Difference between revisions of "Progressive multifocal leukoencephalopathy"

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#redirect [[Neuropathology#Progressive_multifocal_leukoencephalopathy]]
'''Progressive multifocal leukoencephalopathy''', abbreviated '''PML''', is a rare [[neuropathology]] caused by a [[polyomavirus]].
 
==General==
*Caused by ''JC virus'' (a type of [[polyomavirus]]<ref name=pmid21499097>{{Cite journal  | last1 = Berger | first1 = JR. | title = The basis for modeling progressive multifocal leukoencephalopathy pathogenesis. | journal = Curr Opin Neurol | volume = 24 | issue = 3 | pages = 262-7 | month = Jun | year = 2011 | doi = 10.1097/WCO.0b013e328346d2a3 | PMID = 21499097 }}</ref>) in the context of immunodeficiency; usu. in the setting of [[HIV]] infection.<ref name=pmid12709870>{{Cite journal  | last1 = Berger | first1 = JR. | title = Progressive multifocal leukoencephalopathy in acquired immunodeficiency syndrome: explaining the high incidence and disproportionate frequency of the illness relative to other immunosuppressive conditions. | journal = J Neurovirol | volume = 9 Suppl 1 | issue =  | pages = 38-41 | month =  | year = 2003 | doi = 10.1080/13550280390195261 | PMID = 12709870 }}</ref>
**Approximately 5% of HIV patients develop PML.<ref name=pmid12709870/>
**Virus destroys oligodendrocytes -> demyelination results.<ref name=pmid21823157>{{Cite journal  | last1 = Mateen | first1 = FJ. | last2 = Muralidharan | first2 = R. | last3 = Carone | first3 = M. | last4 = van de Beek | first4 = D. | last5 = Harrison | first5 = DM. | last6 = Aksamit | first6 = AJ. | last7 = Gould | first7 = MS. | last8 = Clifford | first8 = DB. | last9 = Nath | first9 = A. | title = Progressive multifocal leukoencephalopathy in transplant recipients. | journal = Ann Neurol | volume = 70 | issue = 2 | pages = 305-22 | month = Aug | year = 2011 | doi = 10.1002/ana.22408 | PMID = 21823157 }}
</ref>
*Suspected cases are biopsied - unlike other demyelinating diseases.<ref>URL: [http://path.upmc.edu/cases/case336/dx.html http://path.upmc.edu/cases/case336/dx.html]. Accessed on: 15 January 2012.</ref>
==Gross==
*Multifocal - as the name suggests.
 
DDx:
*[[Multiple sclerosis]].
 
==Microscopic==
Features:<ref>URL: [http://path.upmc.edu/cases/case120/dx.html http://path.upmc.edu/cases/case120/dx.html]. Accessed on: 3 January 2012.</ref>
*Perivascular inflammatory cells.
*Foamy histiocytes.
*Abnormal appearing glial cells:<ref name=uscf_pml>URL: [http://missinglink.ucsf.edu/lm/ids_104_Demyelination/Didactic/Pml.htm http://missinglink.ucsf.edu/lm/ids_104_Demyelination/Didactic/Pml.htm]. Accessed on: 3 January 2012.</ref>
**Reactive astrocytes.
**Oligodendrocytes with nuclear enlargement and glassy magenta chromatin - '''key feature'''.
**Atypical mitoses - known as ''[[Creutzfeldt cell]]''.
 
Note:
*The chromatin changes remind me of [[Urine_cytopathology#Human_polyomavirus_infection|polyomavirus]] in [[urine cytology]]... perhaps ''not'' surprising as they are related.
 
===Images===
<gallery>
File:PML-HE_brain_biopsy.jpg | Oligodendrocytes with nuclear enlargement and glassy magenta chromatin (WC/jensflorian)
File:PML-SV40.jpg | Same case with SV40-positive immunoreactivity (WC/jensflorian)
</gallery>
 
*[http://path.upmc.edu/cases/case120/micro.html PML - case 1 (upmc.edu)].
*[http://path.upmc.edu/cases/case120/dx.html PML - case 1 (upmc.edu)].
*[http://path.upmc.edu/cases/case336/images/fig03.jpg Creutzfeldt cell - case 2 (upmc.edu)].<ref>URL: [http://path.upmc.edu/cases/case336.html http://path.upmc.edu/cases/case336.html]. Accessed on: 15 January 2012.</ref>
*[http://missinglink.ucsf.edu/lm/ids_104_Demyelination/Figures/PMLHandE.jpg PML oligodendrocyte (ucsf.edu)].<ref name=uscf_pml>URL: [http://missinglink.ucsf.edu/lm/ids_104_Demyelination/Didactic/Pml.htm http://missinglink.ucsf.edu/lm/ids_104_Demyelination/Didactic/Pml.htm]. Accessed on: 3 January 2012.</ref>
*[http://neuro.psychiatryonline.org/data/Journals/NP/3923/RA4831F2.jpeg PML oligodendrocyte (psychiatryonline.org)].<ref>{{Cite journal  | last1 = Hurley | first1 = RA. | last2 = Ernst | first2 = T. | last3 = Khalili | first3 = K. | last4 = Del Valle | first4 = L. | last5 = Simone | first5 = IL. | last6 = Taber | first6 = KH. | title = Identification of HIV-associated progressive multifocal leukoencephalopathy: magnetic resonance imaging and spectroscopy. | journal = J Neuropsychiatry Clin Neurosci | volume = 15 | issue = 1 | pages = 1-6 | month =  | year = 2003 | doi =  | PMID = 12556565 }}</ref>
 
