Multiple System Atrophy (MSA)

Multiple System Atrophy is the third most common of the four atypical parkinsonism disorders. Older terms for MSA include Shy Drager Syndrome, Olivopontocerebellar Atrophy, and Striatonigral Degeneration.


MSA Symptoms

MSA is divided into two types. In the United States, MSA-P is more common. Symptoms vary based on the two types. They are:

  • MSA-Parkinsonism (MSA-P): where parkinsonism symptoms are predominant
  • MSA-Cerebellar (MSA-C): where cerebellar symptoms are predominant

Parkinsonism features can include bradykinesia (slow movement), rigidity, tremor, and postural instability. Cerebellar features can include gait ataxia (wide, irregular steps), ataxic dysarthria, limb ataxia, and nystagmus (an oculomotor issue).

Other common symptoms can include severe orthostatic hypotension (blood pressure falls within three minutes of standing), urinary incontinence or urinary retention, erectile dysfunction in men, and REM sleep behavior disorder (RBD).

Less-common symptoms can include antecollis (neck is flexed forward such that the chin rests on the chest), dystonia of the face and mouth, stridor, low volume of speech, myoclonus, emotional incontinence (inappropriate laughter or crying), camptocormia (severe forward flexion of the spine), Pisa syndrome (severe lean of the body), and contractures of the hands and feet.


MSA Treatments

MSA treatments are few. All treatments are focused on symptoms. So, parkinsonism may be treated by levodopa. (Some with MSA do have a response to this medication for a short period of time.) Orthostatic hypotension may be treated by a variety of medications. Urinary incontinence can be treated with medication, botox injections, catheters, or adult briefs.

Therapy is worth trying: physical therapy, occupational therapy, and speech therapy. In our local support group, we have seen a few people with MSA benefit from LSVT-LOUD speech therapy, designed for Parkinson’s Disease.


Go-To Organizations for MSA Education

A handful of organizations consistently deliver quality information about MSA. Click the link for each organization to review their web sites:

Organization Name Country Focus Web Address
Multiple System Atrophy Coalition USA Education, awareness, support www.multiplesystematrophy.org
Multiple System Atrophy Trust UK Support, education, awareness www.msatrust.org.uk

MSA Top Resources

Brain Support Network continually reviews most of the resources available on MSA. In our opinion, the most helpful resources for families are listed following. This list is also available in printable PDF.

A Guide to Multiple System Atrophy

This 36-page guide, last revised in November 2015, was written by the Multiple System Atrophy Trust in the UK.  It offers a short description of MSA and details on treatment and management of symptoms.

msatrust.org.uk/wp-content/uploads/2015/11/A-Guide-to-MSA.pdf

MSA: NINDS Pamphlet and Fact Sheet

These documents were written by the National Institute of Neurological Disorders and Stroke (NINDS), part of the National Institutes of Health (NIH), and revised in November 2014.

Pamphlet: ninds.nih.gov/disorders/msa/multiple_system_atrophy_pamphlet.pdf
Fact sheet: ninds.nih.gov/disorders/msa/detail_msa.htm

Top Ten Things You Need to Know About Multiple System Atrophy

Two-page document compiled by MSA Awareness from posts to an online MSA-related support group. These are 10 items not normally highlighted in MSA literature.

brainsupportnetwork.org/MSA-Awareness–10-things-to-know-about-MSA.pdf

MSA: Some Answers

This short brochure was published by CurePSP and last printed in October 2017.

psp.org/wp-content/uploads/2017/09/MSA-SOME-ANSWERS-BROCH_PAGES_WEB-9.19.17.pdf

Treatment of and Research on Multiple System Atrophy

Movement disorder specialist Dr. Kathleen gave an indepth presentation on MSA as part of an atypical parkinsonism symposium organized by Stanford University and Brain Support Network in October 2012.

youtube.com/watch?v=osgaPBrB95E

MSA: What You Need to Know

This 57-page document is an in-depth look at treatment of common symptoms in MSA. Produced by The MSA Coalition; updated in 2014.

multiplesystematrophy.org/wp-content/uploads/2014/07/MSA_-_What_You_Need_to_Know_v2.pdf

MSA Brain Donation: Confirm Diagnosis, Support Research for a Cure

Brain Support Network volunteer Candy Welch, whose husband had MSA (confirmed through brain donation), spoke in October 2017 about the importance of brain donation for MSA specifically.

brainsupportnetwork.org/wp-content/uploads/BSN_MSACoalitionConf_Oct2017.pdf

Recent MSA Patient/Family Conferences (Nashville and New Orleans)

There were many terrific speakers at the MSA Coalition’s October 2017 and 2016 all-day events.  Note in particular Dr. Allyson Mayeux’s keynote address in October 2016 about her brother Hal’s MSA journey, ending in brain donation.  Unedited conference recordings are here:

2017: facebook.com/pg/MultipleSystemAtrophyCoalition/videos/

2016: multiplesystematrophy.org/2016-msa-patient-family-conference-live

Slides from 2017:  multiplesystematrophy.org/resources/msa-patient-family-conference-handouts

Recent MSA NJ Patient/Family Conference

There were many terrific speakers at MSA NJ’s March 2017 all-day conference.  Note in particular the talks on practical methods to improve everyday life.

msanj.org/2017-regional-patient-and-caregiver-support-conference/

Orthostatic Hypotension (OH) in Parkinson’s Disease (PD), Multiple System Atrophy (MSA), and Lewy Body Dementia (LBD)

