Progressive Supranuclear Palsy

I've just started a community job and am having to learn more about progressive conditions. Here's some info on PSP.

PSP

• Neurodegenerative parkinsonian disorder.
• Typically presents in middle/late age with progressive unexplained postural instability, falls, supranuclear gaze palsy, pseudobular palsy and mild cognitive disturbances.
• Cause: progressive damage to the basal ganglia and brainstem.
• Begins slowly and leads to continuous decline.

Observations

• Personality changes (apathy/forgetfulness/irritability/lability)
• Blurring of vision, problems controlling eye movement e.g. problems looking down/ with eye contact.
• Slurred speech and dysphagia (often more severe than in Parkinsons).

Dysphagia

• Pneumonia = the most common cause of death
• People may live up to 10 years with proper interventions.

Leopold et al (1997) Dysphagia in progressive supranuclear palsy: radiologic features, dysphagia, 12(3), 140-3.

Key features

• Uncoordinated lingual movements
• Absent velar elevation/retraction
• Impaired lingual displacement
• copious pharyngeal secretions
• non cohesive lingual transfer
• oral bolus leakage into the pharynx
• significant vallecular residue
• reduced oral sensation

Key observations

• Difficulties chewing/ with the oral stage of swallowing mean that softer foods may be easier. 85% of the patients with PSP had oral stage difficulties. 
• Most of the patients had pharyngeal stage problems which may have been caused by the oral stage impairments e.g. pharyngeal overspill.

Example Recommendations

• Avoid mixed textures/ hard chewy foods.
• Decrease distractions
• Put the plate high up if the person cannot look down.
• Encourage a clearing cough post swallow
• Make the patient more aware of the oral stage e.g. write Step 1, 2, 3, 4.

Observations from a videofluoroscopy of a patient with PSP

• decreased coordination of lingual movements, especially the base of tongue. Pre-swallow loss of bolus'.
• +++ vallecular residue on soft food (banana + yogurt) but no aspiration.
• Small amount of aspiration on thin fluids when taken after eating.
• No aspiration on syrup thickened fluids taken after eating - this helped clear the vallecular residue.

Recommendations from the VF

1. Syrup thick fluids at mealtimes.
2. Sips of thin fluids throughout the day (for QOL)
3. SOft mashed diet.

Case Study: Apraxia

Here is one of the most successful therapy interventions i have planned. Initially i tried to set goals around gesture use, picture symbol use and comprehension. As i observed the client in OT and PT sessions it became clear that their apraxia of gestures and of speech made their comprehension appear more severely impaired on impairment based assessment than in reality. Once I set goals around speech, which was motivating for the client he progressed very quickly.

History

·       60, left MCA infarct, LPCA infarct affecting the left frontal lobe and left parietal lobe, history of alcohol excess and cannabis abuse. The patient was very musical, played 3 instruments and enjoyed singing. One nephew visited the patient. Previous SLT report – Comprehension- unable to consistently select the correct pic from verbal stimuli. Diff following simple instructions. Expression – ‘yes’ to all Q’s, no other verbal output, inaccurate pointing.

Assessment

·       Object to pic matching – able to do with everyday objects. Yes/ No response to orientation questions/ simple questions in context – patient was inconsistent due to ideational apraxia and apraxia of speech. Best Yes/no interpretation = from body language and tone of voice.

·       OT/ PT joint sessions – patient was able to follow simple instructions in context. Difficulties copying actions.

·       Word repetition/ apraxia assessment – patient presented with severe apraxia of speech.

·       Language screen – groping for words expressively, unable to follow complex directions e.g. 3-stage (thought to be due to apraxia of gestures). Pre-morbid literacy impairments.

Goals

Long term (0ver 2.5 months)

I will be able to consistently communicate my basic needs by using total communication strategies (pictures, gestures, drawing).

I will be able to spontaneously use 5 functional words in context.

Short term

I will be able to communicate a consistent Yes/No response when prompted to use a communication aid.

I will be able to use pictures to communicate functionally during a kitchen/ wash and dress task.

I will be able to consistently understand picture symbols relating to my everyday needs.

Smart goals

I will be able to imitate the following sounds consistently when given visual prompts: ‘mm’ ‘me’ ‘yo’ ‘yes’ ‘no’.