==IHC==
*[[SV40]] +ve.<ref name=pmid15581180>{{Cite journal  | last1 = Muñoz-Mármol | first1 = AM. | last2 = Mola | first2 = G. | last3 = Fernández-Vasalo | first3 = A. | last4 = Vela | first4 = E. | last5 = Mate | first5 = JL. | last6 = Ariza | first6 = A. | title = JC virus early protein detection by immunohistochemistry in progressive multifocal leukoencephalopathy: a comparative study with in situ hybridization and polymerase chain reaction. | journal = J Neuropathol Exp Neurol | volume = 63 | issue = 11 | pages = 1124-30 | month = Nov | year = 2004 | doi =  | PMID = 15581180 }}</ref>
 
==See also==
*[[Neuropathology]].
 
==References==
{{Reflist|2}}


[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:Neuropathology]]

Latest revision as of 17:23, 29 June 2016

Progressive multifocal leukoencephalopathy, abbreviated PML, is a rare neuropathology caused by a polyomavirus.

General

  • Caused by JC virus (a type of polyomavirus[1]) in the context of immunodeficiency; usu. in the setting of HIV infection.[2]
    • Approximately 5% of HIV patients develop PML.[2]
    • Virus destroys oligodendrocytes -> demyelination results.[3]
  • Suspected cases are biopsied - unlike other demyelinating diseases.[4]

Gross

  • Multifocal - as the name suggests.

DDx:

Microscopic

Features:[5]

  • Perivascular inflammatory cells.
  • Foamy histiocytes.
  • Abnormal appearing glial cells:[6]
    • Reactive astrocytes.
    • Oligodendrocytes with nuclear enlargement and glassy magenta chromatin - key feature.
    • Atypical mitoses - known as Creutzfeldt cell.

Note:

Images

  • Oligodendrocytes with nuclear enlargement and glassy magenta chromatin (WC/jensflorian)

  • Same case with SV40-positive immunoreactivity (WC/jensflorian)

IHC

See also

References

  1. ↑ Berger, JR. (Jun 2011). "The basis for modeling progressive multifocal leukoencephalopathy pathogenesis.". Curr Opin Neurol 24 (3): 262-7. doi:10.1097/WCO.0b013e328346d2a3. PMID 21499097.
  2. ↑ 2.0 2.1 Berger, JR. (2003). "Progressive multifocal leukoencephalopathy in acquired immunodeficiency syndrome: explaining the high incidence and disproportionate frequency of the illness relative to other immunosuppressive conditions.". J Neurovirol 9 Suppl 1: 38-41. doi:10.1080/13550280390195261. PMID 12709870.
  3. ↑ Mateen, FJ.; Muralidharan, R.; Carone, M.; van de Beek, D.; Harrison, DM.; Aksamit, AJ.; Gould, MS.; Clifford, DB. et al. (Aug 2011). "Progressive multifocal leukoencephalopathy in transplant recipients.". Ann Neurol 70 (2): 305-22. doi:10.1002/ana.22408. PMID 21823157.
  4. ↑ URL: http://path.upmc.edu/cases/case336/dx.html. Accessed on: 15 January 2012.
  5. ↑ URL: http://path.upmc.edu/cases/case120/dx.html. Accessed on: 3 January 2012.
  6. ↑ 6.0 6.1 URL: http://missinglink.ucsf.edu/lm/ids_104_Demyelination/Didactic/Pml.htm. Accessed on: 3 January 2012.
  7. ↑ URL: http://path.upmc.edu/cases/case336.html. Accessed on: 15 January 2012.
  8. ↑ Hurley, RA.; Ernst, T.; Khalili, K.; Del Valle, L.; Simone, IL.; Taber, KH. (2003). "Identification of HIV-associated progressive multifocal leukoencephalopathy: magnetic resonance imaging and spectroscopy.". J Neuropsychiatry Clin Neurosci 15 (1): 1-6. PMID 12556565.
  9. ↑ Muñoz-Mármol, AM.; Mola, G.; Fernández-Vasalo, A.; Vela, E.; Mate, JL.; Ariza, A. (Nov 2004). "JC virus early protein detection by immunohistochemistry in progressive multifocal leukoencephalopathy: a comparative study with in situ hybridization and polymerase chain reaction.". J Neuropathol Exp Neurol 63 (11): 1124-30. PMID 15581180.
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