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==Differentiating Progressive multifocal leukoencephalopathy from other Diseases==
==Differentiating Progressive multifocal leukoencephalopathy from other Diseases==
PML is similar to another demyelinating disease, [[multiple sclerosis]], but since it destroys the cells that produce myelin (unlike MS, in which myelin itself is attacked but can be replaced), it progresses much more quickly.
Progressive multifocal leukoencephelopathy (PML) is more common among [[Immunocompromised|immunocompromised patients]] who are at high risk for other [[fungal]], [[bacterial]], and [[viral infections]]. It should be differentiated from the following diseases:
{| class="wikitable"
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Disease
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Differentiating signs and symptoms
! align="center" style="background:#4479BA; color: #FFFFFF;" + |Differentiating tests
|-
|[[Lymphoma|CNS lymphoma]]<ref name="pmid20212226">{{cite journal |vauthors=Gerstner ER, Batchelor TT |title=Primary central nervous system lymphoma |journal=Arch. Neurol. |volume=67 |issue=3 |pages=291–7 |year=2010 |pmid=20212226 |doi=10.1001/archneurol.2010.3 |url=}}</ref>
|
* Patient is [[immunocompetent]]
* Focal symptoms indicative of a mass [[lesion]]
* [[Seizure]]
|
*Single solitary ring enhancing [[lesion]] on [[CT]] or [[MRI]]
|-
|[[Disseminated tuberculosis]]<ref name="pmid21740673">{{cite journal |vauthors=von Reyn CF, Kimambo S, Mtei L, Arbeit RD, Maro I, Bakari M, Matee M, Lahey T, Adams LV, Black W, Mackenzie T, Lyimo J, Tvaroha S, Waddell R, Kreiswirth B, Horsburgh CR, Pallangyo K |title=Disseminated tuberculosis in human immunodeficiency virus infection: ineffective immunity, polyclonal disease and high mortality |journal=Int. J. Tuberc. Lung Dis. |volume=15 |issue=8 |pages=1087–92 |year=2011 |pmid=21740673 |doi=10.5588/ijtld.10.0517 |url=}}</ref>
|
* Prior history of residence in an [[Endemic (epidemiology)|endemic]] area
* Chronic [[cough]], [[weight loss]], [[hemoptysis]]
|
* [[PCR]] of [[CSF]] for [[tuberculosis]]
* Mycobacterial culture of [[CSF]]
* [[Brain]] biopsy for [[acid-fast bacilli]] staining
* Culture and acid stain positive for [[acid-fast bacilli]]
* CXR shows [[Cavitation|cavitations]]
|-
|[[Aspergillosis]]<ref name="pmid10194462">{{cite journal |vauthors=Latgé JP |title=Aspergillus fumigatus and aspergillosis |journal=Clin. Microbiol. Rev. |volume=12 |issue=2 |pages=310–50 |year=1999 |pmid=10194462 |pmc=88920 |doi= |url=}}</ref>
|
* [[Pulmonary]] [[lesions]] in addition to [[CNS]] [[lesions]]
* Symptoms may include [[cough]], [[chest pain]], and [[hemoptysis]]
|
*[[CSF]] fungal culture, [[galactomannan]]
|-
|[[Cryptococcosis]]
|
*Symptoms include [[cough]], [[chest pain]], and [[hemoptysis]]
|
*[[Cryptococcal infection|Cryptococcal]] [[antigen]] from [[CSF]] and [[serum]]
*[[CSF]] fungal culture
|-
|[[Chagas disease]]<ref name="pmid20399979">{{cite journal |vauthors=Rassi A, Rassi A, Marin-Neto JA |title=Chagas disease |journal=Lancet |volume=375 |issue=9723 |pages=1388–402 |year=2010 |pmid=20399979 |doi=10.1016/S0140-6736(10)60061-X |url=}}</ref>
|
*History of residence in Central or  South America
*Acute infection is rarely symptomatic
*[[Encephalitis]] or focal [[brain]] [[lesions]]
*[[Myocarditis]]
*[[Chronic]] [[infections]] in [[immunocompromised]] patients develop into [[encephalitis]] with [[necrotic]] [[brain]] lesions causing a [[mass effect]]
|
*[[Trypanosoma cruzi]] in [[blood]], [[Tissue (biology)|tissue]], or [[CSF]], [[PCR]] of [[Tissue (biology)|tissue]] or [[body fluids]], and [[Serological testing|serologic tests]]
|-
|[[Cytomegalovirus infection|CMV infection]]<ref name="pmid11215290">{{cite journal |vauthors=Emery VC |title=Investigation of CMV disease in immunocompromised patients |journal=J. Clin. Pathol. |volume=54 |issue=2 |pages=84–8 |year=2001 |pmid=11215290 |pmc=1731357 |doi= |url=}}</ref>
|
*Most common [[CNS]] [[opportunistic infection]] in [[AIDS]] patients
