The best treatment to be prescribed for a 10 years old male diagnosed with Dawson's disease is a combination of intraventricular interferon alfa plus oral Isoprinosine.
Dawson's disease is another name for Subacute Sclerosing Panencephalitis (SSPE), which is a progressive brain disorder caused by a defective measles virus. The passage clearly states that a combination of intraventricular interferon alfa plus oral Isoprinosine is currently the best effective treatment available for SSPE. The other options listed are not treatments recommended for this condition.
This presentation briefly summarizes pathophysiology, clinical features, diagnosis and treatment of different types of tuberculosis of brain and spinal cord.
SSPE, dr. amit vatkar, pediatric neurologistDr Amit Vatkar
Subacute sclerosing pan encephalitis (SSPE) also known as Dawson Disease, Dawson encephalitis, and measles encephalitis is a rare and chronic form of progressive brain inflammation caused by a persistent infection with measles virus.
In this presentaion i will a case a sspe and give u some information regarding daignosis and treatment
This presentation briefly summarizes pathophysiology, clinical features, diagnosis and treatment of different types of tuberculosis of brain and spinal cord.
SSPE, dr. amit vatkar, pediatric neurologistDr Amit Vatkar
Subacute sclerosing pan encephalitis (SSPE) also known as Dawson Disease, Dawson encephalitis, and measles encephalitis is a rare and chronic form of progressive brain inflammation caused by a persistent infection with measles virus.
In this presentaion i will a case a sspe and give u some information regarding daignosis and treatment
Progressive multifocal leukoencephalopathy (PML) is a disease of the white matter of the brain, caused by a virus infection that targets cells that make myelin--the material that insulates nerve cells (neurons). Polyomavirus JC (often called JC virus) is carried by a majority of people and is harmless except among those with lowered immune defenses. The disease is rare and occurs in patients undergoing chronic corticosteroid or immunosuppressive therapy for organ transplant, or individuals with cancer (such as Hodgkin’s disease or lymphoma). Individuals with autoimmune conditions such as multiple sclerosis, rheumatoid arthritis, and systemic lupus erythematosus -- some of whom are treated with biological therapies that allow JC virus reactivation -- are at risk for PML as well. PML is most common among individuals with HIV-1 infection / acquired immune deficiency syndrome (AIDS). Currently, the best available therapy is reversal of the immune-deficient state, since there are no effective drugs that block virus infection without toxicity. Reversal may be achieved by using plasma exchange to accelerate the removal of the therapeutic agents that put patients at risk for PML. In the case of HIV-associated PML, immediately beginning anti-retroviral therapy will benefit most individuals. Several new drugs that laboratory tests found effective against infection are being used in PML patients with special permission of the U.S. Food and Drug Administration. Hexadecyloxypropyl-Cidofovir (CMX001) is currently being studied as a treatment option for JVC because of its ability to suppress JVC by inhibiting viral DNA replication.
In general, PML has a mortality rate of 30-50 percent in the first few months following diagnosis but depends on the severity of the underlying disease and treatment received. Those who survive PML can be left with severe neurological disabilities.
Disorders of amino acid metabolism
Disorders of renal amino acid transport
Disorders of carbohydrate metabolism and transport
Carbohydrate-deficient protein syndromes
carbohydrate metabolism and transport
Disorders of fatty acid oxidation
Disorders of purine and pyrimidine metabolism
Disorders of lipid and lipoprotein metabolism
Ceroid lipofuscinosis and other lipidoses.
Disorders of serum lipoproteins
Lysosomal disorders
Peroxisomal disorders
Disorders of metal metabolism
Porphyrias
Progressive multifocal leukoencephalopathy (PML) is a disease of the white matter of the brain, caused by a virus infection that targets cells that make myelin--the material that insulates nerve cells (neurons). Polyomavirus JC (often called JC virus) is carried by a majority of people and is harmless except among those with lowered immune defenses. The disease is rare and occurs in patients undergoing chronic corticosteroid or immunosuppressive therapy for organ transplant, or individuals with cancer (such as Hodgkin’s disease or lymphoma). Individuals with autoimmune conditions such as multiple sclerosis, rheumatoid arthritis, and systemic lupus erythematosus -- some of whom are treated with biological therapies that allow JC virus reactivation -- are at risk for PML as well. PML is most common among individuals with HIV-1 infection / acquired immune deficiency syndrome (AIDS). Currently, the best available therapy is reversal of the immune-deficient state, since there are no effective drugs that block virus infection without toxicity. Reversal may be achieved by using plasma exchange to accelerate the removal of the therapeutic agents that put patients at risk for PML. In the case of HIV-associated PML, immediately beginning anti-retroviral therapy will benefit most individuals. Several new drugs that laboratory tests found effective against infection are being used in PML patients with special permission of the U.S. Food and Drug Administration. Hexadecyloxypropyl-Cidofovir (CMX001) is currently being studied as a treatment option for JVC because of its ability to suppress JVC by inhibiting viral DNA replication.
