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Transmissible Spongiform Encephalopathy (Prion Disease) and Subacute Spongiform Encephalopathy (Creutzfeldt-Jakob Disease)

by Carlo Raj, MD

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    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.


    About the Lecture

    The lecture Transmissible Spongiform Encephalopathy (Prion Disease) and Subacute Spongiform Encephalopathy (Creutzfeldt-Jakob Disease) by Carlo Raj, MD is from the course CNS Infection—Clinical Neurology. It contains the following chapters:

    • Transmissible Spongiform Encephalopathy (Prion Diseases)
    • Creutzfeldt-Jakob Disease

    Included Quiz Questions

    1. Alpha-helix to beta-pleated sheet
    2. Alpha-pleated to beta-helix sheet
    3. Beta-helix to alpha-pleated sheet
    4. Alpha-helix to beta-helix sheet
    5. Alpha-pleated to beta-pleated sheet
    1. A prion's infectious nature comes from its association with a viral particle.
    2. A prion protein acquires relative resistance to digestion with proteases.
    3. Prions are highly infectious particles.
    4. Kuru is an example of prion disease.
    5. A prion protein is usually a normal protein in neurons.
    1. 14-3-3 protein
    2. 14-3-13 protein
    3. 14-13-3 protein
    4. 14-30-13 protein
    5. 14-3-30 protein

    Author of lecture Transmissible Spongiform Encephalopathy (Prion Disease) and Subacute Spongiform Encephalopathy (Creutzfeldt-Jakob Disease)

     Carlo Raj, MD

    Carlo Raj, MD


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