I will be able to spontaneously say ‘yes’ or ‘no’ appropriately when asked an appropriate question.

I will be able to consistently use 5 topic cards (Body, family, therapy, food, activities) with trained members of staff to communicate a piece of information.

I will be able to use ten functional words (tea, coffee, Bob Marley, physio, pain, medication, Thomas, toilet, shower) consistently and spontaneously in context.

I will be able to say 10 functional phrases within specific contexts (e.g. breakfast group, physiotherapy, with the doctors…).

My nephew will be able to identify strategies (using topic cards, yes/no questions, pauses…) to support me to communicate, during a short conversation.

Therapy Activities

1.     Joint sessions with the OT/ PT around comprehending instructions and responding consistently to yes/ No questions.

2.     Apraxia Exercises – starting with vegetal sounds/ sounds that were easy for the patient to make and using topping to facilitate speech. Phonemic prompts and finally modelling of the word were used as a step down.

Yawn (open mouth wide)	‘ah’
Hum (keep mouth closed and vibrate)	‘m’
La la (singing) (curl tongue up and down)	‘l’
Bite lower lip	‘f’
Damming the lips	‘p’
Tut tut	‘t’

Yeah	·       Tap (extended) ·       ‘y’ ‘air’
Oh Gosh	·       tap,  tap (extended) ·       O  gosh
Hello	·       tap tap ·       cough (humpf), laugh ·       he  lo
Yes	·       tap (extended) ·       yeah ·       yeah sss
No	·       tap ·       ‘nnn’ ‘ooo’
Good	·       Tap ·        ‘g’ ‘ood’
Got	·       Tap ·        ‘g’ ‘ot’
Gone	·       Tap (extended) ·        ‘g’ ‘one’
Bye	·       Tap ·        ‘b’ ‘I’
Down	·       Tap ·        ‘d’ ‘own’

3.     Apraxia exercises – semantic clues e.g. you might drink it, pictures and carrier phrases were used to elicit chosen functional words/ phrases. The patients accent was considered e.g. ‘th’ was difficult so ‘d’ was used instead, as with the Caribbean accent.

4.     Topic card use in supported conversations.

5.     Joint sessions with the psychotherapist  - talking about motivating topics e.g. family, coming to the U.K., spiritual beliefs, dealing with a stroke.

Outcomes

-        Consistent yes/ no responses for simple questions.

-        Some functional word/ phrase use in context.

-        Some spontaneous sentences/ and carry over to words not practised in therapy.

Referrals

·       Referral made to community services with potential goals around facilitated conversation with the patient’s nephew and functional communication goals around shopping etc.

Gesture and apraxia

Mengotti, P et al (2003) Selective imitation impairments differentially interact with language processing, Brain, 136, 2602-2618.

Introduction

• Ideational apraxia - patient struggles to come up with a plan/ idea for how to use objects (sequencing may be incorrect..)
• Ideational apraxia - difficulties translating an idea into movement e.g. with imitation.

Methods

• 57 patients with damage to their dominant left-hemisphere were tested on: 1) ability to process language, 2) Use objects functionally, 3) imitate meaningful gestures, and 4) imitate meaningless gestures.

Results

• The patients' ability to imitate meaningful gestures and their performance on naming/ repetition tasks appeared to be related. Whereas their imitation of meaningless gestures was correlated to the patients' comprehension.

Conclusion

• Imitation of familiar gestures relies on the language system because these gestures are linked to the corresponding lexical semantic representations.
• The angular gyrus is important for gesturing.

How does this relate to practice?

• Meaningless gestures - if a patient is unable to imitate meaningful gestures perhaps you could try new gestures which you then develop new representations for e.g. fidt/palm for yes/no.
• Increase in the use of meaningful gestures may be a positive diagnostic sign for naming/ language.
• There are two separate pathways for gesture imitation (one via semantics and one not).
• Working on gesture/ total communication may positively impact language/ naming.

Tongue/ oral control ex's

TONGUE

1.     Open your mouth and poke out your tongue.  Be sure your tongue is straight out, not resting on your lips or pointing to either side.  Hold this position for 2 seconds.  Practise slowly 10 times.

