Immune Reconstitution Inflammatory Syndrome with Recurrent Paradoxical Cerebellar HIV-Associated Progressive Multifocal Leukoencephalopathy
Progressive multifocal leukoencephalopathy (PML), presenting as immune reconstitution inflammatory syndrome (IRIS), is a known complication of antiretroviral therapy (ART) in people living with HIV (PLWH). Typically preceded by ART initiation, IRIS may appear simultaneously/unmasked (PML-s-IRIS) or...
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| Format: | Article |
| Language: | English |
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MDPI AG
2021-06-01
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| Series: | Pathogens |
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| Online Access: | https://www.mdpi.com/2076-0817/10/7/813 |
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| author | Paola Frattaroli Teresa A. Chueng Obinna Abaribe Folusakin Ayoade |
| author_facet | Paola Frattaroli Teresa A. Chueng Obinna Abaribe Folusakin Ayoade |
| author_sort | Paola Frattaroli |
| collection | DOAJ |
| description | Progressive multifocal leukoencephalopathy (PML), presenting as immune reconstitution inflammatory syndrome (IRIS), is a known complication of antiretroviral therapy (ART) in people living with HIV (PLWH). Typically preceded by ART initiation, IRIS may appear simultaneously/unmasked (PML-s-IRIS) or as a delayed/worsening/paradoxical (PML-d-IRIS) presentation of known PML disease. Primary cerebellar tropism continues to be a rare presentation, and paradoxical cerebellar involvement of PML-IRIS syndrome can be a challenge for both diagnosis and management. Steroids have been suggested as a possible therapy in severe cases but the duration of steroid therapy remain elusive. Our case is that of a 34-year-old man with newly diagnosed HIV simultaneously found to have cerebellar PML. His PML lesions however worsened after initiation of ART (PML-d-IRIS) with evidence of increased intracranial pressure. Despite initial favorable response to a short duration of steroids, he had multiple recurrence of his PML lesions after steroids were discontinued. The presence of predominant cerebellar lesions and the question of how long steroids should be provided to prevent or minimize PML recurrence is the highlight of our case. This report emphasizes the need for more controlled studies to assist clinicians in the optimal diagnosis and management of PML-IRIS in PLWH. |
| first_indexed | 2024-03-09T04:48:02Z |
| format | Article |
| id | doaj.art-823ff0997a484f68b4d5514f86fdd778 |
| institution | Directory Open Access Journal |
| issn | 2076-0817 |
| language | English |
| last_indexed | 2024-03-09T04:48:02Z |
| publishDate | 2021-06-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Pathogens |
| spelling | doaj.art-823ff0997a484f68b4d5514f86fdd7782023-12-03T13:13:32ZengMDPI AGPathogens2076-08172021-06-0110781310.3390/pathogens10070813Immune Reconstitution Inflammatory Syndrome with Recurrent Paradoxical Cerebellar HIV-Associated Progressive Multifocal LeukoencephalopathyPaola Frattaroli0Teresa A. Chueng1Obinna Abaribe2Folusakin Ayoade3Jackson Memorial Hospital, 1611 NW 12th Ave, Miami, FL 33136, USAJackson Memorial Hospital, 1611 NW 12th Ave, Miami, FL 33136, USAJackson Memorial Hospital, 1611 NW 12th Ave, Miami, FL 33136, USAJackson Memorial Hospital, 1611 NW 12th Ave, Miami, FL 33136, USAProgressive multifocal leukoencephalopathy (PML), presenting as immune reconstitution inflammatory syndrome (IRIS), is a known complication of antiretroviral therapy (ART) in people living with HIV (PLWH). Typically preceded by ART initiation, IRIS may appear simultaneously/unmasked (PML-s-IRIS) or as a delayed/worsening/paradoxical (PML-d-IRIS) presentation of known PML disease. Primary cerebellar tropism continues to be a rare presentation, and paradoxical cerebellar involvement of PML-IRIS syndrome can be a challenge for both diagnosis and management. Steroids have been suggested as a possible therapy in severe cases but the duration of steroid therapy remain elusive. Our case is that of a 34-year-old man with newly diagnosed HIV simultaneously found to have cerebellar PML. His PML lesions however worsened after initiation of ART (PML-d-IRIS) with evidence of increased intracranial pressure. Despite initial favorable response to a short duration of steroids, he had multiple recurrence of his PML lesions after steroids were discontinued. The presence of predominant cerebellar lesions and the question of how long steroids should be provided to prevent or minimize PML recurrence is the highlight of our case. This report emphasizes the need for more controlled studies to assist clinicians in the optimal diagnosis and management of PML-IRIS in PLWH.https://www.mdpi.com/2076-0817/10/7/813paradoxical PML-IRIScerebellar PML-IRIStreatment PML-IRIS |
| spellingShingle | Paola Frattaroli Teresa A. Chueng Obinna Abaribe Folusakin Ayoade Immune Reconstitution Inflammatory Syndrome with Recurrent Paradoxical Cerebellar HIV-Associated Progressive Multifocal Leukoencephalopathy Pathogens paradoxical PML-IRIS cerebellar PML-IRIS treatment PML-IRIS |
| title | Immune Reconstitution Inflammatory Syndrome with Recurrent Paradoxical Cerebellar HIV-Associated Progressive Multifocal Leukoencephalopathy |
| title_full | Immune Reconstitution Inflammatory Syndrome with Recurrent Paradoxical Cerebellar HIV-Associated Progressive Multifocal Leukoencephalopathy |
| title_fullStr | Immune Reconstitution Inflammatory Syndrome with Recurrent Paradoxical Cerebellar HIV-Associated Progressive Multifocal Leukoencephalopathy |
| title_full_unstemmed | Immune Reconstitution Inflammatory Syndrome with Recurrent Paradoxical Cerebellar HIV-Associated Progressive Multifocal Leukoencephalopathy |
| title_short | Immune Reconstitution Inflammatory Syndrome with Recurrent Paradoxical Cerebellar HIV-Associated Progressive Multifocal Leukoencephalopathy |
| title_sort | immune reconstitution inflammatory syndrome with recurrent paradoxical cerebellar hiv associated progressive multifocal leukoencephalopathy |
| topic | paradoxical PML-IRIS cerebellar PML-IRIS treatment PML-IRIS |
| url | https://www.mdpi.com/2076-0817/10/7/813 |
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