Clinical and paraclinical findings in natalizumab-associated infratentorial progressive multifocal leukoencephalopathy patients: Table 1

Hoepner, Robert; Ahlbrecht, Jonas; Faissner, Simon; Schneider, Ruth; Dahlhaus, Stefanie; Adams, Ortwin; Raab, Peter; Lukas, Carsten; Chan, Andrew; Stangel, Martin; Gold, Ralf

doi:10.1136/jnnp-2014-307582

Cited by 10 publications

(5 citation statements)

References 3 publications

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“…The prominent brainstem involvement in this patient led to central hypopnea which in terms of a predominant infratentorial PML manifestation has previously been described by our group to be associated with a poor functional PML outcome. 11 Nevertheless, interpretation of our data regarding PML survival is limited by the small sample of the subgroup of the PML non-survivor ( n = 1). Furthermore, we found a correlation between age and number of JCV DNA copies in CSF with functional PML outcome.…”

Section: Resultsmentioning

confidence: 81%

Predictors of severity and functional outcome in natalizumab-associated progressive multifocal leukoencephalopathy

et al. 2016

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Section: Resultsmentioning

confidence: 81%

Predictors of severity and functional outcome in natalizumab-associated progressive multifocal leukoencephalopathy

et al. 2016

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“…In addition to patients with AIDS, hematologic malignancies, and transplant recipients, PML has been reported in a growing number of individuals with autoimmune diseases treated with immunomodulatory medications such as natalizumab (Clifford et al 2010; Dahlhaus et al 2013; Gheuens et al 2012b; Hoepner et al 2014; Marousi et al 2012; Tan et al 2011), dimethyl fumarate (Bartsch et al 2015; Dammeier et al 2015; Khatri et al 2015), or efalizumab (Carson et al 2009; Gadzia and Turner 2010; Korman et al 2009; Kothary et al 2011). Since seizures frequently occurred at or soon after PML onset, PML should be included in the differential diagnosis of new onset seizures in the aforementioned high-risk patients.…”

Section: Discussionmentioning

confidence: 99%

Predictors and characteristics of seizures in survivors of progressive multifocal leukoencephalopathy

et al. 2015

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This study aims to determine the risk factors for epileptogenesis and characteristics of seizures in patients with progressive multifocal leukoencephalopathy (PML) who survive more than 1 year from onset of neurological symptoms (PML survivors). We reviewed clinical data including seizure history and MR imaging studies from PML survivors evaluated at our institution between 1997 and 2014. PML progressors who passed away within 1 year and patients with a history of seizures prior to PML diagnosis were excluded from the analysis. Of 64 PML survivors, 28 (44 %) developed seizures. The median time from the onset of PML symptoms to the first seizure was 5.4 months (range 0–159) and 64 % of patients with seizures had them within the first year. The presence of juxtacortical PML lesions was associated with a relative risk of seizures of 3.5 (p<0.02; 95 % confidence interval (CI) 1.3– 9.4) in multivariate analyses. Of all seizure types, 86 % were focal and 60 % most likely originated from the frontal lobes. Among seizure patients, 89 % required treatment, including one (54 %), two (25 %), or three (10.5 %) antiepileptic drugs. Seizures are a frequent complication in PML and can develop throughout the entire course of the disease. However, late onset seizures did not signify PML relapse. Seizures may require treatment with multiple antiepileptic medications and are a significant co-morbidity in PML.

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“…Most frequent symptoms in these patients were ataxia, dysmetria, dysarthria, cerebellar deficits and paresis as the cerebellum and pons were predominantly affected ( Fig. 2c) [46].…”

Section: Infratentorial Pmlmentioning

confidence: 94%

“…Progressive multifocal leukoencephalopathy may also involve infratentorial structures . A recent study in patients with natalizumab‐associated PML observed an initial infratentorial manifestation in 33% with a third of these patients having an exclusive infratentorial manifestation.…”

Section: Clinical Features Of Pml – Typical and Atypical Presentationmentioning

confidence: 99%

The spectrum of progressive multifocal leukoencephalopathy: a practical approach

Bartsch

Rempe

Leypoldt

et al. 2019

Euro J of Neurology

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John Cunningham virus (JCV) infection of the central nervous system causes progressive multifocal leukoencephalopathy (PML) in patients with systemic immunosuppression. With the increased application of modern immunotherapy and biologics in various immune‐mediated disorders, the PML risk spectrum has changed. Thus, new tools and strategies for risk assessment and stratification in drug‐associated PML such as the JCV antibody indices have been introduced. Imaging studies have highlighted atypical presentations of cerebral JCV disease such as granule cell neuronopathy. Imaging markers have been developed to differentiate PML from new multiple sclerosis lesions and to facilitate the early identification of pre‐clinical manifestations of PML and its immune reconstitution inflammatory syndrome. PML can be diagnosed either by brain biopsy or by clinical, radiographic and virological criteria. Experimental treatment options including immunization and modulation of interleukin‐mediated immune response are emerging. PML should be considered in any patient with compromised systemic or central nervous system immune surveillance presenting with progressive neurological symptoms.

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Clinical and paraclinical findings in natalizumab-associated infratentorial progressive multifocal leukoencephalopathy patients: Table 1

Cited by 10 publications

References 3 publications

Predictors of severity and functional outcome in natalizumab-associated progressive multifocal leukoencephalopathy

Predictors of severity and functional outcome in natalizumab-associated progressive multifocal leukoencephalopathy

Predictors and characteristics of seizures in survivors of progressive multifocal leukoencephalopathy

The spectrum of progressive multifocal leukoencephalopathy: a practical approach

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