Gastroparesis – Symptoms, Evaluation, and Treatment

Gastroparesis, or delayed gastric emptying that causes bloating, regurgitation, and early satiety, can be a problem in the neurodegenerative disorders in our group, especially in MSA and LBD. Gastroparesis can lead to weight loss and dehydration.

One gentleman in our local support group with a clinical diagnosis of MSA but a pathologically confirmed diagnosis of LBD had gastroparesis.  It was the worst symptom he had.

Online friend Vera James posted to an MSA-related Yahoo!Group a link to a terrific article on gastroparesis.  Here’s a link to the article, available at no charge:

www.practicalgastro.com/pdf/December06/WoArticle.pdf

Motility and Functional Disorders of the Stomach: Diagnosis and Management of Functional Dyspepsia and Gastroparesis
Practical Gastroenterology
December 2006
by John M. Wo and Henry P. Parkman

See pages 37-45 especially.

The section on “Treatment” is particularly good. These points are discussed in detail:

* eating smaller, more frequent meals

* relying on liquid nutrient

* limiting fatty foods

* avoid salads, raw foods, and red meat

* antiemetic medications.  “The most commonly prescribed traditional antiemetic drugs include promethazine and prochlorperazine.”

* prokinetic medications such as metoclopramide, domperidone (not available in the US), and erythromycin.

* botulinum toxin injection

* gastric electrical stimulation

* jejunal feeding, rather than PEG feeding

I’ve copied below the “Gastroparesis Cardinal Symptom Index.”

Robin

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Table 4.  The Gastroparesis Cardinal Symptom Index
1. Nausea (feeling sick to your stomach as if you were going to vomit or throw up)
2. Retching (heaving as if to vomit, but nothing comes up)
3. Vomiting
4. Stomach fullness
5. Not able to finish a normal-sized meal
6. Feeling excessively full after meals
7. Loss of appetite
8. Bloating (feeling like you need to loosen your clothes)
9. Stomach or belly visibly larger

Each symptom is graded by the patient on a 0 to 5 scale: 0=none; 1= very mild, 2=mild, 3=moderate, 4=severe, 5=very severe.

From Revicki DA, Rentz AM, Dubois D, Kahrilas P, Stanghellini V,  Talley NJ, Tack J. Aliment Pharm Ther, 2003;18:141.

“Speech and swallowing in MSA and other autonomic disorders”

This article on “Speech and swallowing in MSA and other autonomic disorders” will certainly be of interest to the MSAers but some of the questions/answers will be of interest to everyone (especially #1, #7, #8, #9, #12, and #13). (My dad, with PSP, has many of the problems indicated.)

The Sarah Matheson Trust is a UK charitable organization focused on MSA. The Trust’s newsletter, SMarT News, had an article last year in which “speech and language therapist Tricia Gilpin report(ed) on the presentations of speech and swallowing problems and the therapists role in treating people with MSA.” Here’s a link to the Winter ’05 issue of the newsletter:

www.msaweb.co.uk/smart_2005PDF_winter.pdf
Editor’s Note: Link no longer available

There was one surprising sentence in the article: “It is important to realise that exercises will not improve the speech.” I thought that was the whole point of speech therapy. Other than that one quibble, I thought the article was a good introduction to what speech/language pathologists (our term in the US) do and what kinds of speech and swallowing problems there are with these disorders.

A copy of the article follows (note that the numbers in front of the questions were added by me).

————————

Speech and swallowing in MSA and
other autonomic disorders

by Tricia Gilpin
Senior Speech and Language Therapist
SMarT News, Winter ’05 Issue, November 2005
Sarah Matheson Trust newsletter

#1 What does a Speech & Language
Therapist do?

Speech and Language Therapists are
involved with both children and adults
with many different types of disorders.
These include developmental difficulties,
stroke, head injury, brain
tumours and many different degenerative
neurological diseases.

