In This Issue June 2015 Volume 72, Number 6 JAMA Neurology Pages 613-732 Research Opinion Antibodies to Clustered AChRs and MG 642 Viewpoint Rodríguez Cruz and colleagues determine the diagnostic 623 Neurology and the Aff ordable Care Act usefulness of cell-based assays (CBAs) in the diagno- D Gold and Coauthors sis of myasthenia gravis (MG) and compare the clinical 624 Political Correctness of Medical features of patients with antibodies only to clustered Documentation acetylcholine receptors (AChRs) with those of patients JR Berger with seronegative MG. Radioimmunoprecipitation as- Editorial say (RIPA) and CBA were used to test for standard AChR 626 The Human Alzheimer Disease antibodies and antibodies to clustered AChRs in 138 patients. Cell-based assay is a use- Project: A New Call to Arms ful procedure in the routine diagnosis of RIPA-negative MG, particularly in children, and RN Rosenberg ad RC Petersen patients with antibodies only to clustered AChRs appear to be younger and have milder 628 Cognition and Quality-of-Life disease than other patients with MG. Editorial perspective in support of these data is pro- Outcomes in the Targeted Temperature vided by Steven Vernino, MD, PhD. Management Trial for Cardiac Arrest V Aiyagari and MN Diringer Editorial 630 630 Unraveling the Enigma of Continuing Medical Education jamanetworkcme.com Seronegative Myasthenia Gravis S Vernino Biogenesis and Myogenesis in SMA 666 631 Status Epilepticus AC Jongeling and Coauthors Ripolone and coauthors investigate mitochondrial dys- function in a large series of muscle biopsy samples from Clinical Review & Education patients with spinal muscular atrophy (SMA). They stud- ied quadriceps muscle samples from 24 patients with Images in Neurology genetically documented SMA and paraspinal muscle samples from 3 patients with SMA-II undergoing surgery or scoliosis correction. Their results strongly support the conclusion that an altered regulation of myogenesis and a downregulated mitochondrial biogenesis contribute to pathologic change in the muscle of patients with SMA. ACTA1 Mutation in Scapuloperoneal Myopathy 689 Zukosky et al determine the genetic cause of a slow- ly progressive, autosomal dominant, scapuloperoneal neuromuscular disorder by using linkage and exome sequencing. Fourteen aff ected individuals in a 6-gener- ation family with a progressive scapuloperoneal disorder 720 Basilar Artery Aneurysm in a Woman With Syphilis were evaluated. This family defi nes a new scapulopero- neal phenotype associated with an ACTA1 mutation. Clinical Review & Education Brain Stimulation for Torsion Dystonia 713 Fox and Alterman review the evidence and eff ect sizes for Departments treating diff erent types of dystonia with diff erent types 620 Staff Listing of brain stimulation and discuss recent advances relevant 634, 642 CME Articles to patient selection, surgical approach, programming, 730 Classifi ed Advertising and mechanism of action. They report that strong (level 730 Journal Advertiser Index B) evidence supports the use of deep brain stimulation 731 Contact Information (DBS) for the treatment of primary generalized or seg- 732 CME Questions mental dystonia, especially when due to mutation in the Instructions for Authors DYT1 gene, as well as for patients with cervical dystonia. Patients with dystonia that is not www.jamaneurology.com/public adequately controlled with standard medical therapy should be referred for consideration /instructionsforauthors.aspx of DBS, especially patients with generalized, segmental, or cervical dystonia. jamaneurology.com JAMA Neurology June 2015 Volume 72, Number 6 615 Copyright 2015 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/01/2021.