“Physician’s Guide to PSP” (Mentions of CBD and MSA)

This video guide to medical professionals for diagnosing progressive supranuclear palsy (PSP) is well worth watching.  Note that multiple system atrophy (MSA) is mentioned (around 12:30) as well as corticobasal degeneration (CBD) (around 12:40) along with a description of alien limb syndrome.

This 18-minute diagnostic video, “A Physician’s Guide to PSP,” was announced a few months ago by CurePSP, the new name for the Society of PSP.  You can order the video on a DVD but it’s easier to find it online here:

youtu.be/IXMv919LOWE

[Editor’s note:  the above link was updated in 2012 when CurePSP moved video off its website on to YouTube.]

I found the program understandable to a layperson.

Dr. John Steele, who was one of the MDs who defined the disease PSP, is the first speaker.  There’s some interesting video of supranuclear gaze palsy.  I guess so much time is spent on this because downward gaze palsy is a hallmark symptom.  You probably don’t need a dictionary because the terms used (eg, apraxia, spastic) are described in video form.

Dr. David Williams is the second speaker.  He is one of the leading stars of PSP research and practices in Melbourne, Australia.  (He used to work with Dr. Andrew Lees in the UK.)  He talks about brain structures affected by PSP and the neuropathology of PSP.  (Don’t worry – this doesn’t last long!)

After Dr. Williams, we hear the voice of Dr. John Steele who says:

  • “A population of 100,000 could contain 1-2 diagnosed PSP cases and 4-5 undiagnosed”
  • “This is about 5% of the prevalence of Parkinson’s Disease”
  • “The average survival in PSP is 6-8 years after symptom onset”
  • “This is about 2 years less than for Parkinson’s Disease before the L-dopa era”

Dr. Lawrence Golbe is the third speaker.  He indicates that the facial expression, gait, and speech of PSP are unusual.  He says that the speech of PSPers (spastic and ataxic) is distinctive and occurs “in almost no other condition.”  He says that one of the diagnostic criteria for PSP is falls occurring within the first year.  (I’m going to have to look up again what other neurologists say about that.  My dad did have falls this early but I didn’t think everyone did.)  He always orders a brain MRI.  He sometimes orders a SPECT.

The fourth speaker, Dr. Andrew Lees, notes that the arm swing is often preserved in PSPers.  This is unusual and dissimilar from Parkinson’s Disease (PD). He talks about blepharospasm.  He says that eye movements form an important part of the neurological exam.  An eye exam is shown.

Dr. Lees notes that as part of taking a patient’s history, it’s important to ask about previous history of encephalitis.  History of visual hallucinations, psychosis, and memory loss would indicate the patient may have Dementia with Lewy Bodies.  As part of the physical exam, it’s important to exclude autonomic dysfunction.  Symptoms of autonomic dysfunction are more commonly associated with PD and MSA.  Dr. Lees says that CBD should be considered if there is marked asymmetry of symptoms (such as jerky, tremulous movements in one limb) or alien limb.

The video is also intended for families.  It was produced by CurePSP and the PSP Society of Europe, hence the combination of US and UK researchers.

Here’s a description of the video from the CurePSP website:

“NEW!  A Physician’s Guide to PSP – Diagnostic DVD – An important resource for neurologists, family physicians, and medical professionals created by the PSP Europe Association in the United Kingdom and CurePSP in the United States.  This 18 minutes long video features commentary by top neurologists specializing in PSP, including Lawrence I. Golbe, MD, John C. Steele, MD, and Andrew Lees.”

“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.

Warning for those with dementia about anticholinergics

Like Alzheimer’s Disease (AD), those with many other types of dementia have an imbalance of acetylcholine in the brain.  Anticholinergic drugs can be problematic for those with AD and non-AD dementias.

I saw this Q&A recently in my Dad’s AARP Health Care Options newsletter called fyi.

Robin

——————————

Ask Dr. Reed ([email protected])
AARP Health Care Options fyi (newsletter)
Fall 2006

Question:  My husband has Alzheimer’s disease.  His pharmacist told me that certain medicines could further worsen his memory problems.  Any advice?

Answer:  …You are wise to take steps to ensure that your husband’s mental status is not worsened by the effects of his medicines.  As we have mentioned in previous columns, many of us become more sensitive to medicines as we age.  As a result, a variety of medicines could produce unanticipated effects that could worsen mental status and overall function.

Alzheimer’s disease is characterized by low levels of a chemical that transmits signals between nerves called “acetylcholine.”  As a result, medicines called “anticholinergic” drugs that block the effects of this nerve chemical can be especially problematic for people with Alzheimer’s disease.  Unfortunately, these drugs are very common.  They include:

* Certain antihistamines such as diphenhydramine (Benadryl)
* Certain antidepressants such as amitriptyline (Elavil) and doxepin (Sinequan)
* Medicines for bladder problems such as oxybutynin (Ditropan)
* Muscle relaxants such as carisoprodol (Soma), cyclobenzaprine (Flexeril), and methocarbamol (Robaxin)

I always say to write down the name of every medicine that you or your loved one is taking, and review this list regularly with your doctor and your pharmacist…

Warning for those with dementia about anticholinergics

Like Alzheimer’s Disease (AD), those with many other types of dementia have an imbalance of acetylcholine in the brain.  Anticholinergic drugs can be problematic for those with AD and non-AD dementias.

I saw this Q&A recently in my Dad’s AARP Health Care Options newsletter called fyi.