The SLT will see patients with any
of the following problems:

* Dysarthria: a motor speech
disorder, where there is loss of
function or co-ordination in the
breathing mechanism, or in the lips,
tongue or soft palate

* Dysphasia: a disruption to the
language centre in the brain which
may affect understanding of the
spoken word, spoken output,
reading & writing

* Dysphonia: the loss of the voice
due to physical or psychological
difficulties. This must always be
checked by an Ear, Nose and Throat
specialist to eliminate the possibility
of structural damage to the larynx

* Dyspraxia: a motor speech
programming disorder. There is no
loss of movement in the lips,
tongue or soft palate, but the
messages from the brain to them
becomes disrupted

* Dysphagia: this is a disturbance in
the process of swallowing. A person
with dysphagia may report any of
the following signs:
– difficulty with hard, dry foods or
mixed consistencies
– problems with thin liquids
– coughing and choking when
eating and drinking
– food becoming stuck in the mouth
or falling out of the mouth
– extended meal times
– excessive saliva and/or dribbling
– having a ‘gurgly’ voice after
mealtimes or drinks
– difficulty opening the mouth
sufficiently
– weak cough
– weight loss
– chest infection

* Tracheostomy: SLTs also work
with patients who have a
tracheostomy.

#2 Classification of Multiple
System Atrophy

There are three different aspects to
MSA:

* Parkinsonian or Extrapyramidal signs
* Cerebellar signs
* Autonomic Impairment

The disease can present in any of
these three ways, or often as a
combination of two or three of them.
The speech and swallowing
characteristics of the patient will vary
according to the clinical picture, and
may change over time.

#3 What speech characteristics
should I expect if the presentation
is mainly Parkinsonian?

This type of MSA is now called MSA-P.
The type of dysarthria seen in
Parkinson’s Disease, and in MSA-P is
called hypokinetic dysarthria. This is
characterised by:

* festination of speech — that is
difficulty getting started and then
speech coming out in a rush

* quiet speech

* slow and hesitant speech

* speaking in a monotone and at the
same pitch

#4 What should I expect if the
presentation is mainly
Cerebellar?

This used to be called Olivopontocerebellar
Atrophy but is now
called MSA-C.

This type of MSA involves the
cerebellum and brain stem and the type
of dysarthria seen is called ataxic or
cerebellar dysarthria. This is
characterised by:
* staccato (or chopped up) speech
* imprecise consonants
* slow and slurred speech

Unfortunately, this type of dysarthria
can make you sound as if you have been
drinking too much alcohol.

#5 What if it is the Autonomic
Presentation?

The third type of MSA is the
predominantly Autonomic presentation
where the patient often has postural
hypo-tension. This may lead to general
feelings of dizziness and exhaustion
and you may not feel very much like
talking at all.

A drop in the blood pressure can
result in a decrease in the volume of
the speech.

#6 Spastic Dysarthria

In addition some patients present with a
spastic dysarthria where the speech is
characterised by:
* imprecise consonants
* strained or strangled voice
* quiet voice

It is important to remember that speech
characteristics in MSA can be mixed
between the different types of
presentation and therefore the different
types of dysarthria.

A research study by Kluin et al in
1996 looked at 46 patients with MSA
and looked at the speech characteristics
and concluded that:

* Hypokinetic components
predominated in 48%
* Ataxic components predominated in
35%
* Spastic components predominated
in 11%
* the remaining 6% were mixed
dysarthria

#7 What can the SLT do to assess
my speech and communication?

The SLT may decide to do a formal
dysarthria assessment. This consists of
looking at the different aspects of the
speech process:
* respiration (breathing for speech)
* phonation (production of sound)
* movements of the facial musculature
* prosody (intonation patterns — the
ability to change pitch in speech)
* articulation (ability to produce clear
sounds)
* intelligibility

Alternatively, the SLT may assess you
in a more informal way by talking to
you and to your family.

#8 What can the SLT do to help me
with my speech and
communication?

Unfortunately, there is no cure for
the speech difficulties experienced in
MSA but the SLT can help by giving
information to the patient and to
the family.

Many people with MSA experience
other difficulties with their
communication in addition to the
speech.

* many people experience a lack of
facial expression

* others find making and maintaining
eye contact difficult

* some may have a forward head tilt
which hampers communication,
feeding and vision

* others will have displays of
inappropriate laughter or crying

* some people have difficulty
switching attention from one topic
to another

* some people will experience a
low mood

Often it is helpful just to be able to
discuss these matters and try to find a
way, with the therapist, to manage these
difficulties. It can be helpful to discuss
ways to avoid very noisy situations, or
to cut down on background noise when
trying to communicate (even turning off
the TV can be helpful).

