Progressive multifocal leukoencephalopathy: Difference between revisions

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Latest revision as of 14:19, 12 June 2021

This page is about clinical aspects of the disease. For microbiologic aspects of the causative organism(s), see JC virus.

For patient information click here

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Progressive multifocal leucoencephalopathy, Multifocal leucoencephalopathy, Multifocal leukoencephalopathy, PML, PMLE

Differentiating Progressive multifocal leukoencephalopathy from other Diseases

Natural History, Complications and Prognosis

Patients with Progressive Multifocal leukoencephalopathy(PML) have a long-term neurological sequelae and is often fatal. The mortality rate is 30%-50% ^[1] in early months of diagnosis. Those who are immunocompromised are at significant risk of reactivation.

COMPLICATIONS

PML may complicate into the following :

Neurologic Dysfunction
Dementia
Blindness
Seizures

PROGNOSIS

The prognosis is variable and depends on the underlying severity of PML.
Impairment of immune system (eg. HIV) or use of immunosuppressants may worsen prognosis and leads to rapid progression of the disease.^[1]
Patients who survive have substantial morbidity and severe neurological disabilities.^[2]

Reference

Progressive Multifocal Leukoencephalopathy Information Page. NIH-National Institute of Neurological Disorders and Stroke. 29 July 2019. Retrieved 12 June 2021.
Castle, D., & Robertson, N. P. (2019). Treatment of progressive multifocal leukoencephalopathy. Journal of Neurology, 266(10), 2587–2589. https://doi.org/10.1007/s00415-019-09501-y

Diagnosis

Treatment

Medical Therapy | Surgery | Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1

Related Chapters

Leukoencephalopathy with vanishing white matter

Template:Viral diseases de:Progressive multifokale Leukenzephalopathie nl:Progressieve multifocale leukoencefalopathie

