Progressive multifocal leukoencephalopathy in a patient without apparent immunosuppression

Grewal, Jessie; Dalal, Poorvi; Bowman, Michelle R.; Kaya, Behiye; Otero, José; Imitola, Jaime

doi:10.1007/s13365-016-0459-y

Cited by 16 publications

(19 citation statements)

References 22 publications

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“…15,16 In addition, several case reports on immunocompetent patients developing PML have also been published. 17,18 A relatively thin, uniformly linear SWI-hypointense rim was observed in 13 of 18 patients with PML in our study. The rim exclusively affected the U-fibers, following the cortical architecture, and did not correlate with other MR imaging findings.…”

Section: Discussionsupporting

confidence: 55%

Susceptibility-Weighted MR Imaging Hypointense Rim in Progressive Multifocal Leukoencephalopathy: The End Point of Neuroinflammation and a Potential Outcome Predictor

Thurnher

Boban

Rieger³

et al. 2019

AJNR Am J Neuroradiol

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BACKGROUND AND PURPOSE: Progressive multifocal leukoencephalopathy (PML) represents a life-threatening demyelinating disorder of the brain caused by reactivation of a rare opportunistic infection with JC Polyomavirus. The aims of this study were to describe the incidence of a susceptibility-weighted imaging hypointense rim in patients with multifocal leukoencephalopathy and to explore the histologic correlates and prognostic value of the rim with regard to the clinical outcome. MATERIALS AND METHODS: This retrospective study included 18 patients with a definite diagnosis of progressive multifocal leukoencephalopathy. Ten patients were HIV-positive, 3 patients had natalizumab-associated progressive multifocal leukoencephalopathy, 1 patient had multiple myeloma, 3 patients had a history of lymphoma, and 1 was diagnosed with acute myeloid leukemia. Patients were divided into short-(up to 12 months) and long-term (Ͼ12 months) survivors. A total of 93 initial and follow-up MR imaging examinations were reviewed. On SWI, the presence and development of a hypointense rim at the periphery of the progressive multifocal leukoencephalopathy lesions were noted. A postmortem histologic examination was performed in 2 patients: A rim formed in one, and in one, there was no rim. RESULTS: A total of 73 progressive multifocal leukoencephalopathy lesions were observed. In 13 (72.2%) patients, a well-defined thin, linear, hypointense rim at the periphery of the lesion toward the cortical side was present, while in 5 (27.8%) patients, it was completely absent. All 11 long-term survivors and 2 short-term survivors presented with a prominent SWI-hypointense rim, while 5/7 short-term survivors did not have this rim. CONCLUSIONS: The thin, uniformly linear, gyriform SWI-hypointense rim in the paralesional U-fibers in patients with definite progressive multifocal leukoencephalopathy might represent an end-point stage of the neuroinflammatory process in long-term survivors.

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Section: Discussionsupporting

confidence: 55%

Susceptibility-Weighted MR Imaging Hypointense Rim in Progressive Multifocal Leukoencephalopathy: The End Point of Neuroinflammation and a Potential Outcome Predictor

Thurnher

Boban

Rieger³

et al. 2019

AJNR Am J Neuroradiol

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“…29 CD4 lymphocytopenia, occurring as in idiopathic CD4 lymphocytopenia, has been linked to PML. 30 FAEs are known to reduce CD4 + and CD8 + lymphocytes, 31,32 and in all eight cases of PML reported in patients on FAE treatment, exposure to moderate-to-severe lymphocytopenia was present. FAE-induced moderate lymphocytopenia with more pronounced selective reductions in lymphocyte-subsets could predispose to an increased risk for PML.…”

Section: Discussionmentioning

confidence: 93%

“…Experimental studies have shown that FAEs induce lymphocytopenia, including reduction of CD4 + , CD8 + and B lymphocytes . CD4 lymphocytopenia, occurring as in idiopathic CD4 lymphocytopenia, has been linked to PML . FAEs are known to reduce CD4 + and CD8 + lymphocytes, and in all eight cases of PML reported in patients on FAE treatment, exposure to moderate‐to‐severe lymphocytopenia was present.…”

Section: Discussionmentioning

confidence: 99%

Progressive multifocal leukoencephalopathy associated with fumaric acid esters treatment in psoriasis patients

Balak

Hajdarbegovic

Bramer

et al. 2017

Acad Dermatol Venereol

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Progressive multifocal leukoencephalopathy is infrequently linked to FAE treatment, but underreporting cannot be excluded. Physicians treating patients with FAEs should be vigilant for the occurrence of PML, and both clinicians and patients should be alert for onset of new neurological symptoms. Periodic monitoring of lymphocyte counts and FAE discontinuation in case of moderate-to-severe lymphocytopenia is recommended to minimize the risk for PML.

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“…Usually, PML occurs almost exclusively in immunosuppressed individuals. However, there are isolated case reports of PML in patients without apparent immunosuppression [6]. In a retrospective population-based analysis of a health insurance database, the incidence rates of PML in patients with autoimmune vasculitis was 10.8 per 100 000 person years [7].…”

Section: Discussionmentioning

confidence: 99%

Rare but not beyond care: a young female with altered mental status and seizures

Pathireddy

Bose

Baradhi

et al. 2019

Oxford Medical Case Reports

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A 40-year-old Caucasian lady with focal crescentic glomerulonephritis (p-ANCA) demonstrated by kidney biopsy, was treated with intravenous pulse steroids followed by weekly outpatient rituximab infusions (375 mg/m2). Five days after the fourth and final rituximab infusion, she developed headaches, altered mental status and seizures. Upon transfer to our facility, magnetic resonance imaging of the brain revealed cortical white matter changes suggestive of possible progressive multifocal leukoencephalopathy (PML) or posterior reversible encephalopathy syndrome (PRES). She was aggressively treated with antihypertensives, anti-seizure medications, intravenous steroids, plasmapheresis and ventilatory support while awaiting cerebrospinal fluid analysis and polymerase chain reaction on John Cunningham virus DNA. She had a complete recovery and, at 1 year follow up, was found to be doing well. Awareness of potential complications of rituximab therapy, such as PRES or PML is critical in providing appropriate treatment.

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Progressive multifocal leukoencephalopathy in a patient without apparent immunosuppression

Cited by 16 publications

References 22 publications

Susceptibility-Weighted MR Imaging Hypointense Rim in Progressive Multifocal Leukoencephalopathy: The End Point of Neuroinflammation and a Potential Outcome Predictor

Susceptibility-Weighted MR Imaging Hypointense Rim in Progressive Multifocal Leukoencephalopathy: The End Point of Neuroinflammation and a Potential Outcome Predictor

Progressive multifocal leukoencephalopathy associated with fumaric acid esters treatment in psoriasis patients

Rare but not beyond care: a young female with altered mental status and seizures

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