1. Multiple System Atrophy
Jill Lyons
MSA Nurse Specialist

2. Multiple System Atrophy
Progressive, adult onset disorder characterised by a combination of:
Autonomic dysfunction
Parkinsonism
Ataxia
New Consensus Statement 2008 states autonomic involvement must be present

3. Who gets MSA? Adult onset mean age 52 years commonly 40-69 years
never <30years
Men to women 1.2 : 1
UK prevalence 5 per 100,000
1 MSA patient for every 20 PD
Cause unknown
Not thought to be hereditary

4. Parkinsonism Cerebellar Autonomic
Balance, Co-ordination, Speech, Swallow
Slow, Stiff, poor initiation of movement
Parkinsonism
Cerebellar
Autonomic
Erectile Dysfunction, Bladder, Blood Pressure

5. Diagnosis 60% patients diagnosed initially with Parkinson’s or Ataxia
Time to diagnosis: approx. 3 years
No definitive diagnostic tool

6. Diagnosis MRI scan (magnetic resonance imaging)
SPECT/DAT scan (dopamine in brain)
Rule out other neuro disorders
Look at drug history
Try Levodopa
Allow time for symptoms to develop
Possible/probable Diagnosis
Positive Diagnosis only if brain examined after death
SPECT Single photon emission computerised topography - looks at the dopamine in brain

7. Parkinsonism Stiffness Slowness Tremor
Difficulty turning in bed or getting out of a chair
Poor drug responsiveness
Falls
Ante-collis
Muscle weakness

8. Cerebellar Slurred Speech
Gait ataxia: difficulty standing without support
Difficulty negotiating spaces, unsteadiness
Falls
Limb ataxia, intention tremor, difficulty dressing and eating

9. Autonomic Male Erectile Dysfunction
Bladder dysfunction urgency, frequency
nocturia, retention

10. Autonomic Respiratory: inspiratory sighs stridor snoring apnoea
oxygen de-saturation

11. Autonomic Postural hypotension 30mmHg systolic on standing (15d)
dizzy, falls & fatigue
blurred vision
coat hanger pain
altered consciousness
Constipation
70% (MSAT Survey)

12. Autonomic Speech/Swallow
Low volume, monotone
Slurred, one breath delivery
Complete loss of sound production
Poor oral control
Pooling of residue
Coughing/choking/silent aspiration
Drooling of saliva

13. Cognition Emotional lability inappropriate laughing/crying
Sleep problems
restlessness & discomfort
nocturia
sleep apnoea
vivid dreams
REM sleep disorder
Cognitive Slowing
Memory not primary change

14. Red Flags Erectile dysfunction Bladder disturbance
Postural hypotension
Poor response to Levodopa
Cold extremities
Severe dysphonia, dysphagia
Inspiratory sighs/stridor
Rapid progression, wheelchair dependency

15. Prognosis Disease Duration Individual-
80% using wheelchair after 5 years
Survival from Diagnosis
3 – 9 years
20% will survive 12 years
Unpredictable

16. Management MDT Approach
Specialist
Nurse Specialist
Speech and Language Therapist
Physiotherapist
Occupational Therapist
Continence Advisory Service
Social Services
Outreach team
Hospice
Community Matron/Nurse
Specialist = neurologist, care of the elderly physician, movement disorders specialist.
Nurse Specialist = Parkinsons Nurse Specialist, Neurology nurse, Community Specialist Nurse for rare neurological conditions

17. Lifestyle Adaptation Raise the head of the bed during sleep
30 degree head-up tilt
Increase fluid & salt intake
Use calf pump exercises
Eat small meals
Control B/P e.g. fludrocortisone, midodrine
ephedrine
Avoid hot temperatures, dehydration
Abdominal binder

18. Mobility Referral to Physio and OT
Target-specific training e.g. turning in bed
Exercises on bed/chair to avoid hypotension
Plan for wheelchair dependency and hoist transfers
Get the right chair and wheelchair (reclining/tilt in space )
Functional management

19. Speech & Swallow Referral to Speech and Language Therapist
Equipment useful
High tech: amplifiers, light writers
Referral to communication aids team
Low tech: alphabet chart, picture board
Dietician: modified diet
Consideration of artificial feeding

20. Continence Referral to continence advisory service
Intermittent Catheterisation
Supra pubic
Anti-cholinergics DDAVP
Constipation / Loose stools
Early Treatment for UTI (asymptomatic)

21. End of Life Issues Discuss early on before communication difficult
Make wishes known to family/medical team
Advance directives
Resuscitation
Artificial feeding
Antibiotics
Admission to hospital
Brain donation

22. Palliative Care Patient choice Quality of Life Respite care
Symptom control
Counselling
Spiritual care
Family support

23. Cause of Death Expected Aspiration Pneumonia
Unexpected Apnoea Hypotension

24. Sarah Matheson Born in 1937 1960’s Royal College of Art
Dx in 1993 Aged 56
Set up SMT 1997
Died in 1999
Aged 62

25. Multiple System Atrophy Trust
Telephone support line
Information for people living with MSA, carers and professionals
Educational talks/awareness days
Newsletter
Contact scheme
Support Groups
Funding for research
MSAT Office
Nurse Specialists:
Katie Rigg
Jill Lyons
Samantha Pavey

26. Research
To date the causes and disease mechanism in MSA are largely unknown
Astro Zenica Trial
Prospect Study contact
Creating a database and longitudinal study

27. Survey results
A third of respondents (33%) took 3-5 years to get a diagnosis
43% of respondents were not given any information on diagnosis
40% of respondents see a Neurologist 6/12.
18% see a PDNS 6/12

28. On Diagnosis

29. Support Groups

30. Contact Details
MSA Trust Nurse Specialists: Samantha Pavey (South East & East England): / Katie Rigg (Scotland, Ireland and North England): / Jill Lyons (Wales & South West England): / Office:

31. Listening Project