Posters

Presenting Author

Hugo Zamarron

Presenting Author Academic/Professional Position

Medical Student

Academic Level (Author 1)

Medical Student

Academic Level (Author 2)

Fellow

Discipline/Specialty (Author 2)

Neurology

Presentation Type

Poster

Discipline Track

Patient Care

Abstract Type

Case Report

Abstract

Background: Progressive multifocal leukoencephalopathy (PML) is a rare but serious demyelinating disease of the central nervous system, caused by the John Cunningham (JC) virus. While the JC virus is common in the general population, it remains dormant in healthy individuals but can reactivate in immunocompromised patients, particularly those with HIV/AIDS or undergoing immunosuppressive treatments for organ transplants or autoimmune conditions. The virus attacks oligodendrocytes, leading to demyelination and significant neurological deficits. Immunosuppressive therapies such as natalizumab, rituximab, and mycophenolate increase the risk of PML. Diagnosing PML is challenging as its symptoms—focal neurological deficits, sensory-motor issues, and visual disturbances—overlap with conditions like multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Differentiating PML from these diseases is crucial for appropriate treatment, as misdiagnosis can lead to delayed interventions and worse outcomes. This case highlights the importance of early recognition of PML, the need for prompt antiviral therapy, and the cessation of immunosuppressive medications.

Case Presentation: A 47-year-old female with CREST syndrome, chronic immunosuppression, and right hip septic arthritis presented with progressive right-sided weakness and sensory loss over two weeks. Her immunosuppressive regimen included mycophenolate, dupilumab, and prednisone for three years, managed by her rheumatologist. She also had class 3 obesity, chronic bronchitis, drug- induced liver injury, gastritis, and hypothyroidism, with a history of multiple surgeries, including hip and thyroid procedures. A brain MRI revealed a large, T2-hyperintense lesion with vasogenic edema in the left hemisphere, with no gadolinium enhancement. A lumbar puncture showed one oligoclonal band in the cerebrospinal fluid (CSF). Testing for autoimmune encephalitis was negative, but the CSF revealed a low-positive JC virus titer of 50 IU/mL and slightly elevated anti-MOG serum levels (1:10). Given these findings, the patient's immunosuppressive medications were discontinued. She received a 5-day course of high-dose Solu-Medrol (1,000 mg) followed by a prednisone taper. Despite this, her symptoms did not improve. Further CSF tests were negative for other CNS inflammatory diseases. A follow-up MRI showed worsening lesions, prompting a brain biopsy due to her immunocompromised status and the low-positive JC virus titer. The biopsy confirmed PML secondary to chronic immunosuppression.

Conclusions: This case highlights the importance of considering PML in the differential diagnosis of unexplained neurological symptoms in immunocompromised patients. Early diagnosis and appropriate management, including discontinuation of immunosuppressive therapy and antiviral treatment, can improve outcomes. The case also illustrates the difficulty of distinguishing PML from other demyelinating diseases like MS, NMOSD, and MOGAD, stressing the need for advanced diagnostic tools. Timely intervention can prevent severe consequences, underscoring the necessity of vigilance in managing this rare and potentially fatal condition.

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Progressive Multifocal Leukoencephalopathy (PML) Induced by JC Virus: A Case Report and Review of Clinical Implications

Background: Progressive multifocal leukoencephalopathy (PML) is a rare but serious demyelinating disease of the central nervous system, caused by the John Cunningham (JC) virus. While the JC virus is common in the general population, it remains dormant in healthy individuals but can reactivate in immunocompromised patients, particularly those with HIV/AIDS or undergoing immunosuppressive treatments for organ transplants or autoimmune conditions. The virus attacks oligodendrocytes, leading to demyelination and significant neurological deficits. Immunosuppressive therapies such as natalizumab, rituximab, and mycophenolate increase the risk of PML. Diagnosing PML is challenging as its symptoms—focal neurological deficits, sensory-motor issues, and visual disturbances—overlap with conditions like multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Differentiating PML from these diseases is crucial for appropriate treatment, as misdiagnosis can lead to delayed interventions and worse outcomes. This case highlights the importance of early recognition of PML, the need for prompt antiviral therapy, and the cessation of immunosuppressive medications.

Case Presentation: A 47-year-old female with CREST syndrome, chronic immunosuppression, and right hip septic arthritis presented with progressive right-sided weakness and sensory loss over two weeks. Her immunosuppressive regimen included mycophenolate, dupilumab, and prednisone for three years, managed by her rheumatologist. She also had class 3 obesity, chronic bronchitis, drug- induced liver injury, gastritis, and hypothyroidism, with a history of multiple surgeries, including hip and thyroid procedures. A brain MRI revealed a large, T2-hyperintense lesion with vasogenic edema in the left hemisphere, with no gadolinium enhancement. A lumbar puncture showed one oligoclonal band in the cerebrospinal fluid (CSF). Testing for autoimmune encephalitis was negative, but the CSF revealed a low-positive JC virus titer of 50 IU/mL and slightly elevated anti-MOG serum levels (1:10). Given these findings, the patient's immunosuppressive medications were discontinued. She received a 5-day course of high-dose Solu-Medrol (1,000 mg) followed by a prednisone taper. Despite this, her symptoms did not improve. Further CSF tests were negative for other CNS inflammatory diseases. A follow-up MRI showed worsening lesions, prompting a brain biopsy due to her immunocompromised status and the low-positive JC virus titer. The biopsy confirmed PML secondary to chronic immunosuppression.

Conclusions: This case highlights the importance of considering PML in the differential diagnosis of unexplained neurological symptoms in immunocompromised patients. Early diagnosis and appropriate management, including discontinuation of immunosuppressive therapy and antiviral treatment, can improve outcomes. The case also illustrates the difficulty of distinguishing PML from other demyelinating diseases like MS, NMOSD, and MOGAD, stressing the need for advanced diagnostic tools. Timely intervention can prevent severe consequences, underscoring the necessity of vigilance in managing this rare and potentially fatal condition.

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