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Progressive multifocal leukoencephalopathy (view source)
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# | '''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=== | |||
*[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 Crutzfeldt 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]] | |||