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Immune reconstitution is not a prognostic factor in progressive multifocal leukoencephalopathy.

ABSTRACT: Progressive multifocal leukoencephalopathy (PML) is typically associated with minimal inflammation; however, patients may develop an inflammatory response due to immune reconstitution (IRIS). The authors aimed to determine if characteristics and outcomes of PML are altered in those with IRIS. A retrospective records review was performed on 87 patients diagnosed with PML at Johns Hopkins, 27 of which had a syndrome consistent with IRIS. Gadolinium enhancement on MRI occurred in 44.4% of cases of PML-IRIS versus 5.1% in PML (p<0.05), and thus had low diagnostic sensitivity and specificity. In HIV+ cases, CD4 counts were lower in those who later developed IRIS (mean 34.8 vs. 71.7, p<0.05) and was predictive of the development of IRIS (p<0.05). Improved prognosis was seen with higher cerebrospinal fluid (CSF) white blood cell counts and protein levels, but not for gadolinium enhancement and there were no differences in survival for PML versus PML-IRIS.

SUBMITTER: Harrison DM

PROVIDER: S-EPMC4916968 | biostudies-literature | 2011 Sep

REPOSITORIES: biostudies-literature

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Immune reconstitution is not a prognostic factor in progressive multifocal leukoencephalopathy.

Harrison Daniel M DM Newsome Scott D SD Skolasky Richard L RL McArthur Justin C JC Nath Avindra A

Journal of neuroimmunology 20110812 1-2

Progressive multifocal leukoencephalopathy (PML) is typically associated with minimal inflammation; however, patients may develop an inflammatory response due to immune reconstitution (IRIS). The authors aimed to determine if characteristics and outcomes of PML are altered in those with IRIS. A retrospective records review was performed on 87 patients diagnosed with PML at Johns Hopkins, 27 of which had a syndrome consistent with IRIS. Gadolinium enhancement on MRI occurred in 44.4% of cases of ...[more]

PMID: 21840066

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Project description:Progressive multifocal leukoencephalopathy (PML) is a subacute brain infection by the opportunistic John Cunningham (JC) virus. Herein, we describe seven patients with PML, lymphopenia, and sarcoidosis, in three of whom PML was the first manifestation of sarcoidosis. At onset, the clinical picture comprised rapidly progressive spastic hemi- or limb pareses as well as disturbances of vision, speech, and orientation. Cerebral magnetic resonance imaging showed T2-hyperintense, confluent, mainly supratentorial lesions. Four patients developed punctate contrast enhancement as a radiological sign of an immune reconstitution inflammatory syndrome (IRIS), three of them having a fatal course. In the cerebrospinal fluid, the initial JC virus load (8-25,787 copies/ml) did not correlate with interindividual severity; however, virus load corresponded to clinical dynamics. Brain biopsies (n = 2), performed 2 months after symptom onset, showed spotted demyelination and microglial activation. All patients had lymphopenia in the range of 270-1150/µl. To control JC virus, three patients received a combination of mirtazapine and mefloquine, another two patients additionally took cidofovir. One patient was treated with cidofovir only, and one patient had a combined regimen with mirtazapine, mefloquine, cidofovir, intravenous interleukin 2, and JC capsid vaccination. To treat sarcoidosis, the four previously untreated patients received prednisolone. Three patients had taken immunosuppressants prior to PML onset, which were subsequently stopped as a potential accelerator of opportunistic infections. After 6-54 months of follow up, three patients reached an incomplete recovery, one patient progressed, but survived so far, and two patients died. One further patient was additionally diagnosed with lung cancer, which he died from after 24 months. We conclude that the combination of PML and sarcoidosis is a diagnostic and therapeutic challenge. PML can occur as the first sign of sarcoidosis without preceding immunosuppressive treatment. The development of IRIS might be an indicator of poor outcome.

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Immune reconstitution is not a prognostic factor in progressive multifocal leukoencephalopathy.

Publications

Immune reconstitution is not a prognostic factor in progressive multifocal leukoencephalopathy.

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