Browsing by Issue Date, starting with "2024-12"
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- An Unusual Case of Overlapping Immunoglobulin G4-Related Disease and Systemic Lupus Erythematosus.Publication . Burillo Simões, Pilar; Martins, João; Menezes, Maria do Mar; Sousa, João; Jorge, CristinaImmunoglobulin G4-related disease (IgG4-RD) and systemic lupus erythematosus (SLE) are multisystemic autoimmune disorders that can present with renal manifestations. Overlapping cases of these diseases are extremely rare and present both diagnostic and therapeutic challenges. We report the case of a 70-year-old male with a history of autoimmune pancreatitis, who was admitted with fatigue, weight loss, and worsening kidney function. Laboratory tests revealed anemia with a positive Coombs test, leucopenia, elevated IgG4, hypocomplementemia, and positive results for ANA, anti-double-stranded DNA (dsDNA), anti-nucleosome, anti-RP11 antibodies, and rheumatoid factor. A spot urine sample showed subnephrotic proteinuria without hematuria. The patient met the criteria for both SLE and possible IgG4-RD, but the cause of the worsening renal function remained unclear, prompting a kidney biopsy. The biopsy revealed a lymphoplasmacytic infiltrate, storiform fibrosis, and IgG4-positive staining, consistent with IgG4-related tubulointerstitial nephritis, but without evidence of lupus nephritis. The patient was treated with prednisolone, resulting in improvement of both his symptoms and kidney function. However, significant leukopenia, anemia, and elevated anti-dsDNA titers persisted, which were presumed to be secondary to the overlapping SLE. Hydroxychloroquine and azathioprine were added to the treatment regimen, leading to improvement in cytopenias at the three-month follow-up. This case underscores the importance of kidney biopsy in suspected overlapping autoimmune diseases for identifying kidney involvement and guiding treatment, although evidence regarding optimal therapy remains limited.
- Multidisciplinary Approaches and Molecular Diagnostics in New-Onset Refractory Status Epilepticus and Nontuberculous Mycobacterial CNS Infections in the ICU: A Case Report.Publication . Batarda Sena, Pedro Manuel; Ferro, Margarida; Alves Cabrita, Joana; Pontinha, Carlos; Mestre, Ana; Oliveira, Mário; Bento, LuisThe diagnosis and management of complex neurological conditions such as New-Onset Refractory Status Epilepticus (NORSE) and central nervous system (CNS) infections caused by non-tuberculous mycobacteria (NTM) pose substantial difficulties in intensive care units (ICUs). This article combines a case report and a literature review that explores the diagnostic dilemmas and therapeutic strategies for these critical conditions. We report the case of an 83-year-old female with chronic granulomatous meningitis secondary to NTM, presenting a challenging diagnostic and complex management complexity typical of such a rare disease through a period time of five years. Her case emphasized the importance of a multidisciplinary approach in such cases, as the interplay between neurology and intensive care was vital. The need for new molecular diagnostic technologies is shown to be of high significance in identifying the causative pathogens and improving patient outcomes in these rare but critical conditions.
- Acute Symptomatic Seizures in Patients with Recurrent Ischemic Stroke: A Multicentric Study.Publication . Leal Rato, Miguel; Schön, Miguel; Zafra, Maria Paula; Aguiar de Sousa, Diana; Pinho E Melo, Teresa; Franco, Ana Catarina; Peralta, Ana Rita; Ferreira-Atuesta, Carolina; Mayor-Romero, Luis Carlos; Rouhl, Rob P W; Bentes, CarlaObjective: Epileptic seizures occur frequently after stroke due to changes in brain function and structure, and up to around 10% of stroke patients experience stroke recurrence in the first year. We aimed to establish the risk of acute symptomatic seizures in patients with recurrent stroke. Methods: Retrospective cohort study including consecutive admissions to a Stroke Unit due to acute ischemic stroke, during a 5-year period. Additional inclusion of patients admitted to two centers in different countries to corroborate findings (confirmatory cohort). We aimed to compare acute symptomatic seizure incidence in patients with and without previous stroke. Patients with history of epilepsy were excluded. Logistic regression modeling was performed to identify predictors in middle cerebral artery (MCA) stroke. Results: We included 1473 patients (1085 with MCA stroke), of which 117 had a recurrent ischemic stroke (84 with MCA stroke). Patients with recurrent stroke had a seizure risk during hospital stay similar to that of patients with a first-ever stroke (5.1% vs. 4.5%, OR 1.15, 95% CI .48-2.71, p = .75). Risk of acute symptomatic seizures was also similar (5.0% vs. 4.1, OR 1.22, 95% CI .29-5.27, p = .78). Older age, female sex, and hemorrhagic transformation were predictors of seizures in patients with a first MCA ischemic stroke, but not in recurrent stroke patients. Electrographic characteristics were similar between the two groups in patients who had an electroencephalogram (46 with first stroke, 5 with recurrent stroke). The low rate of seizures (1.5%) in the confirmatory cohort (n = 198) precluded full comparison with the initial cohort. Nevertheless, the rate of seizures was not higher in stroke recurrence. Significance: History of previous stroke was not associated with an increased risk of acute symptomatic seizures during hospital stay. Larger, prospective studies, with prospective electrophysiological evaluation, are needed to explore the impact of stroke recurrence on seizure risk.