Session Time: 12:30pm-2:00pm
Location: Research Hub - Kiosk 4
Disclosures: Debbie Lee, MD: Nothing to disclose
Case Description: Patient presented with 2 weeks of dizziness, imbalance, and dysarthria. Workup revealed positive human immunodeficiency virus (HIV)-1 genotype with CD4 count 27, as well as diagnosis of progressive multifocal leukoencephalopathy (PML) based on presence of John Cunningham (JC) virus and findings on magnetic resonance imaging (MRI). Patient was started on highly active antiretroviral therapy (HAART). He was admitted to acute inpatient rehabilitation (AIR) functioning at minimal-assistance to supervision level. Eight days after HAART initiation, he demonstrated acute decline in function. MRI demonstrated progression of PML lesions. Over next 2 weeks, he demonstrated worsening neurological function requiring total-assistance. Repeat MRI and clinical deterioration suggested development of immune reconstitution inflammatory syndrome (IRIS). Infectious-disease specialists recommended pulse steroid followed by prolonged taper.
Setting: Acute inpatient rehabilitation.
Patient: 35-year-old male with newly diagnosed HIV and PML that evolved into IRIS.
Assessment/Results: Despite the steroid course, patient did not demonstrate neurological or functional improvement. Family meetings were held to discuss poor prognosis and goals of care. Patient was discharged to hospice care.
Discussion: To our knowledge, this is the first case of a patient with HIV and PML who developed IRIS while undergoing AIR. PML is a fatal demyelinating disease of the central nervous system, with a 1-year survival rate of approximately 50 percent in HIV-infected patients on HAART. Goal of treatment is to prolong survival by optimizing HAART to restore adaptive immune response. IRIS occurs in 10-20 percent of patients within 3-6 weeks after HAART initiation, wherein there is paradoxical worsening of clinical condition with recovery of immune system. IRIS treatment with steroids remains controversial but is generally recommended for those with worsening clinical or radiologic findings.
Conclusion: In HIV-infected patients with opportunistic infections like PML, it is important to closely monitor neurological status and confer with specialists in the setting of recent HAART initiation. Here, patient developed IRIS within 2 weeks of AIR and was started on steroids, though treatment was ultimately unsuccessful.
Level of Evidence: Level IV
To cite this abstract in AMA style:Lee D, Hurh P. Development of Immune Reconstitution Inflammatory Syndrome from Progressive Multifocal Leukoencephalopathy During Acute Inpatient Rehabilitation: A Case Report [abstract]. PM R. 2019; 11(S2)(suppl 2). https://pmrjabstracts.org/abstract/development-of-immune-reconstitution-inflammatory-syndrome-from-progressive-multifocal-leukoencephalopathy-during-acute-inpatient-rehabilitation-a-case-report/. Accessed December 9, 2023.
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