Medicine and Health Sciences Commons^™

Editorial: Sudden Unexpected Death In Epilepsy: Bio-Markers, Mechanisms, Risk Identification And Prevention., Rainer Surges, Michael R. Sperling, Christopher M. Degiorgio

Department of Neurology Faculty Papers

No abstract provided.

Markers Of Bone And Lipid Metabolism With Eslicarbazepine Acetate Monotherapy., Scott Mintzer, Tawnya Constantino, Barry Gidal, Parul Bhargava, Todd Grinnell, David Blum

Department of Neurology Faculty Papers

OBJECTIVE: To evaluate the impact of eslicarbazepine acetate (ESL) monotherapy on markers of bone and lipid metabolism.

METHODS: We conducted a post-hoc analysis of data pooled from two Phase III, dose-blind, conversion-to-ESL (1600 mg and 1200 mg) monotherapy studies in patients with focal seizures. Laboratory measurements included lipids (total cholesterol [TC]; high-density lipoprotein cholesterol [HDL-C]; low-density lipoprotein cholesterol; and triglycerides) and markers of bone metabolism (alkaline phosphatase; 25-hydroxyvitamin D; osteocalcin; and parathyroid hormone [PTH]); measurements were taken at baseline, Week 18, and Month 12, and analyzed according to enzyme-inducing antiepileptic drugs (EIAEDs) use at baseline (+EIAED and -EIAED subgroups).

RESULTS: …

Case 22-2019: A 65-Year-Old Woman With Myopathy., Marinos C. Dalakas

Department of Neurology Faculty Papers

No abstract provided.

Association Of Β-Amyloid Burden With Sleep Dysfunction And Cognitive Impairment In Elderly Individuals With Cognitive Disorders., Jason C. You, Erica Jones, Devon E. Cross, Abigail C. Lyon, Hyunseung Kang, Andrew B. Newberg, Carol F. Lippa

Department of Neurology Faculty Papers

Importance: Evidence shows that sleep dysfunction and β-amyloid (Aβ) deposition work synergistically to impair brain function in individuals with normal cognition, increasing the risk of developing dementia later in life. However, whether Aβ continues to play an integral role in sleep dysfunction after the onset of cognitive decline in individuals with dementia is unclear.

Objective: To determine whether Aβ deposition in the brain is associated with subjective measures of sleep quality and cognition in elderly individuals with cognitive disorders.

Design, Setting, and Participants: A nested survey study was conducted at the Cognitive Disorders and Comprehensive Alzheimer Disease Center of Thomas …

Smart Lighting Clinical Testbed Pilot Study On Circadian Phase Advancement, Joseph D. Gleason, Meeko Oishi, Michelle Simkulet, Arunas Tuzikas, John P. Hanifin, George C. Brainard, S. R.J. Brueck, Robert F. Karlicek, Jr., Lee K. Brown

Department of Neurology Faculty Papers

Objective: Lighting is a strong synchronizer for circadian rhythms, which in turn drives a wide range of biological functions. The objective of our work is a) to construct a clinical in-patient testbed with smart lighting, and b) evaluate its feasibility for use in future clinical studies.

Methods: A feedback capable, variable spectrum lighting system was installed at the University of New Mexico Hospital. The system consists of variable spectrum lighting troffers, color sensors, occupancy sensors, and computing and communication infrastructure. We conducted a pilot study to demonstrate proof of principle, that 1) this new technology is capable of providing continuous …

Prevalence Of Dyskinesia And Off By 30-Minute Intervals Through The Day And Assessment Of Daily Episodes Of Dyskinesia And Off: Novel Analyses Of Diary Data From Gocovri Pivotal Trials., Robert A. Hauser, Daniel Kremens, Lawrence W. Elmer, David L. Kreitzman, Ryan R. Walsh, Reed Johnson, Robert Howard, Jack T. Nguyen, Rajiv Patni

Department of Neurology Faculty Papers

BACKGROUND: Parkinson's disease (PD) patients using levodopa commonly develop dyskinesia and OFF episodes that reduce quality of life.

