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 Showing 21-40 of 67 Articles
Elizabeth C. Mormino, PhD; Kathryn V. Papp, PhD

In 2011, a National Institute on Aging−Alzheimer’s Association (NIA-AA) work group published criteria for classifying clinically normal older individuals into biomarker-defined stages of preclinical Alzheimer disease using measures of Aβ and neurodegeneration (ND).1 These staging criteria assume a trajectory that begins with aberrant Aβ accumulation, followed by ...

Original Investigation 
Mohammad A. Khoshnoodi, MD; Shaun Truelove, BA; Ahmet Burakgazi, MD; Ahmet Hoke, MD, PhD; Andrew L. Mammen, MD, PhD; Michael Polydefkis, MD, MHS
Includes: Supplemental Content

Importance  Few data are available on the natural history of small fiber neuropathy (SNF). Peripheral neuropathy typically follows a length-dependent pattern, leading us to hypothesize that patients with SFN would lose intraepidermal nerve fibers at the distal leg more quickly than at more proximal thigh sites.


Editorial: Understanding Small Fiber Neuropathy; John T. Kissel, MD; A. Gordon Smith, MD
Original Investigation 
Eugenia Martinez-Hernandez, MD, PhD; Helena Ariño, MD; Andrew McKeon, MD; Takahiro Iizuka, MD; Maarten J. Titulaer, MD, PhD; Mateus M. Simabukuro, MD; Eric Lancaster, MD, PhD; Mar Petit-Pedrol, MS; Jesús Planagumà, PhD; Yolanda Blanco, MD, PhD; Robert J. Harvey, BSc, PhD; Albert Saiz, MD, PhD; Francesc Graus, MD, PhD; Josep Dalmau, MD, PhD
Includes: Supplemental Content

Importance  Symptoms of stiff-person syndrome (SPS), stiff-limb syndrome (SLS), or progressive encephalomyelitis with rigidity, myoclonus, or other symptoms (SPS-plus) can occur with several autoantibodies, but the relative frequency of each antibody, syndrome specificity, and prognostic implications are unclear.

Objective  To report the clinical and immunologic findings ...

Original Investigation 
Anja Soldan, PhD; Corinne Pettigrew, PhD; Qing Cai, MS; Mei-Cheng Wang, PhD; Abhay R. Moghekar, MBBS; Richard J. O’Brien, MD, PhD; Ola A. Selnes, PhD; Marilyn S. Albert, PhD; for the BIOCARD Research Team
Includes: Supplemental Content

Importance  Clinical trials testing treatments for Alzheimer disease (AD) are increasingly focused on cognitively normal individuals in the preclinical phase of the disease. To optimize observing a treatment effect, such trials need to enroll cognitively normal individuals likely to show cognitive decline over the duration of the ...

Editorial: Cognitive Decline in Preclinical Stage 2 Alzheimer Disease; Elizabeth C. Mormino, PhD; Kathryn V. Papp, PhD
JAMA Neurology Clinical Challenge 
Matthias Hoheisel, MD; Matthias Sturzenegger, MD; Niklaus Meier, MD

A 24-year-old woman had a strong unilateral headache located in her left cheek, eye, and forehead that evolved during the evening hours, peaked at night, and decreased again until the morning, without any remarkable trigger along with ipsilateral lacrimation, a red eye, and periorbital swelling but without ptosis. ...

Comment & Response 
Mohamed Y. Rady, BChir, MB(Cantab), MA, MD(Cantab); Joseph L. Verheijde, PhD, MBA, PT

To the Editor Greer et al1 analyzed compliance of hospital protocols on brain death (BD) determination with the 2010 American Academy of Neurology (AAN) guidelines. They posited that this neurologic standard is 100% accurate. We comment on the accuracy claim.

Comment & Response 
David M. Greer, MD, MA; Panayiotis N. Varelas, MD, PhD; Eelco F. M. Wijdicks, MD, PhD

In Reply Rady and Verheijde appear to have missed the point of our article1 or otherwise have chosen to use it as a platform to criticize the American Academy of Neurology Practice Parameters (AANPPs). Our study was designed to look at variability of US hospital policies in ...

Guy Helman, BS; Joshua L. Bonkowsky, MD, PhD; Adeline Vanderver, MD

This Viewpoint assesses the frequency of next-generation sequencing use among neurologists and identifies barriers in its implementation.

Daniel B. Drachman, MD

Come to me, come! New lamps for old!
I'm a Magician from No-One-Knows-Where.
Here are more lamps than my hands can hold,
Bright new lamps of copper and gold,
Full of smooth oil, with a soft and fair
Little flame that throws its beams to the sky.
I'll ...

Reinhard Hohlfeld, MD; Tania Kümpfel, MD

Alemtuzumab is a humanized monoclonal antibody that binds to CD52, a surface molecule expressed by many types of immune cells, including T lymphocytes, B lymphocytes, monocytes, macrophages, and eosinophils.13 After intravenous infusion, the therapeutic antibody binds to CD52-expressing cells in blood and to a lesser ...

David Pleasure, MD

In this issue of JAMA Neurology, Cornett et al1 analyze clinical disability in 520 children and adolescents aged 3 to 20 years with Charcot-Marie-Tooth disease (CMT). Approximately half of the cohort had CMT type 1A (CMT1A), with the remainder distributed roughly equally between rarer forms of ...

