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Multiple System Atrophy - Parkinsonian Type (MSA-P)

An aggressive atypical parkinsonian syndrome characterised by autonomic dysfunction.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Nöroloji department. Book Appointment →

What is Multiple System Atrophy - Parkinsonian Type (MSA-P)?

Multiple system atrophy (MSA) is a sporadic, rapidly progressive neurodegenerative disease classified as alpha-synucleinopathy; characterised by accumulation of glial cytoplasmic inclusions (GCI). It has two main subtypes: MSA-P (parkinsonian, 60-80% in the West) and MSA-C (cerebellar). MSA-P represents the parkinsonian-predominant clinical phenotype.

Mean age at onset is 55-60; mean survival 7-9 years (more aggressive than Parkinson's disease). Cardinal triad: autonomic dysfunction (orthostatic hypotension, neurogenic bladder, erectile dysfunction, anhidrosis) + akinetic-rigid parkinsonism (mostly poor L-dopa response) + early postural instability.

Other suggestive findings: cold blue hands ('cold hand sign'), inspiratory stridor, REM sleep behaviour disorder (RBD), antecollis, anterocollis posturing, pyramidal signs (Babinski, hyperreflexia), pisa syndrome, dysarthria. Diagnostic criteria: 2022 MDS criteria. MRI may show putaminal atrophy and 'hot cross bun' sign (in MSA-C). Treatment: symptomatic L-dopa (limited benefit), midodrine/fludrocortisone for orthostatic hypotension, intermittent catheterisation for bladder, CPAP for stridor, physiotherapy and palliative care.

Symptoms

Symmetrical bradykinesia and rigidity
Poor L-dopa response
Severe orthostatic hypotension
Neurogenic bladder (urgency, incontinence)
Erectile dysfunction (early and severe in men)
Anhidrosis (decreased sweating)
Inspiratory stridor
REM sleep behaviour disorder (RBD)
Antecollis (forward neck flexion)
Pisa syndrome (lateral trunk leaning)
Cold blue hands
Cerebellar findings (ataxia, dysarthria - MSA-C overlap)
Pyramidal findings (Babinski)
Early severe falls

Risk Factors

Sporadic (>95%)
Mean age 50-60
COQ2 gene mutation (rare familial cases)
Environmental factors (controversial)
REM sleep behaviour disorder (preceding)
Pure autonomic failure (preceding)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Symmetrical parkinsonism + autonomic findings
  • L-dopa unresponsive parkinsonism
  • Severe orthostatic hypotension
  • Sudden onset bladder dysfunction
  • REM behaviour disorder + parkinsonism
  • Newly developed inspiratory stridor
  • Recurrent falls
  • Cerebellar findings (ataxia, dysarthria)

Treatment Methods

01
Brain MRI (putaminal atrophy, hot cross bun)
02
MDS-MSA 2022 diagnostic criteria
03
Autonomic function tests (tilt table)
04
Urodynamic study
05
Polysomnography (RBD assessment)
06
Symptomatic L-dopa trial (limited)
07
Midodrine, fludrocortisone, droxidopa (orthostasis)
08
Compression stockings, salt diet
09
Intermittent catheterisation, bladder pacemaker
10
PDE-5 inhibitor (erectile dysfunction)
11
CPAP, tracheostomy (severe stridor)
12
Botulinum toxin (sialorrhoea, dystonia)
13
Physiotherapy and balance training
14
Speech-language and swallowing therapy
15
Palliative care, family education

Which Department to Visit?

You can visit our Nöroloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.