For the family and friends of the
person with MSA it is important to try
to make time to communicate, even
though the process may be slower
than previously. It is often helpful to
be able to watch the face of the
person to help with understanding and
to ask for repetition in a different
way if something cannot be understood.
It is important not to pretend to
understand if you have not, and to ask
for clarification.

#9 Will the SLT give me exercises to
do to improve my speech?

It is important to realise that exercises
will not improve the speech. However,
sometimes the therapist will decide to
focus on a particular aspect of the
speech mechanism to try to maintain
and preserve speech for as long as
possible. It may be helpful to work on:

* exercises to improve facial
expression

* breathing exercises to maintain an
adequate respiratory drive for speech

* relaxation exercises to reduce
tension in the muscles used for
communication

* voice exercises to maximise volume

* speech exercises to improve overall
intelligibility and fluency

#10 Are there any Communication
Aids for people with MSA?

* some people find that a small speech
amplifier can be helpful

* there are other communication aids
such as Lightwriters which allow the
patient to type out their message and
the machine talks for them

* some people prefer to use a pen and
paper if they are finding speech
difficult

#11 I have heard that sometimes
people with MSA need to have a
tracheostomy. Is this true?

Unfortunately, some patients with MSA
may experience difficulty opening the
vocal cords in the larynx sufficiently to
allow the normal amount of airflow.

This may be due to vocal cord palsy and
can result in:
* excessive snoring
* inspirational stridor — a sound like
snoring but on the ‘in’ breath
* sleep difficulties and vivid dreams
* breathing problems, particularly at
night
* sleep apnoea, where the person stops
breathing for a short time during
sleep

In extreme cases the ENT surgeon may
recommend a tracheostomy. This is a
small tube which is fitted into the
neck below the level of the vocal cords,
allowing the patient to breathe
comfortably.

The Speech and Language Therapist
can assist in advising whether or not a
speaking valve is appropriate.

#12 What happens to the normal
swallow in people who have
MSA?

The normal swallow is made up of 3
stages:
* the oral stage
* the pharyngeal stage
* the oesophageal stage

All three stages of the swallow may be
disrupted in MSA. A research study by
Smith & Bryan in 1992 looked at ten
patients with MSA and found the
following:

Oral stage:
* 90% had decreased control of what
was in their mouth
* 40% had poor tongue movements

Pharyngeal stage:
* 80% had a delayed swallow
* 100% reported the feeling of
‘something stuck in the throat’
* 30% experienced ‘silent’ aspiration —
where food or drink goes ‘down the
wrong way’ and enters the lungs, but
the patient does not cough

Oesophageal stage:
* some patients with MSA or other
types of autonomic dysfunction
experience dysmotility of the gut
* this can result in constipation but
also in dysmotility of the
oesophagus with patients reporting a
feeling of ‘something stuck’ in the
centre of their chest

#13 How will the Speech and
Language Therapist assess my
swallow?

The SLT will start by taking a full
history of the disease process and also
of the eating and swallowing problems
>from both the patient, and if possible,
>from the carers.

The SLT will then probably
complete the following assessments:

* a ‘bedside’ assessment of the
swallow including:
– assessing the cranial nerves of the
patient, looking at the functioning
of the face, tongue, lips, soft
palate etc.
– trial the patient on various foods
and drinks, observing their ability
to cope with different
consistencies

* possibly perform a videofluoroscopy
(this is a moving x-ray of the
swallow which is filmed onto
video). This shows the safety and
assist the therapist in making
recommendations regarding:
– appropriate consistencies of food
– positioning
– possible modifications to the diet

It may be that the SLT, in consultation
with other members of the multidisciplinary
team, may suggest a PEG.
This is a Percutaneous Enderscopic
Gastrostomy which is a small tube
which is passed directly into the
stomach to allow the patient to be fed
with liquid feed, should this become
necessary.

