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Powassan virus (POWV) is a tickborne flavivirus that causes encephalitis with severe neurologic sequelae (1). In the United States, POWV infections occur primarily in the Northeast and Great Lakes regions [...]

Background and Objectives: Creatine kinase (CK) is a commonly used screening test for neuromuscular disorders (NMDs). However, hyperCKemia can result from several pathologic and physiologic causes. We analyzed neuromuscular disorders in noninfant children with hyperCKemia including those with no weakness and mild CK elevations (<5 times the upper limit of normal). We hypothesized that children with mild CK elevation and no weakness would be unlikely to have neuromuscular disorders and require additional evaluation. Methods: We retrospectively evaluated patients between 1 and 18 years of age seen at a single children's hospital over a 3-calendar-year period with initial total CK values greater than the upper limit of normal with at least 2 years of follow-up data. Final diagnoses were analyzed and associations with possible risk factors assessed. Receiver operating characteristic curves were generated to assess altering CK cutoff values. Results: Of 260 subjects with hyperCKemia, 18 had a neuromuscular disorder (6.9%, 95% confidence interval [CI] 4.2%-10.9%). Of 166 subjects with CK <5 times the upper limit of normal and no weakness, 8 had a neuromuscular disorder (4.8%, 95% CI 2.3%-9.6%). Weakness (odds ratio [OR] 32.5, 95% CI 4-385, P =.0002), and family history of neuromuscular disorders (OR not calculable, P =.0003) were associated with neuromuscular disorders. An optimal CK threshold of 777 was identified on receiver operating characteristic curve analysis (sensitivity of 72% and specificity of 64%). The most commonly identified neuromuscular disorders were muscular dystrophies, inflammatory myopathies, and metabolic myopathies. Conclusion: Most children with hyperCKemia will not be diagnosed with a neuromuscular disorder, but a significant minority even with mild hyperCKemia and without weakness may warrant additional evaluation. [ABSTRACT FROM AUTHOR]

Introduction/Aims: The purpose of this literature review is to develop an evidence‐based guideline for the use of neuromuscular ultrasound in the diagnosis of ulnar neuropathy at the elbow (UNE). The proposed research question was: "In patients with suspected UNE, does ulnar nerve enlargement as measured with ultrasound accurately identify those patients with UNE?" Methods: A systematic review and meta‐analysis was performed, and studies were classified according to American Academy of Neurology criteria for rating articles for diagnostic accuracy. Results: Based on Class I evidence in four studies, it is probable that neuromuscular ultrasound measurement of the ulnar nerve at the elbow, either of diameter or cross‐sectional area (CSA), is accurate for the diagnosis of UNE. Recommendation For patients with symptoms and signs suggestive of ulnar neuropathy, clinicians should offer ultrasonographic measurement of ulnar nerve cross‐sectional area or diameter to confirm the diagnosis and localize the site of compression (Level B). [ABSTRACT FROM AUTHOR]

Background: Peripheral polyneuropathies (PN) are common neuromuscular conditions. The role of electrodiagnostic study (EDX) in diagnosis of PN is not well-defined.Methods: We performed a retrospective chart review of patients referred for EDX evaluation of PN.Results: Of 162 patients analyzed, 23 had pure peripheral neuropathy (pPN; 14.2%), 29 had peripheral neuropathy and another diagnosis (PN+; 17.9%), 51 had an alternative diagnosis (nonPN; 31.5%), and 59 had normal studies (36.4%). In univariable analysis, age (P < .001) and gender (P = .004) were weakly associated with final diagnosis. In multinomial logistic regression analysis, significant predictors included age (odds ratio [OR] for nonPN/PN+:1.07 per year; 95% confidence interval [CI], 1.03-1.11), gender (OR for PN+:0.2, 95% CI, 0.07-0.61), and diabetes/prediabetes (OR for pPN:3.29; 95% CI, 1.17-9.27).Conclusions: These data suggest that EDX commonly yields additional or nonPNs in patients referred with a diagnosis of PN, and although some variables predict electrodiagnosis, none have a large enough effect to suggest poor utility in any subpopulation. [ABSTRACT FROM AUTHOR]

Pediatric-onset multiple sclerosis (POMS), once thought to be rare, is now being diagnosed in increasing numbers in children. Despite improvements to diagnostic criteria, the diagnosis and management of POMS remains challenging. The aim of this study is to retrospectively describe a growing POMS patient population seen at a single center over a 13 year period. Epidemiologic, clinical, neuroimaging, laboratory features and therapeutic management and outcome data were collected and analyzed. These data support associations between MS and environmental triggers such as obesity and vitamin D deficiency. Presenting symptoms, magnetic resonance imaging and laboratory findings were consistent with the existing literature; however, the prevalence of cortical lesions and abnormal saccadic pursuit is higher than other reports. Data also demonstrate a shift in practice from first- to second-line therapies over the observed period. [ABSTRACT FROM AUTHOR]