In This Issue May 2018 Volume 75, Number 5 JAMA Neurology Pages 525-636

Research Opinion

Amyloid and Tau Accumulation in Young Adults With ADAD 548 Viewpoint 531 Preventing Sudden Unexpected Although the medial temporal lobe is typically the first area of neurofibrillary tangle depo- Death in Epilepsy sition in aging populations, it is not clear if this is the case for younger individuals who are O Devinsky and Coauthors predisposed to autosomal dominant Alzheimer disease (ADAD). In a cross-sectional study, Quiroz and coauthors used positron emission tomography imaging to measure amyloid and Clinical Review & Education tau deposition in 24 participants (mean [SD] age, 38.0 [7.4] years) from a large Colombian Review kindred with ADAD. The authors report that amyloid accumulates in the cortex of unim- paired presenilin 1 E280A mutation carriers 10 to 15 years before symptom onset, whereas tau deposits emerge in the medial temporal lobe approximately 6 years before and then spread into the cortex as carriers move closer to clinical onset. These findings suggest that amyloid prompts the spread of tau pathology beyond the medial temporal lobe and that the presence of tau is closely associated with memory decline. Editorial perspective is pro- vided by McDade and Bateman. Editorial 536

Risk of Serotonin Syndrome With Triptans and Antidepressants 566 In 2006, the US Food and Drug Administration issued a warning regarding the potential risk of serotonin syndrome with coprescription of triptan antimigraine drugs and selective sero- 620 Review of the Neurological toninreuptakeinhibitororselectivenorepinephrinereuptakeinhibitorantidepressantsbased Implications of von Hippel–Lindau Disease D Dornbos III and Coauthors on a small number of case reports. In this population-based study using data from the Part- ners Research Data Registry,Orlova and coauthors identified 47 968 patients who had been JAMA Neurology Clinical Challenge prescribed triptans and 19 017 patients who had been prescribed antidepressants be- tween January 2001 and December 2014. A total of 17 patients had possible serotonin syn- drome, and only 2 patients received a confirmed diagnosis of serotonin syndrome. Affec- tive disorder can likely be treated safely in the patients who also need triptans to treat migraine and who are at a low risk for serotonin syndrome. This study also demonstrates that the frequency of concomitant use of these medications remained stable over the study period, illustrating a clear need to treat both conditions. Continuing Medical Education jamanetwork.com/learning

Developing a Myotonic Dystrophy Type 1 Prognosis Score 573 Life expectancy is greatly shortened in patients with myotonic dystrophy type 1, but there is no reliable prognostic risk score to predict survival. In this longitudinal cohort study from January 2000 to November 2014, Wahbi and coauthors studied 1296 adults (mean [SD] 628 What is your diagnosis? age, 39.8 [13.7] years) with myotonic dystrophy type 1 and developed and validated a prac- ᭿+ Online @ jamaneurology.com tical risk score that predicts 10-year survival based on a set of common patient character- istics, including age, diabetes, need for support when walking, heart rate, systolic blood pres- Peer Reviewers List sure, first-degree atrioventricular block, bundle-branch block, and lung vital capacity. The JAMA Neurology Peer Reviewers in 2017 score may prove useful for patients living with myotonic dystrophy type 1 and physicians.

Circadian Rest-Activity Pattern Changes and Preclinical AD 582 Disturbances of circadian rhythm are associated with both aging and neurodegenerative diseases, but it is not clear if circadian changes are present in the presymptomatic phase of Alzheimer disease (AD). In this cross-sectional study, Musiek and coauthors assessed cir- Departments cadian rest-activity patterns in 189 cognitively normal individuals (mean [SD] age, 66.6 [8.3] 526 Staff Listing years) relative to AD biomarkers. Preclinical AD was associated with fragmentation of cir- 542, 566 CME Articles cadian rest-activity patterns, even after correction for age and sex. These findings indicate 634 Classified Advertising that circadian disturbance occurs very early in AD, prior to any cognitive symptoms. 634 Journal Advertiser Index 635 Contact Information 636 CME Questions

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