Clinical Assessment and Management of Multiple System Atrophy

Multiple System Atrophy (MSA) is a rare, rapidly progressive neurodegenerative disorder characterised by a combination of parkinsonism, cerebellar ataxia and autonomic dysfunction. Clinical assessment hinges on detailed history taking to identify early autonomic signs such as orthostatic hypotension, erectile failure and urinary disturbance, followed by objective autonomic testing and neurological examination to document cerebellar and extrapyramidal features. Neuroimaging may reveal characteristic patterns of cerebellar atrophy or the “hot-cross bun” sign in the pons, while emerging fluid and imaging biomarkers offer promise for earlier and more accurate diagnosis. Management is primarily symptomatic, targeting orthostatic hypotension with volume expansion and vasoconstrictors, controlling parkinsonian features with levodopa and using physiotherapy to maintain mobility. Careful attention to swallowing dysfunction and sleep-related breathing disorders such as stridor is essential to reduce morbidity. Multidisciplinary teams, including neurologists, autonomic specialists and allied health professionals, play a pivotal role in optimising quality of life. Palliative and supportive strategies, including nutritional supplementation and non-pharmacological interventions, are integral to holistic care. Despite the absence of disease-modifying therapies, advances in trial design and biomarker development are shaping the future landscape of therapeutic intervention in MSA.

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Clinical Assessment and Management of Multiple System Atrophy publication trend

The graph below shows the total number of articles in clinical assessment and management of multiple system atrophy across all publications each year (not limited to Nature Index journals).

Technical terms

Multiple System Atrophy (MSA): A neurodegenerative synucleinopathy marked by parkinsonism, cerebellar ataxia and autonomic failure.

Autonomic failure: Impairment of involuntary functions such as blood pressure regulation, bladder control and gastrointestinal motility.

Parkinsonism: A syndrome of tremor, rigidity, bradykinesia and postural instability often seen in MSA‐P subtype.

Cerebellar ataxia: Loss of coordinated movements and balance due to cerebellar involvement, characteristic of MSA‐C subtype.

REM sleep behaviour disorder (RBD): A parasomnia in which muscle atonia is lost during REM sleep, leading to dream enactment movements.

Neurofilament light chain: An axonal structural protein released into biofluids during neurodegeneration, used as a prognostic biomarker.

References

1. Longitudinal evolution of sleep disturbances in early multiple system atrophy: a 2‐year prospective cohort study. BMC Medicine (2023).
2. Patient-perceived progression in multiple system atrophy: natural history of quality of life. Journal of Neurology Neurosurgery & Psychiatry (2024).
3. Neurofilament light levels predict clinical progression and death in multiple system atrophy. Brain (2022).