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 Showing 1-20 of 43 Articles
Original Investigation 
Vladimir Coric, MD; Stephen Salloway, MD; Christopher H. van Dyck, MD; Bruno Dubois, MD; Niels Andreasen, MD, PhD; Mark Brody, MD; Craig Curtis, MD; Hilkka Soininen, MD; Stephen Thein, PhD; Thomas Shiovitz, MD; Gary Pilcher, PhD; Steven Ferris, PhD; Susan Colby, BA; Wendy Kerselaers, BA; Randy Dockens, PhD; Holly Soares, PhD; Stephen Kaplita, MSc; Feng Luo, PhD; Chahin Pachai, PhD; Luc Bracoud, MSc; Mark Mintun, MD; Joshua D. Grill, PhD; Ken Marek, MD; John Seibyl, MD; Jesse M. Cedarbaum, MD; Charles Albright, PhD; Howard H. Feldman, MD; Robert M. Berman, MD
Includes: Supplemental Content

Importance  Early identification of Alzheimer disease (AD) is important for clinical management and affords the opportunity to assess potential disease-modifying agents in clinical trials. To our knowledge, this is the first report of a randomized trial to prospectively enrich a study population with prodromal AD (PDAD) defined ...

Original Investigation 
Francoise Pousset, MD; Lise Legrand, MD; Marie-Lorraine Monin, MD; Claire Ewenczyk, MD; Perrine Charles, MD, PhD; Michel Komajda, MD; Alexis Brice, MD; Massimo Pandolfo, MD; Richard Isnard, MD, PhD; Sophie Tezenas du Montcel, MD, PhD; Alexandra Durr, MD, PhD
Includes: Supplemental Content

Importance  Friedreich ataxia (FRDA) is the most common genetic sensory ataxia, and myocardial involvement is a major determinant of survival.

Objective  To assess FRDA survival and cardiac outcome to adapt future therapeutic trials.

Design, Setting, and Participants  In a longitudinal follow-up study, all patients with ...

Original Investigation  FREE
Amy L. Jones, MD; Eoin P. Flanagan, MD; Sean J. Pittock, MD; Jay N. Mandrekar, PhD; Scott D. Eggers, MD; J. Eric Ahlskog, PhD, MD; Andrew McKeon, MD
Includes: Supplemental Content

Importance  Classic Purkinje cell cytoplasmic antibody type 1 (PCA-1, or anti-Yo) paraneoplastic cerebellar ataxia has a poor prognosis, yet little has been published otherwise regarding treatment responses and outcomes among patients with autoimmune cerebellar ataxia.

Objectives  To investigate treatment responses and outcomes in adults with autoimmune ...

Images in Neurology 
Jung E. Park, MD; Nora Vanegas-Arroyave, MD; Mark Hallett, MD; Codrin Lungu, MD

This case report describes a 27-year-old woman with a THAP1 mutation and cervical dystonia that responded well to deep brain stimulation of the globus pallidus internus.

Images in Neurology 
Eelco F. M. Wijdicks, MD, PhD

This case report describes very small cortical contusions that were identified after a seizure in a patient with head trauma.

Editorial  FREE
Roger N. Rosenberg, MD

This theme issue on “Circuits and Circuit Disorders: Approaches to Neuromodulation” is the result of a symposium held at the annual meeting of the American Neurological Association in Chicago, Illinois, on September 26, 2015, and cosponsored by JAMA Neurology and the Annals of Neurology. The speakers were ...

Original Investigation  FREE
Svjetlana Miocinovic, MD, PhD; Coralie de Hemptinne, PhD; Salman Qasim, BA; Jill L. Ostrem, MD; Philip A. Starr, MD, PhD
Includes: Supplemental Content

Importance  Isolated dystonia and Parkinson disease (PD) are disorders of the basal gangliothalamocortical network. They have largely distinct clinical profiles, but both disorders respond to deep brain stimulation (DBS) in the same subcortical targets using similar stimulation paradigms, suggesting pathophysiologic overlap. We hypothesized that, similar to PD, ...

Original Investigation 
Ki Sueng Choi, PhD; Patricio Riva-Posse, MD; Robert E. Gross, MD, PhD; Helen S. Mayberg, MD

Importance  The clinical utility of monitoring behavioral changes during intraoperative testing of subcallosal cingulate deep brain stimulation is unknown.

