A 43-year-old man presented to the emergency room complaining of tongue paresthesia, slurred speech, and weakness. These symptoms had developed insidiously and progressively over the past 4 weeks. The patient described muscular weakness initially involving the left side of the body, which soon included the right side. He also referred a decrease in sensitivity in the left half of the body. He denied fever, headache, weight loss, dyspnea, convulsions, visual symptoms, incontinence, and vertigo. His past medical history was unremarkable, except for smoking (15 pack-years) and recreational alcohol consumption.
The neurological examination revealed a hyposensitivity in the right hemiface, as in the right half of the body, and grade 4 of muscle strength of the left upper limb. The remaining physical examination was normal as were his vital signs. The initial laboratory work-up was normal; however, further investigation revealed a positive serology for HIV infection by the enzyme-linked immunosorbent assay and Western blotting. The TCD4+ peripheral count was 75 cells/microL, and the HIV-1 RNA viral load in the blood was 97,911 copies/mL (branched DNA) or Log 4991. The brain computed tomography (CT) revealed two hypodense foci in the right cerebral hemisphere's white matter, in the right frontal lobe, and in the high parietal region, without contrast enhancement or midline shift.
Due to the imaging findings in a patient with HIV, neurotoxoplasmosis was the initial working diagnosis. Therefore, pyrimethamine and sulfadiazine were promptly prescribed. Two weeks later, this antibiotic regimen was withdrawn since (i) the patient's clinical features did not improve; (ii) the serology for toxoplasmosis tested negative for immunoglobulin IgG and IgM; and (iii) the lesions increased in size and became more evident in controlled CT. The brain magnetic resonance imaging (MRI) showed extensive discontinuous hyper signal areas on T2- and FLAIR-weighted images in the hemispheric white matter, with predominance in the right frontotemporal subcortical region, as well as in the splenium of the corpus callosum and the brainstem (Figure 1). These findings raised the diagnosis of PML. At this time, the cerebrospinal fluid (CSF) examination showed leukocytes of 2 cells, erythrocytes of 14 cells, protein of 25 mg/dL, and glucose of 58 mg/dL. CSF culture was negative for bacteria, mycobacteria, and fungus. The polymerase chain reactions (PCR) for Mycobacterium tuberculosis, Cryptococcus spp, Toxoplasma gondii, and JCV were negative, which permitted the initiation of highly active antiretroviral therapy (HAART).
The patient's outcome was troublesome with progressive worsening of his neurological status, and slow progression to spastic paraparesis. At the end of the fourth week of hospitalization the patient maintained only the distal movement of the right upper limb, hyperreflexia, tetraplegia in a pyramidal pattern, generalized spasticity and hypertonia, ophthalmoplegia, facial amimia, gaze fixation inability, anarthria, and the inability to swallow. According to this outcome, the diagnosis of PML was highly considered, and because of the deterioration of the neurological status concomitantly after HAART, the hypothesis of immune reconstitution inflammatory syndrome-PML (IRIS-PML) was considered. Thus, prednisone 1 mg/kg was started. However, no improvement was observed.
Meanwhile, the patient presented an episode of bronchoaspiration and septic shock, and died after 7 weeks of hospitalization.
An autopsy was performed after the informed consent signed by his wife.