OBJECTIVE: Evaluate prevalence of troublesome dyskinesia and OFF through the day, assessed by 30-minute intervals, as well as the mean number and duration of troublesome dyskinesia and OFF episodes, transitions between PD states, and effects of Gocovri® (amantadine) extended release capsules on these episodes.

METHODS: Evaluate diary data from pooled Gocovri phase 3, placebo-controlled trials-analyzed for 17 hours following wake-up-at baseline and week 12.

RESULTS: Diaries were evaluable for 162 patients. At baseline, 67% of patients woke up OFF, with prevalence decreasing …

Mdivi-1, A Mitochondrial Fission Inhibitor, Modulates T Helper Cells And Suppresses The Development Of Experimental Autoimmune Encephalomyelitis., Yan-Hua Li, Fang Xu, Rodolfo Thome, Min-Fang Guo, Man-Luan Sun, Guo-Bin Song, Rui-Lan Li, Zhi Chai, Bogoljub Ciric, A. M. Rostami, Mark T. Curtis, Cun-Gen Ma, Guang-Xian Zhang

Department of Neurology Faculty Papers

BACKGROUND: Unrestrained activation of Th1 and Th17 cells is associated with the pathogenesis of multiple sclerosis and its animal model, experimental autoimmune encephalomyelitis (EAE). While inactivation of dynamin-related protein 1 (Drp1), a GTPase that regulates mitochondrial fission, can reduce EAE severity by protecting myelin from demyelination, its effect on immune responses in EAE has not yet been studied.

METHODS: We investigated the effect of Mdivi-1, a small molecule inhibitor of Drp1, on EAE. Clinical scores, inflammation, demyelination and Drp1 activation in the central nervous system (CNS), and T cell responses in both CNS and periphery were determined.

RESULTS: Mdivi-1 effectively …

Biochemical And Clinical Response After Umbilical Cord Blood Transplant In A Boy With Early Childhood-Onset Beta-Mannosidosis., Troy C. Lund, Weston P. Miller, Julie B. Eisengart, Katrina Simmons, Laura Pollard, Deborah L. Renaud, David A. Wenger, Marc C. Patterson, Paul J. Orchard

Department of Neurology Faculty Papers

BACKGROUND: Deficiency in the enzyme β-mannosidase was described over three decades ago. Although rare in occurrence, the presentation of childhood-onset β-mannosidase deficiency consists of hypotonia in the newborn period followed by global development delay, behavior problems, and intellectual disability. No effective pharmacologic treatments have been available.

METHODS: We report 2-year outcomes following the first umbilical cord blood transplant in a 4-year-old boy with early childhood-onset disease.

RESULTS: We show restoration of leukocyte β-mannosidase activity which remained normal at 2 years posttransplant, and a simultaneous increase in plasma β-mannosidase activity and dramatic decrease in urine-free oligosaccharides were also observed. MRI of …

Trial Of Canakinumab, An Il-1Β Receptor Antagonist, In Patients With Inclusion Body Myositis., Michalis L Kosmidis, Dimitris Pikazis, Panayotis Vlachoyiannopoulos, Athanasios G Tzioufas, Marinos C. Dalakas

Department of Neurology Faculty Papers

Objective: To assess whether canakinumab, a monoclonal antibody against IL-1β approved for autoinflammatory diseases, is effective as target-specific therapy in patients with sporadic inclusion body myositis (sIBM).

