Category Archives: Parkinson’s

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:

www.pdf.org/en/spring07_gastrointestinal_and_urinary_dysfunction_in_pd

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.

Robin

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Excerpts from:

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

Dysphagia
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.

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 pubmed.gov for this abstract)

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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.”

Robin

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

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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.