Template:WikiDoc Sources

@@ Line 1: / Line 1: @@
-{{DiseaseDisorder infobox |
+__NOTOC__
-  Name        = Progressive multifocal leukoencephalopathy |
+{{Progressive multifocal leukoencephalopathy}}
-  ICD10       = {{ICD10|A|81|2|a|80}} |
+{{About1|JC virus}}
-  ICD9        = {{ICD9|046.3}} |
+'''For patient information click [[Progressive multifocal leukoencephalopathy (patient information)|here]]'''
-  MedlinePlus = 000674 |
-}}
-{{Progressive multifocal leukoencephalopathy}}'''For patient information click [[Progressive multifocal leukoencephalopathy (patient information)|here]]'''
 {{CMG}}
-==Overview==
-==Cause and epidemiology==
+{{SK}} Progressive multifocal leucoencephalopathy, Multifocal leucoencephalopathy, Multifocal leukoencephalopathy, PML, PMLE
-==Disease process==
+==[[Progressive multifocal leukoencephalopathy overview|Overview]]==
-==Diagnosis==
+==[[Progressive multifocal leukoencephalopathy historical perspective|Historical Perspective]]==
-PML is diagnosed by testing for JC virus [[DNA]] in [[cerebrospinal fluid]] or in a brain [[biopsy]] specimen. Characteristic evidence of the damage caused by PML in the brain can also be detected on [[magnetic resonance imaging|MRI]] images.
+==[[Progressive multifocal leukoencephalopathy pathophysiology|Pathophysiology]]==
+==[[Progressive multifocal leukoencephalopathy causes|Causes]]==
+==[[Progressive multifocal leukoencephalopathy differential diagnosis|Differentiating Progressive multifocal leukoencephalopathy from other Diseases]]==
+==[[Progressive multifocal leukoencephalopathy epidemiology and demographics|Epidemiology and Demographics]]==
+==[[Progressive multifocal leukoencephalopathy risk factors|Risk Factors]]==
+==[[Progressive multifocal leukoencephalopathy natural history|Natural History, Complications and Prognosis]]==
+Patients with Progressive Multifocal leukoencephalopathy(PML) have a long-term neurological sequelae and is often fatal. The mortality rate is 30%-50% <sup>[1]</sup> in early months of diagnosis.
+Those who are immunocompromised are at significant risk of reactivation.
+===== '''COMPLICATIONS''' =====
+PML may complicate into the following :
+* Neurologic Dysfunction
+* Dementia
+* Blindness
+* Seizures
+===== '''PROGNOSIS''' =====
+* The prognosis is variable and depends on the underlying severity of PML.
+* Impairment of immune system (eg. HIV) or use of immunosuppressants may worsen prognosis and leads to rapid progression of the disease.<sup>[1]</sup>
+* Patients who survive have substantial morbidity and severe neurological disabilities.<sup>[2]</sup>
+== Reference ==
+# [https://www.ninds.nih.gov/disorders/all-disorders/progressive-multifocal-leukoencephalopathy-information-page  Progressive Multifocal Leukoencephalopathy Information Page]. NIH-National Institute of Neurological Disorders and Stroke. 29 July 2019. Retrieved 12 June 2021.
+# Castle, D., &amp; Robertson, N. P. (2019). Treatment of progressive multifocal leukoencephalopathy. Journal of Neurology, 266(10), 2587–2589. https://doi.org/10.1007/s00415-019-09501-y
+==[[Progressive multifocal leukoencephalopathy diagnosis|Diagnosis]]==
+[[Progressive multifocal leukoencephalopathy history and symptoms|History and Symptoms]] | [[Progressive multifocal leukoencephalopathy physical examination|Physical Examination]] | [[Progressive multifocal leukoencephalopathy laboratory tests|Laboratory Findings]] | [[Progressive multifocal leukoencephalopathy x ray|X Ray]] | [[Progressive multifocal leukoencephalopathy CT|CT]] | [[Progressive multifocal leukoencephalopathy MRI|MRI]] | [[Progressive multifocal leukoencephalopathy echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Progressive multifocal leukoencephalopathy other imaging findings|Other Imaging Findings]] | [[Progressive multifocal leukoencephalopathy other diagnostic studies|Other Diagnostic Studies]]
 ==Treatment==
+[[Progressive multifocal leukoencephalopathy medical therapy|Medical Therapy]] | [[Progressive multifocal leukoencephalopathy surgery|Surgery]] | [[Progressive multifocal leukoencephalopathy prevention|Prevention]] | [[Progressive multifocal leukoencephalopathy cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Progressive multifocal leukoencephalopathy future or investigational therapies|Future or Investigational Therapies]]
-==References==
+==Case Studies==
-* Aksamit, A. J. (2001) Treatment of non-AIDS progressive multifocal leukoencephalopathy with cytosin arabinoside. J Neurovirol 2001;7:386.
+[[Progressive multifocal leukoencephalopathy case study one|Case #1]]
-* Berger, J.R. (2003) Progressive multifocal leukoencephalopathy in acquired immunodeficiency syndrome: explaining the high incidence and disproportionate frequency of the illness relative to other immunosuppressive conditions. ''Journal of Neurovirology'' '''9''' (supplement), 38-41. PMID 12709870
-* Langer-Gould, A., Atlas, S. W., Bollen, A. W., Pelletier, D. (2005) Progressive Multifocal Leukoencephalopathy in a Patient Treated with Natalizumab. N Engl J Med 2005;353:375-81.
-* Vendrely, A., Bienvenu, B., Gasnault, J., Thiebault, J.B., Salmon, D. and Gray, F. (2005) Fulminant inflammatory leukoencephalopathy associated with HAART-induced immune restoration in AIDS-related progressive multifocal leukoencephalopathy. ''Acta Neuropathologica'' '''109''', 449-455. PMID 15739098
-* Wyen, C., Hoffmann, C., Schmeisser, N., Wohrmann, A., Qurishi, N., Rockstroh, J., Esser, S., Rieke, A., Ross, B., Lorenzen, T., Schmitz, K., Stenzel, W., Salzberger, B. and Fatkenheuer, G. (2004) Progressive multifocal leukencephalopathy in patients on highly active antiretroviral therapy: survival and risk factors of death. ''Journal of Acquired Immune Deficiency Syndrome'' '''37''', 1263-1268. PMID 15385733
-== See also ==
+== Related Chapters ==
 * [[Leukoencephalopathy with vanishing white matter]]
@@ Line 38: / Line 63: @@
 [[Category:Mental disorders due to a general medical condition]]
 [[Category:Neurology]]
-[[Category:Infectious disease]]
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