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Slow Virus Diseases

Dr. Hind AbdulRahman Unit: Dr Sumaia Al Asad

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.

Absence of Immune response.


Genetic predisposition. Leads to Fatal illness.

Virus JC Virus

Virus family Polyomavirus

Disease Progressive multifocal leukoencephalopat hy BK Nephropathy Subacute Sclerosing Panencephalitis Progressive Rubella Panencephalitis AIDS Adult T-cell Leukemia/Lympho ma Atypical Hairy Cell Leukemia

Typical latency Years to Life

Epidemiology Unknown; Contaminated water?

BK Virus

Polyomavirus

Years to life

Unknown; respiratory spread/urine?


Respiratory droplets Respiratory droplets Infected blood or other body fluids Infected blood or other body fluids Infected blood or other body fluids Bite of an infected animal

Rubeola (Measles)

Paramyxovirus

1-10 years

Rubella HIV HTLV 1 HTLV 2

Togaviridae Retrovirus Retrovirus Retrovirus

10-20 years 5-10 years 10-30 years 10-30 years

Rabies Virus
JC

Rhabdoviridae

Rabies

3-12 weeks

virus & BK virus only cause disease in immuno-compromised patients

Classification
Group A: Group C: Sheep Caused by Lentivirus Subacute sclerosing Group B: Prions CNS Scrapie Mink

panencephalitis, Progressive multifocal Leucoencephalopathy

encephalopathy Kuru Creutzfeldt-Jacob disease Subacute spongiform viral encephalopathy

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

What are Prion Diseases?


Prion diseases are among the most intriguing infectious diseases and are associated with unconventional Proteinaceous infectious agents known as prions. Prions seem to lack nucleic acid and propagate by transmission of protein misfolding. The nature of prions and their unique mode of transmission present challenges for early diagnosis of prion disease

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

Prions and Resistance:


Formaldehyde 3.7% Boiling, Ethanol 50% Ionizing Radiation House hold bleach. Autoclaving at 121 c I1 hour

Resistant to all above methods of Sterilization

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

Inherited Prion Disorders


CJD Gerstmann Straussler Syndrome Fatal Familial insomnia Human to Human Kuru Iatrogenic From Bovine to Human Variant of CJD Investigation at Autopsy Pr Psc Accumulates in the CNS Detection with Western Blot

test
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Acquired Prion Diseases


Iatrogenic CJD Human to Human 1974 Corneal graft Instruments Human growth Hormone. Incubation 20 years

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Sub acute sclerosing pan encephalitis (SSPE)


Definition Subacute sclerosing panencephalitis is a rare, progressive brain disorder caused by an abnormal immune response to the measles virus. Description This fatal condition is a complication of measles affects children and young adults before the age of 20 usually occurs in boys more often than in girls extremely rare, appearing in only one out of a million cases of measles.

Causes and symptoms a form of measles encephalitis (swelling of the

brain)
caused by an improper response by the immune

system to the measles virus.


The condition begins with: behavioral changes

memory loss
Irritability problems with school work.

As the neurological damage increases, the

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

Dementia, Neurological Involvement


Effects person with Genetic predisposition.

Gerstmann Strassler Scheinker syndrome


Dementia Pr p plaques.

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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Transmission of prion via medications & surgical equipment

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

Disease (vCJD), is caused by the consumption of BSE infected meat products


Variant CJD seems to affect

mostly young patients


The first 10 cases of variant

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

Mad Cow Disease


Scientific Name: Bovine Spongiform

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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Initially thought to have been caused by a

slow viruses, these were classified as slow Viral Diseases


Now there is evidence to point out Prions

as the causative factor

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Mad Cow Disease: What is it?


The Mad Cow Disease in Cows, Scrapie in Sheep, the Creutzfeldt-Jakob Disease in human beings belong to a class of disease called Transmissible Spongiform Encephalopathy (TSEs) Initially thought to be due to slow viruses, due to the long incubation period between time of infection and appearance of disease, these are now known to be caused by agents called prions.
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Kuru: shivering or trembling


in Fore tribe of New Guinea , who used to eat dead human body.

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Women and children are high risk.

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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