Immune Reconstitution Inflammatory Syndrome with Recurrent Paradoxical Cerebellar HIV-Associated Progressive Multifocal Leukoencephalopathy

Abstract

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.

Leucoencefalopatía multifocal progresiva secundaria al síndrome de reconstitución inmune

“…28 Es importante destacar que, en cuanto al pronóstico, la LMP por SRI se asocia a un mayor número de lesiones radiológicas, disminución en la sobrevida y aumento en la mortalidad, en comparación con otras etiologías. 29 En este grupo de pacientes, el tratamiento se basa en el uso de corticosteroides a pesar de que no existen hasta este momento ensayos clínicos aleatorizados que demuestren su efectividad. Se necesitan estudios para dilucidar nuevas estrategias terapéuticas para mejorar el pronóstico de la LMP, sin embargo; con los avances en el seguimiento y diagnóstico, así como las maniobras para restablecer el sistema inmune dependiendo de la etiología, se ha alcanzado un progreso importante; no obstante, aún hay mucho por hacer, especialmente en el contexto de VIH y LMP por SRI.…”