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

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.