Methods: Because in sIBM IL-1β colocalizes with amyloid precursor protein and upregulates amyloid aggregates enhancing degeneration, targeting IL-1β with canakinumab may arrest disease progression. On this basis, 5 ambulatory patients with sIBM participated in an institutional review board--approved open-labeled study with 150 mg canakinumab [4 bimonthly, then monthly subcutaneous injections] for a mean period of 15.8 months. Patients were assessed bimonthly with a manual dynamometer in 12 proximal and distal muscles and with …

Astragaloside Iv Protects Neurons From Microglia-Mediated Cell Damage Through Promoting Microglia Polarization., Jingwen Yu, Minfang Guo, Yanhua Li, Huiyu Zhang, Zhi Chai, Qing Wang, Yuqing Yan, Jiezhong Yu, Chunyun Liu, Guang-Xian Zhang, Ma Cungen

Department of Neurology Faculty Papers

Astragaloside IV (AST-IV) is a major active ingredient of astragalus, with a neuroprotective effect. The current study is aimed to investigate the impact of AST-IV on the M1/M2 microglial activation in response to lipopolysaccharide (LPS) stimulation, how AST-IV attenuated microglia-mediated neuronal damage, and the molecular mechanisms underlying AST-IV's protection of neurons against microglia-mediated neuronal damage. Our results showed that AST-IV partially protected microglia from death evoked by LPS and downregulated the release of pro-inflammatory (M1) mediators including interleukin (IL)-1β, IL-6, tumour necrosis factor α (TNF-α) and nitric oxide, as well as the expression of Toll-like receptors 4 (TLR4), MyD88, and …

Galcanezumab In Episodic Migraine: Subgroup Analyses Of Efficacy By High Versus Low Frequency Of Migraine Headaches In Phase 3 Studies (Evolve-1 & Evolve-2)., Stephen D. Silberstein, Virginia L. Stauffer, Katie A. Day, Sarah Lipsius, Maria-Carmen Wilson

Department of Neurology Faculty Papers

BACKGROUND: Patients with high-frequency episodic migraine (HFEM) have a greater disease burden than those with low-frequency episodic migraine (LFEM). Acute treatment overuse increases the risk of migraine chronification in patients with HFEM. Galcanezumab, a humanized monoclonal antibody binding calcitonin gene-related peptide (CGRP), is effective for migraine prevention with a favorable safety profile. Here, we investigate whether there are differences in galcanezumab efficacy in patients with LFEM or with HFEM.

METHODS: Data were pooled from two double-blind, placebo-controlled phase 3 trials; EVOLVE-1 and EVOLVE-2. Patients were 18-65 years old, experienced 4-14 monthly migraine headache days (MHDs) for ≥1 year prior, with …

Stat3 Genotypic Variant Rs744166 And Increased Tyrosine Phosphorylation Of Stat3 In Il-23 Responsive Innate Lymphoid Cells During Pathogenesis Of Crohn's Disease, Ying Tang, Sanda A. Tan, Atif Iqbal, Jian Li, Sarah C. Glover

Department of Neurology Faculty Papers

Crohn's disease (CD) results from dysregulated immune responses to gut microbiota in genetically susceptible individuals, affecting multiple areas of the gastrointestinal tract. Innate lymphoid cells (ILCs) are tissue-resident innate effector lymphocytes which play crucial roles in mucosal immune defense, tissue repair, and maintenance of homeostasis. The accumulation of IFN-γ-producing ILC1s and increased level of proinflammatory cytokines produced by ILCs has been observed in the inflamed terminal ileum of CD patients. To date, the precise mechanisms of ILC plasticity and gene regulatory pathways in ILCs remain unclear. Signal transducer and activator of transcription 3 (STAT3) regulates gene expression in …

Immunomodulatory And Neuroprotective Mechanisms Of Huangqi Glycoprotein Treatment In Experimental Autoimmune Encephalomyelitis., Yanxia Xing, Binyu Liu, Yijin Zhao, Lihong Zhang, Rodolfo Thome, Huiqing Xue, Peijun Zhang, Cungen Ma

