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In This Issue of JAMA Neurology
February 2017


JAMA Neurol. 2017;74(2):137. doi:10.1001/jamaneurol.2016.3979


Bongianni and colleagues develop an algorithm for accurate and early diagnosis of Creutzfeldt-Jakob disease (CJD) by using the real-time quaking-induced conversion (RT-QuIC) assay on cerebrospinal fluid (CSF) samples, olfactory mucosa (OM) samples, or both. Among the 86 patients (37 men [43%] and 49 women [57%]; mean [SD] age, 65.7 [11.5] years) included for analysis, all 61 patients with sporadic CJD had positive RT-QuIC findings using OM or CSF samples or both for an overall RT-QuIC diagnostic sensitivity of 100% (95% CI, 93%-100%). A proposed diagnostic algorithm for sporadic CJD combines CSF and OM RT-QuIC testing to provide virtually 100% diagnostic sensitivity and specificity in the clinical phase of the disease. Editorial perspective is provided by Paul Brown, MD.


CME and Author Audio Interview

Shahnawaz and coauthors develop a novel assay with high sensitivity and specificity to detect small quantities of α-synuclein (αSyn) aggregates circulating in cerebrospinal fluid (CSF) of patients with Parkinson disease (PD) and related synucleinopathies. Studies with synthetic αSyn aggregates showed that αSyn–protein misfolding cyclic amplification (PMCA) enabled the detection of as little as 0.1 pg/mL of αSyn oligomers. The findings suggest that detection of αSyn oligomers by αSyn-PMCA in the CSF of patients with PD may offer a good opportunity for a sensitive and specific biochemical diagnosis of the disease. Editorial perspective is provided by Tim Bartels, PhD.


Fokkink and colleagues assess whether there is an association between serum albumin levels, a widely used and relatively easily measurable biomarker of health and inflammation, and the clinical course and outcome of Guillain-Barré syndrome (GBS) in patients treated with intravenous immunoglobulin (IVIG). Hypoalbuminemia was associated with an increased chance of respiratory failure before (16 [36.4%] of 44, P = .001) or after (29 [54.7%] of 53, P < .001) IVIG treatment, inability to walk unaided (21 [35.0%] of 60 vs 6 [5.3%] of 114, P < .001), and severe muscle weakness at 4 weeks (Medical Research Council sum score, 31.8 vs 52.9, P < .001) and 6 months (Medical Research Council sum score, 49.4 vs 58.4, P < .001). Patients with GBS may develop hypoalbuminemia after treatment with IVIG, which is related to a more severe clinical course and a poorer outcome. Editorial perspective is provided by Richard A. C. Hughes, MD, FRCP, FMedSci, FEAN.


Zissimopoulos et al analyze the association between statin exposure and Alzheimer disease (AD) incidence among Medicare beneficiaries. High exposure to statins was associated with a lower risk of AD diagnosis for women (hazard ratio [HR], 0.85; 95% CI, 0.82-0.89; P<.001) and men (HR, 0.88; 95% CI, 0.83-0.93; P<.001). The reduction in AD risk varied across statin molecules, sex, and race/ethnicity.