A PEG is often used in conjunction
with continuing to eat and drink. It may
be that the patient is finding eating and
drinking very time consuming, slow and
difficult and may find it much more
pleasurable to get all the necessary
hydration and nutrition through the PEG
leaving the opportunity to eat and/or
drink small amounts for pleasure.

#14 The Multi-Disciplinary Team and
the Speech and Language
Therapist

The SLT may discuss a number of
complex issues with you regarding
your speech and swallowing and
also have discussions with you
regarding the placing of a PEG tube or
a tracheostomy.

It is important to remember that any
complex decision that has to be made
will always be after joint discussion
between members of the MDT, the
patient and his/her family. The MDT
may include the Neurologist, the GP, the
clinical nurse specialist for MSA, other
nurses, the dietician, the physiotherapist,
the occupational therapist and the speech
and language therapist.

In the discussions regarding the
placement of a PEG or a tracheostomy
it is vital to allow time for discussion
with the patient and his family and to
take into account the individuals right to
make decisions about his or her quality
of life.

Article on Alternative Therapies and PD

PSP Forum –
Sorry for the screwy formatting of this post. These are two emails below that I sent in August ’06 to the local (SF Bay Area) support group on “alternative therapies” (including CoQ10) and Parkinson’s Disease. (I thought the author worked at Scripps but the institution is Cedars-Sinai.) It’s just easier to copy-and-paste rather than re-work the two emails. If you are interested in seeing these sorts of posts from me, note that I am a regular poster on pspinformation.com (Yahoo!Groups). I don’t regularly come to the Forum (because I dislike using anything but email to post and read).
Thanks,
Robin

Date: Wed, 23 Aug 2006 18:38:47 -0700
To: Local Support Group
From: Robin Riddle <[email protected]>
Subject: Fwd: “Alternative Therapies” & PD (Co-Q10, vitamin C, creatine, etc)

This updated email will be of interest to those considering NADH, glutathione, massage therapy, and exercise. There’s also a resource list for additional info on alternative therapies.

The “Alternative Therapies” article provided answers to seven questions. I found an earlier article by the same author, Dr. Jill Marjama-Lyons, that had ten questions. Perhaps three questions were eliminated to fit the article on one page in the APDA Newsletter Spring 2006. The earlier article was written perhaps in 2004 or early 2005. Here are the three new questions along with the exercise question answered again (because the earlier article gave a longer answer), a resource list (books, etc) for holistic therapies (“holistic” is the preferred term), and some info on the author:

Should persons with PD take NADH? Similar to Co-Q10, NADH (nicotinamide adenine dinucleotide hydrogen) is an enzyme that is involved in energy production of living cells. NADH is not a proven treatment for Parkinson’s disease. Several open-label (patients and examiners were not blinded) studies have shown motor improvement in persons with PD who took NADH. One small double-blinded, controlled study of 10 persons with Parkinson’s disease who took intravenous NADH did not indicate that any improvement occurred in patients.

Should a person take IV(intravenous) glutathione for Parkinson’s? Glutathione is not an approved treatment for Parkinson’s disease. Similar to Co-Q10, glutathione levels have been shown to be lower in persons with PD. Despite many personal stories of patients feeling better with use of IV glutathione, currently there are no published controlled studies proving or disproving it as a therapy for PD.

Can massage therapy help people with Parkinson’s disease? Some persons with PD report massage therapy to lessen muscle stiffness (rigidity) and pain, though the benefit is often transitory and last a few hours or days.

Does exercise really make a difference? Exercise of any kind that does not increase one’s risk of falling is always recommended to increase endurance, improve delivery of oxygen to the brain, heart and muscles, increase muscle strength and mass, and improve coordination, balance and flexibility. Exercises such as yoga and tai chi focus on the mind-body connection and improve balance and mobility for persons with Parkinson’s and many PD centers and health clubs offer these exercise classes. Even for someone with advanced Parkinson’s disease, exercise can make a big difference; chair and bed/floor stretches/movements as well as swimming can be performed by almost anyone. (Note from Robin: the last sentence is not in the APDA Newsletter Spring ’06.)

Resource List for Holistic Therapies
(Note from Robin: the author of the article has a book listed below – third item down. It was recommended to me by someone who used to work with Dr. Marjama-Lyons at Cedars-Sinai Medical Center.)