Objective  To characterize the structural connectivity correlates of deep brain stimulation–evoked behavioral effects using probabilistic tractography in depression.

Design, Setting, and Participants  Categorization of acute behavioral ...

Mahlon R. DeLong, MD; Thomas Wichmann, MD

Importance  The revival of stereotactic surgery for Parkinson disease (PD) in the 1990s, with pallidotomy and then with high-frequency deep brain stimulation (DBS), has led to a renaissance in functional surgery for movement and other neuropsychiatric disorders.

Objective  To examine the scientific foundations and rationale for ...

Clinical Implications of Basic Neuroscience Research 
Justin G. English, PhD; Bryan L. Roth, MD, PhD

Neurologic disorders are frequently a result of inappropriate electrical and/or chemical signaling of neurons and glia. Ultimate remediation would necessitate reprogramming these signals. Historically, correcting neuronal and glial signaling is accomplished via drug therapy/administration, although they frequently fail to effectively and fully treat the underlying disorder. Developments in ...

Seemant Chaturvedi, MD; Ralph L. Sacco, MD, MS

In the past 2 decades, 2 large multicenter trials have demonstrated the efficacy of carotid endarterectomy (CEA) for patients with asymptomatic carotid stenosis: the Asymptomatic Carotid Atherosclerosis Study (ACAS) and the Asymptomatic Carotid Surgery Trial (ACST).1,2 These trials found that CEA, compared with best medical ...

Original Investigation 
Catherine Yang, BHSc; Chrysi Bogiatzi, MD; J. David Spence, MD
Includes: Supplemental Content

Importance  Many patients with asymptomatic carotid stenosis are offered carotid stenting for the prevention of carotid occlusion. However, this treatment may be inappropriate if the risk of stroke is low at the time of occlusion and with intensive medical therapy.

Objectives  To determine the risk resulting ...

Editorial: Current Risks of Asymptomatic Carotid Stenosis; Seemant Chaturvedi, MD; Ralph L. Sacco, MD, MS
Original Investigation 
Renée F. A. G. de Bruijn, MD; Jan Heeringa, MD, PhD; Frank J. Wolters, MD; Oscar H. Franco, MD, PhD; Bruno H. C. Stricker, MD, PhD; Albert Hofman, MD, PhD; Peter J. Koudstaal, MD, PhD; M. Arfan Ikram, MD, PhD

Importance  Atrial fibrillation (AF) has been suggested as a risk factor for dementia since it may lead to chronic cerebral hypoperfusion and stroke. However, longitudinal studies assessing the association between AF and dementia have shown inconsistent results.

Objective  To determine the effect of AF on the ...

Bharath Wootla, PhD; Jens O. Watzlawik, PhD; Arthur E. Warrington, PhD; Nathan J. Wittenberg, PhD; Aleksandar Denic, MD, PhD; Xiaohua Xu, MD, PhD; Luke R. Jordan, BA; Louisa M. Papke, BS; Laurie J. Zoecklein, MS; Mabel L. Pierce; Sang-Hyun Oh, PhD; Orhun H. Kantarci, MD; Moses Rodriguez, MD

Importance  Modulating the immune system does not reverse long-term disability in neurologic disorders. Better neuroregenerative and neuroprotective treatment strategies are needed for neuroinflammatory and neurodegenerative diseases.

Objective  To review the role of monoclonal, naturally occurring antibodies (NAbs) as novel therapeutic molecules for treatment of neurologic disorders....

JAMA Neurology Clinical Challenge 
Lawrence S. Honig, MD, PhD

A woman in her 50s presented with a 1-month history of gait unsteadiness and imbalance, suspected to be due to neuropathy, but she then returned to the emergency department with 2 successive transient episodes, each lasting less than 1 hour, of acute dizziness, disorientation, gait dysfunction, and confusion. ...

Henrik Zetterberg, MD, PhD; Kaj Blennow, MD, PhD

Recent biomarker studies suggest that the pathological changes of Alzheimer disease (AD) start with Aβ deposition as early as 20 to 30 years prior to symptom onset,1 a window of time that holds promise for therapeutic intervention to reverse or slow down the disease. Aβ deposition is ...

