Moderate viral or prion maladies of the focal sensory system

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Moderate viral or prion ailments of the focal sensory system

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Slow popular ailments of the focal sensory system beat of clinical sickness extended brooding period (may likewise be extended course of illness) numerous neurological indications

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SLOW INFECTIONS IN HUMANS VIRUSES SV40-like infections (PML) measles infection (SSPE) rubella infection (PRP) ATYPICAL AGENTS Kuru, Creutzfeld-Jakob ailment (CJD) (new) variation CJD malady (vCJD=nvCJD)

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Progressive multifocal leukoencephalopathy Polyoma infection family, SV40-like (JC infection and so forth) dynamic, normally deadly, connected with resistant concealment HAART may draw out life in AIDS patients however little impact on PML frequency commonly non incendiary yet can get a provocative reaction in the mind after HAART treatment (insusceptible reconstitution fiery disorder) demyelination (oligodendrocytes tainted)

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SYMPTOMS weakness discourse issues psychological issues cerebral pains walk issues visual issues tactile misfortune seizures http://library.med.utah.edu/WebPath/TUTORIAL/AIDS/AIDS076.html

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Progressive multifocal leukoencephalopathy reactivation of idle disease 70-80% populace are seropositive connected with immunosuppression 1979: 1.5 for each 10,000,000 populace 2004: 1 in 20 AIDS patients

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BK infection (polyoma) Associated with urinary tract infections in immunosuppression Possibly contributory figure prostate tumor???

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MEASLES VIRUS paramyxovirus family (morbillivirus variety) sub-intense sclerosing panencephalitis fiery sickness imperfect infection ~1-10 yrs after beginning contamination early contamination with measles is a hazard figure uncommon intricacy of measles (7-70 cases for each 1,000,000 cases measles) immunization ensures against SSPE

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RUBELLA VIRUS togavirus family (rubrivirus class) dynamic rubella panencephalitis incendiary ailment years after introductory contamination intrinsic/early contaminations exceptionally uncommon

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transmissible subacute spongiform encephalopathies transmissible cerebral amyloidoses prion ailments

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http://www.cdc.gov/ncidod/dvrd/cjd/( Ermias Belay)

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TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES (TSEs, TRANSMISSIBLE CEREBRAL AMYLOIDOSES, PRION DISEASES) human Kuru Creutzfeldt-Jakob ailment (CJD) Gerstmann-Straussler-Scheinker disorder (GSS) deadly familial a sleeping disorder (FFI) variation CJD ('human BSE') creature scrapie (sheep and goats) ox-like spongiform encephalopathy (BSE) transmissible mink encephalopathy and so on

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ATYPICAL AGENTS atypical infections atypical operators prions

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SIMILAR TO VIRUSES little filterable need have cells no apparatus for vitality era or protein amalgamation DIFFERENT FROM VIRUSES no perceptible virions in tainted tissues no recognizable virions in purged irresistible material if nucleic corrosive is available, little extremely impervious to inactivation ATYPICAL AGENTS

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RESISTANT TO OR ONLY PARTIALLY INACTIVATED BY : formaldehyde ethanol glutaraldehyde bright and ionizing illumination non-ionic cleansers INACTIVATED BY : autoclaving (121C for 60 minutes) (> standard) 5% sodium hypochlorite sodium hydroxide proteases , urea, other protein denaturants

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decontaminated irresistible material protein exhibit (PrP) proteases inactivate nucleic corrosive disputable however little or none PRION

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PRION DISEASE CNS LONG INCUBATION SLOW COURSE OF DISEASE (FATAL) SPONGIFORM ENCEPHALOPATHY VACUOLATION OF NEURONS FIBRILLAR AGGREGATES, AMYLOID-TYPE MATERIAL (frame plaques) RARE IN MAN http://www.cdc.gov/ncidod/dvrd/cjd/( Ermias Belay)

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Helical - Happy Beta-creased sheet - Bad PRION PROTEIN (PrP) (have cell quality) PrP or PrP C alpha-helical protease touchy PrP RES or PrP SC beta-creased sheet protease safe