In general, PML has a mortality rate of 30-50 percent in the first few months following diagnosis but depends on the severity of the underlying disease and treatment received. Those who survive PML can be left with severe neurological disabilities.
Disorders of amino acid metabolism
Disorders of renal amino acid transport
Disorders of carbohydrate metabolism and transport
Carbohydrate-deficient protein syndromes
carbohydrate metabolism and transport
Disorders of fatty acid oxidation
Disorders of purine and pyrimidine metabolism
Disorders of lipid and lipoprotein metabolism
Ceroid lipofuscinosis and other lipidoses.
Disorders of serum lipoproteins
Lysosomal disorders
Peroxisomal disorders
Disorders of metal metabolism
Porphyrias
Avian encephalomyelitis (AE) is a viral disease of the CNS of young chickens, turkeys, Japanese quail, pheasants, and pigeons. Turkeys are less susceptible to natural infection and generally develop a milder clinical disease than chickens. Ducklings and guinea fowl are susceptible to experimental infection
1. Al-Quds University
School of Medicine
Microbiology
SSPE
Prepared by : LAYTH HUSSEIN
Autumn semester-2012
2.
3. Subacute Sclerosing Panencephalitis (SSPE) is
a serious disorder of the central nervous system.
It is a slow virus infection caused by defective
measles virus.
Dawson, Pette and Doring, Greenfield.
(SSPE) is a progressive, debilitating, and deadly
brain disorder.
4.
5. SSPE has been reported from all parts of the
world.
IN US (one per million) childhood population. IN
INDIA (21 per million) ,in comparison with (2.4 per
million) in the Middle East.
Primary measles infection at an early age (<2
years), which is followed, after a latent period of
6–8 years .
male/female ratio 3:1. not for measles.
Widespread immunization has produced greater
than 90% reduction in the incidence of SSPE
6. Measles is caused by an RNA virus, which belongs to the
marbillivirus.
Despite the long interval between the acute infection and
symptoms of SSPE, there is evidence that measles virus infection
of brain occurs soon after the acute infection with subsequent
spread throughout the brain.
Measles virus is thought to reach the brain through infection of
cerebral endothelial cells OR by circulating inflammatory cells.
Recently, a trans-synaptic transmission of virus has been
suggested.
extensive mutations (SLOW) in viral genome-that encodes the
envelop proteins. (still UNCLEAR).
may include several immunological factors. For example, in
tissue culture, the addition of antibodies against measles virus
may alter the pattern of viral gene expression .
7. The defective virus:
A virus that by mutation has lost the ability to be replicated in the
host cell without the aid of a helper virus. The virus particles
(virions) contain all the viral structural components; they can
attach, penetrate, and release their nucleic acid (RNA; DNA) within
the host cell. However, since the mutation has destroyed an
essential function, new virions will not be made unless the cell was
simultaneously infected with the helper virus, which can provide
the missing function. Only then will the cell produce a mixed
population of new helper and defective viruses.
The most important group of defective viruses are deletion
mutants. They are derived from their homologous nondefective
(wild-type) virus through errors in the nucleic acid replication that
result in the deletion of a fragment in the
newly synthesized molecules.
8. Signs
Parents and teachers may notice progressive
deterioration in scholastic performance.
mild intellectual deterioration and behavioral
changes.
Myoclonic jerks initially involve the head and
subsequently trunk and limbs. Muscular contraction
is followed by 1–2 seconds of relaxation.
Papillo-oedema,Papillitis,Optic atrophy, blindness.
Seizures ,Unsteady gait.
9. diagnosis
There may be a history of measles in an unvaccinated child. A
physical examination may reveal:
Damage to the optic nerve, which is responsible for
sight
Damage to the retina, the part of the eye that
receives light
Muscle twitching
Poor performance on motor (movement)
coordination tests.
The following tests may be performed:
Electroencephalogram (EEG).
Brain MRI.
ELIZA.
Spinal tap.
10.
11.
12.
13.
14. Expectations (prognosis)
Persons with this disease
frequently die 1 to 2 years
after diagnosis, but some
may survive for longer
periods. The condition is
always deadly.
15. Treatment
Prevention is the corner stone-Immunization
against measles is the only known prevention
for SSPE. The measles vaccine has been highly
effective in reducing the numbers of affected
children.
Combination of intraventricular interferon alfa
plus oral Isoprinosine is the best effective
treatment available.
16. A 10 years old male, was diagnosed of
dawson’s disease. The best to be
prescribed in this case is :
1-colchecine.
2-penicillin.
3- Isoprinosine.
4-interferon alpha.
5-3+4