Презентация на тему The brain eater

leads to dementia and, ultimately, death.


Symptoms of Creutzfeldt-Jakob disease

(CJD) sometimes resemble those of other dementia-like brain disorders.


Creutzfeldt-Jakob is rapidly progressive.

What is Creutzfeldt-Jakob disease?

1920.
In 1921 Alfons M. Jakob described 4 cases with

2 resembling what today is referred to as CJD.





In 1974, a case iatrogenic CJD was reported via corneal transplantation.

History of CJD

of spread through contaminated human derived growth hormone.

approximately equal to one per million
May be underestimated.
More common

in individuals above 60 years.
vCJD is more prevalent in younger individuals.
Average life span after onset of symptoms is 4 months.

and animal diseases known as transmissible spongiform encephalopathies (TSEs).



Etiology

abnormal protein known as a prion.
Prions were first discovered

in the 1960's by radiation biologist Tikvah Alper and the mathematician John Stanley Griffith.
Prions are proteins with an abnormal fold known as an amyloid fold.
They have very stable structures in the form of beta pleated sheets.
Prions do not multiply in the host organism that they infect.

Etiology

mutation of an individual’s own normal proteins

Variant CJD- acquired

from using contaminated human growth hormone or consuming contaminated meat (bovine or human).

Familial CJD- inheritance of a mutated gene for PrP.

Iatrogenic- through contaminated surgical sources.

Transmission

is present in CJD CNS tissue.
The normal variant of

this protein is  PrPC.
PrPSc deposits in the CNS of CJD patients causing dysfunction, and in the presence of PrPSc, PrPC is converted to PrPSc.
In the case of familial CJD, a mutated form of the prion protein gene appears to lead to prion protein deposition.

Pathogenesis

of a mutated prion protein gene as a transgene

in mice was found to induce a spongiform neuropathology.
This suggests that the mutant PrPSc is sufficient to produce disease.
The pathogenesis of sporadic Creutzfeldt-Jakob disease remains unclear. 
It has been hypothesized that a spontaneous somatic change in conformation of prion protein in the CNS initiates the disease. 

Pathogenesis

demonstrate the presence of prion-like elements in yeast.
Experiments show

that these elements lead to aggregation and amyloid formation of a protein.
These studies suggest that prions as a cause of abnormal phenotypes may be more widespread than realized.

Pathogenesis

a number of neurodegenerative diseases.
These diseases may not be

transmissible in the same way as the sub acute spongiform encephalopathies such as CJD are.
However, they are assumed to affect the CNS in a prion like mechanism.
In addition, the pathogenic proteins are also misfolded. 

Pathogenesis

evident cerebral atrophy.
Microscopic findings are similar to those

of other prion diseases with neuronal loss, astrocytosis, and the development of cytoplasmic vacuoles in neurons and astrocytes.
Amyloid plaques that contain the abnormal PrP are found in the areas of infected tissue in most cases.
There is no inflammation.
The cortex and basal ganglia are most affected, but all parts of the neuraxis may be involved.
Early lesions are more severe in the gray matter

Pathology

dementia in middle or late life.
Vague, prodromal symptoms of

anxiety, fatigue, dizziness, headache, impaired judgment, and unusual behavior may occur.
Once memory loss starts, it progresses rapidly, and other characteristic signs appear, sometimes abruptly.

Clinical Manifestations

are pyramidal tract disease
weakness
stiffness of the

limbs
accompanying reflex changes
Extrapyramidal signs
Tremor
rigidity,
Dysarthria
slowness of movement
myoclonus (often stimulus sensitive).

Clinical Manifestations

difficulties with movement, swallowing and talking.
In the final stage,

patients lose all mental and physical function and may lapse into a coma.
Many patients die from an infection such as pneumonia.
The average duration of disease from the onset of symptoms to death is four to six months.
Ninety percent of patients die within a year. 

Clinical manifestations

and/or Western blot
confirmed protease-resistant rP; and /or presence of
scrapie-associated

fibrils

Diagnostic Criteria CDC

out of the following four clinical features:
Myoclonus
Visual or cerebellar

signs
Pyramidal/extrapyramidal signs
Akinetic mutism
AND  a positive result on at least one of the following laboratory tests:
a typical EEG (periodic sharp wave complexes) during an illness of any duration; and/or
a positive 14-3-3 cerebrospinal fluid (CSF) assay in patients with a disease duration of less than 2 years
Magnetic resonance imaging (MRI) high signal abnormalities in caudate nucleus and/or putamen on diffusion-weighted imaging (DWI) or fluid attenuated inversion recovery (FLAIR).

Diagnostic Criteria CDC

cadaveric-derived pituitary hormone
Sporadic CJD with a recognized exposure risk,

e.g., antecedent neurosurgery with dura mater implantation.

Diagnostic Criteria CDC

a first degree relative
Neuropsychiatric disorder plus disease-specific PrP gene mutation.
Diagnostic Criteria

CDC

occurring at intervals of 0.5-1.0 seconds.

excluded from blood, organ or other body tissue donations.
Identify

source of infection
MANAGEMENT OF CONTACTS
Patients with potential exposure to CJD should be informed of their risk

Control

to slow down CJD's progression
Treatment on trial

Creutzfeldt-Jakob disease Changes not only in the brain?. Neurology, 79(10), 965-966.
de

Villemeur, T. B. (2012). Creutzfeldt-Jakob disease. Handbook of clinical neurology, 112, 1191-1193.
Merritt, H. H. (2010). Merritt's neurology. L. P. Rowland, & T. A. Pedley (Eds.). Lippincott Williams & Wilkins.
Riley, D. E., Lang, A. E., & Lewis, A. (2010). Creutzfeldt–Jacob Disease.Encyclopedia of Movement Disorders, 1, 263.
Sikorska, B., Knight, R., Ironside, J. W., & Liberski, P. P. (2012). Creutzfeldt-Jakob disease. In Neurodegenerative Diseases (pp. 76-90). Springer US.

References