Robin

——————————

Ask Dr. Reed ([email protected])
AARP Health Care Options fyi (newsletter)
Fall 2006

Question:  My husband has Alzheimer’s disease.  His pharmacist told me that certain medicines could further worsen his memory problems.  Any advice?

Answer:  …You are wise to take steps to ensure that your husband’s mental status is not worsened by the effects of his medicines.  As we have mentioned in previous columns, many of us become more sensitive to medicines as we age.  As a result, a variety of medicines could produce unanticipated effects that could worsen mental status and overall function.

Alzheimer’s disease is characterized by low levels of a chemical that transmits signals between nerves called “acetylcholine.”  As a result, medicines called “anticholinergic” drugs that block the effects of this nerve chemical can be especially problematic for people with Alzheimer’s disease.  Unfortunately, these drugs are very common.  They include:

* Certain antihistamines such as diphenhydramine (Benadryl)
* Certain antidepressants such as amitriptyline (Elavil) and doxepin (Sinequan)
* Medicines for bladder problems such as oxybutynin (Ditropan)
* Muscle relaxants such as carisoprodol (Soma), cyclobenzaprine (Flexeril), and methocarbamol (Robaxin)

I always say to write down the name of every medicine that you or your loved one is taking, and review this list regularly with your doctor and your pharmacist.

“Understanding Difficult Behaviors”- recommended book

This post might be of interest to those who have loved ones with dementia — all of the LBD, some of the PSP, and some of the CBD (especially late stage) folks…

There are a couple of great books with practical suggestions on how to cope with Alzheimer’s Disease and similar illnesses. One is “Understanding Difficult Behaviors” by Anne Robinson, Beth Spencer, and Laurie White, 1989, published by Eastern Michigan University.

You can purchase the book at local offices of the Alzheimer’s Association, which are in Mountain View, Lafayette, San Rafael, Sacramento, Santa Cruz, etc.

The difficult behaviors this book deals with are: angry, agitated behavior; hallucinations, paranoia; incontinence problems; problems with bathing; problems with dressing; problems with eating; problems with sleeping; problems with wandering; repetitive actions; screaming, verbal noises; and wanting to go home.

Copied below are some excerpts from the four-page section on “Screaming, Verbal Noises.”

Robin


 

Excerpts from

“Screaming, Verbal Noises”
in
Understanding Difficult Behaviors

by Anne Robinson, Beth Spencer, and Laurie White
1989
Published by Eastern Michigan University

POSSIBLE CAUSES

Physiological or Medical Causes
* hunger
* incontinence (wetness, etc)
* need to go to the bathroom
* fatigue
* need for help changing position in bed or wheelchair
* vision or hearing loss that causes misperception of the environment
* impaired ability to speak or be understood
* acute medical problems that result in feeling ill or pain and discomfort

Environmental Causes
* too much noise
* overstimulation or sensory overload
* use of physical restraints
* upset by behavior of other residents

Other Causes
* procedures which are uncomfortable or not understood, such as having an enema, having a dressing changed, being catherized, etc.
* bathing – person may be cold or feel exposed
* dressing – person may be cold or feel exposed
* purpose of mouth care not understood
* touch/turning/repositioning – uncomfortable or not understood
* fear/anxiety
* feeling threatened
* need for attention
* frustration
* boredom/lack of stimulation

COPING STRATEGIES

* Have a good medical evaluation to check for illness, infections, pain/discomfort, or impaction

* Provide adequate meals/snacks to minimize hunger

* Institute regular toileting schedule to minimize incontinence

* Change promptly after incontinent episodes

* Try rest periods to minimize fatigue

* Make sure there are frequent (at least every 1-2 hours) position changes if person is bedridden or restrained in chair

* Maximize sensory input. (Check to see whether hearing aids and eyeglasses are in place and working properly.)

* Lower stress; create a relaxing environment:
– minimize noise
– avoid overstimulation/sensory overload
– avoid use of restraints
– play soft, soothing music

* Use relaxation strategies to minimize fear, threat, anxiety. For example:
– try massage/therapeutic touch, stroking person’s head, arms, hands
– try placing your arms around the person and gently rocking back and forth
– talk in a soothing voice
– play soft, soothing music or soothing sounds such as tape of rainfall, waves breaking on shore, etc.

* Try these communication suggestions:
– approach person with soothing voice; call person by his/her name; identify yourself
– explain/prepare person for what is to be done using simple, clear, short sentences
– break task into short steps briefly explaining each one
– think of other ways for the person to communicate, such as using a bell. This can enhance the person’s sense of security by feeling that he/she is able to communicate needs to caregiver

* For staff in long term care settings:
– use consistent routines for activities such as bathing, meals, getting ready for bed; keep to the same schedule each day
– identify staff who work well with certain individuals. Consistency in staffing is important.
– plan time to socialize with the person for a few minutes in addition to assisting with activities of daily living
– encourage participation in meaningful activities to minimize boredom and frustration.

* Softly read to person.

* Medication should be used cautiously when other interventions have been unsuccessful and when the vocal behavior is very stressful to the caregiver(s) and/or residents living in the area. This medication should be monitored carefully by a physician/psychiatrist.

OTHER CONSIDERATIONS

Vocal behaviors are most commonly seen in the later stages of progressive dementia. Many people who shout or cry out are physically immobile – wheelchair or bed-bound. The underlying problem is the person’s inability to communicate his/her needs, wishes, thoughts, etc.