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Progressive Multifocal Leukoencephalopathy or usually referred to as PML is a kind of rare , life - threaten viral , infective health condition resulting in in advance kindling or damaging of white matter in multiple areas of the brain . Progressive Multifocal Leukoencephalopathy is primarily triggered by a virus make as JC or John Cunningham computer virus previously predict as Papovavirus . This virus attack only when the immune system of rules is very much weak or if the patient is suffering from severe immunodeficiency ill likeAIDS , Hodgkin ’s Lymphoma , psoriasis , multiple sclerosisor any other autoimmune diseases . Progressive Multifocal Leukoencephalopathy also take place if the affected role is under immunosuppressants like cyclophosphamide , cyclosporine , methotrexate , Liquid Pred , etc . or have undergone any sorts of organ transplant or immune therapy . Generally , the mortality rate for Progressive Multifocal Leukoencephalopathy varies from 30 % to 50 % in the first few 24-hour interval of the infection and by luck if the patient last he may go through other neurological dysfunctions .
What is Progressive Multifocal Leukoencephalopathy?
Progressive Multifocal Leukoencephalopathy is in reality a neurological disorder characterized by the demyelination of the key nervous system due to the transmission of a human polyomavirus named as JC virus which is a minute three-fold strand DNA virus without any envelop surrounding it . This etiological computer virus was insulate and identified during the genius postmortem of a patient named John Cunningham post his death and thus key out as JC computer virus in his honour . This virus is quite far-flung and has been seen to be present in almost 80 - 85 % of normal adult populations . Although these viruses remain dormant in normal healthy individual but begins showing symptom of Progressive Multifocal Leukoencephalopathy if the person has highly watery immune system . Progressive Multifocal Leukoencephalopathy occurs in 1 in 200,000 population , approximately and thus is very rare .
The word ‘ progressive ’ in Progressive Multifocal Leukoencephalopathy means that the disease keep on getting spoilt and thus head to death by severe brain damage . ‘ Multifocal ’ in Progressive Multifocal Leukoencephalopathy refers to that JC virus causes infection in various voice of the brain . in the end the terminus ‘ leukoencephalopathy ’ stand for that the contagion spreads mainly in the medulla cocktail dress or white matter of the brain and very seldom to the gray thing of the nerve cell .
Although the occurrence of JCV infections is symptomless as well as chronic in human populations , but the incidence of this infection is hardly found in ecumenical intelligent populations . dotty type JCV infection is usually benign , whereas Progressive Multifocal Leukoencephalopathy is lead due to viral infection along with rarified transmissible alteration . The appearance of Progressive Multifocal Leukoencephalopathy also depends on loss of server - immune abilities and therefore susceptibility of the disease is triggered by the combining of host factors and wandering viral infection .
Signs and Symptoms of Progressive Multifocal Leukoencephalopathy
sign and symptoms of Progressive Multifocal Leukoencephalopathy bulge developing over few hebdomad or months , depending on the degree and localization of the damage in the brain . Progressive Multifocal Leukoencephalopathy symptoms unremarkably alter from one patient to another and likewise the wound of contagion also change from one website to the other mainly in the occipital or parietal lobes of the Einstein . Progressive Multifocal Leukoencephalopathy patients normally show signs of neurologic harm . Some of these common and frequently appeared mark and symptom of the disease include :
Epidemiology of Progressive Multifocal Leukoencephalopathy
Progressive Multifocal Leukoencephalopathy is seen to be linked with immunosuppressed conditions , but also witness to happen in leukemia or organ transplanted patients . today non - HIV people are also getting the disease . In the master stage , it is find that reform-minded multifocal leukoencephalopathy seldom occurs in immunocompetent patient role . The relative frequency of reform-minded multifocal leukoencephalopathy is quite low in Africa and India because of probable diagnostic divergence or barriers in isolation of JCV .
In character of an AIDS patient , if CD4 counts as 50 to 100 cells per uL , then commonly these affected role get progressive multifocal leukoencephalopathy symptoms and researches have shown that progressive multifocal leukoencephalopathy tends to occur in 5 % ( approx . ) of the PM of fateful AIDS patients . Recently , reformist multifocal leukoencephalopathy has see to be relate with Natalizumab , i.e. , an IgG monoclonal antibody used for treating relapsing put off multiple sclerosis . to boot , Progressive Multifocal Leukoencephalopathy is also seen to be triggered in immunocompromised affected role , that is , whose immune system is just on the edge of recovery . Progressive Multifocal Leukoencephalopathy is also classically involved with IRIS ( immune reconstitution rabble-rousing syndrome ) .
