 Welcome to the ProScan Case Review Series, and today's case, a very interesting neurodegenerative one, is part of our core, our board review, and our recredentialing review with questions for those of you that are taking upcoming examinations so we can help you not only raise the bar of care, but do well on your examination. Our first case today is an uncommon, but a very interesting one. Who you pick for Super Bowl? I'm here with my colleague, a technical colleague, Alex Kolesnikov, a Muscovite from Russia, and Mr. Kolesnikov, I'm focused on the case and the education of the people that are watching this educational program, focused on the thing directly in front of me. Okay, what do you think of the Seahawks defense? Please. Mr. Kolesnikov, I'm focused on only one thing, and that is the case material and the educational value of what I am about to present. Will Real Madrid will be in Super Bowl? Will win Super Bowl? Okay. Mr. Kolesnikov, Real Madrid is a soccer team. They are not playing in the Super Bowl, and I am focused on only one thing. The education, material, and quality of the information we're about to present to the audience. So let's get started with the case. This is a 67-year-old man with a gate disturbance. I am showing you on the far left a T1 in the middle of flair, and on the right a diffusion restriction image. Let's look at the first question, shall we? All may be included in the differential diagnosis of restricted diffusion in the cerebral cortex and subcortical region except A, Milos, mitochondrial encephalopathy, lactic acidosis and stroke-like episodes, B, venous hypertensive encephalopathy, C, fatal familial insomnia, D, HIV encephalopathy, and E, prion disease. You can pause the educational program if you wish, but HIV encephalopathy is characterized with patchy or confluent white matter abnormalities, often asymmetric with atrophy, especially cortical atrophy, but it is not typically associated with diffusion restriction on diffusion imaging. Question number two, MRI findings of CJD, also known as Crutsfeld, Yacob disease include all of the following except abnormal T2 and flair, high signal intensity in the cerebral cortex, A, B, cerebral atrophy, C, abnormal T2 intensity in the caudate basal ganglion thalamus, D, restricted regional diffusion in a gyro pattern, sometimes symmetric, and E, post-contrast regional cortical enhancement. The answer is E. Typically, in CJD, there is little to no contrast enhancement, which helps you differentiate this from, say, bilateral cortical infarctions, which at some point will enhance. All of the following are true regarding Crutsfeld-Yacob disease except CJD may be sporadic, familial, iatrogenic, or variant slash mixed. That's A. B. Variant CJD is associated with bovine, spongiform encephalopathy, and transmitted through consumption of contaminated meat. In other words, it's transmitted to humans by eating cow. C. Typical presentation of CJD is slowly progressive dementia and motor dysfunction. D. Lesions in the pulvanar nuclei of the thalamus, the so-called pulvanar or hockey stick sign, can be seen sometimes with sporadic and mostly with variant CJD. The answer to this one is C. CJD patients typically present with more rapidly, but progressive dementia. Myoclonus and multifocal neurologic dysfunction with motor dysfunction being a lesser symptom only in the end game. Question number four. Pryon-mediated disease may present with A. Myoclonus. B. Dementia. C. Parametal signs. D. Cerebellar signs. E. All of the above. The answer is E. All of the above. In addition to MRI, question number five. Diagnostic aids for CJD and other spongiform encephalopathy include sporadic spikes on EEG. A. B. Urine, aerosulphatase or sulfatide. C. Fatty acid screen. D. IgM measurement in C. S. F. E. Serum protein electrophoresis. And the answer is periodic synchronous discharges on the EEG are typical or characteristic for CJD. Another diagnostic test for CJD is the immunoassay for the 14-3-3 protein in the cerebral spinal fluid. There are several diseases that fall into the category of spongiform encephalopathies. There are in fact two major variants of CJD. There's the classic one which is seen in older individuals around age 68 and then the variant one at about age 28 to 30. In the classic disease it is rapidly progressive. Usually resulting in death within four to five months and the variant one they live longer. Perhaps 14 months. In the classic one they have dementia and the variant one they often have psychiatric disturbance. In the classic one they do have the spiked EEG findings. The pulvinar side or the hockey stick side is more typical of variant CJD seen in 75% of individuals. Involvement of the caudate, the butamen and thalamus. Very typical in variant CJD. And diffusion restriction more common as seen here in the cortex in variant CJD. We have it in the motor area in the perirolandic area and along the posterior parietal occipital cortex along with hyperintensity on the flare but little evidence of abnormality on the contrast enhanced T1 weighted image. So now I think I'll turn the tables on my colleague Mr. Kolesnikov. And I have several questions to ask you about the culture in Russia. Do you think dementia is a big problem in Russia as it is in the United States with the aging population? No. Man a few words. Do you worry about eating meat in Russia? No. Man a few words. All right. I think I'll turn the tables on well. I think I'll throw you a zinger then. There are only two animals in which these prions can be transmitted to humans from animals. One of them is the cow so called mad cow disease. I'm willing to bet you don't know what the other animal is. Of course. It's the mink. Well as you can see I'm humbled and stunned. That is the correct answer. Transmissible mink encephalopathy is the other spongiform encephalopathy that is transmitted from animals to humans. This concludes our Super Bowl week of case review preparing you for your examinations and boards. Hope you have a great week and enjoy the game. Go Real Madrid. Go Real Madrid. Thank you. Thank you.