Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating central nervous system illness encountered in the setting of immunosuppressive conditions like human immunodeficiency virus / acquired immunodeficiency syndrome, autoimmune diseases and hematologic malignancies. We had a 54-year-old woman with systemic lupus erythematosus and coexisting autoimmune hepatitis who presented with progressive cognitive decline, right hemiparesis and ataxia who was found to have PML. She had severe CD4 lymphopenia. She was managed with low-dose prednisolone and plasma exchange after which she showed significant clinical improvement. This case highlights the diagnostic and therapeutic challenges encountered in managing a case of PML in the setting of autoimmune conditions with profound lymphopenia.
Background and purpose <break> <break>The benefit of endovascular treatment in large anterior circulation ischemic strokes with low ASPECTS score(<6) is uncertain. Recent studies have demonstrated the benefit of endovascular treatment (EVT) in large ischemic strokes. The present meta-analysis aims to assess the combined effect of these studies on efficacy and safety of endovascular treatment in this group of patients.<break> <break>Materials and Methods<break> <break>We conducted a systematic review and meta-analysis according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. Databases MEDLINE, PubMed, EMBASE, SCOPUS, Google Scholar, Tripdatabase were searched for randomised controlled trials with at least 50 participants. The primary efficacy outcome analysed was the relative risk of functional independence defined as mRS- 0-2 at 90 days. Secondary efficacy outcomes included early neurological improvement, death due to any cause at 90 days and proportion of patients requiring decompressive hemicraniectomy. The primary safety outcome was the risk of developing symptomatic intracerebral haemorrhage.<break> <break>Results <break> <break>A total of 3 studies( RESCUE Japan-LIMIT,SELECT 2 and ANGEL ASPECTS) involving 1011 patients ; 510 in the EVT arm and 501 in the medical management (MM) arm met the defined criteria (ASPECTS-3-5). The combined RR for the primary outcome of mRS 0-2 was 2.53 [1.84-3.47] (p=<0.0001) favouring EVT over MM. The primary safety outcome of symptomatic intracerebral haemorrhage was not significant in the EVT arm with a combined RR of 1.84 [0.94-3.60] (p=0.5157). Mortality rates were similar in both arms (26.67% in EVT arm vs 27.94% in MM arm) with a combined RR of 0.95 [0.78; 1.16] (p=1.000). <break> <break>Conclusion<break>In patients with LVO and low ASPECTS (3-5), EVT was associated with higher likelihood of achieving functional independence and early neurologic improvement but did not provide any mortality benefit. The rates of symptomatic ICH were similar in both the groups whereas the risk of any ICH was significantly higher in the EVT arm.
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