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Unusual disease that causes acute confusion may be underdiagnosed

Date:
February 25, 2015
Source:
Loyola University Health System
Summary:
An unusual disease called Susac syndrome, which can cause acute confusion and problems with hearing and eyesight, is rare but probably under reported, physicians report.
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An unusual disease called Susac syndrome, which can cause acute confusion and problems with hearing and eyesight, is rare but probably under reported, Loyola University Medical Center physicians report in the Journal of Stroke and Cerebrovascular Diseases.

Classical neurology textbooks do not list Susac syndrome as a possible diagnosis of acute confusional states. And Susac syndrome is often misdiagnosed early on as multiple sclerosis or a similar disorder called acute disseminated encephalomyelitis (ADEM).

Susac syndrome affects three times as many women as men, and has been seen in patients ranging in age from 9 to 72 years.

Susac syndrome was first described in 1979 by Dr. John Susac in the journal Neurology. It is believed to be an autoimmune disease, in which the immune system attacks endothelial cells that line blood vessels in the brain, inner ears and retinas. This causes the endothelial cells to swell up and partially or completely block blood flow to affected organs.

The Loyola authors describe two Susac syndrome patients who presented with acute confusion and abnormal MRIs and spinal fluid:

- A 32-year-old man was found by police on a highway in the wrong state. During the previous three weeks, he had displayed unusually violent behavior, incoherent speech and increasing inattention. He later developed hearing loss and blockages in the retinal arteries called branch retinal artery occlusions (BRAOs).

- A 57-year-old woman suffered confusion, forgetfulness, dizziness, blurred vision, fatigue, diminished hearing in the left ear, unsteady gait, facial weakness, numbness in her hands and inappropriate behavior.

Both patients recovered after being put on drugs to suppress their immune systems.

The authors propose that Susac syndrome be considered as a possible diagnosis in young patients with otherwise unexplained acute onset of confusion, along with abnormal spinal fluid tests and MRI results.


Story Source:

Materials provided by Loyola University Health System. Note: Content may be edited for style and length.


Journal Reference:

  1. Michael Star, Rick Gill, Maria Bruzzone, Felipe De Alba, Michael J. Schneck, José Biller. Do Not Forget Susac Syndrome in Patients with Unexplained Acute Confusion. Journal of Stroke and Cerebrovascular Diseases, 2015; DOI: 10.1016/j.jstrokecerebrovasdis.2014.11.028

Cite This Page:

Loyola University Health System. "Unusual disease that causes acute confusion may be underdiagnosed." ScienceDaily. ScienceDaily, 25 February 2015. <www.sciencedaily.com/releases/2015/02/150225142317.htm>.
Loyola University Health System. (2015, February 25). Unusual disease that causes acute confusion may be underdiagnosed. ScienceDaily. Retrieved May 28, 2017 from www.sciencedaily.com/releases/2015/02/150225142317.htm
Loyola University Health System. "Unusual disease that causes acute confusion may be underdiagnosed." ScienceDaily. www.sciencedaily.com/releases/2015/02/150225142317.htm (accessed May 28, 2017).

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