The American Holistic Health Association’s Complete Guide to Alternative Medicine, by William Collinge, M.P.H., Ph.D.

Alternative Medicine: The Definitive Guide, edited by Burton Goldberg (Future Medicine, 1998), features over 400 holistic practitioners

What Your Doctor May Not Tell You About Parkinson’s Disease: A Holistic Program for Optimal Wellness, by Jill Marjama-Lyons, MD and Mary Shomon (Warner Books, 2003)

PDR for Herbal Medicine, (First Edition, 1999) Medical Economics Company

Prescription for Nutritional Healing, by James Balch, MD and Phylis Balch (Avery Penguin Putnam, 2000)

Tyler’s Herbs of Choice: The Therapeutic Use of Phytomedicinals, by James E. Robbers and Varro E. Tyler

Eat Well, Stay Well With Parkinson’s Disease,by Kathrynn Holden, M.S., R.D. (Five Star Living, 1998)

The Brain Wellness Plan, by Jay Lombard, M.D. and Carl Germono

American Holistic Health Association P.O. Box 17400, Anaheim, California 92817 / (714-779-6152/ www.ahha.org

NIH National Center for Complementary and Alternative Medicine (NCCAM) 888-644-6226 / www.nccam.nih.gov

Mind Body Medical Institute 110 Francis St., Boston, Massachusettes 02215 / (617-632-9530) / www.mbmi.org

American Association of Oriental Medicine 433 Front St., Catasauqua, Pennsylvania 18032 / (888-555-7999) / www.aaom.org

Acupuncture Page Listing licensed acupuncturists in each state, www.acupuncture.com

The Homeopathy Home Page www.homeopathy.com

Nutritional Web Site www.nutrition.about.com

About the Author: Dr. Marjama-Lyons received her bachelors degree in psychology from the University of North Carolina in Chapel Hill and her medical degree from S.U.N.Y Health Sciences Center in Syracuse, NY. She completed her internship at the University of Rochester and her neurology residency at the University of Arizona followed by a fellowship in Parkinson’s disease at Kansas University. She was assistant professor and medical director of The Parkinson Center at the University of Florida in Jacksonville, and currently is regional director of PADRECC (Parkinson Disease Research Education and Clinical Center) at the Albuquerque VA Hospital, Director of the NPF Parkinson Outreach Program serving Navajo persons with Parkinson’s and medical director of a new Deep Brain Stimulation program in Albuquerque. She is co-author of What Your Doctor May Not Tell You About Parkinson’s Disease: A Holistic Program for Optimal Wellness published by Warner Books (February 2003) and author of the National Parkinson Foundation Medication and Deep Brain Stimulation Manuals. She is dedicated to patient education and believes in a holistic approach to care and life.

(Note from Robin: The author’s name was misspelled in the APDA Newsletter Spring ’06.)

3 Forms of PSP – RS, PSP-P, and PPFG

The Society for PSP had an International PSP Resarch Symposium on 11/17/05, right after the annual meeting of the Society for Neuroscience in Washington, DC.  The latest issues of the PSP Advocate has an interesting series of articles on some of the presentations made by scientists at the Symposium.

One scientific abstract I found worthwhile was written by David Williams, MBBS, FRACP, of the Queen Square Brain Bank (QSBB), the Sara Koe PSP Research Center, etc., London.

Dr. Williams said that clinicians can use some key differences between PD and some types of PSP in differentiating PD from some types of PSP in a clinical setting.  Specifically, the “timing of falls, the presence of visual hallucinations and testing of smell should be helpful in distinguishing PSP-P and PPFG from PD.”  (PD patients start falling later, nearly half have sustained visual hallucinations, and a decreased sense of smell.)

Also, he identified three distinct types of PSP:

  • Richardson’s Syndrome (RS):  early onset of postural instability and falls, gaze difficulties, and dementia.  54% of cases in the study.
  • PSP-parkinsonism (PSP-P):  non-symmetrical symptom onset, tremor, and a moderate initial therapeutic response to levodopa.  32% of cases in the study.  Obviously this type is often confused with Parkinson’s Disease.
  • Primary progressive freezing gait (PPFG):  early onset of gait freezing without other neurological signs. 4% of cases in the study.