Original Investigation 
Mahdi Ghani, MD, PhD; Christiane Reitz, MD, PhD; Rong Cheng, PhD; Badri Narayan Vardarajan, PhD; Gyungah Jun, PhD; Christine Sato, MSc; Adam Naj, PhD; Ruchita Rajbhandary, MPH; Li-San Wang, PhD; Otto Valladares, MS; Chiao-Feng Lin, PhD; Eric B. Larson, MD, MPH; Neill R. Graff-Radford, MD; Denis Evans, MD; Philip L. De Jager, MD, PhD; Paul K. Crane, MD, MPH; Joseph D. Buxbaum, PhD; Jill R. Murrell, PhD; Towfique Raj, PhD; Nilufer Ertekin-Taner, MD, PhD; Mark Logue, PhD; Clinton T. Baldwin, PhD; Robert C. Green, MD, MPH; Lisa L. Barnes, PhD; Laura B. Cantwell, MPH; M. Daniele Fallin, PhD; Rodney C. P. Go, PhD; Patrick A. Griffith, MD; Thomas O. Obisesan, MD; Jennifer J. Manly, PhD; Kathryn L. Lunetta, PhD; M. Ilyas Kamboh, PhD; Oscar L. Lopez, MD; David A. Bennett, MD; Hugh Hendrie, MB, ChB, DSc; Kathleen S. Hall, PhD; Alison M. Goate, PhD; Goldie S. Byrd, PhD; Walter A. Kukull, PhD; Tatiana M. Foroud, PhD; Jonathan L. Haines, PhD; Lindsay A. Farrer, PhD; Margaret A. Pericak-Vance, PhD; Joseph H. Lee, PhD; Gerard D. Schellenberg, PhD; Peter St. George-Hyslop, MD; Richard Mayeux, MD; Ekaterina Rogaeva, PhD; for the Alzheimer’s Disease Genetics Consortium
Includes: Supplemental Content

Importance  Mutations in known causal Alzheimer disease (AD) genes account for only 1% to 3% of patients and almost all are dominantly inherited. Recessive inheritance of complex phenotypes can be linked to long (>1-megabase [Mb]) runs of homozygosity (ROHs) detectable by single-nucleotide polymorphism (SNP) arrays.

Objective  ...

Original Investigation 
Maartje I. Kester, MD, PhD; Charlotte E. Teunissen, PhD; Daniel L. Crimmins, PhD; Elizabeth M. Herries, BA; Jack. H. Ladenson, PhD; Philip Scheltens, MD, PhD; Wiesje M. van der Flier, PhD; John C. Morris, MD; David M. Holtzman, MD; Anne M. Fagan, PhD

Importance  Neurogranin (NGRN) seems to be a promising novel cerebrospinal fluid (CSF) biomarker for synaptic loss; however, clinical, and especially longitudinal, data are sparse.

Objective  To examine the utility of NGRN, with repeated CSF sampling, for diagnosis, prognosis, and monitoring of Alzheimer disease (AD).

Design, Setting, ...

Editorial: Neurogranin Levels in Cerebrospinal Fluid; Henrik Zetterberg, MD, PhD; Kaj Blennow, MD, PhD
Original Investigation 
Joshua W. Miller, PhD; Danielle J. Harvey, PhD; Laurel A. Beckett, PhD; Ralph Green, MD, PhD; Sarah Tomaszewski Farias, PhD; Bruce R. Reed, PhD; John M. Olichney, MD; Dan M. Mungas, PhD; Charles DeCarli, MD
Includes: Supplemental Content

Importance  Vitamin D (VitD) deficiency is associated with brain structural abnormalities, cognitive decline, and incident dementia.

Objective  To assess associations between VitD status and trajectories of change in subdomains of cognitive function in a cohort of ethnically diverse older adults.

Design, Setting, and Participants  Longitudinal ...

Images in Neurology 
Lia D. Ernst, MD; Kenneth Brock, BA; Luis H. Barraza, MD; Adam Davis, MD; Melissa J. Nirenberg, MD, PhD

This case report describes a previously healthy 30-year-old man who developed limb paresthesias and incoordination. Initial magnetic resonance imaging was normal, but over the subsequent 8 weeks, he developed a bandlike sensation across the torso and profound sensory ataxia.

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