Short answer · Medically reviewed summary · Last updated: 2026-05-08
Multiple System Atrophy (MSA) was historically recognized through the distinct descriptions of Shy-Drager syndrome and olivopontocerebellar atrophy, which were unified under the current diagnostic criteria in the late 20th century. While once considered a collection of separate disorders, modern medicine now understands Multiple System Atrophy as a single, progressive neurodegenerative disease characterized by the accumulation of misfolded alpha-synuclein proteins in the brain. How was Multiple System Atrophy first identified? The history of Multiple System Atrophy dates back to the early 20th century when various researchers described isolated symptoms.
2 people with Multiple Systems Atrophy have shared their first-person experience on this question at DiseaseMaps.
What is the history of Multiple Systems Atrophy?
History of Multiple Systems Atrophy: when and how it was discovered, and the milestones in research since, medically reviewed.
Multiple System Atrophy (MSA) was historically recognized through the distinct descriptions of Shy-Drager syndrome and olivopontocerebellar atrophy, which were unified under the current diagnostic criteria in the late 20th century. While once considered a collection of separate disorders, modern medicine now understands Multiple System Atrophy as a single, progressive neurodegenerative disease characterized by the accumulation of misfolded alpha-synuclein proteins in the brain.
How was Multiple System Atrophy first identified?
The history of Multiple System Atrophy dates back to the early 20th century when various researchers described isolated symptoms. In 1960, neurologists Milton Shy and Glenn Drager published a landmark paper describing patients with autonomic failure, parkinsonism, and cerebellar ataxia, a condition later known as Shy-Drager syndrome. Before this, other clinicians identified related neurodegenerative patterns like striatonigral degeneration and sporadic olivopontocerebellar atrophy. It was not until the 1990s that consensus criteria formally grouped these distinct clinical presentations under the umbrella of Multiple System Atrophy.
How has our understanding of the disease evolved?
The shift in understanding Multiple System Atrophy occurred primarily due to advancements in neuropathology. In 1989, researchers identified glial cytoplasmic inclusions (GCIs) as the pathological hallmark of the disease. These inclusions contain alpha-synuclein, placing Multiple System Atrophy in the category of "synucleinopathies," alongside Parkinson’s disease and Lewy body dementia. This discovery allowed clinicians to move beyond symptom-based labels to a unified biological diagnosis.
What are the major historical milestones in research?
- 1960: Shy and Drager formally report the association between autonomic failure and parkinsonian features.
- 1989: Discovery of alpha-synuclein-positive glial cytoplasmic inclusions, providing a diagnostic marker.
- 1998: Establishment of the first consensus criteria to standardize the clinical diagnosis of Multiple System Atrophy.
- Current Era: Utilization of advanced neuroimaging (like MRI and PET scans) to distinguish Multiple System Atrophy from other parkinsonian disorders.
How has patient advocacy changed the landscape?
With 911 members currently sharing their experiences on DiseaseMaps.org, the Multiple System Atrophy community has become a vital force in driving awareness and research funding. Historically, patients were often misdiagnosed with Parkinson’s disease, leading to isolation. Today, patient-led organizations provide essential support and advocate for clinical trials, shifting the focus from simply managing symptoms to seeking disease-modifying therapies.
Next steps
- Consult with a movement disorder specialist or neurologist to discuss the latest diagnostic imaging techniques.
- Join the 911+ members on DiseaseMaps.org to share experiences and find support.
- Monitor clinical trial databases for emerging research on alpha-synuclein-targeted therapies.
Medical Disclaimer: This information is for educational purposes and should not replace professional medical advice, diagnosis, or treatment.
References
- NIH Genetic and Rare Diseases Information Center (GARD): Multiple System Atrophy
- Orphanet: Multiple System Atrophy (ORPHA: 102)
- OMIM (Online Mendelian Inheritance in Man): Multiple System Atrophy
- The MSA Coalition: Understanding the History of MSA
In 1969 the term multiple system atrophy was adopted to include these three disorders. In 1998 it was discovered that all three diseases had the same brain autopsy finding of alpha-synuclein glial cytoplasmic inclusions (GCIs). These were observed as a build up of toxic alpha-synuclein protein within the oligodendroglial cells (glial cells are a form of helper cells to the neurons that make up the myelin sheath or outer insulating coating of the neurons) within the brain and spinal cord.
Multiple system atrophy is now classified as two separate types: MSA-P (parkinson like symptoms) and MSA-C (cerebellar ataxia symptoms). Both types include autonomic or urinary dysfunction as a defining symptom. You can read the full diagnostic criteria for multiple system atrophy at this link: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2676993/
Posted Jul 11, 2017 by Pam Bower 2952
Today, it is classified as separate, with two types (with much overlap) MSA P (or with more Parkinson’s type symptoms) and MSA C which seems to focus more in the autonomic part of the brain.
As the disease spreads through the brain, the overlap becomes greater.
Posted Mar 23, 2018 by [email protected] 3020