Here are some excerpts from the scientific abstract about survival time and other symptoms:

“…We have recently identified two apparently distinct clinical types of PSP by analyzing clinical case notes of patients with a pathological diagnosis, archived at the Queen Square Brain Bank, London. We have named these two clinical types: Richardson’s syndrome (RS) and PSP-Parkinsonism (P).

Disease duration in RS was shorter (average 5.9 vs. 9.1 years), falls occurred earlier and age at death was younger (72.1 vs. 75.5 years) than in PSP-P. …

Primary progressive freezing gait (PPFG) … had longer disease duration (average 12.8 years) and late onset of gaze palsy and dementia compared with RS.

Other clinical analysis at QSBB has shown that sustained visual hallucinations are exceedingly rare in PSP (3%), but relatively common in Parkinson’s disease (PD, 49%). A decreased sense of smell is usual in Parkinson’s disease but not common in PSP. The time from symptom onset to first fall is early in PSP: 57% of patients with PSP fall within two years, 
compared to 6% with PD. The timing of falls, the presence of visual hallucinations and testing of smell should be helpful in distinguishing PSP-P and PPFG from Parkinson’s disease in the clinic…”

You can find this scientific abstract and the related lay abstract along with all the other articles online at:

www.psp.org/media/pdf/1q2006.pdf

Robin

 

“Foiling Chemical Event May Combat Brain Breakdown”

This post is about an interesting article on preventing misfolded protein accumulations (which occurs in AD, PD, and many related diseases), protecting an enzyme (called called protein disulphide isomerase), and stopping a chemical (nitric oxide) from wreaking havoc in the brain.

The Bloomberg News article talks about a study just published in the journal Nature. The aarticle notes:

“While previous studies showed that nitric oxide can be involved in the degeneration of brain cells through a variety of mechanisms, the new study shows a previously unrecognized relationship between the chemical and protein misfolding.”

Here’s a link to the article:

www.bloomberg.com/apps/news?pid=10000082&sid=avdCh4_MT55U

Foiling Chemical Event May Combat Brain Breakdown, Study Says
Bloomberg.com
May 24, 2006

“A Surprising Clue to Parkinson’s” (MIT Technology Review)

I saw an article in the May/June ’06 issue of Technology Review, an MIT publication, related to protein aggregations in those with PD and other diseases. What I found interesting was the fact that there is not scientific agreement over whether abnormal protein aggregations in the brain are a bad thing. The author says that the MIT biotechnology study demonstrates “that increased protein aggregation can improve the health of cells modeling neurodegenerative disease.”

That led to a related article published on the Technology Review’s website on 3/7/06. Excerpts from that March article:

“It’s easy to distinguish a healthy person from a person with advanced Parkinson’s: the Parkinson’s sufferer has large clumps of proteins in his or her brain cells. Scientists disagree, however, about the nature of these clumps. Some contend that they lead to the massive neuronal cell death and resulting movement disorders in Parkinson’s, others that the clumps are merely a byproduct of the disease — and some scientists contend that the clumps are actually protective, sequestering toxic proteins so they can’t hurt the cell. Mounting evidence points to the last possibility.”

“Existing research already suggests that the biggest clumps, known as inclusions, are helpful. Cells that form clumps of the mutant Huntington protein, for example, survive longer than clump-free cells. Now MIT scientists have discovered a compound that increases clumps in cell models of Huntington’s and Parkinson’s disease and makes the cells healthier. Scientists aren’t sure how the compound works, but they think it might be helping cells get rid of toxic forms of the proteins floating around in the cell by isolating them into clumps.”

Here’s a link to the older article:

www.technologyreview.com/read_article.aspx?id=16517&ch=biotech

A Surprising Clue to Parkinson’s
Drugs that boost the protein clumping that occurs during neurodegenerative disease could provide a new route to treatment.
Technology Review (MIT)
By Emily Singer
March 7, 2006

And a link to the newer article:

www.technologyreview.com/read_article.aspx?ch=biotech&sc=&id=16825&pg=2

A Curious Clue to Parkinson’s
Drugs that boost protein aggregation could provide a new route to treatment
Technology Review (MIT)
May/June 2006

Journal article on MRIs to distinguish PSP, MSA, and PD

This article in the Movement Disorder Society magazine (of the UK) is on using MRIs to distinguish between Progressive supranuclear palsy (PSP), Multiple system atrophy (MSA), and Parkinson’s disease (PD). Apparently the technique is most (only?) effective in later stages. This was published online on 4/6/06. The abstract is below.

