Severe Susac syndrome The world is suddenly upside down

Münster University Hospital informs about the Susac-Snydrom
Vision disorders, hearing disorders, headaches, and confused acts can be the result of the rare Susac syndrome. The disease "suddenly turns the world of those affected on its head," say physicians at the University Hospital Münster (UKM).

Sudden confusion, memory lapses or even personality changes can loudly take a fulminant course in those affected and often the patients themselves - in the truest sense of the word - hardly recognize each other.

For example, patients with Susac syndrome "suddenly and for no apparent reason" put their shoes in the fridge or start talking to family and friends overnight in a foreign language, "reports the UKM. Most of the time the neurological disorder hits patients out of the blue. It can take some time until the diagnosis is made, because the syndrome is extremely rare and the symptoms can be extremely different. Thirty-five affected people visited the UKM on Friday and shared their experiences with the disease.

Susac syndrome can manifest itself in different ways. In addition to headaches, visual and hearing impairments, these include confused acts such as placing shoes in the refrigerator. (Image: fuzzbones / fotolia.com)

Especially young women affected
According to the UKM, an estimated 300 people worldwide suffer from Susac syndrome. Typical of the disease is the "classic triad of encephalopathy, with symptoms such as severe headaches and neurological deficits from visual and hearing disorders", explains Dr. med. Ilka Kleffner, senior physician at the Department of General Neurology at the UKM. In principle, the Susac syndrome occurs in individual episodes and progressively in batches.

Especially young women between the ages of 20 and 40 are affected, but men also suffer from the syndrome (gender ratio of about 3: 1). According to the UKM, the cause is "inflammatory processes of the smallest blood vessels of the brain, the retina and the inner ear with presumably autoimmune roots."

Hearing disorders and blurred vision unsettle sufferers
The patient Inga Fritz from Münsterland reports in the press release of the UKM on her experiences with the disease and makes it clear that her illness did not turn out quite as dramatic as with other patients. The first symptoms were in the summer of 2014 and rather diffuse.

"When suddenly I could no longer hear correctly, I first believed in a hearing loss", so the statement of the patient. With her husband, she had just built a house and attributed the complaints to the stress. But one morning she woke up and suddenly saw everything in the right eye with a black frame. Since "I was a bit scared," says the 43-year-old. It took almost half a year until the diagnosis was more likely to be accidental. On the recommendation of a friendly ophthalmologist, who suspected Susac syndrome behind the symptoms, Inga Fritz came to the neurologist Dr. med. Kleffner.

Good treatment options
In general, according to the UKM, the inflammatory activity of Susac syndrome can be reasonably well controlled. With cortisone, modern immunotherapies and ASA (acetylsalicylic acid), the disease can often be treated well. Thanks to the therapy, Inga Fritz is now in a position to live almost without any restrictions and she continues to work in her profession as a primary school teacher, s the message from the UKM. (Fp)