxation techniques to help yourself forget worries and get to sleep.  Even counting sheep.

Psycho-therapies are being modified and written expressively for those with PD.  Best to find a psychiatrist or psychologist who has experience with PD.

Exercise is a good treatment for depression.

NIH funded an 8-week trial of PD and paroxetine CR (an SSRI), nortriptyline (a tricyclic), and placebo.  This is the largest and first of placebo-controlled studies on antidepressants and PD.  This study is not yet published.  (Other studies are in the works.)  Big improvement in depression for both anti-depressants but nortriptyline was much better than paroxetine.  [paroxetine CR = Paxil CR; nortriptyline = Pamelor]  Nortriptyline affects both serotonin and norepinephrine in the brain.  Paroxetine affects only serotonin.  This study calls into question the use of SSRIs as first line treatment in PD.  Two other newer drugs that affect both serotonin and norepinephrine are Cymbalta and Effexor.  Paxil was effective for some in the study.  (23:48)

Sleep problems in PD are more common than would be expected from age alone.  50-75% of people with PD have trouble with sleep.  In their current study, sleep was the #1 predictor of quality of life (even more than motor problems).  The most common sleep problem is difficulty staying asleep (74-88% of patients).  Other problems:  poor quality sleep, difficulty falling asleep, muscle movements (PLMS and RLS; up to 15%), sleep apnea (up to 12%), RBD, morning headaches.

RBD (REM behavior disorder) is a particular concern.  PD accounts for 27% of RBD cases.  32% had injured themselves and 64% had assaulted their spouse.  Acting out dreams.  Can be a few vocalizations or something more dramatic.  People are reluctant to talk to MDs about this.  There’s a good treatment for RBD that works most of the time.

Excessive daytime sleepiness (EDS) occurs in up to 51% of PD patients.

Sleep attacks (sudden onset of sleep, usually without much warning) are associated with nearly every dopaminergic medication but especially Mirapex and Requip.  3.8% of PD patients had sudden attacks while driving.  Some still debate whether these attacks are caused by these drugs or EDS.

Sleep disturbances in PD may be related to nocturia (frequent need to urinate at night), pain, dystonia, akinesia, difficulty turning, etc.  Sleep is regulated by adrenergic, serotonergic, cholinergic, and various peptidergic symptoms which are disrupted (variably) in PD.  Depression is a major risk factor.  Dopaminergics can also worsen sleep (produce arousal and suppress REM).  (33:00)

Treatment of sleep disturbances includes:  exercise, sleep hygiene, intermittent use of sleep meds (Lunesta, Ambien, etc).  Some medications may help daytime sleepiness including Provigil, Ritalin (an older stimulant), and sometimes amantadine (Symmetrel).

Sleep hygiene:  regular sleep hours; avoid excess time in bed; regular get-up time regardless of sleep quality; avoid daytime naps (of 2 hours in length; 15 minutes is OK); use bed for sleep or sex; relaxation; physical activity; sunlight in morning; bedroom quality (noise, temperature, humidity); avoid evening stimulants; avoid large evening meals.  If you are worrying, get out of bed.  (36:34)

Two categories of unusual behaviors that sometimes accompany meds given for PD:  psychosis (hallucinations and delusions); impulse control disorders (including gambling, binge eating, buying, hypersexuality).

Psychosis is rare in untreated PD.  Can be caused by all PD meds though psychosis is somewhat more frequent with dopaminergic receptor agonists.  Biggest risk factor for developing psychosis is memory impairment.

Hallucinations (seeing something that isn’t really there) occurs in approximately 30% of PD patients.  Usually these are mild.  The problem is when hallucinations are frightening.

Delusions (belief that isn’t shared by other people in your world) occur in 3-17% of patients.  Can cause major problems and be very disruptive.  Generally later in illness when memory begins to fail.  Typically persecutory (eg, fear of being poisoned, infidelity).  Please bring these up with your MD.

