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Multiple system atrophy: What PCPs need to know
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Multiple system atrophy — whose worldwide awareness day is commemorated every Oct. 3 — includes disorders that historically had been referred to as Shy-Drager syndrome, olivopontocerebellar atrophy and striatonigral degeneration, according to the NIH.
A report in The New England Journal of Medicine puts the disease’s estimated prevalence at 3.4 to 4.9 cases per 100,000 population, a figure that rises to 7.8 per 100,000 among those older than 40 years.
The rarity of multiple system atrophy is not the only trait that makes it hard for some clinicians to recognize.
The disease’s resemblance to other neurological conditions also often delays diagnosis, according to Paola Sandroni MD, PhD, practice chair of the neurology department at the Mayo Clinic in Minnesota and chair of the autonomic disorder division.
“There are a lot of similarities between multiple system atrophy and Parkinson’s disease, so often patients receive the Parkinson’s diagnosis first,” she told Healio Family Medicine.
“Only after a neurological exam and tests such as autonomic screening (to assess BP, heart rate control, and sweat capacity), bladder function, MRI and positron emission tomography can be closer to saying a patient has multiple system atrophy. But in many instances, it is only at autopsy than we can definitively make a diagnosis,” Sandroni continued.
She said the exact factors that put one at risk for the disease remain unclear, but there are several possibilities.
“At least one study has suggested that multiple concussions are a risk factor, but there may also be genes that predispose certain patients to developing the disease. The jury is still out on this one,” Sandroni said.
According to the Mayo Clinic and NIH, the primary sign of the disease is orthostatic hypotension, but other signs include CV problems, urinary and bowel dysfunction, constipation, incontinence, sweating abnormalities, reduced production of bodily fluids, heat intolerance due to reduced sweating, impaired body temperature control, sleep disorders and agitated sleep, irregular nighttime breathing, sexual dysfunction, impotence, loss of libido, and problems controlling emotions.
Sandroni, who is also a fellow of the American Academy of Neurology, added that motor symptoms may precede the onset of autonomic ones, occur concomitantly or become apparent later in the course of the disease.
“This is a very difficult, complicated disease to understand in primary care. As soon as a primary care physician notices or a patient reports the symptoms along with Parkinsonian disorder, a neurologist should be consulted,” Sandroni said.
She added that patients with multiple symptom atrophy can be treated with medications and/or make lifestyle changes, but the disease ultimately proves fatal. The median lifespan for a patient with suspected multiple system atrophy is 6 to 10 years once a diagnosis is made.
The Mayo Clinic and NIH also said the disease impacts the body's involuntary functions, including breathing, bladder function, BP and muscle control.
“[Multiple system atrophy] is a horrible disease for patients,” Sandroni told Healio Family Medicine. “We need to work on getting a cure for it.” – by Janel Miller
References:
Fanciulli A, Wenning GK. NEJM. 2018;doi:10.1056/NEJMra1311488.
Mayo Clinic. “Multiple system atrophy.” https://www.mayoclinic.org/diseases-conditions/multiple-system-atrophy/symptoms-causes/syc-20356153. Accessed Sept. 26, 2018.
NIH. “Multiple System Atrophy Fact Sheet.” https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Multiple-System-Atrophy. Accessed Sept. 26, 2018.
World MSA Day. “Home.” http://www.world-msa-day.org/World-MSA-Day/WELCOME.html, Accessed Sept. 26, 2018.
Disclosure: Sandroni reports no relevant financial disclosures.