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PrP + PrP SC

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unconstrained change SPORADIC gained transformation ACQUIRED obtained PrP SC germline transformation, unconstrained change more probable INHERITED substantial transformation, unconstrained transformation more probable SPORADIC

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WHY ARE DIFFERENT PRION DISEASES DIFFERENT? Appears to be MORE THAN ONE CONFORMATION FOR THE PrP SC FORM

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PrP 1 + PrP SC PrP 2 + illness brought about by adaptation 1 may contrast from that created by compliance 2 PrP SC

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WHY ARE PRION DISEASES SOMETIMES INHERITED? Transformations IN THE PrP GENE CAN INCREASE THE CHANCE OF PrP SC FORMATION

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germline transformation 1 INHERITED germline transformation 2 INHERITED ailment brought about by germline transformation 1 may contrast from that created by germline transformation 2

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SCRAPIE sheep loss of strong control squandering glial multiplication vacuolation of neurons amyloid plaques anomalous properties irresistible material does not appear to cross sheep/human species boundary

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KURU human illness tremors, ataxia, shortcoming dementia, passing amyloid plaques spongiform changes transmission – contact with irresistible material

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CREUTZFELDT-JAKOB DISEASE spongiform appearance of cerebrum at post-mortem examination dementia, myoclonus, ataxia 16-80+, more often than not 50-70 Median age at death in US=68 yrs 10% familial likewise sporadic shape additionally obtained frame (eg. iatrogenic CJD) a few hundred passings in US for each year

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CREUTZFELDT-JAKOB DISEASE traditional shape no confirmation for direct individual to individual transmission blood drain other body liquids imply social contact

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CREUTZFELDT-JAKOB DISEASE iatrogenic CJD human corpse development hormone human dead body gonadotropin dural mater unites corneal transplantation neurosurgical instruments stereotactic EEG terminals

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variation CJD (vCJD) patients more youthful at presentation, more extended course of sickness middle age at death for UK vCJD patients=28 yrs frequently patients give psychiatric side effects BSE association appears to be cow-like/human hindrance is less demanding to cross than sheep/human obstruction unmistakable neurotic appearance particular properties of the PrP res operator is in some fringe tissues lymphoid tissues 2 plausible situations where transmitted by blood future? two cases in US – both had invested energy in UK

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Belay et al (2005) Emerging Infectious Diseases • www.cdc.gov/eid • 11: 1351

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CDC/Teresa Hammett , Photo Credit: Sherif Zaki; MD; PhD; Wun-Ju Shieh; MD; PhD; MPH

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Amino corrosive 129 Met or Val? - both variations found in the human populace Britain 37% of the UK populace is MM 100% of clinical vCJD cases are MM Are MV, VV invulnerable to vCJD, or will they create vCJD later on? 83% of sporadic CJD cases are MM Britain, France, Japan overabundance of VV in development hormone beneficiaries with iatrogenic CJD does heterozygosity (M/V) offer some security?

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OTHER HUMAN PRION DISEASES Gerstmann-Sträussler-Scheinker disorder (GSS) ( familial ) engine now and again viewed as subclass of CJD deadly familial a sleeping disorder (FFI) circadian cadence issues hypothalamus

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IMMUNE RESPONSE no fiery reaction no interferon acceptance no counter acting agent reaction no cell-intervened reaction

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TREATMENT perpetually lethal endeavors at medication treatment baffling blood mind obstruction

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DIAGNOSIS CLINICAL PICTURE, EEG, MRI (vCJD) USUALLY CONFIRMED POST-MORTEM NOW HAVE ANTIBODIES RAISED IN RECOMBINANT MICE can use on biopsy of cerebrum (or fringe lymphoid tissue in vCJD)

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PLAQUES PrP NOT THE SAME AS IN ALZHEIMERS

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http://www.cdc.gov/ncidod/dvrd/vcjd/

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Belay, E. http://www.cdc.gov/ncidod/dvrd/vcjd/chart_percent_vcjd_cjd_deaths.htm

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