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|Year : 2022 | Volume
| Issue : 4 | Page : 1762-1763
Progressive Multifocal Leukoencephalopathy in Patient with Primary Immunodeficiency Syndrome
Malorzata Blauciak1, Joanna Bladowska2, Mieszko Zagrajek1, Konstanty Guranski1, Boguslaw Paradowski1
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 Submission||03-Dec-2019|
|Date of Decision||06-Mar-2020|
|Date of Acceptance||02-Mar-2020|
|Date of Web Publication||30-Aug-2022|
Department of Neurology, Wroclaw Medical University, Borowska 213, Wroclaw, 50-556
Source of Support: None, Conflict of Interest: None
|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 7];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., Treatment is ineffective and the prognosis is unfavorable.
|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)|
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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.
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Conflicts of interest
There are no conflicts of interest.
| » References|| |
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.
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.
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.