Multiple Systems Atrophy (MSA)

Diagnosis:

  • Multiple systems controlling movement and autonomic involuntary actions are affected (functions like blood pressure and bladder regulation). Motor + Autonomic failure is present.
  • Usually develops when the person is 50-60 years old.
  • No diagnostic tests available to confirm MSA – recommendation needed from a neurologist specialising in movement disorders.
  • Currently there is no cure, however, drugs and therapies are available to manage symptoms.

Early Presentation

Image retrieved here

Other Early Symptoms:
Postural Hypotension (decreased BP)
Erectile dysfunction

Lifespan from Diagnosis:
On average, people live ~7-10 years after diagnosis of MSA.
Death often due to respiratory problems.
Sleep Disorders may be one of the first symptoms displayed in MSA.

Nonmotor and Motor impairments:

    • Decreased BP, problems with HR (falls risk)
    • Impotence in me
    • Incontinence
      Ataxia + Parkinsonian changes
    • Mood + Cognitive changes
    • Eye movements can be affected
    • Slowness
    • Rigidity
    • Poor coordination of walking and limb movements (ataxia)
    • Difficulty starting movement (akinesia)
    • Poor balance, unsteadiness
    • Speech + Swallowing + Breathing
    • Tremor
    • Dystonia (abnormal postures, sustained muscle activity)
    • Extreme postures

Differential diagnosis:

  • MSA: Different parts of the brain affected
  • Most common form of MSA: Changes in the Basal Ganglia causing Parkinsonian movement changes.
    • Parkinson’s Disease (PD) also affects the area of the Basal Ganglia.
    • Imbalance will manifest earlier and progress faster in MSA than PD.
  • 20% people with MSA experience changes in the cerebellum causing incoordination (cerebellar ataxia).
  • MSA has faster progression vs PD & does not respond well to medications.

Tests to help confirm MSA Diagnosis:

  • Sphincter EMG
  • Cardiovascular Autonomic Function Test
  • Clonidine Testing
  • Brain Scans (MRI or SPECT)

Specialists / Healthcare Providers and their Roles (MDC Team):

  • Neurologists: Advise on medical treatments + treat presenting symptoms relating to medications especially levodopa and Botulinum Toxin A (for dystonic/spastic musculature).
  • Neuropsychologist: Cognitive assessments.
  • Nurse: Provide education and medication to caregivers regarding symptoms, disease progression, management strategies, etc. Arrange referrals to other health professionals in the MDC where relevant.
  • Caregiver: Provide care and assistance esp. as MSA progresses, position changes, good posture for eating + drinking.
  • Speech Pathologist: Assess speaking and swallowing. Works with dieticians to create a specialised thickened (liquid) diet in late stages where risk of aspiration is present.
  • Occupational Therapists: Assess home environment and suggest ways to improve safety, safety aids (rails, shower + toilet), planning of home modifications, equipment such as seating, cushions and mattresses.
  • Physiotherapists:
    ❖ Early stages: Assess walking and balance. Reduce falls risk. Maintain independence for as long as possible. Equipment: wheelchairs, protective garments e.g. hip garment to protect against fractures. Home Exercise Program: strength, fitness, ROM. Objective measures used in the early stage of MSA – Strength, 6MWT, BBS, TUG, etc.
    ❖ Middle: Managing impairments as they present (Parkinsonian changes become more evident).
    ❖ Late: Stretch tight muscles (contracture), relieve symptoms, monitor the risk of aspiration.
  • OT & PT: Advise on appropriate seating.

Management Strategies:

  • Focusing on optimisation of QoL + Relieving symptoms (contracture, secretion clearance, weakness)
  • Assist with ADLs.
  • Continence management: Medications e.g. oxybutynin + intermittent self-catheterisation.
  • Low blood pressure management: Medications, drinking adequate fluids, high salt diet, eating small and frequent meals, pressure stockings, raising the head of the bed when sleeping.
  • Pressure sores: Constant changing of position, supportive mattresses and seating.
  • Breathing disorders: CPAP to keep upper airways open (sleep apnoea – mask worn during sleep). MSA can be associated with stridor, sleep apnoea and dyspnoea – there is a need to treat respiratory impairments as these cardiopulmonary complications are often the cause of death in individuals with MSA (more noticeable in late stages of the condition).

References

  • Multiple system atrophy. Monash Health. (n.d.). https://monashhealth.org/services/movement-disorders-program/understanding-movement-disorders/multiple-system-atrophy/#:~:text=Multiple%20System%20Atrophy%20(MSA)%20is,blood%20pressure%20and%20bladder%20control
  • Mayo Foundation for Medical Education and Research. (2022, July 13). Multiple system atrophy (MSA). Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/multiple-system-atrophy/symptoms-causes/syc-20356153#:~:text=People%20typically%20live%20about%207,the%20lungs%20(pulmonary%20embolus)
  • Krismer, F., Jellinger, K. A., Scholz, S. W., Seppi, K., Stefanova, N., Antonini, A., Poewe, W., & Wenning, G. K. (2014). Multiple system atrophy as emerging template for accelerated drug discovery in α-synucleinopathies. Parkinsonism & Related Disorders, 20(8), 793–799. https://doi.org/10.1016/j.parkreldis.2014.05.005
  • Sarah Jividen, R. (2021, September 21). What are parkinson’s plus syndromes? Verywell Health. https://www.verywellhealth.com/parkinsons-plus-syndromes-5200635
  • Multiple system atrophy treatment with stem cells. (n.d.). https://www.startstemcells.com/atrophy.html
  • Coon, E. A., & Ahlskog, J. E. (2021). My treatment approach to multiple system atrophy. Mayo Clinic Proceedings, 96(3), 708–719. https://doi.org/10.1016/j.mayocp.2020.10.005
  • Jala, S. (2019, February) 8). Multiple system atrophy: A common form of atypical parkinsonism. Clinical Advisor. https://www.clinicaladvisor.com/home/topics/neurology-information-center/multiple-system-atrophy-a-common-form-of-atypical-parkinsonism/
Blog written by Tess Koparan