Progressive multifocal leukoencephalopathy in patients with sarcoidosis

Jamilloux, Yvan; Néel, A.; Lecouffe-Desprets, Marie; Fèvre, Anne; Kerever, Sébastien; Guillon, B.; Bouvry, Diane; Varron, L.; Redares, Cécile; Dominique, S.; Roux, Mareille; Chapelon-Abric, C.; Valeyre, Dominique; Ducray, François; Bernard, C.; Broussolle, C.; Hamidou, M.; Sève, P.

doi:10.1212/wnl.0000000000000318

Cited by 42 publications

(50 citation statements)

References 38 publications

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“…This was despite the fact that his brain MRI lacked contrast enhancement, which is very atypical for neurosarcoidosis. Very importantly, sarcoidosis is a known risk factor for PML and frequently causes a delay in diagnosis [5] . This was definitely the case for patient D, who was treated for neuro-sarcoidosis for months before we decided to do a brain biopsy.…”

Section: Discussionmentioning

confidence: 99%

Progressive multifocal leukoencephalitis in a patient with sarcoidosis on hydroxychloroquine with negative cerebrospinal fluid testing for the John Cunningham virus

Inamullah¹,

Farin²,

Tran³

et al. 2018

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Progressive multifocal leukoencephalopathy (PML) is a severe demyelinating disease of the central nervous system caused by the John Cunningham (JC) virus typically seen in immuno-compromised patients. Several drugs that suppress that immune system have already been known to cause PML such as natalizumab and rituximab. We present a patient with sarcoidosis who develops PML in the rare setting of minimal immunosuppression with only hydroxychloroquine. There was significant delay in the diagnosis due to negative cerebrospinal fluid testing for JC virus and concern for neuro-sarcoidosis, but eventually a diagnosis of PML was made via brain biopsy.

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Section: Discussionmentioning

confidence: 99%

Progressive multifocal leukoencephalitis in a patient with sarcoidosis on hydroxychloroquine with negative cerebrospinal fluid testing for the John Cunningham virus

Inamullah¹,

Farin²,

Tran³

et al. 2018

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“…When clinical deterioration progresses despite intensive immunemodulating treatment in neurosarcoidosis, a progressive multifocal leukoencephalopathy should be considered. This potentially fatal treatment complication in immunocompromised patients can be easily missed (131).…”

Section: Treatmentmentioning

confidence: 99%

Challenges in the diagnosis and treatment of neurosarcoidosis

et al. 2015

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The diagnosis and treatment of neurosarcoidosis can be very challenging for several reasons. It affects clinically 5%-10% of sarcoidosis patients, but can be found in up to 25% of autopsies. These data reveal that a high percentage of asymptomatic or misdiagnosed cases can be missed at an initial diagnostic approach. Clinical and imaging findings are often non-specific since they can be found in a large number of neurological disorders. Histopathology can also be confounding if not performed by an expert pathologist and not placed in an appropriate clinical context. In this review, we discuss clinical features, laboratory findings, imaging, and histology of neurosarcoidosis, and we report current evidence regarding drug therapy. We conclude that a correct diagnostic approach should include a multidisciplinary evaluation involving clinicians, radiologists, and pathologists and that future studies should evaluate the genetic signature of neurosarcoidosis as they could be helpful in the assessment of this uncommon disease. With head-to-head comparisons of medical treatment for neurosarcoidosis still lacking due to the rarity of the disease and an increasing number of immunomodulating therapies at hand, novel therapeutic approaches are to be expected within the next few years. Key messages Neurosarcoidosis is a rare disorder that affects clinically 5%-10% of sarcoidosis patients, but can be found in up to 25% of autopsies, revealing that a high percentage of asymptomatic or misdiagnosed cases can be missed at an initial diagnostic approach. A multidisciplinary evaluation is useful to achieve a correct diagnosis because clinical and imaging findings are often non-specific. Corticosteroids are the first-line treatment for neurosarcoidosis, followed by steroid-sparing immune-modulating agents if prednisone therapy is insufficient. Dear Author, Please check these proofs carefully. It is the responsibility of the corresponding author to check against the original manuscript and approve or amend these proofs. A second proof is not normally provided. Informa Healthcare cannot be held responsible for uncorrected errors, even if introduced during the composition process. The journal reserves the right to charge for excessive author alterations, or for changes requested after the proofing stage has concluded.The following queries have arisen during the editing of your manuscript and are marked in the margins of the proofs. Unless advised otherwise, submit all corrections using the CATS online correction form. Once you have added all your corrections, please ensure you press the ''Submit All Corrections'' button.Please review the table of contributors below and confirm that the first and last names are structured correctly and that the authors are listed in the correct order of contribution. The diagnosis and treatment of neurosarcoidosis can be very challenging for several reasons. It affects clinically 5%-10% of sarcoidosis patients, but can be found in up to 25% of autopsies. These data reveal that a high percenta...