Movement disorder specialist Dr. Veronica Santini describes the symptoms of OH, lists the conversative and medication interventions, and answers questions.  This September 2017 webinar was co-hosted by Stanford University and Brain Support Network.  Detailed notes:

brainsupportnetwork.org/notes-orthostatic-hypotension-pd-msa-lbd-webinar/

Recording:  https://youtu.be/-FzsgUfQ_xI

MSA Update by Aaron Haug

Update was given by neurologist Dr. Aaron Haug at the CurePSP family conference in Denver in October 2013.

youtube.com/watch?v=iwWpAyg9zVs

Exploring Diagnosis, Management and Best Practice Interventions in Relation to PSP, MSA, and CBD

One of the world’s top researchers of MSA, Dr. David Williams presents on several disorders at a November 2009 conference for allied health professionals (RNs, PTs, OTs, STs, social workers, etc) in Melbourne, Australia. His presentations are divided into a series of 10-minute online video segments. The MSA presentation…

Starts here: youtube.com/watch?v=-v02RilqZdc&start=242
Continues until timestamp 2:30: youtube.com/watch?v=zxPS0UVCKzg

Fact Sheets

In 2015, the Multiple System Atrophy Trust in the UK published 25 fact sheets on topics such as bowel management, continence, fatigue, Parkinson’s medications, monitoring blood pressure, saliva, driving, and useful equipment for hygiene.

Main page: msatrust.org.uk/support-for-you/factsheets/

Carer’s Guide

This short guide, published in June 2014, was written by the Multiple System Atrophy Trust in the UK. It includes a list of things to think about early: practical aspects, physical aspects, and emotional aspects of caring for someone with MSA.

msatrust.org.uk/wp-content/uploads/2014/06/MSA-Trust-Carers-Guide_v1.0.pdf


MSA Diagnostic Criteria

The diagnostic criteria for MSA presented here reflect those reported by Gilman, et al, Second Consensus statement on the diagnosis of multiple system atrophy, Neurology. 2008 Aug 26;71(9):670-6.

Definitive Diagnosis of MSA

  • Brain autopsy required

Probable Diagnosis of MSA (“Table 1”)

A sporadic, progressive, adult (>30 years) onset disease characterized by:

Probable Diagnosis of MSA-P Probable Diagnosis of MSA-C
Autonomic failure involving urinary incontinence (inability to control the release of urine from the bladder, with erectile dysfunction in males) or
An orthostatic decrease of blood pressure within 3 min of standing by at least 30 mm Hg systolic or 15 mm Hg diastolic.
[SYMPTOMS ABOVE ARE REQUIRED FOR BOTH MSA-P AND MSA-C]
Poorly levodopa-responsive parkinsonism (bradykinesia with rigidity, tremor, or
postural instability)
Cerebellar syndrome (gait ataxia with cerebellar dysarthria, limb ataxia, or
cerebellar oculomotor dysfunction)

Possible Diagnosis of MSA (“Table 2”)

Note that “Possible Diagnosis” requires that at least one additional symptom from the list of “Additional Symptoms of Possible MSA”.  These are listed in “Table 3”.  A sporadic, progressive, adult (>30 years) onset disease characterized by:

Possible Diagnosis of MSA-P Possible Diagnosis of MSA-C
Parkinsonism:

  • Bradykinesia with rigidity
  • Tremor, or
  • Postural instability)
A cerebellar syndrome:

  • Gait ataxia with cerebellar dysarthria,
  • Limb ataxia, or
  • Cerebellar oculomotor dysfunction
At least one feature suggesting autonomic dysfunction (otherwise unexplained urinary urgency, frequency or incomplete bladder emptying, erectile dysfunction in males, or significant orthostatic blood pressure decline that does not meet the level required in probable MSA)
[SYMPTOMS ABOVE ARE REQUIRED FOR BOTH MSA-P AND MSA-C]
At least one of the additional features shown in Table 3
[SYMPTOMS ABOVE ARE REQUIRED FOR BOTH MSA-P AND MSA-C]

Additional symptoms of possible MSA (“Table 3”)

Additional symptoms of possible MSA-P Additional symptoms of possible MSA-C
  • Babinski sign with hyperreflexia
  • Stridor

[SYMPTOMS ABOVE MAY BE PRESENT IN BOTH MSA-P AND MSA-C]

  • Rapidly progressive parkinsonism
  • Poor response to levodopa
  • Postural instability within 3 y of motor onset
  • Gait ataxia, cerebellar dysarthria, limb ataxia, or cerebellar oculomotor dysfunction
  • Dysphagia within 5 y of motor onset
  • Atrophy on MRI of putamen, middle cerebellar peduncle, pons, or cerebellum
  • Hypometabolism on FDG-PET in putamen, brainstem, or cerebellum
  • Parkinsonism (bradykinesia and rigidity)
  • Atrophy on MRI of putamen, middle cerebellar peduncle, or pons
  • Hypometabolism on FDG-PET in putamen
  • Presynaptic nigrostriatal dopaminergic denervation on SPECT or PET

Symptoms supporting a diagnosis of MSA (“Table 4”)

  • Orofacial dystonia
  • Disproportionate antecollis
  • Camptocormia (severe anterior flexion of the spine) and/or Pisa syndrome (severe lateral flexion of the spine)
  • Contractures of hands or feet
  • Inspiratory sighs
  • Severe dysphonia
  • Severe dysarthria
  • New or increased snoring
  • Cold hands and feet
  • Pathologic laughter or crying
  • Jerky, myoclonic postural/action tremor

Cautionary Symptoms not supporting a diagnosis of MSA (“Table 4”)

  • Classic pill-rolling rest tremor
  • Clinically significant neuropathy
  • Hallucinations not induced by drugs
  • Onset after age 75 years
  • Family history of ataxia or parkinsonism
  • Dementia (on DSM-IV)
  • White matter lesions suggesting multiple sclerosis