Progressive multifocal leukoencephalopathy developing in advanced pulmonal sarcoidosis

Völker, Hans-Ullrich; Kraft, K.; Arnold, Eva; Steinhoff, Silke; Kolios, George; Sommer, Stephanie

doi:10.1016/j.clineuro.2007.05.009

Cited by 26 publications

(19 citation statements)

References 17 publications

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“…Until now, only few cases on the coincidence of PML and sarcoidosis have been reported 5, 6, 7, 8. Our case series adds new important observations and thus helps raising the level of alertness to this rare brain infection in these conditions.…”

Section: Discussionmentioning

confidence: 53%

Can pulmonary sarcoidosis trigger a progressive multifocal leukoencephalopathy? Considerations from a case series and a review of literature

Gamperl

Enzinger

Pichler

et al. 2018

Clinical Case Reports

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Section: Discussionmentioning

confidence: 53%

Can pulmonary sarcoidosis trigger a progressive multifocal leukoencephalopathy? Considerations from a case series and a review of literature

Gamperl

Enzinger

Pichler

et al. 2018

Clinical Case Reports

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“…No curative treatment of PML has been established. Three cases of patients with PML and sarcoidosis are reported to be treated with the antiviral medication cidofovir [1,10], one in combination with mirtazapine [7]. Two of these patients could be stabilised and neurological symptoms improved, while the other patient died of respiratory failure 5 months after onset of symptoms.…”

Section: Sirsmentioning

confidence: 96%

“…MRI in PML typically shows hyperintense lesions in T2-weighted images mainly located in the white matter of parieto-occipital and frontal lobes. A few cases of purely infratentorial involvement have been reported in PML [2,3,5,10,11]. Infratentorial involvement in conjunction with supratentorial lesions is seen more frequently [11].…”

Section: Sirsmentioning

confidence: 96%

“…d MRI, 1.5 Tesla, axial T2-TSE demonstrates similar, smaller focal T2-hyperintensities located supratentorially in the periventricular and frontal white matter JCV PCR in CSF and stereotactic brain biopsy led to the diagnosis of PML. A co-prevalence of PML and sarcoidosis in HIV-negative patients has been previously described in a few cases [1,[6][7][8]10]. It can be speculated that the re-activation of JCV infection in sarcoidosis is a direct result of immunosuppressive treatment with corticoids.…”

Section: Sirsmentioning

confidence: 96%

“…Moreover, PML has been described in three cases of untreated sarcoidosis [1,6,8]. It has been hypothesized that the shift of CD4/CD8 ratio in sarcoidosis with a lack of JC-virus specific cytotoxic cells could facilitate the development of PML [10]. Albeit it is improbable that corticosteroids generally cause PML they do seem to accelerate the progression of the disease.…”

Section: Sirsmentioning

confidence: 98%

See 2 more Smart Citations

Infratentorial progressive multifocal leukencephalopathy in a patient with pulmonary sarcoidosis

et al. 2009

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Characteristics of Progressive Multifocal Leukoencephalopathy Associated With Sarcoidosis Without Therapeutic Immune Suppression

et al. 2023

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ImportanceProgressive multifocal leukoencephalopathy can occur in the context of systemic sarcoidosis (S-PML) in the absence of therapeutic immune suppression and can initially be mistaken for neurosarcoidosis or other complications of sarcoidosis. Earlier recognition of S-PML could lead to more effective treatment of the disease.ObjectiveTo describe characteristics of patients with S-PML.Design, Setting, and ParticipantsFor this case series, records from 8 academic medical centers in the United States were reviewed from 2004 to 2022. A systematic review of literature from 1955 to 2022 yielded data for additional patients. Included were patients with S-PML who were not receiving therapeutic immune suppression. The median follow-up time for patients who survived the acute range of illness was 19 months (range, 2-99). Data were analyzed in February 2023.ExposuresSarcoidosis without active therapeutic immune suppression.Main Outcomes and MeasuresClinical, laboratory, and radiographic features of patients with S-PML.ResultsTwenty-one patients with S-PML not receiving therapeutic immune suppression were included in this study, and data for 37 patients were collected from literature review. The median age of the 21 study patients was 56 years (range, 33-72), 4 patients (19%) were female, and 17 (81%) were male. The median age of the literature review patients was 49 years (range, 21-74); 12 of 34 patients (33%) with reported sex were female, and 22 (67%) were male. Nine of 21 study patients (43%) and 18 of 31 literature review patients (58%) had simultaneous presentation of systemic sarcoidosis and PML. Six of 14 study patients (43%) and 11 of 19 literature review patients (58%) had a CD4+ T-cell count greater than 200/μL. In 2 study patients, a systemic flare of sarcoidosis closely preceded S-PML development. Ten of 17 study patients (59%) and 21 of 35 literature review patients (60%) died during the acute phase of illness. No meaningful predictive differences were found between patients who survived S-PML and those who did not.Conclusions and RelevanceIn this case series, patients with sarcoidosis developed PML in the absence of therapeutic immune suppression, and peripheral blood proxies of immune function were often only mildly abnormal. Systemic sarcoidosis flares may rarely herald the onset of S-PML. Clinicians should consider PML in any patient with sarcoidosis and new white matter lesions on brain magnetic resonance imaging.

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Progressive multifocal leukoencephalopathy developing in advanced pulmonal sarcoidosis

Cited by 26 publications

References 17 publications

Can pulmonary sarcoidosis trigger a progressive multifocal leukoencephalopathy? Considerations from a case series and a review of literature

Can pulmonary sarcoidosis trigger a progressive multifocal leukoencephalopathy? Considerations from a case series and a review of literature

Infratentorial progressive multifocal leukencephalopathy in a patient with pulmonary sarcoidosis

Characteristics of Progressive Multifocal Leukoencephalopathy Associated With Sarcoidosis Without Therapeutic Immune Suppression

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