Department of Neurology Faculty Papers

Previous studies have shown that Huangqi glycoprotein (HQGP) has an anti-inflammatory effect in vitro, and suppressed experimental autoimmune encephalomyelitis (EAE), an animal model of multiple sclerosis; however, the mechanism underlying its effect is largely unknown. In this manuscript we investigated the mechanisms by which HQGP protect mice from EAE. HQGP was extracted from Astragalus membranaceus and purified by anion-exchange and gel filtration chromatography. HQGP delayed disease onset, reduced disease severity and alleviated inflammation and demyelination in the central nervous system (CNS). Moreover, HQGP reduced the infiltration of pathogenic immune cells and increased the expression of microtubule-associated protein 2 (MAP-2) and …

Generation Of Oligodendrocyte Progenitor Cells From Mouse Bone Marrow Cells., Yuan Zhang, Xin-Yu Lu, Giacomo Casella, Jing Tian, Ze-Qing Ye, Ting Yang, Juan-Juan Han, Ling-Yu Jia, A. M. Rostami, Xing Li

Department of Neurology Faculty Papers

Oligodendrocyte progenitor cells (OPCs) are a subtype of glial cells responsible for myelin regeneration. Oligodendrocytes (OLGs) originate from OPCs and are the myelinating cells in the central nervous system (CNS). OLGs play an important role in the context of lesions in which myelin loss occurs. Even though many protocols for isolating OPCs have been published, their cellular yield remains a limit for clinical application. The protocol proposed here is novel and has practical value; in fact, OPCs can be generated from a source of autologous cells without gene manipulation. Our method represents a rapid, and high-efficiency differentiation protocol for generating …

Peripheral Synucleinopathy In A Dj1 Patient With Parkinson Disease, Cataracts, And Hearing Loss, Derek P. Narendra, Risa Isonaka, Diana Nguyen, Alice B. Schindler, Angela D. Kokkinis, Debra Ehrlich, Tanya M. Bardakjian, David S. Goldstein, Tsao-Wei Liang, Pedro Gonzalez-Alegre

Department of Neurology Faculty Papers

No abstract provided.

Brainstem Atrophy In Focal Epilepsy Destabilizes Brainstem-Brain Interactions: Preliminary Findings., Susanne G. Mueller, Lisa M. Bateman, Maromi Nei, Alica M. Goldman, Kenneth D. Laxer

Department of Neurology Faculty Papers

BACKGROUND: MR Imaging has shown atrophy in brainstem regions that were linked to autonomic dysfunction in epilepsy patients. The brainstem projects to and modulates the activation state of several wide-spread cortical/subcortical regions. The goal was to investigate 1. Impact of brainstem atrophy on gray matter connectivity of cortical/subcortical structures and autonomic control. 2. Impact on the modulation of cortical/subcortical functional connectivity.

METHODS: 11 controls and 18 patients with non-lesional focal epilepsy (FE) underwent heart rate variability (HRV) measurements and a 3 T MRI (T1 in all subjects, task-free fMRI in 7 controls/ 12 FE). The brainstem was extracted, and atrophy …

The Endocannabinoid System In Migraine: From Bench To Pharmacy And Back., Cristina Tassorelli, Rosaria Greco, Stephen D. Silberstein

Department of Neurology Faculty Papers

PURPOSE OF REVIEW: Migraine is a common, highly disabling disorder. Its treatment involves acute and preventive therapy. Many of available preventive medications are not well tolerated, which results in poor compliance and limited effectiveness. Cannabinoids have been proposed for the treatment of migraine but their efficacy and tolerability are controversial.

RECENT FINDINGS: Cannabinoids modulate functions and activity of signaling pathways that have a key role in pain control. Growing preclinical evidence and initial clinical findings suggest that modulation of the endocannabinoid system, via endogenous or exogenous cannabinoids may be relevant for migraine via multiple mechanisms.

SUMMARY: The endocannabinoid system qualifies …

Erenumab In Chronic Migraine: Patient-Reported Outcomes In A Randomized Double-Blind Study., Richard B. Lipton, Stewart J. Tepper, Uwe Reuter, Stephen Silberstein, Walter F. Stewart, Jon Nilsen, Dean K. Leonardi, Pooja Desai, Sunfa Cheng, Daniel D. Mikol, Robert Lenz

Department of Neurology Faculty Papers

OBJECTIVE: To determine the effect of erenumab, a human monoclonal antibody targeting the calcitonin gene-related peptide receptor, on health-related quality of life (HRQoL), headache impact, and disability in patients with chronic migraine (CM).

