Overview: Prions and Prion-induced Diseases.pptx

An overview of Prions and Prion-induced Diseases, from the aspect of a 3rd year medical school student

1. Prions and Prion-induced Diseases

2. Prion's property pure protein with malignant change/misfolding “self-replication”/“infectious” transmittable to normal protein Prp related membranous interaction only exception of central dogma located on short chain in 20th chromosome amyloid formation/spongiform encephalopathy absolute fatality with low rate of proper diagnosis chronic change in CNS only susceptible to protein related disinfect method could be inherited

3. Main property of human prion disease degenerative CNS change spongiform encephalopathy loss of neurons without inflammation abnormal Prp accumulation a taxia myoclonus /muscle spasm dementia positive sign of pyramidal system or extrapyramidal damage

4. Examples of human prion disease Creutzfeldt–Jakob disease (spontaneously,induced by tissues or inherited) nvCJD / Bovine Spongiform Encephalopathies Fatal familial insomnia Gerstmann syndrome Kuru disease

5. Creutzfeldt–Jakob disease and nvCJD Creutzfeldt–Jakob disease Early symptoms : memory problems, behavioral changes, poor coordination, and visual disturbances. Later symptoms: dementia, involuntary movements, blindness, weakness, and coma occur . About 90% of people die within a year of diagnosis ,radpidly progress. nvCJD/ Bovine Spongiform Encephalopathies Similar symptoms to CJD,inaddition to CJD,nvCJD's patients may suffer from mental disorders at early stage. Slow progress,can be caused by prion containing food.Most cases appear in Britain for eating beef. First dementia,then ataxia.

6. Fatal familial insomnia Autosomal dominant inheritance progressive insomnia with Inattention, memory loss, nervousness and hallucinations. rare cases with dementia ACTH↓,glucocorticoids↑ catecholamine↑, abnormal rhythm of GH, prolactin and melatonin secrection

7. Gerstmann syndrome predominantly in middle-age progressive cerebellar ataxia with atrophy of lower extermities later with mental disorder and dementia spongiform foams in brain amyloid change of CNS neuron loss with glial cells hrperplasia few leukocytes infiltration

8. Kuru disease first discribed infectious degenerative neural disease appeared only in a tribe with cannibalism in africa not much record after ceasing the custom spongiform foams in brain amyloid change of CNS neuron loss with glial cells hrperplasia few leukocytes infiltration First ataxia,then dementia,along with proimnent tremor.

9. Clinical examination EEG MRI examination of cephalospinal fluid biopsy autopsy immunochemistry examination of PrPsc

10. Criteria of diagnosis progressive dementia myoclonus typical EEG change no other indication of other diseases spongiform degenerative encephalopathy neuron loss glial cells hyperplasia PrPsc postive area in brain

11. Treatment and prophylaxis No effective treatment only control of disease progression and elevate patient's life quality no effective vaccine only can control by isolation of sick animals and patients,killing of sick animals