Title : Navigating progressive multifocal leukoencephalopathy in HIV: A treatment journey
Abstract:
Introduction:
Progressive multifocal leukoencephalopathy (PML) is a severe demyelinating disease caused by the JC virus, predominantly affecting immunocompromised individuals, particularly those with HIV. The current era sees AIDS as the most common predisposing disorder for PML. PML presents with various neurological symptoms and can lead to significant morbidity and mortality if left untreated. Differentiating PML from HIV encephalopathy is crucial for appropriate management.
Case Presentation:
We report the case of a 42-year-old female with HIV and poor adherence to antiretroviral therapy (ART), who developed neurological symptoms. She presented with a one-month history of headache and projectile vomiting, and a five-day history of focal seizures followed by right-sided weakness. Neurological examination revealed right upper motor neuron facial palsy, weakness in the right upper and lower limbs, and brisk reflexes. MRI of the brain showed asymmetrical white matter hyperintensities, more pronounced on the left side, with involvement of subcortical fibers, midline shift, and partial effacement of the lateral ventricle. Differential diagnoses included CNS infections (tuberculosis, toxoplasmosis), CNS lymphoma, PML, and HIV encephalopathy. The lack of enhancing lesions on MRI and asymmetrical white matter involvement suggested PML. Despite a negative CSF JC virus PCR, the clinical and imaging findings supported PML. The patient was started on second-line ART and anti-seizure medications.
Discussion:
The diagnosis of PML can be challenging due to its non-specific presenting symptoms, including weakness, sensory deficits, cognitive dysfunction, and seizures. The clinical presentations of PML form a continuum influenced by the host's immune response, ranging from classic PML with minimal immune activity to PML-IRIS with extensive inflammation. Differentiating PML from HIV encephalopathy, which typically presents with cognitive deficits and symmetric lesions on imaging, is crucial for effective management. Other differential diagnoses considered included tuberculoma, CNS toxoplasmosis, primary CNS lymphoma, and CMV encephalitis. Each condition has distinct imaging characteristics: tuberculoma presents as ring-enhancing lesions, CNS toxoplasmosis as large ring-enhancing lesions involving deep grey matter, primary CNS lymphoma as enhancing periventricular lesions, and CMV encephalitis as multifocal diffuse micro-nodules.
Conclusion:
This case highlights the importance of early recognition and management of PML in HIV-positive individuals, especially those with poor ART adherence. Prompt initiation of appropriate antiretroviral therapy and supportive care can lead to favorable outcomes, as demonstrated by the resolution of lesions and clinical improvement in our patient. Differentiating PML from HIV encephalopathy is essential for tailored management strategies and optimal patient outcomes. This case underscores the challenges of managing PML in HIV-positive patients and the necessity of distinguishing it from other potential diagnoses for effective clinical management.
Audience Take Away Notes:
- Clinical Presentation and Diagnosis: Understanding the varied neurological symptoms associated with PML, such as weakness, sensory deficits, and seizures, and the challenges in diagnosing PML versus other neurological conditions like HIV encephalopathy.
- Imaging and Differential Diagnosis: Recognizing the distinctive MRI findings of PML, such as asymmetrical white matter hyperintensities, and distinguishing these from imaging patterns seen in conditions like CNS infections and lymphoma.
- Outcome and Prognosis: Appreciating the potential for clinical improvement and lesion resolution with timely intervention, highlighting the evolving prognosis of PML in the era of widespread ART use.