METHODS: In this double-blind, placebo-controlled study, 667 adults with CM were randomized (3:2:2) to placebo or erenumab (70 or 140 mg monthly). Exploratory endpoints included migraine-specific HRQoL (Migraine-Specific Quality-of-Life Questionnaire [MSQ]), headache impact (Headache Impact Test-6 [HIT-6]), migraine-related disability (Migraine Disability Assessment [MIDAS] test), and pain interference (Patient-Reported Outcomes Measurement Information System [PROMIS] Pain Interference Scale short form 6b).

RESULTS: Improvements were observed for all endpoints in …

Lewy Body Dementia Association's Research Centers Of Excellence Program: Inaugural Meeting Proceedings., Bethany Peterson, Melissa Armstrong, Douglas Galasko, James E. Galvin, Jennifer Goldman, David Irwin, Henry Paulson, Daniel Kaufer, James Leverenz, Angela Lunde, Ian G. Mckeith, Andrew Siderowf, Angela Taylor, Katherine Amodeo, Matt Barrett, Kimiko Domoto-Reilly, John Duda, Stephen Gomperts, Neill Graff-Radford, Samantha Holden, Lawrence Honig, Daniel Huddleston, Carol Lippa, Irene Litvan, Carol Manning, Karen Marder, Charbel Moussa, Chiadi Onyike, Fernando Pagan, Alexander Pantelyat, Victoria Pelak, Kathleen Poston, Joseph Quinn, Irene Richard, Liana S. Rosenthal, Marwan Sabbagh, Douglas Scharre, Sharon Sha, Holly Shill, Yasar Torres-Yaghi, Tina Christie, Todd Graham, Ian Richards, Mike Koehler, Brad Boeve

Department of Neurology Faculty Papers

The first Lewy Body Dementia Association (LBDA) Research Centers of Excellence (RCOE) Investigator's meeting was held on December 14, 2017, in New Orleans. The program was established to increase patient access to clinical experts on Lewy body dementia (LBD), which includes dementia with Lewy bodies (DLB) and Parkinson's disease dementia (PDD), and to create a clinical trials-ready network. Four working groups (WG) were created to pursue the LBDA RCOE aims: (1) increase access to high-quality clinical care, (2) increase access to support for people living with LBD and their caregivers, (3) increase knowledge of LBD among medical and allied (or …

Human Verbal Memory Encoding Is Hierarchically Distributed In A Continuous Processing Stream., Michal T. Kucewicz, Krishnakant Saboo, Brent M. Berry, Vaclav Kremen, Laura R. Miller, Fatemeh Khadjevand, Cory S. Inman, Paul Wanda, Michael R. Sperling, Richard Gorniak, Kathryn A. Davis, Barbara C. Jobst, Bradley Lega, Sameer A. Sheth, Daniel S. Rizzuto, Ravishankar K. Iyer, Michael J. Kahana, Gregory A. Worrell

Department of Neurology Faculty Papers

Processing of memory is supported by coordinated activity in a network of sensory, association, and motor brain regions. It remains a major challenge to determine where memory is encoded for later retrieval. Here, we used direct intracranial brain recordings from epilepsy patients performing free recall tasks to determine the temporal pattern and anatomical distribution of verbal memory encoding across the entire human cortex. High γ frequency activity (65-115 Hz) showed consistent power responses during encoding of subsequently recalled and forgotten words on a subset of electrodes localized in 16 distinct cortical areas activated in the tasks. More of the high …

Study Of The Cytological Features Of Bone Marrow Mesenchymal Stem Cells From Patients With Neuromyelitis Optica., Chunsheng Yang, Yang Yang, Li Ma, Guang-Xian Zhang, Fu-Dong Shi, Yaping Yan, Guoqiang Chang

