Fulminant inflammatory leukoencephalopathy associated with HAART-induced immune restoration in AIDS-related progressive multifocal leukoencephalopathy

  title={Fulminant inflammatory leukoencephalopathy associated with HAART-induced immune restoration in AIDS-related progressive multifocal leukoencephalopathy},
  author={Aur{\'e}lie Vendrely and B. Bienvenu and Jacques Gasnault and J.-B. Thiebault and D Salmon and Françoise Gray},
  journal={Acta Neuropathologica},
HAART-induced immune restoration is beneficial for patients with AIDS-related progressive multifocal leukoencephalopathy (PML). However, in rare instances, an immune-reconstitution inflammatory syndrome (IRIS) may cause paradoxical clinical deterioration. We report the neuropathological study of an AIDS patient who presented with progressive cognitive deterioration; CD4+ count was 117 and the HIV viral load >104; imaging showed non-enhancing lesions consistent with PML. Following initiation of… 

Progressive multifocal leukoencephalopathy and immune reconstitution inflammatory syndrome in an HIV patient with favorable outcome using combination of antiretroviral therapy and systemic corticosteroids: a case report.

The diagnosis and management of a 50-year-old Afro-Caribbean man who was found to have AIDS, treated with antiretroviral regimen but soon develop worsening of neurological symptoms and poor functional performance status is described.

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A 37-year-old African woman with HIV infection diagnosed at 13 years of age was admitted to the emergency department after experiencing backache, severe headache, acute aphasia and psychomotor slowing, but the patient died from brainstem herniation 48 hours after admission.

Pathology of immune reconstitution inflammatory syndrome in multiple sclerosis with natalizumab-associated progressive multifocal leukoencephalopathy

Natalizumab is an approved medication for highly active multiple sclerosis (MS). Progressive multifocal leukoencephalopathy (PML) may occur as a severe side effect of this drug. Here, we describe

Progressive multifocal leukoencephalopathy and immune reconstitution inflammatory syndrome (IRIS)

It is shown that PML and PML–IRIS result from predominant JC virus infection of oligodendrocytes and, to a lesser extent, of infected neurons, and that inflammation in these encephalitides seems to be driven by a dominant cytotoxic T cell response which is massively exaggerated during IRIS.

Progressive Multifocal Leukoencephalopathy Complicating HIV-1 Infection

While there is no specific treatment, reversal of immunosuppression by cART leads to clinical and MRI stabilization in 50-60% of PML patients and JCV clearance from CSF.

Immune Reconstitution Associated With Progressive Multifocal Leukoencephalopathy in Human Immunodeficiency Virus: A Case Discussion and Review of the Literature

These features clinically, radiologically, and histopathologically suggest an added component in the form of immune reconstitution syndrome to PML, which substantiates the role of corticosteroids in carefully considered cases of HIV with leukoencephalopathy secondary to immune reconStitution.

Progressive multifocal leukoencephalopathy: a review of the neuroimaging features and differential diagnosis

Patients under treatment with monoclonal antibodies in routine practice, or new ones in ongoing clinical trials, differentiating PML from new MS lesions on brain MRI is critical for both neurologists and neuroradiologists.

Immune reconstitution after potent antiretroviral therapy in AIDS patients with progressive multifocal leukoencephalopathy

The following 2 cases of immune reconstitution PML described in this report illustrate the varied radiological manifestations and clinical outcomes that can develop in AIDS patients with PML receiving potent ART and to the authors' knowledge are the first reports of immune Reconstitution isolated to the cerebellum in such patients.

Immune Reconstitution Inflammatory Syndrome Unmasking or Worsening AIDS-Related Progressive Multifocal Leukoencephalopathy: A Literature Review

Incidence of progressive multifocal leukoencephalopathy (PML) in HIV-infected patients has declined in the combined antiretroviral therapy (cART) era although a growing number of acquired



New insights into progressive multifocal leukoencephalopathy

  • I. Koralnik
  • Medicine, Biology
    Current opinion in neurology
  • 2004
Advances in the understanding of JC virus biology have shed new light on the pathogenesis of progressive multifocal leukoencephalopathy, and on its possible role in cerebellar atrophy in HIV-infected individuals.

Severe, demyelinating leukoencephalopathy in AIDS patients on antiretroviral therapy

Evidence is provided for the emergence of a severe form of HIV-associated leukoencephalopathy in AIDS patients failing highly active antiretroviral therapy and its relationship to clinical and neuroimaging findings, and hypotheses regarding pathogenesis are suggested.

Progressive multifocal leukoencephalopathy in patients with AIDS receiving highly active antiretroviral therapy.

The observations suggest that PML can develop in patients who have shown clinical response to HAART, and that definitive therapy is still needed for PML.

Progressive multifocal leucoencephalopathy with unusual inflammatory response during antiretroviral treatment

Evidence is given that AIDS associated inflammatory PML must be considered another clinical entity in the expanding range of diseases now commonly referred to as the immune reconstitution syndrome.

Prolonged survival without neurological improvement in patients with AIDS-related progressive multifocal leukoencephalopathy on potent combined antiretroviral therapy.

The results demonstrate a benefit of CART on survival of AIDS-related PML patients and suggest the need for an early, specific anti-JC virus treatment to limit the neurological deterioration.

Cerebral CD8+ lymphocytosis in HIV-1 infected patients with immune restoration induced by HAART

It is suggested that the rapid immune reconstitution induced by HAART in these two patients led to a redistribution of lymphocytes into peripheral blood, followed by recruitment of CD8+ lymphocyte into the brain, which resulted in the diffuse infiltration described.

Inflammatory reactions in progressive multifocal leukoencephalopathy after highly active antiretroviral therapy.

Three patients with progressive multifocal leukoencephalopathy treated with highly active antiretroviral therapy (HAART) worsened clinically and radiologically and all three had reduced HIV viraemia and increased CD4 cell counts at the time of deterioration.


Immune reconstitution resulting from use of highly active antiretroviral therapy in patients infected with human immunodeficiency virus type 1 (HIV-1) has been associated with a significant decrease

Predictive factors for prolonged survival in acquired immunodeficiency syndrome—associated progressive multifocal leukoencephalopathy

Predictors of long‐term survival in AIDS patients with PML include PML as the heralding manifestation of AIDS, high CD4 T‐lymphocyte count at disease onset, lesion enhacement on computed tomographic scan or magnetic resonance imaging, and evidence of recovery of neurological function.

Survival improvement of AIDS-related progressive multifocal leukoencephalopathy in the era of protease inhibitors. Clinical Epidemiology Group. French Hospital Database on HIV.

This study of a large cohort of patients diagnosed with PML provides evidence that a combination antiretroviral regimen, especially one including PI, confers marked survival benefits.