00:02 Here, we have transmissible spongiform encephalopathy In other words, our prion diseases. 00:07 And please make sure that you’re quite familiar with spongiform encephalopathy. 00:12 Unbelievably, it behaves like an infection. 00:15 However, the prions are very, very difficult to kill. 00:20 They are extremely refractory. 00:22 You do everything and these things just live. 00:24 It’s amazing, like survival. 00:27 Here, the diseases include mad cow disease. 00:30 You've heard of Creutzfield-Jakob disease, Gerstmann–Sträussler–Scheinker syndrome, fatal familial insomnia, and kuru. 00:41 These are all different types of prion diseases based on different sources of the prion, which is then causing these particular pathologies. 00:50 A different twist in pathology and microbiology all together prion is. 00:58 So what’s prion disease? All associated with abnormal forms of specific protein, a prion protein, which behaves like it’s become infected, and then worse, it becomes transmissible, fascinating. 01:13 Prion protein, usually, a normal protein neuron. 01:16 Stop there. 01:17 So you have a prion protein, which is normal. 01:20 All of a sudden, it got exposed to something, Creutzfield-Jakob, Kuru, what have you, in which now the prion becomes “infectious.” Prion protein undergoes conformational change from alpha to beta-pleated sheet. 01:37 Make sure that you memorize this. 01:39 This is when it becomes “transmissible.” Now, the prion protein acquires relative resistance. 01:48 When I say relative resistance, this thing, man, it doesn’t want to die. 01:52 It’s like a cockroach. 01:54 Ever tried to kill a cockroach? You try to step on this cockroach and you want to come back and you want to put it into a trash can. 02:00 And you come back, the cockroach is gone. 02:03 It’s unbelievable. 02:04 Prion behaves the same way. 02:06 Very, very resistant to digestion with proteases. 02:11 Infectious nature comes from ability to corrupt the integrity of the normal cellular components. 02:17 Very tricky, this disease, isn’t it, in general? Prion. 02:20 Transmissible spongiform. 02:23 Clinical features of Creutzfield-Jakob disease. 02:28 Dementia, usually. 02:30 It’s like Alzheimer’s. 02:31 Let’s do multiinfarct dementia. 02:34 Is it rapidly or is it insidious in terms of progression? Insidious. 02:38 It takes time, right? But here, we have rapidly progressive dementia. 02:43 It should clue you in. 02:44 Oh, my goodness, does my patient have Creutzfield-Jakob? Seventh decade is the peak. 02:50 Iatrogenic transmissible is possible. 02:54 Subtle changes in memory and behavior followed by rapidly progressive dementia with involuntary jerking movement. 03:00 Clinical presentation of Creutzfield-Jakob disease. 03:05 We do have variants. 03:07 And we have Gerstmann–Sträussler–Scheinker syndrome. 03:10 Inherited disease. 03:12 Progressive dementia with cerebellar ataxia, just a couple of words just so that you’ve heard of such as prion disease. 03:18 Not commonly asked, but still know that it exists, please. 03:22 Let’s do diagnosis of our prion disease. 03:25 Difficult. 03:26 Imaging with our MRI with diffusion-weighted image CSF, this is important. 03:32 Memorize 14-3-3 protein. 03:34 There has to be something that’s been given to you in a stem of a question or clinically in which you are seriously suspecting a prion disease. 03:42 Please memorize 14-3-3 protein in cerebrospinal fluid. 03:47 And you might find abnormal EEG changes, electroencephalogram.
The lecture Transmissible Spongiform Encephalopathy (Prion Disease) and Subacute Spongiform Encephalopathy (Creutzfeldt-Jakob Disease) by Carlo Raj, MD is from the course CNS Infections - Clinical Neurology. It contains the following chapters:
What conformational change occurs in a prion protein when it becomes transmissible?
Which of the following statements regarding prion disease is NOT true?
What is the classic CSF finding found in transmissible spongiform encephalopathy?
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