Leveron&Nexovas
Neurology India
menu-bar5 Open access journal indexed with Index Medicus
  Users online: 146  
 Home | Login 
About Editorial board Articlesmenu-bullet NSI Publicationsmenu-bullet Search Instructions Online Submission Subscribe Videos Etcetera Contact
  Navigate Here 
 Search
 
  
 Resource Links
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Article in PDF (941 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this Article
   References
   Article Figures

 Article Access Statistics
    Viewed280    
    Printed10    
    Emailed0    
    PDF Downloaded6    
    Comments [Add]    

Recommend this journal

 


 
Table of Contents    
NEUROIMAGE
Year : 2022  |  Volume : 70  |  Issue : 4  |  Page : 1762-1763

Progressive Multifocal Leukoencephalopathy in Patient with Primary Immunodeficiency Syndrome


1 Department of Neurology, Interventional Radiology and Neuroradiology, Wroclaw Medical University, Wroclaw, Poland
2 Department of General Radiology, Interventional Radiology and Neuroradiology, Wroclaw Medical University, Wroclaw, Poland

Date of Submission03-Dec-2019
Date of Decision06-Mar-2020
Date of Acceptance02-Mar-2020
Date of Web Publication30-Aug-2022

Correspondence Address:
Malorzata Blauciak
Department of Neurology, Wroclaw Medical University, Borowska 213, Wroclaw, 50-556
Poland
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.355187

Rights and Permissions



How to cite this article:
Blauciak M, Bladowska J, Zagrajek M, Guranski K, Paradowski B. Progressive Multifocal Leukoencephalopathy in Patient with Primary Immunodeficiency Syndrome. Neurol India 2022;70:1762-3

How to cite this URL:
Blauciak M, Bladowska J, Zagrajek M, Guranski K, Paradowski B. Progressive Multifocal Leukoencephalopathy in Patient with Primary Immunodeficiency Syndrome. Neurol India [serial online] 2022 [cited 2022 Oct 2];70:1762-3. Available from: https://www.neurologyindia.com/text.asp?2022/70/4/1762/355187




A 22-year-old male with T-cell immunodeficiency was admitted with aphasia and spastic paresis of the right extremities. MRI showed extensive white matter hyperintensities involving subcortical short association U-fibers [Figure 1]. CSF examination showed JCV viremia with 612,400 copies/mL. Therapy with Cidofovir and immunoglobulins was administered. Follow-up examination found focal seizures in the right extremities and consciousness disturbances. MRI revealed progression of lesions in the right temporal and parietal areas. The patient died after 6 months. PML is a rare condition among patients with genetically determined primary immunodeficiency.[1],[2] Treatment is ineffective and the prognosis is unfavorable.[3]
Figure 1: MRI examination, axial T2-weighted (a), T1-weighted (b), diffusion-weighted (c), and T1-weighted contrast-enhanced (d) images as well as perfusion MRI (e) and MRI spectroscopy (f). The images show diffuse hyperintensities involving the white matter, including U-fibers. There is no contrast enhancement within the affected areas (d). Perfusion MRI (e) does not reveal any regions of hyperperfusion. MRI spectroscopy (f) demonstrates a decreased N-acetyl aspartate (NAA) level, while choline (Cho) and myo-inositol (MI) peaks are increased. There is also an elevated lactate peak (Lac)

Click here to view


Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Berger JR, Aksamit AJ, Clifford DB, Davis L, Koralnik IJ, Sejvar JJ, et al. PML diagnostic criteria: Consensus statement from the AAN neuroinfectious disease section. Neurology 2013;80:1430-8.  Back to cited text no. 1
    
2.
Zerbe CS, Marciano BE, Katial RK, Santos CB, Adamo N, Hsu AP, et al. Progressive multifocal leukoencephalopathy in primary immune deficiencies: Stat1 gain of function and review of the literature. Clin Infect Dis 2016;62:986-94.  Back to cited text no. 2
    
3.
Pavlovic D, Patera AC, Nyberg F, Gerber M, Liu M. Progressive multifocal leukoencephalopathy: Current treatment options and future perspectives. Ther Adv Neurol Disord 2015;8:255-73.  Back to cited text no. 3
    


    Figures

  [Figure 1]



 

Top
Print this article  Email this article
   
Online since 20th March '04
Published by Wolters Kluwer - Medknow