*Presents with [[encephalitis]], [[retinitis]], progressive [[myelitis]], or [[polyradiculitis]]
*In [[disseminated disease]], it involves both the [[liver]] and kidneys
|
*[[Brain]] [[CT]]/[[MRI]]/[[biopsy]]: location of [[lesions]] is usually near the [[brain stem]] or periventricular areas
*[[PCR]] of [[CSF]] with detectable [[virus]] is diagnostic
*[[Brain biopsy]] with + [[staining]] for [[CMV]] or evidence of owl's eyes is also diagnostic, but it is rarely performed because of the location of [[brain]] lesions
|-
|[[HSV|HSV infection]]<ref name="pmid1919640">{{cite journal |vauthors=Bustamante CI, Wade JC |title=Herpes simplex virus infection in the immunocompromised cancer patient |journal=J. Clin. Oncol. |volume=9 |issue=10 |pages=1903–15 |year=1991 |pmid=1919640 |doi=10.1200/JCO.1991.9.10.1903 |url=}}</ref>
|
*[[Seizures]], [[headache]], [[confusion]] and/or [[urinary retention]] can be seen in [[disseminated disease]], which usually affects only the [[immunocompromised]] or acute [[infections]]
*In [[pregnant]] women, it may be associated with concurrent [[genital]]/[[oral]] [[lesions]]; can be spread to the [[neonate]] during acute infection in the mother, or via [[viral shedding]] in the [[birth canal]]
*[[Neonatal]] [[Herpes simplex virus|HSV]] can range from localized [[Skin and soft-tissue infections|skin infections]] to [[encephalitis]], [[pneumonitis]], and [[disseminated disease]]
|
*[[Brain]] [[CT]]/[[MRI]]/[[biopsy]]: location of [[lesions]] is usually the [[medial]] [[temporal lobe]] or the [[Orbital cavity|orbital]] surface of the [[frontal lobe]].
*[[PCR]] of [[CSF]] with detectable [[virus]] is diagnostic
|-
|[[Chickenpox|Varicella Zoster infection]]<ref name="pmid15864101">{{cite journal |vauthors=Hambleton S |title=Chickenpox |journal=Curr. Opin. Infect. Dis. |volume=18 |issue=3 |pages=235–40 |year=2005 |pmid=15864101 |doi= |url=}}</ref>
|
*Multifocal involvement has subacute course, usually only in [[immunosuppressed]], with [[headache]], [[fever]], focal deficits, and [[seizures]].
*Unifocal involvement is more typically seen in [[immunocompetent]] hosts, occurring after [[contralateral]] [[cranial nerve]] [[herpes zoster]], with [[Altered mental status|mental status changes]], [[TIA|TIAs]], and [[stroke]]
*[[Disseminated disease|Disseminated]] [[varicella zoster virus]] can occur in adults during primary [[infection]], presenting with [[pneumonitis]] and/or [[hepatitis]]
*Disease is a [[Vasculitis|vasculopathy]] with [[hemorrhage]] and [[stroke]]
|
*[[PCR]] of [[CSF]] with detectable [[virus]] is diagnostic
|-
|[[Brain abscess]]<ref name="pmid24174804">{{cite journal |vauthors=Alvis Miranda H, Castellar-Leones SM, Elzain MA, Moscote-Salazar LR |title=Brain abscess: Current management |journal=J Neurosci Rural Pract |volume=4 |issue=Suppl 1 |pages=S67–81 |year=2013 |pmid=24174804 |pmc=3808066 |doi=10.4103/0976-3147.116472 |url=}}</ref><ref name="pmid25360205">{{cite journal |vauthors=Patel K, Clifford DB |title=Bacterial brain abscess |journal=Neurohospitalist |volume=4 |issue=4 |pages=196–204 |year=2014 |pmid=25360205 |pmc=4212419 |doi=10.1177/1941874414540684 |url=}}</ref>
|
*Associated with [[sinusitis]] (abutting the sinuses) or with [[bacteremia]]
*Signs and symptoms includes [[fever]] and [[necrotizing]] [[brain]] [[lesions]] with [[mass effect]]
|
*[[CSF]] culture or culture of [[brain abscess]]
|-
|[[Progressive multifocal leukoencephalopathy]]<ref name="pmid20298966">{{cite journal |vauthors=Tan CS, Koralnik IJ |title=Progressive multifocal leukoencephalopathy and other disorders caused by JC virus: clinical features and pathogenesis |journal=Lancet Neurol |volume=9 |issue=4 |pages=425–37 |year=2010 |pmid=20298966 |pmc=2880524 |doi=10.1016/S1474-4422(10)70040-5 |url=}}</ref>
|
*Symptoms are often more insidious in onset and progress over months. Symptoms include progressive [[weakness]], poor [[coordination]], with gradual slowing of [[mental]] function. Only seen in the [[immunosuppressed]]. Rarely associated with [[fever]] or other systemic symptoms
 
