Multiple System Atrophy (MSA)

Primer

Multiple System Atrophy (MSA) is a rapidly progressive neurodegenerative disorder characterized by any combination of Parkinsonism, cerebellar ataxia, and autonomic failure. The autonomic failure can include severe impairments such as orthostatic hypotension, post-prandial hypotension, postural instability, falls, and constipation. It is also the only neurodegenerative disorder with cerebellar dysfunction (ataxia, kinetic tremors). Although more typically considered as a neurodegenerative movement disorder, MSA is increasingly recognized as having a cognitive impairment component as well, in particular with impairments in verbal memory and verbal fluency.[1]

Epidemiology
  • MSA affects men and women equally, with an average age of onset of approximately 55 years.[2]
    • The mean life expectancy following diagnosis is 7 years
    • Thus, late-onset presentations at age 70 and above is unusal.
Prognosis
  • The progression of MSA is usually rapid and relentless with a mean survival of 6–10 years.[3]
Comorbidity
Risk Factors

Signs and Symptoms

  • Severe orthostatic hypotension
  • Incontinence

Neuroimaging

  • On MRI, iron accumulation in the globus pallidus is suggestive of MSA.[4]

Differential Diagnosis

MSA can be difficult to differentiate from Dementia with Lewy Bodies (DLB), Parkinson's Disease (PD), and Progressive Supranuclear Palsy (PSP) in both early and late stages of the disease process. These disease are all within the family of Parkinson's-plus disorders.[5]

Treatment

References

1) Brown, R. G., Lacomblez, L., Landwehrmeyer, B. G., Bak, T., Uttner, I., Dubois, B., ... & Leigh, N. P. (2010). Cognitive impairment in patients with multiple system atrophy and progressive supranuclear palsy. Brain, 133(8), 2382-2393.
2) McKay, J. H., & Cheshire, W. P. (2018). First symptoms in multiple system atrophy. Clinical Autonomic Research, 28, 215-221.
3) Wenning, G. K., Geser, F., Krismer, F., Seppi, K., Duerr, S., Boesch, S., ... & Pellecchia, M. T. (2013). The natural history of multiple system atrophy: a prospective European cohort study. The lancet neurology, 12(3), 264-274.
4) Kruer, M. C., & Boddaert, N. (2012, June). Neurodegeneration with brain iron accumulation: a diagnostic algorithm. In Seminars in pediatric neurology (Vol. 19, No. 2, pp. 67-74). WB Saunders.
5) Koga, S., Aoki, N., Uitti, R. J., Van Gerpen, J. A., Cheshire, W. P., Josephs, K. A., ... & Dickson, D. W. (2015). When DLB, PD, and PSP masquerade as MSA: an autopsy study of 134 patients. Neurology, 85(5), 404-412.