Posterior fossa progressive multifocal leukoencephalopathy: first presentation of an unknown autoimmune disease

Scholten, Paulette; Kralt, Peter; Jacobs, Bram

doi:10.1136/bcr-2017-220990

Cited by 9 publications

(10 citation statements)

References 13 publications

(12 reference statements)

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…14 Since the AIDS epidemic, PML was reported exclusively in association with HIV, but more recently, there has been a shift toward other immunosuppressive conditions, including patients on immune-suppression therapy and patients with long-standing autoimmune inflammatory or hematologic malignancies. 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.…”

Section: Discussionmentioning

confidence: 99%

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

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionmentioning

confidence: 99%

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

View full text Add to dashboard Cite

show abstract

“…Corpus callosum83 or deep grey matter may be affected. Posterior fossa involvement has been reported, most commonly involving the cerebellum and middle cerebellar peduncles 84. Lesions have sharp borders toward gray matter contrasting with ill-defined borders toward white matter, and increased signal intensity on T2-weighted and diffusion-weighted images (DWI) 85.…”

Section: Diagnosis Of Pmlmentioning

confidence: 99%

<p>Progressive Multifocal Leukoencephalopathy: Current Insights</p>

Kartau¹,

Sipilä²,

Auvinen³

et al. 2019

DNND

View full text Add to dashboard Cite

Cases of PML should be evaluated according to predisposing factors, as these subgroups differ by incidence rate, clinical course, and prognosis. The three most significant groups at risk of PML are patients with hematological malignancies mostly previously treated with immunotherapies but also untreated, patients with HIV infection, and patients using monoclonal antibody (mAb) treatments. Epidemiological data is scarce and partly conflicting, but the distribution of the subgroups appears to have changed. While there is no specific anti-JCPyV treatment, restoration of the immune function is the most effective approach to PML treatment. Research is warranted to determine whether immune checkpoint inhibitors could benefit certain PML subgroups. There are no systematic national or international records of PML diagnoses or a risk stratification algorithm, except for MS patients receiving natalizumab (NTZ). These are needed to improve PML risk assessment and to tailor better prevention strategies.

show abstract

“…Typical imaging features are multifocal and asymmetric lesions within supratentorial white matter and subcortical frontal and parieto-occipital regions are common locations. 3,7 Thalami, basal ganglia, brainstem, and cerebellum also can be involved, but only 1 out of 10 patients has brainstem or cerebellar lesions. 3,7 Isolated posterior fossa involvement is uncommon.…”

Section: Discussionmentioning

confidence: 99%

“…3,7 Thalami, basal ganglia, brainstem, and cerebellum also can be involved, but only 1 out of 10 patients has brainstem or cerebellar lesions. 3,7 Isolated posterior fossa involvement is uncommon. 2,[4][5][6][7][8][9] There is little or no mass effect or enhancement except in immune reconstitution inflammatory syndrome.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Isolated cerebellar progressive multifocal leukoencephalopathy after allogeneic bone marrow transplantation: focus on imaging

Mazzai

Facchinelli

Borghero

et al. 2021

Tumori

View full text Add to dashboard Cite

Introduction: Progressive multifocal leukoencephalopathy (PML) is caused by JC virus opportunistic infection in the setting of immunodeficiency. Typical imaging features are multifocal and asymmetric lesions within supratentorial subcortical white matter in parieto-occipital regions. Case description: A 47-year-old patient experienced a relapse of acute myeloid leukemia 21 months after hematopoietic stem cell transplantation. He also had visual impairment and magnetic resonance imaging showed an isolated cerebellar lesion without mass effect or enhancement. Common opportunistic infections and leukemic central nervous system involvement were excluded by cerebrospinal fluid (CSF) analysis. Given the worsening clinical and radiologic scenario, PML was suspected, and CSF protein chain reaction analysis was positive for JC virus. Conclusions: Given its potential curability, PML should be thoroughly investigated in patients with hematologic neoplasms and atypical isolated cerebellar presentation.

show abstract

Posterior fossa progressive multifocal leukoencephalopathy: first presentation of an unknown autoimmune disease

Cited by 9 publications

References 13 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

<p>Progressive Multifocal Leukoencephalopathy: Current Insights</p>

Isolated cerebellar progressive multifocal leukoencephalopathy after allogeneic bone marrow transplantation: focus on imaging

Contact Info

Product

Resources

About