Unusual disease that causes acute confusion may be underdiagnosed | | Loyola Medicine
Thursday, February 26, 2015

Unusual disease that causes acute confusion may be underdiagnosed

Often confused with multiple sclerosis, Loyola experts report

MAYWOOD, Ill. 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 Chicago medical experts 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.

The article is titled “Do Not Forget Susac Syndrome in Patients with Unexplained Acute Confusion.” Authors from Loyola’s Stritch School of Medicine are Michael Star, MD, (first author); Rick Gill, MD; Maria Bruzzone, MD; Michael J. Schneck, MD; and Jose Biller, MD (senior author); and Felipe De Alba, MD.

About Loyola Medicine and Trinity Health

Loyola Medicine, a member of Trinity Health, is a quaternary care system based in the western suburbs of Chicago that includes Loyola University Medical Center (LUMC), Gottlieb Memorial Hospital, MacNeal Hospital and convenient locations offering primary and specialty care services from 1,877 physicians throughout Cook, Will and DuPage counties. LUMC is a 547-licensed-bed hospital in Maywood that includes the William G. and Mary A. Ryan Center for Heart & Vascular Medicine, the Cardinal Bernardin Cancer Center, a Level 1 trauma center, Illinois's largest burn center, a certified comprehensive stroke center and a children’s hospital. Having delivered compassionate care for over 50 years, Loyola also trains the next generation of caregivers through its teaching affiliation with Loyola University Chicago’s Stritch School of Medicine and Marcella Niehoff School of Nursing. Gottlieb is a 247-licensed-bed community hospital in Melrose Park with 150 physician offices, an adult day care program, the Gottlieb Center for Fitness, the Loyola Center for Metabolic Surgery and Bariatric Care and the Loyola Cancer Care & Research at the Marjorie G. Weinberg Cancer Center at Melrose Park. MacNeal Hospital is a 374-bed teaching hospital in Berwyn with advanced inpatient and outpatient medical, surgical and psychiatric services, advanced diagnostics and treatments. MacNeal has a 12-bed acute rehabilitation unit, a 25-bed inpatient skilled nursing facility, and a 68-bed behavioral health program and community clinics. MacNeal has provided quality, patient-centered care to the near west suburbs since 1919.

Trinity Health is one of the largest multi-institutional Catholic healthcare systems in the nation, serving diverse communities that include more than 30 million people across 22 states. Trinity Health includes 94 hospitals, as well as 109 continuing care locations that include PACE programs, senior living facilities and home care and hospice services. Its continuing care programs provide nearly 2.5 million visits annually. Based in Livonia, Mich., and with annual operating revenues of $18.3 billion and assets of $26.2 billion, the organization returns $1.1 billion to its communities annually in the form of charity care and other community benefit programs. Trinity employs about 133,000 colleagues, including 7,800 employed physicians and clinicians. Committed to those who are poor and underserved in its communities, Trinity is known for its focus on the country's aging population. As a single, unified ministry, the organization is the innovator of Senior Emergency Departments, the largest not-for-profit provider of home health care services—ranked by number of visits—in the nation, as well as the nation’s leading provider of PACE (Program of All Inclusive Care for the Elderly) based on the number of available programs.