AbstractObjectiveThis study aimed to evaluate the diagnostic accuracy of dried blood spot (DBS) compared with conventional serum Aquaporin‐4‐IgG (AQP4‐IgG) testing.MethodsProspective multicenter diagnostic study was conducted between April 2018 and October 2023 across medical centers in the United States, Uganda, and the Republic of Guinea. Neuromyelitis optica spectrum disorder (NMOSD) patients and controls collected blood on filter paper cards along with concurrent serum samples. These samples underwent analysis using flow cytometric live‐cell‐based assays (CBA) and enzyme‐linked immunosorbent assay (ELISA) to determine AQP4 serostatus. The accuracy of AQP4‐IgG detection between DBS and serum (gold standard) was compared.ResultsAmong 150 participants (47 cases, 103 controls), there was a strong correlation between DBS and serum samples (Spearman's correlation coefficient of 0.82). The AUC was 0.97 (95% CI: 0.92–0.99). AQP4‐IgG detection through DBS showed 87.0% sensitivity (95% CI: 0.74–0.95) and 100% specificity (95% CI: 0.96–1.00) using CBA, and 65.2% sensitivity (95% CI: 0.43–0.84) and 95.2% specificity (95% CI: 0.76–0.99) using ELISA. Serum ELISA demonstrated 69.6% sensitivity (95% CI: 0.47–0.87) and 98.4% specificity (95% CI: 0.91–0.99). The stability of DBS in detecting AQP4‐IgG persisted over 24 months for most cases.InterpretationThe DBS represents a viable alternative for detecting AQP4‐IgG in resource‐limited settings to diagnose NMOSD, offering high sensitivity and specificity comparable to serum testing. Moreover, DBS has low shipping costs, is easy to administer, and is suitable for point‐of‐care testing.
High risk of postpartum relapses in neuromyelitis optica spectrum disorder
Background and purposeThe objective of our study was to identify neurological factors associated with poor outcome in adult patients with fulminant bacterial meningitis.MethodsThis was a retrospective review of consecutive adult patients with fulminant bacterial meningitis, defined as meningitis causing coma within 24–48 h of hospitalization, at Mayo Clinic Rochester between January 2000 and November 2010. Functional status was assessed at discharge and upon last follow‐up using the modified Rankin scale (mRS). The primary end‐point was death or new major disability (increase of >2 on the mRS) at last follow‐up.ResultsThirty‐nine patients were identified. Encephalopathy (44%), coma (28%), focal seizures (3%) or a combination of these (26%) were present on admission. The most common pathogen was Streptococcus pneumoniae (57%). All patients were treated with broad spectrum antibiotics and 51% received steroids. Serious systemic complications were seen in 23 patients. Sixteen patients (41%) died during hospitalization. Median mRS at hospital discharge for surviving patients was 3; four patients had new major disability with a mean follow‐up of 11 months. Predictors of death or new major disability included lower Glasgow Coma Scale score at nadir [P = 0.002; age‐ and sex‐adjusted odds ratio (OR) 0.46, 95% confidence interval (CI) 0.28–0.48], longer duration of symptoms before hospitalization (P = 0.045; adjusted OR 2.34, 95% CI 1.02–5.37), abnormal head imaging at presentation (P = 0.008; adjusted OR 9.40, 95% CI 1.78−49.6) and use of intracranial pressure monitoring (P = 0.010, adjusted OR 51.0, 95% CI 2.51−1036).ConclusionMany adult patients who survive hospitalization are able to regain their pre‐morbid level of function. Aggressive management of bacterial meningitis is justified even in comatose adult patients.
Long-term cognitive outcomes following out-of-hospital cardiac arrest
Extraosseous Ewing's sarcoma (EES) involving the central nervous system is rare, but can be diagnosed and distinguished from other primitive neuroectodermal tumors (PNET) by identification of the chromosomal translocation (11;22)(q24;q12). We report EES arising from the spinal intradural extramedullary space, based on imaging, histopathological, and molecular data in two men, ages 50 and 60 years old and a review of the literature using PubMed (1970–2009). Reverse transcriptase polymerase chain reaction (RT-PCR) identified the fusion product FL1-EWS. Multimodal therapy, including radiation and alternating chemotherapy including vincristine, cyclophosphamide, doxorubicin and ifosfamide and etoposide led to local tumor control and an initial, favorable therapeutic response. No systemic involvement was seen from the time of diagnosis to the time of last follow-up (26 months) or death (4 years). This report confirms that EES is not confined to the earliest decades of life, and like its rare occurrence as an extra-axial meningeal based mass intracranially, can occasionally present as an intradural mass in the spinal canal without evidence of systemic tumor. Gross total resection followed by multimodal therapy may provide for extended progression free and overall survival.
“Pinheads”: The exhibition of neurologic disorders at “The Greatest Show on Earth”