Faculty Opinions recommendation of Progressive multifocal leukoencephalopathy: why gray and white matter.

Author(s):  
Joseph Berger
2021 ◽  
pp. 135245852199965
Author(s):  
Kedar R Mahajan ◽  
Moein Amin ◽  
Matthew Poturalski ◽  
Jonathan Lee ◽  
Danielle Herman ◽  
...  

Objective: Describe magnetic resonance imaging (MRI) susceptibility changes in progressive multifocal leukoencephalopathy (PML) and identify neuropathological correlates. Methods: PML cases and matched controls with primary central nervous system lymphoma (PCNSL) were retrospectively identified. MRI brain at 3 T and 7 T were reviewed. MRI-pathology correlations in fixed brain autopsy tissue were conducted in three subjects with confirmed PML. Results: With PML ( n = 26 total, n = 5 multiple sclerosis natalizumab-associated), juxtacortical changes on susceptibility-weighted imaging (SWI) or gradient echo (GRE) sequences were noted in 3/3 cases on 7 T MRI and 14/22 cases (63.6%) on 1.5 T or 8/22 (36.4%) 3 T MRI. Similar findings were only noted in 3/25 (12.0%) of PCNSL patients (odds ratio (OR) 12.83, 95% confidence interval (CI), 2.9–56.7, p < 0.001) on 1.5 or 3 T MRI. On susceptibility sequences available prior to diagnosis of PML, 7 (87.5%) had changes present on average 2.7 ± 1.8 months (mean ± SD) prior to diagnosis. Postmortem 7 T MRI showed SWI changes corresponded to areas of increased iron density along the gray–white matter (GM-WM) junction predominantly in macrophages. Conclusion: Susceptibility changes in PML along the GM-WM junction can precede noticeable fluid-attenuated inversion recovery (FLAIR) changes and correlates with iron accumulation in macrophages.


Neurosurgery ◽  
1986 ◽  
Vol 18 (4) ◽  
pp. 407-414 ◽  
Author(s):  
Michael Schlitt ◽  
Richard B. Morawetz ◽  
Jose Bonnin ◽  
B. Chandra-Sekar ◽  
John J. Curtiss ◽  
...  

Abstract Since 1980, three immunocompromised patients have been proved to have progressive multifocal leukoencephalopathy (PML) by brain biopsy at the University of Alabama at Birmingham. Two patients presented with focal neurological findings, and the third presented with dementia. Computed tomography (CT) revealed white matter low density lesions in areas appropriate to the neurological abnormalities. Brain biopsy of areas that were abnormal on CT produced diagnostic tissue in all three patients. No patient suffered ill effects from the biopsy. Neuropathological findings on light microscopy were compatible with PML in each case, although there was diversity within the group. Involvement of gray and white matter was present in all biopsy specimens; oligodendrocytes, astrocytes, and neurons were affected. Electron microscopic demonstration of particles compatible with polyoma virus confirmed the diagnosis in each case. Immunosuppressive medication was discontinued in two of the patients; these two have survived more than 2 years after diagnosis. One of these two has gradually improved and is independent in simple activities of daily life. Brain stem and cerebellar involvement and seizure disorders have been present in all reported cases. PML can be accurately and rapidly diagnosed by brain biopsy, enabling therapeutic manipulations that may prolong survival.


2013 ◽  
Vol 115 (5) ◽  
pp. 638-640 ◽  
Author(s):  
Daniel Zeller ◽  
Falko Markulin ◽  
Camelia-Maria Monoranu ◽  
László Solymosi ◽  
Guido Stoll

2016 ◽  
Vol 23 (4) ◽  
pp. 614-616 ◽  
Author(s):  
Marine Boudot de la Motte ◽  
Céline Louapre ◽  
Anne Bertrand ◽  
Pauline Reach ◽  
Catherine Lubetzki ◽  
...  

Background: Fingolimod is a widely used treatment for highly active relapsing–remitting multiple sclerosis. Objectives: To describe the case of a 27-year-old patient treated for more than 2 years by Fingolimod, who presented hemiplegia and speech disturbances associated with extensive white matter lesions on brain magnetic resonance imaging (MRI). Methods: Case study. Results: Although initial presentation questioned the possibility of progressive multifocal leukoencephalopathy, final diagnosis was multiple sclerosis (MS) relapse. Appropriate treatment resulted in clinical and radiological improvement. Conclusion: Severe MS relapses under Fingolimod have been reported, but late onset after treatment initiation and extensive subcortical topography is unusual and represents a diagnostic challenge.


Author(s):  
Steven M. Le Vine ◽  
David L. Wetzel

In situ FT-IR microspectroscopy has allowed spatially resolved interrogation of different parts of brain tissue. In previous work the spectrrscopic features of normal barin tissue were characterized. The white matter, gray matter and basal ganglia were mapped from appropriate peak area measurements from spectra obtained in a grid pattern. Bands prevalent in white matter were mostly associated with the lipid. These included 2927 and 1469 cm-1 due to CH2 as well as carbonyl at 1740 cm-1. Also 1235 and 1085 cm-1 due to phospholipid and galactocerebroside, respectively (Figs 1and2). Localized chemical changes in the white matter as a result of white matter diseases have been studied. This involved the documentation of localized chemical evidence of demyelination in shiverer mice in which the spectra of white matter lacked the marked contrast between it and gray matter exhibited in the white matter of normal mice (Fig. 3).The twitcher mouse, a model of Krabbe’s desease, was also studied. The purpose in this case was to look for a localized build-up of psychosine in the white matter caused by deficiencies in the enzyme responsible for its breakdown under normal conditions.


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