Robin

——————————–

Regional brain volumes distinguish PSP, MSA-P, and PD: MRI-based clinico-radiological correlations
Dominic C. Paviour, MRCP, Shona L. Price, BSc, Marjan Jahanshahi, PhD , Andrew J. Lees, MD, FRCP , Nick C. Fox, MD, FRCP

Progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) are neurodegenerative disorders, each with a prevalence of around 5 per 100,000. Regional brain atrophy patterns differ in the two disorders, however, and magnetic resonance imaging is sometimes helpful in distinguishing them in the later stages. We measured whole brain and regional volumes, including cerebellum, pons, midbrain, superior cerebellar peduncle (SCP), and ventricular volumes as well as frontal and posterior-inferior cerebral regions in 18 subjects with PSP, 9 with MSA-P (parkinsonian phenotype), 9 with Parkinson’s disease (PD), and 18 healthy controls. Associations between these volumes, cognitive profiles, and clinical measures of disease severity and motor disability were assessed. Mean midbrain volume was 30% smaller in PSP than in PD or controls (P < 0.001) and 15% smaller than in MSA-P (P = 0.009). The mean SCP volume in PSP was 30% smaller than in MSA-P, PD, or controls (P < 0.001). Mean cerebellar volumes in MSA-P were 20% smaller than in controls and PD and 18% smaller than in PSP (P = 0.01). Mean pontine volume in MSA-P was 30% smaller than in PD or controls (P < 0.001) and 25% smaller than in PSP (P = 0.01). Motor disability was most strongly associated with midbrain volume, and more severe executive dysfunction was associated with reduced frontal volume. These distinct patterns of cortical and subcortical atrophy, when considered together rather than independently, better differentiate PSP and MSA-P from each other and also from healthy controls. © 2006 Movement Disorder Society

“Applause sign” to diagnose PSP?

The “applause sign” test is useful in diagnosing progressive supranuclear palsy (PSP), according to recent research.  The “applause sign” is where you ask someone who might have PSP to clap.  While clapping, you tell them to stop.  The person with PSP continues to clap; it takes them awhile to stop.

In a study done by Dubois, 30 out of 42 patients diagnosed with PSP could not stop applauding immediately after being told to stop.  None of those with frontotemporal dementia (FTD) or Parkinson’s Disease (PD) had trouble stopping.

So I guess the part of the brain that controls this process is not affected by PD or FTD…?  The “applause sign” is an indication of “motor perseveration.” Perhaps this is controlled by the frontal lobe so interesting that it’s not affected in FTD.

I’ll ask the neurologist to do this in Dad’s next appt.  I’m interested in seeing this.

Copied below is part of the abstract of the Dubois article (published 6/05 in Neurology) from PubMed (pubmed.gov).

Robin

————————

“Applause sign” helps to discriminate PSP from FTD and PD

The “applause sign” is a simple test of motor control that helps to differentiate PSP from frontal or striatofrontal degenerative diseases. It was found in 0/39 controls, 0 of 24 patients with frontotemporal dementia (FTD), 0 of 17 patients with Parkinson disease (PD), and 30/42 patients with progressive supranuclear palsy (PSP). It discriminated PSP from FTD (p < 0.001) and PD (p < 0.00). The “three clap test” correctly identified 81.8% of the patients in the comparison PSP and FTD and 75% of the patients in the comparison of PSP and PD.

NIH/NINDS Creatine Study Press Release

If you are interested in research going on in the Parkinson’s world that might affect the atypical parkinsonism disorders, this post may be of value to you.

The National Institute of Neurological Disorders and Stroke (NINDS) of the National Institutes of Health (NIH) has organized a nationwide effort called NET-PD (Neuroprotection Exploratory Trials in Parkinson’s Disease), a randomized, double-blind futility trial, to study compounds that may slow the clinical decline of Parkinson’s disease.  Creatine and the antibiotic minocycline were identified as agents worthy of preliminary study.