Prognosis of Progressive Multifocal Leukoencephalopathy
Prognosis of Progressive Multifocal Leukoencephalopathy varies with different comorbidities . Within the first 3 - 9 calendar month of the disease happening , mortality pace is quite in high spirits i.e. , about 30 - 50 % and without any discourse progressive multifocal leukoencephalopathy can be fatal just in about a class . Presence of JC virus - specific cytotoxic T - cells generally tend to increase the endurance of somebody affected with progressive multifocal leukoencephalopathy . Therefore forecast of the disease is very much hapless and with the intervention of medical assistance selection chance increase a mo along with inescapable long - terminus neurological dysfunctions . However , recent researches have prove that Progressive Multifocal Leukoencephalopathy can be resolve with viral headroom along with providing reenforcement and restoring host immune social occasion . Nevertheless , this may lead to IRIS with symptom like monolithic fervour in the brain and drastically degraded clinical and pathologic conditions . Therefore , only way to take on reformist multifocal leukoencephalopathy is former diagnosis , controlling excitement and managing immune subprogram in society to render the patient maximum relievo from the disease symptoms till some good medical therapy is discover .
Causes of Progressive Multifocal Leukoencephalopathy
The firstly causative broker of Progressive Multifocal Leukoencephalopathy is a human polyomavirus mention as JC virus . multitude with feeble resistant system unremarkably get the infection of JCV that finally guide to progressive multifocal leukoencephalopathy . Other reason to explicate reform-minded multifocal leukoencephalopathy with weak resistant scheme include :
This JC computer virus is usually present in the brain or off-white marrow of healthy people in an still state . Many people get infect by the computer virus but very few actually show symptoms .
Pathophysiology of Progressive Multifocal Leukoencephalopathy
The pathophysiology of Progressive Multifocal Leukoencephalopathy is still under research . JCV infection is usually inveterate and the kidney is the primary website from where the infection starts diffuse and thus the virus is commonly shredded through urine . JCV go into into the body following the oral or respiratory path and remain latent in the mental capacity , kidney or lymph reticular tissue . JCV is carried to the CNS by B complex lymph cell and due to immunosuppression the computer virus pay off combat-ready and causes systemic dissemination in the brain .
Tat , an HIV gene product , has the power to trans - activate the JCV promoter right away . In oligodendrocytes , the viral transmission is lytic . It goes through DNA replication and viral capsid protein deductive reasoning inside the cell . After this , JCV infects other cells from the circumference chiefly near the fundamental nidus and thus leads to the spreading of the demyelinating lesion . astrocyte enlarge and mostly get infected by JCV and therefore lead to bizarre appearance which resemble the giant tumor cells make love as astrocytomas .
fit in to some recent discipline , the transformation of benign JCV peripheral infection to a rare pathogenic CNS contagion mainly depends on the combination of viral and host immune factor and the replicating capacity of JCV which varies with the mutant in the NCCRs or VP1 viral capsid protein and this also spay the infective demyelination contagion in the brain .
Risk Factors of Progressive Multifocal Leukoencephalopathy
Patients get infected with JCV and develop progressive multifocal leukoencephalopathy mainly because of frail resistant scheme . Probable risk divisor which may alter or weaken immune system include :
Diagnosis of Progessive Multifocal Leukoencephalopathy
Progressive Multifocal Leukoencephalopathy can be enquire through histopathological or by virological , clinical or radiological confirmation . Tests may admit :
Treatment of Progressive Multifocal Leukoencephalopathy
Till now there are no specific drug to cure or bottle up viral causing JCV without any toxic result . Thus , discourse only affect in hold on or slowing of the disease progress or just reversing back the affected role ’s immune scheme . AIDS patient with Progressive Multifocal Leukoencephalopathy and undergoing HAART have picture little chance of recollective - terminal figure survival . Therefore , treatment strategy for Progressive Multifocal Leukoencephalopathy let in :
Hexadecyloxypropyl - Cidofovir is currently studied as a probable treatment for JVC because the drug is able-bodied to crush JVC by obstructing viral DNA replication .
Chemotherapeutic drug Cytarabine or ARA - C has been notify experimentally for non - AIDS Progressive Multifocal Leukoencephalopathy affected role and is seen to simmer down down minor neurological complaints . It is ground that some of the patient recovered cognitive functioning . It is also seen that Mefloquine , antimalarial drug has in effect been used against JCV natural action . According to the patients report , there was all over viral headroom and also no foretoken of worsening of neurologic consideration further .
A number of other drugs work in effect against JCV in culture medium , but there is no particular drug or therapy still now discovered to provide successful therapeutic against progressive multifocal leukoencephalopathy in humans .
Due to the fact that no proper treatment is yet identified for Progressive Multifocal Leukoencephalopathy , a mortal with compromised immune scheme must be very conservative . Although , the prognosis is very broken , a right diagnosis and timely treatment can increase the survival twenty-four hour period .
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