 Good morning. Okay, so today we're going to talk about a case that I got to see the first week that I was here Which was pretty exciting for me. We had a 49 year old Lady come in. She was previously healthy. She was a retired business professor Had retired at 49 because they they'd sold some type of pharmaceutical company So she'd done pretty well for herself and she had a two month history of lower extremity weakness hearing loss and Nuan set decreased visual field loss in the right eye So this at all began about a couple months prior August 2010 She kind of seen sir been seen at several different hospitals She presented initially to Provo, Utah with some weakness and sensory loss in her legs Urinary incontinence Vertigo and gait instability She had an MRI at the outside hospital and that showed some increased T2 flare signals in the deep cortex She also had an EMG there that showed some decreased amplitude in the tibial nerves She was diagnosed there with Guillain Bray. She was treated with five days of IVIG Discharged with physical therapy and she thought for the the next six weeks that she was improving She had from then she had an onset of gradual hearing loss in her right ear Worsening gait instability. They started on started her on prednisone and BAM cycle of ear She also had a new onset of a severe Biotemporal headache some increased weakness and Kind of numbness in the lower extremities worse on the right. She had another MRI at that time which showed Some more signal in on T2 flare, which looked like MS And she was experiencing some pretty severe vertigo at that time and she was transferred to a Colorado hospital She had an INO. She was hyper reflexic She had an LP there, but the CSF showed no oligoclonal bands, but did show some elevated protein Pretty much pan normal lab work up as you can see Kind of got the full full spectrum line HSV Rocky Mountain spotted fever and she was treated with three days of IV steroids at that time So she was discharged. She was still having some of the symptoms She got a dose of IV IG didn't really improve And then she started having some confusion some word-binding difficulties some cognitive slowing And so when she presented to the University of Utah She had a right foot drop She had hearing loss on the right side and she'd woken up the day prior with some decreased visual field on the right side There was no pain with Extracular movement her acuity was great. She was 2020 with correction and over top As far as past medical history as I mentioned at the beginning She was previously healthy had some gestational diabetes and a bout of Acute gastroenteritis while traveling in Ireland pretty recently her only medications were a multivitamin She'd been started on Lyrica a couple months prior for The worsening headaches for migraine and one of the physicians had started on doxy for a suspected Lyme disease Ocular history she'd worn glasses since the age of 12 And notice in quotations it says ocular migraines because that's those are the patients words I know that's not a preferred term here and past neurological history just the migraines with or since age of eight but Maybe one every six months and pretty good relief with ibuprofen One episode of I lost the consciousness when she was nine due to a skiing accident and she was out for a couple days there Family history was significant for a cerebral aneurysm and a sister at age 40 and a maternal grandmother who'd also had a hemorrhage No family history of a mess So on exam again visual acuity was really good. She'd 2020 OU pressures were great kind of really the only abnormality there is stereo and then fields to confrontation she had peripheral also central sparing and No deficits in the left eye and she did not have an I know when she presented here So here are her prior scans This was the first one that we have and you can see the a couple of hyper intense signals here This is the scan the next scan that we have the second MRI that she had and you can notice a little area of hypo Intensity right here in the corpus callosum She continued to have several hyper intense regions This is when they started really seriously considering MS and then the scan that she had when she presented here Again, you can see these areas of hypo intense regions. I think also kind of here You can start to see those Again, several hyper intense lesions on MRI kind of scattered throughout and then fundus photographs So right eye and she's got an occlusion right here left. I look pretty good initially And I'm got FA and some clear abnormalities here these areas of hyper intensity and then you can see the Articulation left. I also showed the areas of hyper intensity and in the periphery another occlusion We were able to get her records from I Believe was Colorado where they got this study and it showed Wyoming And it showed low frequency hearing loss and so differential diagnosis So kind of on the list at that time was multiple sclerosis Atom Lyme disease. She'd been diagnosed previously with Guillain-Barre and then to sex syndrome So she actually kind of meets all the criteria for Seuss X syndrome. It was first described in 1979 There's at least 200 cases reported It usually affects young women and it favors women three to one The classic triad is encephalopathy Hearing loss and branch retinal artery occlusions. They're usually bilateral and so we see all three in this case The pathology of it is it's thought to be an immune mediated endothelial apathy It affects the brain the retina and the cochlea on a this is From a paper where they were able to do a retinal biopsy of a patient who unfortunately passed away while they were experiencing Seuss X syndrome in it You can see these serious deposits kind of in between the the membrane and the vasculature Interiorly and those are found Kind of intermittently throughout The arteries and also there's some thickening of the walls in the areas where there's not the serious deposits And so that the thickening is noted to be different from diabetic thickening. It's serious versus Aglaculated PAS positive with diabetes and not all vessels were thickened with Seuss X patients There is no evidence of thrombus in any of