2.     Open your mouth and this time pull your tongue right to the back of your mouth.  Practise this slowly 10 times.

3.     Poke out your tongue and move it slowly from corner to corner of your lips.  Practise this slowly 10 times.

4.     Poke out your tongue and move it from corner to corner as quickly as you can be sure to check that it is moving right into each corner.  Try this 5 times.

5.     Poke out your tongue and point it down towards your chin.  Hold it there for 2 seconds.  Practise this slowly and carefully 10 times.

6.     Poke out your tongue and try to point it up towards your nose.  Hold it there for 2 seconds.  Practise this slowly 10 times.

7.     Lick your lips slowly and carefully.  Practise this 10 times.

8.     Put your hand on your cheek (your stronger side).  Now put your tongue in your cheek and push against your hand.  Practise this 5 times. Now try exactly the same thing but on your weaker side.

9.     Practise clicking your tongue off the roof of your mouth. 

Do this 10 times.

Tongue/ oral control exercises

-       Chewing ice/ moving around in mouth.

-       Moving a polo on a string in the mouth

-       Use a spoon as a reference point – touch the spoon with your tongue.

Stroke Unit Reflections

I'm leaving the stroke unit for a community job in another trust. Here are some of my final reflections on different aspects of working on a stroke unit. I definately enjoy the MDT atmosphere and mixture of acute and rehab patients, i'll be back....

Caseload

Patient

SLT

Team

Diagnosis/ relevant/ discharge information

Current swallowing goals treatment plans and progress

Current communication goals/ treatment plans and progress

How frequently should they be seen and by SLT or TA?

-        Form updated by SLT’s on an open drive, so that for MDT meetings/ when people were away it was clear where the patient were in terms of rehab.

-        Weekly timetabling meeting – tick off who is seeing which patient + can see if any patients were missed.

-        Daily patient timetabling – flexible – can see new patients/ + often patients go for investigations. Joint sessions, goal planning, groups TOMS and MDM’s pre planned.

-        Try to record as much of what you do as possible

Care packages

-        Consider what service patients are given across all of the SLT’s e.g. informal screen – recommendations – further assessment – therapy. This would allow us to explain to commissioners what package of care we offer.

-        Write care plans with Long term, short term and session goals on them.

Goal Setting

-        Involving patients and their families early on in finding functional meaningful goals lead to positive outcomes.

-        Introducing goals and having an informal discussion with patients about goals was useful before then making MDT goals.

-        Accessing therapy was a good goal – i.e. ability to follow instructions/ order food/ communicate basic needs/ communicate what activities done in hospital to family/ friends/ communicate with peers.

-        Frequently reviewing SMART or short term goals was important in order to focus goals on achieving long term aims.

-        Impairment based goals needed more explanation to clients.

Family meetings

-        Prepare information beforehand and note down specific examples of behaviours e.g. patient not initiating communication.

-        Explain the SLT role and the type of interactions the patient is receiving and why.

-        Briefly explain what you may expect to find with the neurology.

AAC

-        AAC often did not work on the stroke unit due to: 1) patients continually changing/ priorities changing 2) many patients had cognitive impairment and were unable to access AAC 3) High variety of staff – difficult to get everyone to use the AAC

-        Topic cards with a main topic + picture on them and a list of mini topics on the back (attatched together by a pin) worked well with aphasic patients to support people to understand what they were trying to communicate and were easy to model.

-        Picture charts worked well to enable patient to communicate basic needs/ make orders of food.

Oesophageal/ voice patients

-        Refer to ENT/ gastro for more information before recommending oral intake/ giving voice exercises.

Low arousal patients/ Global aphasia patients

-        Monitor with the Wessex Head Injury Matrix – looking for: eye contact/ opening/ vocalisation etc.

-        Regular mouthcare, sometimes moving on to flavoured mouthcare.

-        Look for spontaneous swallows.

-        Assess Yes/No response e.g. Putney Yes/no assessment with objects

-        Joint sessions with PT/OT since these patients were most responsive when getting moved etc.

-        Communication history – find out interests etc – play music/ ask about family etc.

-        Object to object matching? Gesturing object function, picture to object matching – assess ability to use AAC – see what supports comprehension e.g. objects of reference or clear choices by holding up objects.

