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Susac's Syndrome

I have a close friend who is now a patient in the neuro science ward at a university hospital. She has suffered a very quick downward spiral in her condition. First symptoms she has were vertigo, numbness in her arm & leg, and pain in her eyes. This was 8 weeks ago.  In the last 30 days she has gone from bad to worse, in the hospital undergoing multiple tests. Unstedy gait, lost contact with reality and confusion are now added to her symptoms.  They have found lesions in the brain on MRI and the one thing they seem to be leaning towards is an Auto Immune Disease of some sort. Another idea they are thinking is Susac's Syndrome. What is this syndrome and what are treatments and long term effects?
7 Responses
Avatar universal
Hi trin,
How is your friend now?
What are the symptoms associated with your friend?
Does he have deafness?
Susac's syndrome is a classical symptom diagnosed with 3 clinical entity called encephalopathy (These patients are often encountered on the psychiatric floor with personality change and bizarre and paranoid behavior), branch retinal artery occlusions (problem with vision) and hearing loss.
MRI is always diagnostic, the white matter, especially the corpus callosum, is always involved. There is a preferential involvement of the central portion of the corpus callosum with relative preservation of the peripheral portion. The clinical course is self-limited, ranging from one to four years, but usually lasts from two to four years in most cases. During this fluctuating clinical course, the patient may have had one or more episodes of the triad but there is a definite remission noted at four years.
Can you tell me what the MRI report is?
Avatar universal
I will find out some more information over the weekend.  They are now waiting for one last test that they will do on Monday to confirm Susac's Syndrome. From what I have read Susac's is extremely rare with only 200 reported cases in the world. They are still not ruling out MS.  Being she is in a University Hospital every student wants to come now and observe and check into her eyes. Her symptoms are as followed:

Early on she had severe cases over dizziness (which the doctors perscribed Antivert).
Early on she also had occasions of numbness in her arms and legs. Then came a feeling of her ears being (what she would call) plugged up.  Also ringing in her ears. (No detection of ear infection was found, yet at that the time was suggested she see an ENT). Uninsured at this time was a main problem though at this time.
Unstedy gait (she started walking like she had a 12 pack) occured next, which she still continues to have. Around 30 days ago, a major difference in her behavior started, at first I thought she was having some sort of depreession.  But that was quickly ruled out.  And admitted to the hospital she has been.  Her behavior is very childlike, her speech is impaired (her words don't come out very fast and she doesn't make sense most of the time). She is very agitated, getting up and down constantly out of the bed. She has 24 hour CNA at her side.Her leg shakes uncontrolably at times (can't remember if its just one or if its alternating) She has had 5 MRI's, 3 of them unsucessful cause she can't lie still.  White lesions have showed up on the MRI's though. A series of eye tests have been performed. On Monday they are performing one more test which they believe will confirm Susac's. They do have her on steroids now. I am concerned with what sort of normality will her life return to?
Avatar universal
It is beyond expectations and anybody’s reach to think of her having a life as good as before these symptoms.
I would like to know what test they are performing and what the result is.
Do post us back on newer findings.
Avatar universal
Hi, my name is Carla and I was reading your questions on Susac's Syndrome. One year and a half ago I had the  almost identical illness happen to me. I was a basically healthy 39 year old mother of three boys. My only medical problems at this point was an anxiety disorder and I had  episodes of post pardom depression after the birth of each of my children which was treated with Zoloft successfully. By anxiety I am refering to panic attacks. I had been on Zoloft for 12 years with no further depression. My Primary care doctor had temporarily put me on a .25 xanax at bedtime to stop the panic attacks that I had that woke me up out of my sleep. this worked well,but my doctor's office had a policy that they could't percribe any controlled substances eventhough this was an extremly low dose of xanax. They refered me to a phyciatric clinic to get this medication. The doctor there immediatley had a problem with the xanax because he said it could become addictive. He decided to take me off the xanax and also the Zoloft and replace them with (Paroxetine)which is the generic Paxil. He did't tapper either drug and told me to start the paroxetine the next day. Six weeks into the Paroxetine I was standing in the grocery store with my then 5 year old son, looking at chicken when suddenly I became very confussed  and could't figure out what the prices said. my vision was messing up in a very wierd way. over the next week I experienced sevierly distorted vision, extreme light sensitivity,flashing and flickering lights.  I called the doctor who percribed the paroxetine because I suspected the new medicine since nothing else had been changed. He told me it was unheard of six weeks into the medicine and raised the dose from 30mg to 40mg. I never made it to the pharmacy to fill the new dose. These symtoms worsened and I got scared of this medicine and did'nt take it again. The doctor wouldn't see me until he had an opening which was three weeks later. I just kept getting sicker by the day. Other symtoms that started were personality changes(extreme irritability, sevier depression and axiety worse than anything I had ever experienced in the   past. vomiting, diareah, shooting pain in my abdomin and upper legs, headache, and distorted vision so bad I couldn't recognize my own children's faces. I went to two major ER's where they took me to a phsyciatic hospital. I kept telling them I couldn't see, but they were convinced that I was crazy.  After I made myily come and get me out of there I went to a private phycologist who put me immediately back on my Zoloft and Xanax. Both doses had to be raised to get  me back right with my depression and anxiety. Anyway after 6 eye doctors they finally done a visual field test because they could't find any thing wrong with my eyes and I had 20/20 vision. the visual field test showed a visual field defect on the left side of both my eyes which is called a harmonious inferior quadrantopia. They then believed me when   I said I couldn"t see and got  me to a neurologist. The first neuroligist done an MRI  and said I had white matter lesions on my brain and diagnosed me with MS. He hadn't  even done a blood test on me. He sent  me home with about ten needles full of MS medicine to give myself shots of this . I had been studing this over the eight months that it took them to take me seriosly that I couldn't see and I knew he had'nt done enough testing to   diagnose me with MS, so I got antother opinion from another nerologist who started doing the other test(bloodwork, enhanced MRI, spinal tap, Pollyomerse chain reaction to rule out other diseases. All of  my test came back negitive and I was also Negative for the oblonical banding that is characteristic in MS. So this neurologist temporarlly diagnosed me with ADEM. Since then I have had MRI's every six months to check for more lesions to see if I have MS(4 scans in all) None of these showed new lesions. So since I am practacally a self learned neurologist and have been studing this on the computer(after my vision improved enough to see the screen , I put in my three main symtoms which were white matter lesions in the corpus collosum ,vertigo/tinnitis, and vision changes. THE computer pulled up Susac's syndrome and it sounded identical to what I have been through. So I called My neurologist and asked him to read about this syndrome and he did. He said he is going to have the radiologist study up on it and compare my lesions to the ones in th literature and has ordered a hearing test which I am going to this week. I would love to talk to   your friend or you to compare our symptoms and maybe help eachother get through this nightmare.  If you want to reach me my phone number is (865-429-3424) and my name is Carla Goins.
Avatar universal
Susac syndrome is a self-limited syndrome, presumably autoimmune, consisting of a clinical triad of encephalopathy, hearing loss and branch retinal artery occlusions. All three elements of the triad may NOT be present or recognized, and MR imaging is most likely necessary to establish the diagnosis. I believe there are only about 200-201 people in the world who are known to have Susac's Syndrome, that's extremely rare!

Treatments may consist of:  intravenous methylprednisolone, followed by oral steroids in conjunction with the use of intravenous immunoglobulin (IVIG). In some instances, they have added cyclophosphamide (Cytoxan) to these two agents at the very onset, but would reserve plasmapheresis (is a blood purification procedure used to treat several autoimmune diseases. It is also known as therapeutic plasma exchange.) for any breakthrough symptomatology ( the combined symptoms of a disease.) or if the patient is not improving with the above. I am not sure what the outcome is. I have read that the relatively infrequent recognition of this syndrome has provided few cases for systematic study of possible causative factors.I hope this is of help....

6754866 tn?1384459690
Can't believe this, omg.. i have had Susac Syndrome' since the start of this year i'm the latest on the list..I am the 1st and only 1 in Ireland.Omg your friend defo has it. they need to get her on treatment fast. Before she completely loses her sight and hearing i have lost both partially, but i'm managing..... I lost my mind as well didn't know anyone at the start.. Omg so much to tell you i don't even know where to start.. Contact me any time.. Im only learning to cope at the moment, life was normal before all this, she may get constant Tinnitus, like i have, tell her not to be afraid..xxxx
6754866 tn?1384459690
I have Susac's syndrome and this sounds a fair bit similar to how it started with me, Keep on at your doctors as quickly as you can and don't leave with answers and treatment straight away. Please!! For yourself and your kids,, Goodluck with it all...x
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