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 sheet to beta-helix
    3. Beta-helix to alpha-pleated sheet
    4. Alpha-helix to beta-helix
    5. Alpha-pleated sheet to beta-pleated sheet
    1. Prion's infectious nature comes from it's association with a viral particle.
    2. Prion protein acquires relative resistance to digestion with proteases.
    3. Prions are highly infectious particles.
    4. Kuru is an example of prion disease.
    5. 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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