Department of Neurology Faculty Papers

Neuromyelitis optica (NMO) is a refractory autoimmune inflammatory disease of the central nervous system without an effective cure. Autologous bone marrow‑derived mesenchymal stem cells (BM‑MSCs) are considered to be promising therapeutic agents for this disease due to their potential regenerative, immune regulatory and neurotrophic effects. However, little is known about the cytological features of BM‑MSCs from patients with NMO, which may influence any therapeutic effects. The present study aimed to compare the proliferation, differentiation and senescence of BM‑MSCs from patients with NMO with that of age‑ and sex‑matched healthy subjects. It was revealed that there were no significant differences in …

Guidelines Of The International Headache Society For Controlled Trials Of Acute Treatment Of Migraine Attacks In Adults: Fourth Edition., Hans-Christoph Diener, Cristina Tassorelli, David W. Dodick, Stephen Silberstein, Richard B. Lipton, Messoud Ashina, Werner J. Becker, Michel D. Ferrari, Peter J. Goadsby, Patricia Pozo-Rosich, Shuu-Jiun Wang, Jay Mandrekar

Department of Neurology Faculty Papers

The quality of clinical trials is an essential part of the evidence base for the treatment of headache disorders. In 1991, the International Headache Society Clinical Trials Standing Committee developed and published the first edition of the Guidelines for controlled trials of drugs in migraine. Scientific and clinical developments in headache medicine led to second and third editions in 2000 and 2012, respectively. The current, fourth edition of the Guidelines retains the structure and much content from previous editions. However, it also incorporates evidence from clinical trials published after the third edition as well as feedback from meetings with regulators, …

Development And Validation Of The Fsiq-Rms: A New Patient-Reported Questionnaire To Assess Symptoms And Impacts Of Fatigue In Relapsing Multiple Sclerosis, Stacie Hudgens, René Schüler, Jonathan Stokes, Sonya Eremenco, Elke Hunsche, Thomas P. Leist

Department of Neurology Faculty Papers

Objectives: A new patient-reported outcome (PRO) instrument to measure fatigue symptoms and impacts in relapsing multiple sclerosis (RMS) was developed in a qualitative stage, followed by psychometric validation and migration from paper to an electronic format.

Methods: Adult patients with relapsing-remitting multiple sclerosis (RRMS) were interviewed to elicit fatigue-related symptoms and impacts. A draft questionnaire was debriefed in cognitive interviews with further RRMS patients, and revised. Content confirmation interviews were conducted with patients with progressive-relapsing multiple sclerosis (PRMS) and relapsing secondary-progressive multiple sclerosis (RSPMS). Psychometric analyses used data from adult patients with different RMS subtypes and matched non-RMS controls in …

A Closer Look At Arsa Activity In A Patient With Metachromatic Leukodystrophy., Kathleen Doherty, S. Barron Frazier, Matthew Clark, Anna Childers, Sumit Pruthi, David A. Wenger, Jessica Duis

Department of Neurology Faculty Papers

Metachromatic leukodystrophy (MLD) is an autosomal recessive lysosomal storage disease mainly caused by a deficiency of arylsulfatase A activity. The typical clinical course of patients with the late infantile form includes a regression in motor skills with progression to dysphagia, seizures, hypotonia and death. We present a case of a 4-year-old female with rapidly progressive developmental regression with loss of motor milestones, spasticity and dysphagia. MRI showed volume loss and markedly abnormal deep white matter. Enzymatic testing in one laboratory showed arylsulfatase A activity in their normal range. However, extraction of urine showed a large increase in sulfatide excretion in …

Early Progression Of Krabbe Disease In Patients With Symptom Onset Between 0 And 5 Months., Maria L. Beltran-Quintero, Nicholas A. Bascou, Michele D. Poe, David A. Wenger, Carlos A. Saavedra-Matiz, Matthew J. Nichols, Maria L. Escolar

Department of Neurology Faculty Papers

BACKGROUND: Krabbe disease is a rare neurological disorder caused by a deficiency in the lysosomal enzyme, β-galactocerebrosidase, resulting in demyelination of the central and peripheral nervous systems. If left without treatment, Krabbe disease results in progressive neurodegeneration with reduced quality of life and early death. The purpose of this prospective study was to describe the natural progression of early onset Krabbe disease in a large cohort of patients.