the occluded vessels It looked like it was just from the endothelial swelling and some of the articles actually kind of compared it to juvenile Dramatomyositis, which also has occluded vessels from endothelial swelling and it kind of hinted at maybe some glial cell dysfunction With the sequestration of the fluid None of the brain biopsies showed any necrosis in the vessel wall so it's not a vasculitis It's a vasculopathy And the mechanism is still really unknown But some of the theories that have been put out have been either Antidethyloantibodies or a T cell attack or maybe compliment mediated damage the classic findings on MRI Really the corpus closer Is patho mnemonic and then cephalopathic patient it's best seen on a sagittal T2 flare Which may not always be ordered so if you suspect Susak you want to get a sagittal T2 flares MRI the micro infarcts and prefer the the central portion of the corpus closum and it's described as snowballs with Evolving to holes and it occurs only in Susak Another finding is the string of pearls and it's a studying of the internal capsule by micro infarcts I've got the pictures of that best shown on diffusion weighted imaging Leptomine and geol enhancement is also classic in gray matter involvement So here are the snowball pictures in the corpus closum and that Evolved into the holes, which we saw actually with our patient and the string of pearls Picture here on diffusion weighted imaging So an FA you'll see arterial wall hyperfluorescence. It's often multifocal With Brent's retinal artery occlusions, and they may be peripheral again as we saw in our patient and left eye There was one at the far periphery If it's normal initially and you suspect Susak they recommend doing repeated FAs At differing intervals another unusual feature of this syndrome is are the gas plaques and they're actually kind of mid-segment Plaques in the arterials which are different from the hole in horse plaques, which are usually at the bivocations Then cephalopathy evolves subacutely Often these patients will present with psychiatric features. They may be found in the psych wards They'll have extensor planter responses may have seizures and frequently have headaches hearing loss due to embarkation of cochlea Frequently associated with vertigo much like our patient presented with low frequency hearing loss and this is one of the in terms of prognosis one of the the main residual Factors for Susak's is that they often continue to have hearing loss and they'll require cochlear implants So often the the patients kind of get worked up for atypical MS or atypical atum And so some of the differences between that In MS and a them the closer involvement is usually under the close them and in Susak's the central fibers of the close and more involved and It's demyelination versus microembarks MS doesn't usually have leptomeningial enhancement And deep gray matter involvement is usually also rare in MS as is deafness So there's a lot of a lot of words on the slide, but kind of The the basic is the traditional treatment is usually pulse treatment with IV methyl prednisone Followed by high-dose oral prednisone and then IV IG And then cyclophosphamide or or micro phenylite some papers discuss a differing treatment plan for those patients who just present with out encephalopathy Although it follows this a similar pattern IV IG steroids They it may be at a Slower taper, but they caution that that you know with the unpredictability of encephalopathy that you may still want to treat pretty aggressively for this Additional treatment options are Ritux Mab, which is a monoclonal antibody to just CD 20 positive B cells and those are found only on mature B cells not on the stem cells or the plasma cells It takes about eight months to replace those cells If patients aren't responding to conventional therapy, you might want to consider plasmapheresis For those patients who were initially diagnosed with MS and were treated with interferon It has not been shown to be successful for treatment and often those patients worsen clinically Monitoring includes serial ophthalmology exams Following with audiology neuro psych testing and repeat MRIs The prognosis can vary it can be monocyclic there can be remission after a couple of years and they never have another episode of this It can be polycyclic where it recurs again kind of in a short period Or it can be chronic where they have prolonged continuous course in which the disease is active They frequently see that for the patients who just present with Branch retinal artery occlusions there was one patient that had a kind of a chronic course over about 30 years So in summary C-section syndrome, it's frequently misdiagnosed It's a triad of encephalopathy hearing loss and branch retinal artery occlusion the pathology is thought to be an autoimmune endothelial disorder and the treatment is usually with steroids With the length of treatment depending on patient response The newest therapies for tuximab and you want to follow the patients closely if during the steroid taper They're decompensating You want to increase the dosage prognosis again varies from complete remission to Continued cognitive impairment and hearing loss But blindness is actually rare So for our patient her clinical course She received a three-day course of IVIG and developed some leucopenia pretty severe Her husband was kind of concerned at that time and actually wanted to work back up for Lyme disease Which they did and got an infectious disease consult But it was all negative. She didn't they didn't agree to steroid treatment and she got three days of solumedrol And I was going to be started on retuximab therapy closer to home as Far as her condition on discharge. She was still having pretty unsteady gait. She's still at the right of her field visual defect and was Still having some weakness in her legs Questions. I didn't show the visual fields, you're right And I didn't because they were not very reliable. She when she came to us. She was she was very very tired very confused and I Think kind of fell asleep during the visual fields actually. I believe they're from the the vascular occlusions Yes Any other questions, okay, thank you