In a press release on the study that was released in conjunction with the World Parkinson Congress in Feb ’06, the lead investigator said that a “clinical trial with 200 Parkinson’s disease patients has shown that creatine and minocycline may warrant further consideration for study in a large trial.”

Here’s a link to the press release titled
“Preliminary Results Shows Creatine and Minocycline May Warrant Further Study in Parkinson’s Disease”:

www.ninds.nih.gov/news_and_events/press_releases/pressrelease_parkinsons_20060223.htm

The bottom line is this:

“The trial investigators note that while encouraging, this pilot study does not have sufficient numbers of patients or duration of follow-up to recommend that patients with Parkinson’s take either agent.  In fact, the investigators caution Parkinson’s patients and their physicians not to interpret the results of this study as suggesting such a course of treatment.  Further study is required before the researchers can conclude whether creatine or minocycline is in fact helpful, harmful or has no significant impact.”

Robin

Points from an expert physical therapist – on PD and parkinsonism

I attended Marilyn Basham’s presentation this afternoon on “Caregiving Made Easy for Parkinson’s Individuals.”  She’s the physical therapist (PT) at The Parkinson’s Institute (TPI).  I picked up a few tidbits at the presentation that I thought I’d pass along.  As the presentation was focused on Parkinson’s Disease (PD), not everything applied to the situations we are dealing with but there were still many interesting points that apply.

Here are the points I found interesting….  (with some of my comments in parantheses)

People with PD and Parkinsonism MUST use a walker or wheelchair to make them as safe as possible.  It’s very important to have mobility and postural strategies worked out with a physical therapist and/or neurologist.

PD is evident when 60-80% of the cells in the basal ganglia have died.

The “automatic motor programs” we have are stored in the basal ganglia.  One of these “programs” is what tells us that to stand up from a low chair, we need to scoot to the edge, put our feet underneath us, lean forward, and push up.  PD folks must either receive cues as to the steps of these programs, or they must practice it so many times that doing it becomes somewhat automatic again.

To overcome freezing (called “gait initiation failure”), you can put masking tape on the floor to provide a visual cue.  Put the tape at thresholds or where ever the person often has the freezing problem.  (Of course this won’t work for those with PSP who have downward gaze palsy.)

A suggested verbal cue to give someone who wants to speak is:  “Swallow.”  (pause to let the person swallow)  “Take a deep breath in and then, at the top of your breath tell me what you want.”  (pause to let this happen)  Swallowing is important because fluid accumulates in the back of the throat and those with PD are not aware of it.  You can give them gum to initiate a swallow response.

Before someone with MSA (or PD with blood pressure fluctuations) stands up, give them a glass of water with salt in it or Gatoraid.  This will increase the blood pressure.  Obviously the person’s diet and blood pressure medication needs to be taken into account before following this suggestion.

We must give time for those with these diseases to process information!  Be patient!  Give long pauses.  Don’t overload them.  Don’t give them more than one complex task at a time.  Walking is a complex task.

(Some of you know that my father and I communicate by our holding up fingers to designate an answer.  Example, “do you want 1 for coffee, 2 for tea, or 3 for nothing,” and I hold up 1, 2, and 3 fingers.  He answers by holding up fingers.  Long after the fingers come up, he may try to verbalize the answer.)  I asked Marilyn why my father could hold up fingers faster than he could verbalize a response.  Marilyn said she didn’t know why but pointed out that parents of small children teach their children sign language long before the children can verbalize.

Dementia is rare in PD.  (It’s definitely common in the Atypical Parkinsonism diseases.)  PD folks may lose their keys but they still remember what keys are and how to use them.  (I thought that was a good story for remembering what dementia is.  My dad, for example, cannot remember how to use an ATM card.  I see the dementia very clearly.)

A patch for Sinemet is in the works.  (Some of your loved ones take Sinemet.)

The head of TPI thinks that PD is the most curable of all the neurodegenerative diseases.  (Let’s hope he’s right because hopefully those diseases related to PD can be cured quickly too.)

Regards,
Robin