PMC:4925684 / 1256-5081 JSONTXT

{"project":"2_test","denotations":[{"id":"26727910-2876640-63343915","span":{"begin":748,"end":752},"obj":"2876640"},{"id":"26727910-2876640-63343915","span":{"begin":748,"end":752},"obj":"2876640"},{"id":"T2090","span":{"begin":748,"end":752},"obj":"2876640"},{"id":"T27884","span":{"begin":748,"end":752},"obj":"2876640"}],"text":"Case one\nA 49 year old man presented with chronic headache in 2013, on a background of HIV diagnosed in 1987.\nIn the early 2000s his CD4+ T cell count was at a lifetime nadir of 70 cells/μl, plasma HIV RNA 5 · 35 log, and cerebrospinal fluid (CSF) 4 · 69 log. At that time he had cognitive impairment consistent with HIV associated neurocognitive disorder (HAND) with an MRI brain showing multifocal grey and white matter hyperintensities. CSF was normal including negative PCR for JC virus (JCV) DNA, but a right frontal lobe biopsy showed oligodendrocyte nuclei with viral inclusions, prominent reactive astrocytes including bizarre forms, and was strongly positive for JCV DNA by PCR. HIV associated encephalitis was also present (Anders et al. 1986).\nHe commenced HAART for the first time with efavirenz, lopinavir/ritonavir, lamivudine, and stavudine (the latter two later switched to raltegravir for tolerability), with consequent CD4+ T cell count rise to over 300 cells/μl. He was clinically stable but required institutional care due to poor cognition that did not improve with HAART.\nBy 2013 his CD4+ T cell count had been over 500 cells/μl for 7 years, and HIV RNA had been undetectable for a decade. Annual MRI brain scans since 2008 (Figs. 1, 2 and 3) showed progressive white matter atrophy, and since 2010 asymptomatic fluctuating contrast enhancement without mass effect, in both cortical grey and subcortical white matter.\nFig. 1 Case one, 2008: Fluid attenuated inversion recovery (FLAIR) MRI sequences showing multifocal hyperintensity in the subcortical U-fibres of the right basifrontal, right temporal, and left parieto-occipital regions (see arrows). The corresponding T1 post contrast images (lower row), demonstrate an absence of enhancement\nFig. 2 Case one, 2011: FLAIR MRI sequences from 2011 again show hyperintensity in the right basifrontal (see arrows) and left parieto-occipital regions, now with enhancement on corresponding post contrast T1 images (lower row) in the right basifrontal region only\nFig. 3 Case one, 2013: FLAIR hyperintensity has increased in the left parieto-occipital region (see arrows, top images), with new gyriform enhancement (see arrows, bottom images) on corresponding pre-contrast FLAIR sequences. The right basifrontal contrast enhancement has shown a complete resolution\nExamination when he presented with headache in 2013 revealed a new right inferior quadrantanopia, preexisting cognitive impairment, mild upper limb action tremor, and impaired tandem gait. CD4+ T cell count was 648 cells/μl. CSF was bland; cryptococcal antigen and HIV RNA were undetectable. However CSF JCV DNA was detected (below 30 copies/mL, the lower limit of quantification of the assay), using primers targeting the VP2 gene. The sequenced 131 base pair PCR product was a 93 % match to JCV CPN1 strain using the Basic Local Alignment Search Tool. MRI brain showed increased subcortical signal hyperintensity and cortical enhancement in the left posterior temporal lobe extending into the parieto-occipital region (Fig. 3). He was diagnosed with possible recurrent PML.\nHis headache spontaneously improved only to recur several months later. An MRI showed new enhancing lesions in the left temporoparietal grey and subcortical white matter. Serial MR spectroscopic studies showed increasing choline and myoinositol in these regions with markedly reduced N-acetylaspartate implying ongoing gliosis of the subcortical U-fibres. CSF again showed low JC viral load. A stereotactic-guided biopsy of the left temporoparietal lesion was performed within a month of presentation, and repeated six months later because of the unusual findings, with essentially the same result. The patient has shown no clinical progression on history or examination since the biopsies. MRI has not been repeated."}