METHODS: Patients with early onset Krabbe disease were prospectively evaluated between 1999 and 2018. Data sources included diagnostic testing, parent questionnaires, standardized multidisciplinary neurodevelopmental assessments, and neuroradiological and neurophysiological tests.

RESULTS: We …

Hemi-Eses Associated With Agenesis Of The Corpus Callosum And Normal Cognition., Mahmoud Mohammadi, Safoura Kowkabi, Ali Akbar Asadi-Pooya, Reza Azizi Malamiri, Reza Shervin Badv

Department of Neurology Faculty Papers

• Corpus callosum plays the important role in bilateral synchronous expression of focal discharges of ESES.
• Sparing dominant hemisphere form continuous spike and slow waves during sleep accounts for normal cognitive scores.
• Early detection and treatment of ESES have a great impact on cognitive and language scores and final prognosis.

Combination Therapy With Fingolimod And Neural Stem Cells Promotes Functional Myelination, Yuan Zhang, Xin-Yu Lu, Ze-Qin Ye, Bogoljub Ciric, Cun-Gen Ma, A. M. Rostami, Xing Li, Guang-Xian Zhang

Department of Neurology Faculty Papers

Myelination, which occurs predominantly postnatally and continues throughout life, is important for proper neurologic function of the mammalian central nervous system (CNS). We have previously demonstrated that the combination therapy of fingolimod (FTY720) and transplanted neural stem cells (NSCs) had a significantly enhanced therapeutic effect on the chronic stage of experimental autoimmune encephalomyelitis, an animal model of CNS autoimmunity, compared to using either one of them alone. However, reduced disease severity may be secondary to the immunomodulatory effects of FTY720 and NSCs, while whether this therapy directly affects myelinogenesis remains unknown. To investigate this important question, we used three myelination …

Quantitative Clinical And Autoimmune Assessments In Stiff Person Syndrome: Evidence For A Progressive Disorder., Goran Rakocevic, Harry Alexopoulos, Marinos C. Dalakas

Department of Neurology Faculty Papers

BACKGROUND: Stiff Person Syndrome (SPS) is an under-diagnosed disorder that affects mobility and the quality of life of affected patients. The aim of the study is to describe the natural history of SPS, the extent of accumulated disability and the associated clinical and immunological features in patients followed for up to 8 years in a single center.

METHODS: Our collective cohort included 57 SPS patients. Additionally, 32 of these patients were examined every 6 months for a two-year period in a longitudinal study protocol, to assess disease progression using quantitative measures of stiffness and heightened sensitivity.

RESULTS: The most frequent …

Randomized Trial Of Polychromatic Blue-Enriched Light For Circadian Phase Shifting, Melatonin Suppression, And Alerting Responses., J. P. Hanifin, S. W. Lockley, K. Cecil, K. West, M. Jablonski, B. Warfield, M. James, M. Ayers, B. Byrne, E. Gerner, C. Pineda, M. Rollag, G. C. Brainard

Department of Neurology Faculty Papers

Wavelength comparisons have indicated that circadian phase-shifting and enhancement of subjective and EEG-correlates of alertness have a higher sensitivity to short wavelength visible light. The aim of the current study was to test whether polychromatic light enriched in the blue portion of the spectrum (17,000 K) has increased efficacy for melatonin suppression, circadian phase-shifting, and alertness as compared to an equal photon density exposure to a standard white polychromatic light (4000 K). Twenty healthy participants were studied in a time-free environment for 7 days. The protocol included two baseline days followed by a 26-h constant routine (CR1) to assess initial …