Locked in patients/ significant expressive aphasia/ dysarthria

-        Using a diary to record daily activities enabled me to communicate with the clients family and gave me information to use in supported conversations/ yes/no questions/ gesture therapy etc.

-        AEIOU – alphabet board with an ‘end of word’ facilitated patients to communicate complicated information.

-        Buzzer with a recorded message = simple functional way of communicating basic needs when paired with a list of yes/ no questions e.g. Toilet, back to bed, re-positioning, drink/food.

-        Yes?no – look for a consistent response and handover to the team.

Dementia patients

-        Find out baseline – may be able to make some recovery from the stroke damage if cognitively able to engage in activities.

-        History – preferred foods/ activities.

-        Assess swallowing .

-        Often finger foods / sweet flavourful foods are best e.g. jam sandwich/ yogurt.

-        Feeding guidelines – set up meal

-        Breakfast/ lunch groups often good feeding environment.

-        Do not recommend tube feeding e.g. PEG unless you think they may recover from stroke damage.

-        Communication guidelines for the ward

-        Refer to the dieticians since often variable intake

Aphasia

-        Initial communication screen – naming bedside objects, following 2-3 stage instructions, yes/no questions, automatic speech

-        The cognitive screen on the CAT was a good indicator of whether a patient would be able to access any impairment based therapy e.g. if 3minute recognition memory was impaired – unlikely.

-        The stroke handbook – patients wanted to know what had happened to them – can use pictures in the handbook + information form the doctors to explain why they had a stroke and how it might have affected them.

-        First topics – family, = good topic, work/ home = good topics.

-        Communication history from family

-        SMART session goals – record quantitative/ qualitative information during sessions.

Severe Expressive

-        Automatic speech – try to elicit any speech

-        Reading/ writing assessment

-        Activities – personalised cuing therapy, semantic associations….

Severe Receptive

-        Joint work with family/ in functional situations

Group work

Communication group

-        Varied between total communication e.g. with pictures chosen that were relevant to the patients e.g. Bob Marley, having blood taken…. And supported conversation work.
- Real life resources/ props were engaging for the patients e.g. map/tube map/ menu....

-        Topics for supported conversation: Food (menus, photo's, recipe's) , work, leisure (cinema, reading, shopping, where to go with friends), holidays (map), Geography (where from, where live, where the hospital is..), Hobbies, music (with CD's e.g. discuss/ put hand up if you like the song), Sport (pictures/ news articles).

Newspaper group

-        Newspapers used as prompts – have pictures, headings, summaries and whole articles as a hierarchy.

-        Each patient had their own goal for the group – the SLT facilitated discussions etc.

Dysarthria

-        Functional - communication – guidelines for staff – pad and paper to write keywords/sentences, ask yes/no questions, encourage them to give keywords/ single word answers, deep breath go slowly over articulate. – trial using a communication chart to communicate basic needs on the ward. Considered use of AAC e.g. lightwriter/ i-pad

-        Impairment based – work upwards with dysarthria (bottom down) therefore if someone has breathing/ respiratory difficulties you would start on these before any work on articulation/ intelligibility.  Minimal pairs work, telephone work, barrier activities, use the frenchay intelligibility section for a baseline.

-        Use a hierarchy e.g. strategies in one to one – in small group….

Apraxia of speech

-         

Cognitive Communication Disorders

•                  Group work – to increase initiation, get feedback on communication, practice turn taking etc.

•                  Create a communication passport – information about the client and their preferences and interests (used for a patient with severe cognitive impairments unable to initiate communication).

•                  Functional tasks – e.g. sequencing/ planning when making recipes in breakfast group.

•                  FAVORS – assessment for higher level functional assessment of executive skills, memory etc.

•                  LA – Trobe assessment – looks at communication behaviours and how they have changed, has a section for the client + a family member – you can combine these and discuss possible goals.

•                  Access to therapy – are the cognitive communication defits affecting therapy participation.

Communication screen

1.     Object picture matching

2.     Spoken word – object matching

3.     Written word – picture matcing

4.     Naming

5.     Repeating

6.     Informal conversation

7.     Attention/cognition

8.     Gesturing object use