|
*[[Polymerase chain reaction|PCR]] of [[CSF]] for [[JC virus]]
*[[Biopsy]] reveals [[white matter]] [[lesions]] and not well-circumscribed [[lesions]].
|}


==References==
==References==

Revision as of 16:09, 14 August 2017


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Differentiating Progressive multifocal leukoencephalopathy from other Diseases

Progressive multifocal leukoencephelopathy (PML) is more common among immunocompromised patients who are at high risk for other fungal, bacterial, and viral infections. It should be differentiated from the following diseases:

Disease Differentiating signs and symptoms Differentiating tests
CNS lymphoma[1]
Disseminated tuberculosis[2]
Aspergillosis[3]
Cryptococcosis
Chagas disease[4]
CMV infection[5]
HSV infection[6]
Varicella Zoster infection[7]
Brain abscess[8][9]
Progressive multifocal leukoencephalopathy[10]
  • Symptoms are often more insidious in onset and progress over months. Symptoms include progressive weakness, poor coordination, with gradual slowing of mental function. Only seen in the immunosuppressed. Rarely associated with fever or other systemic symptoms

References

  1. Gerstner ER, Batchelor TT (2010). "Primary central nervous system lymphoma". Arch. Neurol. 67 (3): 291–7. doi:10.1001/archneurol.2010.3. PMID 20212226.
  2. von Reyn CF, Kimambo S, Mtei L, Arbeit RD, Maro I, Bakari M, Matee M, Lahey T, Adams LV, Black W, Mackenzie T, Lyimo J, Tvaroha S, Waddell R, Kreiswirth B, Horsburgh CR, Pallangyo K (2011). "Disseminated tuberculosis in human immunodeficiency virus infection: ineffective immunity, polyclonal disease and high mortality". Int. J. Tuberc. Lung Dis. 15 (8): 1087–92. doi:10.5588/ijtld.10.0517. PMID 21740673.
  3. Latgé JP (1999). "Aspergillus fumigatus and aspergillosis". Clin. Microbiol. Rev. 12 (2): 310–50. PMC 88920. PMID 10194462.
  4. Rassi A, Rassi A, Marin-Neto JA (2010). "Chagas disease". Lancet. 375 (9723): 1388–402. doi:10.1016/S0140-6736(10)60061-X. PMID 20399979.
  5. Emery VC (2001). "Investigation of CMV disease in immunocompromised patients". J. Clin. Pathol. 54 (2): 84–8. PMC 1731357. PMID 11215290.
  6. Bustamante CI, Wade JC (1991). "Herpes simplex virus infection in the immunocompromised cancer patient". J. Clin. Oncol. 9 (10): 1903–15. doi:10.1200/JCO.1991.9.10.1903. PMID 1919640.
  7. Hambleton S (2005). "Chickenpox". Curr. Opin. Infect. Dis. 18 (3): 235–40. PMID 15864101.
  8. Alvis Miranda H, Castellar-Leones SM, Elzain MA, Moscote-Salazar LR (2013). "Brain abscess: Current management". J Neurosci Rural Pract. 4 (Suppl 1): S67–81. doi:10.4103/0976-3147.116472. PMC 3808066. PMID 24174804.
  9. Patel K, Clifford DB (2014). "Bacterial brain abscess". Neurohospitalist. 4 (4): 196–204. doi:10.1177/1941874414540684. PMC 4212419. PMID 25360205.
  10. Tan CS, Koralnik IJ (2010). "Progressive multifocal leukoencephalopathy and other disorders caused by JC virus: clinical features and pathogenesis". Lancet Neurol. 9 (4): 425–37. doi:10.1016/S1474-4422(10)70040-5. PMC 2880524. PMID 20298966.

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