There was just a large study on impulse control behaviors just discussed.  Seem to occur more frequently with Mirapex and Requip but can also occur with Sinemet.  (42:03)

In the face of these problems (psychosis or impulse control disorders), the first thing MDs do is reduce the dopamine medication.  “Motion-emotion conundrum.”  If reducing the parkinson meds doesn’t solve the problem, then MDs look to antipsychotics.  (43:08)  The first antipsychotic given is Seroquel.  If that doesn’t work, then Clozaril is tried.  Clozaril requires a weekly blood sample.  Clozaril is very effective.  (43:42)

In early PD, most develop a little of what could be described as “mild cognitive impairment.”  This is impairment of tasks requiring the frontal lobe of the brain — planning, judgment, and recall memory.  This doesn’t cause major problems.  Dopamine replacement leads to some improvement.  (44:39)

The more difficult thing is the more serious memory impairment that happens later in the disease.  Quite a few people develop this.  This is not Alzheimer’s.  This is much, much slower in development than Alzheimer’s, and generally less severe.  There are trials out there looking at the typical AD drugs (such as Exelon) in PD.  In one study, Exelon had a slightly positive response, and some with PD can take this drug.  It’s worth trying.  There is a question if Namenda will work.

Dealing with cognitive impairment includes:  household safety (and preventing wandering), reminders as to the structure of the house, day care, in-home help.

It’s important to educate yourself about psychiatric issues.  Sometimes you have to educate your physician about psychiatric issues in PD.  (46:39)

Question: Is bipolar a precursor to PD?
Answer:  I don’t think so but on the other hand certainly people with PD can develop bipolar disorder (though this would be unusual).  Bipolar disorder hits people early in life.  (48:00)

Question:  Can you elaborate on Lewy bodies?
Answer:  In Lewy body disease, there is wide distribution of Parkinson’s pathology across the brain.  This is a variant of PD where the cognitive impairment and memory impairment progresses much, much more rapidly than in normal PD.  And the individuals are much more sensitive to the adverse side effects of PD.  It’s a much more rapidly progressive illness than normal PD.  Not much is known about LBD.  It can be quite a trial.  (49:10)

Question:  What were the side effects related to nortriptyline?  (50:06)
Answer:  The newer antidepressants are better tolerated.  Surprisingly, nortriptyline (an older med) was well tolerated.  There was more constipation in the nortriptyline group.  Our lesson from the study:  don’t start with an SSRI.  (51:13)

Question:  When will a cure happen?
Answer:  Someday there will be a cure.  But what do you do now?  We need research on the problems we are having right now.  (51:56)

Question:  Can you comment on the French clozapine study?
Answer:  There have been two well-controlled Clozaril trials showing Clozaril is better than placebo for psychosis.  Weekly blood draws for six months can be a problem for some.  Some of the Seroquel trials did not show that Seroquel was any better than placebo.

Question:  Can we view panic reactions as a behavioral equivalent of a motor tremor?
Answer:  I suppose you could look at them that way.  Sometimes those with anxiety have panic attacks.  I don’t know if it’s the same neurochemically.

Question:  Depression vs. anger.  Can anger be a stimulus?
Answer:  Anger can motivate people to make changes in behavior.  If people are having a lot of anger, it’s usually based on relationships and chronic problems.  Counseling may be helpful in determining cause of anger.  Is the anger a personality change?

Question:  What determines sexual dysfunction?  Compared to what you used to do?  Frequency?
Answer:  These problems are happening in people who are older, some of whom have less interest in sex.  Autonomic dysfunction can contribute.  There are also relationship issues.  If something has dramatically changed compared to the past, then we look to some cause (medication?) for the problem.  (56:09)

Question:  If a person with PD is on bipolar meds, and needs an antidepressant, is there a conflict?  Are they more likely to become manic?  Any connection?
Answer:  This is complicated.  Lithium (a bipolar med) can sometimes not be at all well-tolerated in PD.  Med changes have to be made.  But there are some bipolar meds that those with PD can take.  Depression would be seen as a function of bipolar disorder.  In bipolar disorder, we generally try not to use antidepressants because this can worsen the mania.  But we do use them in conjunction with a mood stabilizer.  (58:10)

Question:  My husband becomes agitated and anxious.
Answer:  Agitation can be many things.  Impulsivity can be a personality change.  Is there a change in impulsivity?

Question:  How effective is Remeron for depression in PD?
Answer:  We have no idea.  Remeron is a new kind of antidepressant.  It works differently than Paxil, Prozac, that class.  Remeron tends to be very helpful with sleep.  It has a lot of antihistaminic and anticholinergic effects so it can make constipation worse.  Each antidepressant needs to be studied individually:  does this drug work in this person?

Question:  Is depression in PD hereditary?  (00:48)
Answer:  We don’t know.  We think that in non-PD depression has a hereditary component (“somewhat more likely” to develop PD).  Many people, however, with no family history develop depression, and many people with a family history of depression who never develop it.  Is there something different about PD and depression compared to PD without depression?  We don’t know but it’s a good question.