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Dr.T.V.Rao MD
Definition:
A slow virus disease is a disease that, after
an extended period of latency, follows a slow, progressive course spanning months to years, frequently involving the central nervous system and ultimately leading to death
Characteristics:
Incubation from months to years. Illness from months to years. Involvement of CNS.
Virus JC Virus
Disease Progressive multifocal leukoencephalopat hy BK Nephropathy Subacute Sclerosing Panencephalitis Progressive Rubella Panencephalitis AIDS Adult T-cell Leukemia/Lympho ma Atypical Hairy Cell Leukemia
BK Virus
Polyomavirus
Years to life
Rubeola (Measles)
Paramyxovirus
1-10 years
Rabies Virus
JC
Rhabdoviridae
Rabies
3-12 weeks
Classification
Group A: Group C: Sheep Caused by Lentivirus Subacute sclerosing Group B: Prions CNS Scrapie Mink
Prions
Shortened form of Proteinaceous infectious particles Prions are single molecules containing about 250 amino acids They are abnormal variants of proteins which normally occur in cells Prions have the ability to convert the normal forms that they come into contact with into abnormal forms
Prion Characteristics
No antibiotics can cure disease caused by prions They are not typical of a prokaryotic organism or a eukaryotic organism All that is present in this pathogen is the protein PrPSc, the mutation of PrPC PrPSc is resistant to any form of digestion Prions are non immunogens and do not induce an immune response Prions are not easy to decompose biologically They are resistant to high temperatures
& disinfectants
PrP is a normal cellular protein referred to as PrPc Diseased brain contains aberrant PrP which is referred to as PrPSc PrPSc has the ability to convert PrPc to itself A chain reaction follows, resulting in a cluster of tangled, nonfunctional proteins called plaques, which are aggregates of PrPSc in the brain The body defences remove these PrPSc aggregates leaving behind holes This causes degeneration of the brain cells leading to mental changes and ultimately, death
Prion Hypothesis
test
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brain)
caused by an improper response by the immune
memory loss
Irritability problems with school work.
child experiences: Seizures involuntary movements further neurological deterioration Eventually, the child starts suffering from progressive dementia The optic nerve begins to shrink and weaken (atrophy) and subsequently the child becomes blind.
Diagnosis
Blood tests and spinal fluid reveal high levels
of antibodies to measles virus, and there is a characteristically abnormal electroencephalogram (EEG), or brain wave test. Typically, there is a history of measles infection two to ten years before symptoms begin.
Treatment
There is no standard treatment, and a number
of antiviral drugs have been tested with little success. Treatment of symptoms, including the use of anticonvulsant drugs, can be helpful.
Prognosis
While there may be periodic remissions during
the course of this disease, it is usually fatal (often from pneumonia) within one to three years after onset.
CJD
1/ 1 million
CJD
A Human and Animal / Fatal disease.
Neurodegenerative disorder
Spongiform changes Reactive Amyloidal plaques. Contain Pr P sc Pr P sc is derived from Pr pc Chromosome 20 is location
of abnormality on neurons.
Origins of CJD
Bovine Spongiform Encephalopathy (BSE) was first described in the UK in 1985 as a prionbased disease that affected cattle. In 1996 it was first detected in a human being It was suspected at that time (which turned out to be correct) that humans were contracting the disease from eating cows that had been infected with BSE In humans, it is has been named the Creutzfeldt-Jakob Disease (CJD)
Types of CJD
CJD is classified into 2 forms: Classic CJD & Variant CJD
Classic CJD can be transmitted to other species, however other animals cannot carry it.
Sub classified into: Sporadic CJD and Iatrogenic CJD Sporadic CJD - >85% of Classic CJD cases Most common between 50 75 years Characterized by rapidly increasing dementia Iatrogenic CJD - < 5% Classic CJD cases
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Variant of CJD
BSE Cattle potential Implications in human diseases. Food chain Young can also be infected 12-74 years Psychiatric sensory symptoms Ataxia Myoclones,Dementia Accumulation of Pr psc Amyloid plaques. With Spongiform chains Acquired by oral route, Possible transmission of variant CJD Can happen through surgical Instruments. How Many infected ? Incubation ?
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What is vCJD
Variant Creutzfeldtt-Jakob
CJD were observed in 1996, ten years after the outbreak of BSE in the UK
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Clinical Symptoms
CJD causes fatal degradation of brain tissue & the
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nervous system The symptoms include loss of expressiveness, muscular tremble, spasm, impaired muscle coordination, loss of memory & dementia vCJD patients also display unusual psychiatric problems There is no cure for CJD The condition of the patient deteriorates, finally resulting in death
Encephalopathy It is found on any type of cloven hoofed animals such as: pigs, sheep, and cattle Sheep: Scrapie Spongiform Encephalopathy. There is a human form called Creutzfeldt-Jakob's Disease
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Kuru
Kuru is a disease of man. It causes tremors and
ataxia and often, in later stages, dementia. It is transmitted by rites for the dead which included autopsy and cannibalism in Fore people in Papua/New Guinea. No one born since these practices ceased has acquired Kuru. There is no evidence for transmission to fetus, transmission via milk or intimate social contact.
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