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“…5 In rare cases, patients with sarcoidosis developed PML with-out immunosuppressive therapies. [6][7][8] These cases suggest that systemic sarcoidosis itself might cause PML. Although not fully understood, the pathogenesis of PML in sarcoidosis might be explained by a regulatory T cell/ Th1 imbalance and redistribution of T cells to the sites of disease involvement.…”

Section: Discussionmentioning

confidence: 89%

“…A study of 58 HIV‐negative patients with PML showed that 9% had sarcoidosis as the underlying disease . In rare cases, patients with sarcoidosis developed PML without immunosuppressive therapies . These cases suggest that systemic sarcoidosis itself might cause PML.…”

Section: Discussionmentioning

confidence: 99%

Mirtazapine treatment ceased the progression of progressive multifocal leukoencephalopathy associated with systemic sarcoidosis

Matsudaira

Araki

Oishi

et al. 2014

Neurology & Clinical Neurosc

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Progressive multifocal leukoencephalopathy occurs almost exclusively in immunosuppressed individuals. Mirtazapine, a 5-hydroxytryptamine-2A antagonist, has been used empirically against progressive multifocal leukoencephalopathy. A 60-year-old man who was diagnosed with sarcoidosis at 20 years-of-age and had not taken immunosuppressive therapies developed dysarthria, left hemiparesis and dressing apraxia. Cranial fluid-attenuated inversion-recovery magnetic resonance imaging showed hyperintense areas in the right frontal and temporoparietal white matter. The initial diagnosis was neurosarcoidosis, based on non-caseating granuloma in a lymph node biopsy. Immunosuppressive therapy was initiated; however, this aggravated his neurological symptoms. Immunohistochemistry of the brain biopsy showed John Cunningham virus infected large ballooned oligodendrocytes, leading to a diagnosis of progressive multifocal leukoencephalopathy. Treatment with mirtazapine improved the neurological symptoms, and the magnetic resonance imaging abnormality did not progress. We stress the diagnostic difficulties of distinguishing progressive multifocal leukoencephalopathy from neurosarcoidosis, and suggest that mirtazapine treatment is effective against progressive multifocal leukoencephalopathy associated with sarcoidosis.

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Progressive multifocal leukoencephalopathy in patients with sarcoidosis

Abstract: PML during sarcoidosis is often misdiagnosed. It is not associated with severe CD4 lymphocytopenia. Fatality rate is high in comparison with PML associated with other conditions. Interrupting immunosuppression remains the mainstay of treatment.

Cited by 42 publications

References 38 publications

Progressive multifocal leukoencephalitis in a patient with sarcoidosis on hydroxychloroquine with negative cerebrospinal fluid testing for the John Cunningham virus

Progressive multifocal leukoencephalitis in a patient with sarcoidosis on hydroxychloroquine with negative cerebrospinal fluid testing for the John Cunningham virus

Challenges in the diagnosis and treatment of neurosarcoidosis

Mirtazapine treatment ceased the progression of progressive multifocal leukoencephalopathy associated with systemic sarcoidosis

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