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

—————————–

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.

LBDA on Surgery/Anesthesia

I saw this second post last week by Angela Taylor, the president of the Lewy Body Dementia Association, in a couple of places. She mentions these medications for which I’m adding the brand names (in parentheses): donepezil (Aricept), rivastigmine (Exelon), and galantamine (Razadyne, which used to be called Reminyl).

Robin
———————

Anesthesia and LBD
Posted by: “jaektaylor”
Date: Thu Jan 11, 2007

We’ve all talked about how our loved ones respond poorly to most
anesthesia. The following verbiage is going to be included in the
next version of the LBDA’s informational pamphlet. Here it is for
you, before it’s even ‘hot off the presses!’

“When considering any surgery, caregivers should meet with the
anesthesiologist in advance. People with LBD often respond to certain
anesthetics and surgery with acute confusional states (delirium)
and/or may have a precipitous drop in functional abilities which may
or may not be permanent. The pros and cons of stopping donepezil,
rivastigmine, or galantamine should be carefully considered. If a
spinal block or regional block can be used instead of general
anesthesia, this would be preferred as those methods are less likely
to result in postoperative confusion.”

LBDA on Excessive Daytime Sleepiness + Use of Provigil

I saw this post last week by Angela Taylor, the president of the
Lewy Body Dementia Association, on the LBDcaregivers online
discussion group. The topics are excessive daytime sleepiness and
the use of the medication Provigil.

Robin

———————

Treating excessive daytime sleepiness/Provigil
Posted by: “jaektaylor”
Date: Thu Jan 11, 2007 6:02 am ((PST))

….The LBDA’s Scientific Advisory Council is also writing a short
overview on the issue of excessive daytime sleepiness, and the use
of psychostimulants like Provigil. The paper isn’t ready for
release to the public yet, but here are a few things that I’ve come
to understand as a lay caregiver that may be of help.

1. The use of psychostimulants does not appear to be a common
treatment in LBD, and should be done with caution. A neurologist
who specializes in sleep disorders might be the best person to
manage this type of medication.

2. There are a number of reasons that patients may experience
excessive sleepiness, which should be addressed BEFORE considering
using psychostimulants, like depression, delirium, inactivity, etc.

3. With the use of psychostimulants, there are some risks of
increased psychosis in some patients. Fluctuating pulse and blood
pressure and falls is also a risk.

4. A full sleep evaluation is recommended before using
psychostimulants to address what other sleep disorders may be
contributing to the problem, that wouldn’t be resolved by the use of
a psychostimulant.

Hope that helps,
Angela

“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).

Robin

————————

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.

New Zealand Pamphlet on MSA

On the Shy-Drager online support group today, Vera posted a link to a pamphlet done by the Parkinsonism Society of New Zealand on multiple system atrophy (MSA).  The link is:

www.parkinsons.org.nz/books/msa%20pamphlet.pdf

Editor’s Note: Link is no longer active

This short pamphlet would be something you could send to family members to provide a general introduction to MSA.  Or it could be given to new caregivers.

Copied below is most of the inside page of the 2-page New Zealand pamphlet that gives details on MSA.

Robin

—————————————–

What is MSA?
Multiple system atrophy (MSA) is a rare
progressive neurological disorder presenting
with similar symptoms to Parkinson’s disease.
The condition is marked by a combination of
symptoms affecting movement, blood
pressure, and other body functions; hence the
label multiple system atrophy.

Various forms of MSA
Symptoms of MSA vary from person to
person. Because of this, three different
diseases were initially described to encompass
this range of symptoms: Shy-Drager
syndrome, striatonigral degeneration, and
olivopontocerebellar atrophy.

What causes MSA?
The cause of MSA is unknown.

Symptoms of MSA?
MSA can cause a wide range of symptoms,
including:
.. orthostatic hypotension, or a significant fall
in blood pressure when standing, causing
dizziness, lightheadedness, fainting, or
blurred vision
.. male impotence
.. loss of control of bowel or bladder
.. stiffness or rigidity
.. freezing or slowed movements
.. postural instability; loss of balance; lack of
coordination
.. speech and swallowing difficulties blurred
vision
.. changes in facial expression.

Who gets MSA?
MSA usually starts between the ages of 50-60
years, although it can affect people younger and
older than this. Around 4 in 100,000 people
are affected by MSA in New Zealand. MSA
does not appear to be hereditary and is not
infectious or contagious. It is a sporadic
disorder that occurs at random.

How is MSA diagnosed?
Often symptoms are vague and diagnosis is
difficult. MSA is often mistaken for Parkinson’s,
especially in the early stages of the condition.
Diagnosis should be made by a specialist,
usually a neurologist.

What is the treatment?
Currently there is no specific treatment for
MSA. A variety of medications, including some
drugs used for Parkinson’s, and other forms of
therapy can help control the symptoms.
Treatment may focus on alleviating the
symptoms, so people with MSA could benefit
>from working with physiotherapists, speech
therapists, dieticians, continence nurses, and
occupational therapists.

What is the prognosis?
MSA is a progressive disorder, the rate of
progression differs in every person.

To the person with MSA and their
family
The diagnosis of MSA has significant impact
on those close to the patient. As the
condition progresses so does the need for
care. Carers/families may feel isolated,
frustrated and chronically tired. Infinite
patience is needed. Carers/families will
need support and should utilise support
services. Support services can be contacted
directly or referral can be made through
your doctor or health professional.