Original Investigation 
Adam Bryant, MD; Harold Atkins, MD, FRCPC; C. Elizabeth Pringle, MD; David Allan, MD, MSc; Grizel Anstee, MD; Isabelle Bence-Bruckler, MD, FRCPC; Linda Hamelin, MScN; Michael Hodgins, MD; Harry Hopkins, RPh, FCSHP; Lothar Huebsch, MD, FRCPC; Sheryl McDiarmid, MBA; Mitchell Sabloff, MD, FRCPC; Dawn Sheppard, MD, MSc, FRCPC; Jason Tay, MD, MSc, FRCPC; Christopher Bredeson, MD, MSc, FRCPC
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Importance  Some patients with myasthenia gravis (MG) do not respond to conventional treatment and have severe or life-threatening symptoms. Alternate and emerging therapies have not yet proved consistently or durably effective. Autologous hematopoietic stem cell transplant (HSCT) has been effective in treating other severe autoimmune neurologic conditions ...

Editorial: New Lymphs for Old; Daniel B. Drachman, MD
Original Investigation 
Kayla MD Cornett, BHK, MSc; Manoj P Menezes, MBBS, FRACP, PhD; Paula Bray, PhD; Mark Halaki, PhD; Rosemary R. Shy, MD; Sabrina W. Yum, MD; Timothy Estilow, OTR/L; Isabella Moroni, MD; Maria Foscan, PT; Emanuela Pagliano, MD; Davide Pareyson, MD; Matilde Laurá, MD, PhD; Trupti Bhandari, PT; Francesco Muntoni, MD, FRCPCH, FMed Sci; Mary M. Reilly, MD, FRCP; Richard S. Finkel, MD; Janet Sowden; Katy J. Eichinger, PT, DPT, NCS; David N. Herrmann, MBBCh; Michael E. Shy, MD; Joshua Burns, PhD; for the Inherited Neuropathies Consortium
Includes: Supplemental Content

Importance  Disease severity of childhood Charcot-Marie-Tooth disease (CMT) has not been extensively characterized, either within or between types of CMT to date.

Objective  To assess the variability of disease severity in a large cohort of children and adolescents with CMT.

Design, Setting, and Participants  A ...

Horst Penkert, MD; Claire Delbridge, MD; Nina Wantia, MD; Benedikt Wiestler, MD; Thomas Korn, MD

This case report describes a case of fulminant central nervous system nocardiosis at 5 months after initiation of alemtuzumab in a patient with multiple sclerosis.

Editorial: Alemtuzumab and Multiple Sclerosis; Reinhard Hohlfeld, MD; Tania Kümpfel, MD

To the Editor Rosebud and colleagues1 reported on a longitudinal study strengthening the idea that olfactory impairment has potential to be a biomarker for diagnosing mild cognitive impairment (MCI) and Alzheimer disease (AD) or early detection for cognitively normal elderly individuals who would likely progress to MCI ...

Steven T. DeKosky, MD; Todd Golde, MD, PhD

Biomarkers as aids to diagnosis, prognosis, and effective therapies have become holy grails of translational neuroscience. In neurodegenerative disorders, especially Alzheimer disease (AD), imaging and cerebrospinal fluid (CSF) biomarkers for the amyloid-β (Aβ) protein and tau have found their place in research and clinical trials, enabling much more ...

Traumatic brain injury (TBI) is an important critical neurologic illness that requires neurocritical care. Traumatic brain injury is an active disease process in which primarily injured tissue manifests as brain hemorrhage and subsequent edema, which can lead to elevated intracranial pressure (ICP). For many years, secondary brain ischemia ...

Tanya Bogoslovsky, MD, PhD; Ramon Diaz-Arrastia, MD, PhD

Biomarkers are molecules that can be measured in accessible biological fluids that reflect physiological, pharmacological, or disease processes and can suggest the etiology of, susceptibility to, activity levels of, or progress of a disease. Biomarkers have historically been critical to patient progress in a broad range of clinical ...

Original Investigation 
Linda Papa, MDCM, MSc; Gretchen M. Brophy, PharmD; Robert D. Welch, MD, MS; Lawrence M. Lewis, MD; Carolina F. Braga, BA; Ciara N. Tan, BS, MHSH; Neema J. Ameli, BS; Marco A. Lopez, AS; Crystal A. Haeussler, BS; Diego I. Mendez Giordano, BS; Salvatore Silvestri, MD; Philip Giordano, MD; Kurt D. Weber, MD; Crystal Hill-Pryor, PhD; Dallas C. Hack, MD, MPH
Includes: Supplemental Content

Importance  Glial fibrillary acidic protein (GFAP) and ubiquitin C-terminal hydrolase L1 (UCH-L1) have been widely studied and show promise for clinical usefulness in suspected traumatic brain injury (TBI) and concussion. Understanding their diagnostic accuracy over time will help translate them into clinical practice.

Objectives  To evaluate ...

Editorial: Temporal Profiles of Neuronal and Axonal Damage After Mild TBI; Tanya Bogoslovsky, MD, PhD; Ramon Diaz-Arrastia, MD, PhD
Original Investigation 
Rawan Tarawneh, MD; Gina D’Angelo, PhD; Dan Crimmins, PhD; Elizabeth Herries, BA; Terry Griest, BS; Anne M. Fagan, PhD; Gregory J. Zipfel, MD; Jack H. Ladenson, PhD; John C. Morris, MD; David M. Holtzman, MD
Includes: Supplemental Content

Importance  Synaptic loss is an early pathologic substrate of Alzheimer disease (AD). Neurogranin is a postsynaptic neuronal protein that has demonstrated utility as a cerebrospinal fluid (CSF) marker of synaptic loss in AD.

Objective  To investigate the diagnostic and prognostic utility of CSF neurogranin levels in ...

Editorial: Cerebrospinal Biomarkers in Alzheimer Disease; Steven T. DeKosky, MD; Todd Golde, MD, PhD

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