 All right, welcome everybody to Grand Rounds this morning. We have a neuro Grand Rounds from neurology residents who are gracious enough to come and teach us a little bit. So our first presenter is going to be Alicia Bennett. She's a 30-year resident as well as me. So she's from Oregon and then did her medical school in Des Moines University. And she's going to present a case about Seussac syndrome. Thank you. Okay, well thank you. I chose this case in part because I had never seen a patient with Seussac syndrome and then I'm gonna be doing a stroke fellowship in the next year and a half. And so I will eventually be taking care of patients with this disorder. So I wanted to learn a little bit more about it. So to start off with a case, this is a patient of Dr. Warners who I saw in follow-up in clinic. And she initially presented in October of 2010. She was 49 years old at that time and she was referred to neuro ophthalmology clinic for evaluation of right eye vision loss. Two months prior to this, she had experienced ascending weakness and sensory loss in both legs with associated incontinence, vertigo and gait instability. And EMG had been done at that time and it suggested that she had Guillain-Barre syndrome so she was treated with a course of IVIG and she did have improvement of her symptoms with this treatment. Six weeks after this, she developed hearing loss on the right, she had worsening gait and she was noted to have an INO and hyperreflexia. She had an audiogram that showed sensory neural hearing loss on the right and she was treated with prednisone but did not have any benefit. So when she came to the University of Utah, she had an MRI that showed T2 hyperintensities and these were in deep and sub-portical locations as well as in the cerebellum. She had a lumbar puncture done which was normal except for an elevated protein of 78 but olivoclonal bands and other studies were negative. On the day prior to her evaluation in clinic, she had sudden loss of vision in her right eye. In clinic, back in 2010, her exam showed encephalopathy and then she had visual field testing that was not consistent due to this encephalopathy but she was noted to have a branch retinal artery occlusion in the inferior nasal vessel of the right eye. So a diagnosis of Susax was made and she was started on rituximab and then restarted a course of IVIG. So this is her visual field at the time but like I said, not totally consistent due to her encephalopathy. Here are pictures of her right eye and you can see here that this vessel is not normal here and then on fluorescent angio again, we're looking closer at this vessel and here are her pictures at 16 and 25 seconds and again you can see that the inferior nasal vessel is not filling normally and then she has some hyperfluorescence in this vessel over here which is not occluded. So to talk about Susax syndrome, it was first described by Susax in 1979 after he took care of two patients that had branch retinal artery occlusions, hearing loss and neurologic symptoms. He first termed the disorder microangiopathy of the brain and retina and then in 1996, it was suggested that the disease be called Susax syndrome. It affects women more than men in a ratio of three to one and the mean age of onset is 28 but cases have reported patients from the ages of 18 to 59 and then the triad consists of B-R-A-O, hearing loss and neurologic symptoms. It's often chronic and has a relapsing course. So to go through the triad, the ophthalmologic findings typically patients will complain of blurred vision and photopsia. There can be multiple B-R-A-Os and it's typically bilateral. Oftentimes, the B-R-A-Os will be in the periphery and patients won't complain of clinical symptoms but these occlusions can also affect larger branches. There can be retinal artery wall hyperfluorescence like we saw in our patient and this can occur remotely from the occluded vessels. If you see the hyperfluorescence in combination with other symptoms like the hearing loss or neurologic symptoms and this can be pathodemonic for Susac and then the last finding would be retinal artery wall plaques or also known as gas plaques and these are typically located randomly between arterial bifurcations which is in contrast to emboli which will be at the bifurcation and these can be transient and disappear. So here are examples of all these findings. So in the right picture at the arrows you can see the gas plaques which again are between the bifurcations and then on the left you can see retinal artery occlusion such as here with the hyperfluorescence in this case next to the occlusion. So the neurologic symptoms can be quite variable. Most patients will complain of headache and this will often be a first symptom that can be months before the other symptoms arise and typically the headache will be migraness. Other possible symptoms include stroke-like symptoms, neuropsychological deficits, long track signs, focal neurologic signs and even seizures and these can be the most disabling parts of the disease for these patients. There's also a multitude of MRI findings that you can see. So often times you'll see multifocal lesions. There can be multiple infarcts in the white and gray matter. There will be snowball-like lesions in the center of the corpus callosum which I'll show you in a second and then leptomeningeal or parenchymal enhancement. So here is an example of these snowball lesions and I think the right image is a little bit better but you can see just these kind of punctate focus of hyperintensity in the corpus callosum and these are different than the Dawson's fingers of multiple sclerosis which are going to be a finger-like projection that's projected into the white matter. So just a little bit different there. Yeah, I think just small vessel infarcts. So then the progressive hearing loss. This will often be very sudden and can occur even overnight. It can affect both ears and it's typically of low and middle frequencies. It can be accompanied by tinnitus and vertigo and it's thought to be from occlusion of the cochlear pericapillary arterials and those of the semicircular canals. Many patients will require cochlear implants and hearing devices. So the pathogenesis is thought to be from an autoimmune process leading to damage and inflammation related to occlusion of the microvessals and these are typically seen in the brain retina on ear thus causing the triad that we've been talking about. There have been case reports of an anti-endothelial cell antibodies which support the autoimmune hypothesis and these have also been seen in Shogun syndrome. There's not perivascular information seen in this disorder like you see in typical vasculitis. So the diagnosis is typically made when you see the combination of the clinical features that I've told you about. Most times you'll have documentation of a BRAAO by Flores and Angio or the retinal artery wall plaques or the retinal artery wall hyperfluorescence. So any combination of those. The characteristic findings on MRI, the clinical course and the headaches can all be supportive of this diagnosis. So there's two common courses of this disease. The first is a monophasic predominantly encephalopathic course that is shorter and typically limited to one to two years. The prognosis is good in these patients if you treat them early and aggressively. Then there's a recurrent course that involves a BRAAO and hearing loss and this will be longer and the encephalopathy is typically less severe. Treatment has not been well established because this is so rare and there have not been any randomized control trials but it is agreed that treatments will either be of a combination with these treatments. So IV steroids followed by an oral taper. Some will be treated with IVIG and I saw some papers even talking about doing plasmapheresis in these patients and then immunomodulators like psychophosphamide, mycophenolate, mophitil and rtoxamab had been used. Most clinicians will treat with aspirin to reduce the procoagulopathic state and then other supportive treatment like cochlear implants are typically used. So just to follow up with our case, she did have remission of her branch retinal artery occlusion but then had recurrence in April of 2011 with recurrent vasculitis and a recurrent BRAAO in the same region of her initial occlusion. She's currently being treated with cell sept and IVIG every four weeks. She was treated with rtoxamab for approximately four months and then was treated with psychophosphamide for a period of time but she's remained fairly stable on the cell sept and IVIG at this time. And then she was later also diagnosed with subacute combined degeneration from B12 deficiency and so she's being followed in neurology clinic for this as well as her seusax and then in neuro-optimology clinic and that's all. Any questions? Yeah?