Severe Susac syndrome: the world suddenly turns upside down

University Hospital Münster provides information about the Susac snydrome
Vision problems, hearing disorders, headaches and confused actions can result from the rare Susac syndrome. The disease "suddenly turns the world of those affected upside down," reports doctors from the University Hospital of Münster (UKM).

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

For example, patients with Susac syndrome "suddenly put their shoes in the fridge for no apparent reason or start talking overnight with family and friends in a foreign language," reports the UKM. Most often, the neurological disorder strikes the patient out of the blue. It may take some time until the diagnosis is made because the syndrome is extremely rare and the symptoms can vary greatly. 30 sufferers were guests at the UKM on Friday and exchanged their experiences with the disease.

Young women especially affected
According to the UKM, an estimated 300 people worldwide suffer from Susac syndrome. The classic triad from encephalopathy, with symptoms such as severe headaches and neurological deficits from vision and hearing disorders, is typical of the clinical picture, explains Dr. Ilka Kleffner, senior physician at the Clinic for General Neurology at the UKM. Basically, Susac syndrome occurs in individual episodes and runs in batches.

Young women between the ages of 20 and 40 are particularly affected, but men also develop the syndrome (gender ratio of about 3: 1). According to the UKM, the cause is "inflammatory processes in the smallest blood vessels in the brain, retina and inner ear, which are believed to have autoimmune roots."

Hearing disorders and visual disturbances unsettle those affected
Inga Fritz, a patient from Münsterland, reported in the UKM press release about her experience with the disease and made it clear that her course of the disease was not quite as dramatic as with other sufferers. The first symptoms appeared in summer 2014 and were rather diffuse.

"When I suddenly couldn't hear properly, I first thought I had a hearing loss," the patient said. 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 with a black frame in her right eye. “I was getting a little scared,” reports the 43-year-old. It took almost half a year for the diagnosis to be made by chance. On the recommendation of an ophthalmologist friend who suspected the Susac syndrome was behind the symptoms, Inga Fritz came to the neurologist Dr. Kleffner.

Good treatment options
In general, the inflammatory activity of Susac syndrome can be kept relatively well in check, according to the UKM. The disease can often be treated well with cortisone, modern immunotherapy and ASA (acetylsalicylic acid). Thanks to the therapy, Inga Fritz is now able to live with almost no restrictions and she continues to work as a primary school teacher, s the UKM announcement. (fp)

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