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What is Susac Syndrome?
Susac Syndrome is an highly rare disorder which is characterized by abnormalities in the three most major organs of the trunk which is the brainpower , the eyes , and the ears . In Susac Syndrome , the affected person will experience encephalopathy with impaired functioning of the nous , branch retinal artery blockage damaging the retina leading to visual constipation , and internal pinna disease damaging the cochlea run to progressivehearing loss .
There are basically three shape of Susac Syndrome depending on which part of the body is more affected . In the first part , the brain is the most affected leave in severe encephalopathy . In the 2d part , the middle are the most stirred with severe damage to the retina due to branch nephritic artery blockage and in the third part the disease start up off with brain disorder but as the disease work up , the eye and the ear job take center stage and the brain disorder more or less resolves . The first form of Susac Syndrome which is the encephalopathic form commonly resolves ad lib with time which may range from one to four years whereas the second and the third forms of Susac Syndrome take a mess more meter and discourse to go away . The mainstay of treatment for Susac Syndrome is immunosuppressive discourse with steroid .
The reason behind Susac Syndrome being consider uncommon is because this consideration is often at times misdiagnosed as atypical multiple sclerosis but with more and more case of this condition being reported there is an increased awareness in the aesculapian sodality about Susac Syndrome .
This is basically an autoimmune shape in which an individual ’s resistant organization attacks and injures the endothelium which results in swelling of the endothelial cells causing partial or complete blockage of the vessels of the psyche , retina , and the inner ear ensue in the characteristic symptoms of what we term as Susac Syndrome .
What are the Causes of Susac Syndrome?
As stated , Susac Syndrome is an autoimmune disorder in which the affected individual ’s immune system of rules attacks the endothelium or the tiny blood vessel which supply line of descent to the genius , retina , and privileged ear . This results in hump of the endothelium causing partial or complete blockage of the vessels of the mentality , retina , and internal ear causing Susac Syndrome .
The accurate rationality as to why the immune mechanism is triggered against the endothelium is still not known resulting in the symptoms of Susac Syndrome .
What are the Symptoms of Susac Syndrome?
The symptom of Susac Syndrome are quite varying and differ from individual to individual . In most of the cases of Susac Syndrome , all the three forms of abnormality do not occur together meaning that abnormalities in the brain , ear , and eye will not come at once . Some people with Susac Syndrome may only have pinna and center symptoms and their brain may be entirely normal whereas in some cases only the brain may be touch .
In majority of cases of Susac Syndrome , dogged headaches are the first presenting symptom which will then be follow by other symptoms of Susac Syndrome . There may be a emcee of neurologic abnormalities that may develop in an somebody with Susac Syndrome like gait abnormalities , speech abnormalities , and cognitive handicap . The individual may also show personality and behavioural changes as a result of the brain disease because of Susac Syndrome .
The abnormalities due to leg retinal artery occluded front may range from seeing black pip in the visual sense to haloing in the theatre of operations of visual sense . Visual scotoma may also be experienced by the patient role .
Another symptom which will be experienced by an individual with Susac Syndrome is hearing loss which is due to damage to the cochlea or the internal capitulum . The audience loss may be fond or complete as a result of Susac Syndrome .
How is Susac Syndrome Diagnosed?
The diagnosis of Susac Syndrome begin with a elaborate account of the affected role and a thorough clinical rating which will involve evaluation by an ophthalmologist as well as an audiologist to check out the status of the middle and the ears .
to boot , specialized tests will be conducted to confirm the diagnosis of Susac Syndrome and to rule other causes of the symptom . A detailed neurologic rating will also be conducted to check for neurologic alteration which are classic for someone with Susac Syndrome .
AnMRIof the brain will be conducted which will clearly show change in the corpus callosum pointing towards Susac Syndrome . An ophthalmologic evaluation will fee-tail checking of the visual fields of the patient role which will clearly show the retinal arteria occlusion obscuring the affected role ’s vision .
An audiometry test will substantiate intimate ear disfunction with impairment to the cochlea thus fulfilling all the three characteristics and sustain the diagnosing of Susac Syndrome . It should be notice here that when the patient first presents to the Dr. then he or she may not have all the symptom which are suggestive of Susac Syndrome and this makes the diagnosing more difficult of Susac Syndrome .
How is Susac Syndrome Treated?
In most of the case , Susac Syndrome is ego limit and requires no particular treatment . The brain disease triggered by Susac Syndrome firmness of purpose by itself within a span of one to four years , although other intervention may speed up up the convalescence process and also help avoid any lasting neurological deficits , or potentially any visual or earshot red ink which may become irreversible if other treatment is not give way .
Immunosuppressive agents and steroid are the front business line intervention for Susac Syndrome . endovenous gammaglobulins are also quite in effect in treatment of Susac Syndrome .
Additionally , CellCept , Imuran , and rituximab have also shown quite a bit of effectiveness in the treatment of Susac Syndrome . For individuals with significanthearing exit , ahearing aidmight be prescribe to allow them to hear unremarkably along with the treatments mentioned above for Susac Syndrome .
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