Brain Scan
A brain scan in an undated image Reuters/Handout

Acute confusional states accompanied by hearing loss and vision problems may be a sign of a strange syndrome rarely analysed or most likely misdiagnosed. A recent report by doctors at the Loyola University Medical Center describes the distinct symptoms of this disease called Susac syndrome. The cause, effects, and development of this brain disease is not thoroughly understood.

Patients who have been diagnosed with the disease have ages ranging from 9-72 and are mostly women. However, there are a number of cases where Susac syndrome is misdiagnosed in advance as another disorder such as multiple sclerosis (MS) and acute disseminated encephalomyelitis (ADEM). Thus, there could be higher prevalence than the reported cases.

The Loyola report which was published in the Journal of Stroke and Cerebrovascular Diseases, discourses the case of two patients diagnosed with Susac syndrome. Both were in a serious state of disorientation, had abnormal brain scans and cerebrospinal fluid findings. Violent conduct, jumbled speech, and lack of concentration was observe in the first patient weeks before he was found by the police in confused state. He also developed hearing and eyesight problems.

The second patient, a 57-year-old lady, suffered similar symptoms, felt dizziness, weakness and numbness in the arms and face, and behaved unbecomingly. The patients were able to recover from the disease after receiving treatment that suppressed the immune system.

Susac syndrome is an autoimmune disease. When endothelial cells covering blood cells in the brain, ears and retina are damaged by the immune system, these cells expand and disrupt blood circulation to these organs. Acute confusional state has been observed in a number of patients diagnosed with the illness, but this symptoms has not been associated with Susac syndrome in existing neurology literature.

Authors of this report recommend that Susac syndrome be included in the diagnosis of patients with unexplained acute confusional states and abnormal MRI and cerebrospinal fluid tests. There is increasing awareness about this rarely diagnosed disease. As of 2013, there are at least 304 case reports from different countries on Susac syndrome; most of which are individual cases. More studies on this provide more bases for understanding the disease and for improving clinical diagnosis and treatment.

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