Brain white matter volume abnormalities in Lesch-Nyhan disease and its variants.

abstract：OBJECTIVE:To determine the minimum incidence and minimum prevalence rates of small-fiber neuropathy (SFN) in a well-defined region in the southern part of the Netherlands. METHODS:In this cross-sectional study with retrospective data collection, we used data of patients diagnosed with pure SFN at our Small Fiber Neuro...

journal_title：Neurology

authors： Peters MJ,Bakkers M,Merkies IS,Hoeijmakers JG,van Raak EP,Faber CG

更新日期：2013-10-08 00:00:00

abstract：:We assessed the ability to recognize epileptiform abnormalities on a video review of ambulatory cassette EEGs (A/EEG) by comparing two independent interpretations of an A/EEG to those derived from a routine recording and intensive monitoring by cable telemetry (C/EEG) of the same 40 patients. Both A/EEG readings concu...

journal_title：Neurology

authors： Ebersole JS,Leroy RF

更新日期：1983-07-01 00:00:00

abstract：OBJECTIVE:To assess longitudinal follow-up of the incidence of multiple sclerosis (MS) through five decades and estimate the prevalence rate in Hordaland County, Norway, on January 1, 2003. METHODS:All patients with MS diagnosed from 1953 to 2003 were identified in the patient records of the Department of Neurology, H...

journal_title：Neurology

authors： Grytten N,Glad SB,Aarseth JH,Nyland H,Midgard R,Myhr KM

更新日期：2006-01-24 00:00:00

abstract：:A 11-year-old boy of short stature had recurrent right temporal pounding headaches of 7 months' duration, and progressive visual loss for 3 days. There was a left hemianopia, alexia without agraphia, and diffuse muscle weakness. Investigation established the presence of a mitochondrial myopathy with pyruvate and lacti...

journal_title：Neurology

authors： Skoglund RR

更新日期：1979-05-01 00:00:00

abstract：OBJECTIVES:To investigate clinical, imaging, and pathologic associations of the cingulate island sign (CIS) in dementia with Lewy bodies (DLB). METHODS:We retrospectively identified and compared patients with a clinical diagnosis of DLB (n=39); patients with Alzheimer disease (AD) matched by age, sex, and education (n...

journal_title：Neurology

authors： Graff-Radford J,Murray ME,Lowe VJ,Boeve BF,Ferman TJ,Przybelski SA,Lesnick TG,Senjem ML,Gunter JL,Smith GE,Knopman DS,Jack CR Jr,Dickson DW,Petersen RC,Kantarci K

更新日期：2014-08-26 00:00:00

abstract：:We investigated the modulatory effects of human interferon beta (IFN-beta) and gamma (IFN-gamma) on expression of class I and II major histocompatibility complex (MHC) antigens and intercellular adhesion molecule-1 (ICAM-1) in fetal human astrocytes in culture using flow cytometry and immunocytochemistry. Under the ba...

journal_title：Neurology

authors： Satoh J,Paty DW,Kim SU

更新日期：1995-02-01 00:00:00

abstract：BACKGROUND:In the late 19th century, a man with a psychogenic movement disorder was evaluated by many of the Philadelphia neurologists associated with Silas Weir Mitchell. In 1885, prior to the development of movie cameras or projectors, the patient was photographed by pioneering photographer Eadweard Muybridge, in col...

journal_title：Neurology

authors： Lanska DJ

更新日期：2015-01-27 00:00:00

abstract：BACKGROUND:Mutations in the early growth response 2 (EGR2) gene have recently been found in patients with congenital hypomyelinating neuropathy and Charcot-Marie-Tooth type 1 (CMT1) disease. OBJECTIVE:To determine the frequency of EGR2 mutations in patients with a diagnosis of CMT1, Dejerine-Sottas syndrome (DSS), or ...

journal_title：Neurology

authors： Timmerman V,De Jonghe P,Ceuterick C,De Vriendt E,Löfgren A,Nelis E,Warner LE,Lupski JR,Martin JJ,Van Broeckhoven C

更新日期：1999-06-10 00:00:00

abstract：OBJECTIVE:We sought to determine the range and extent of neurologic complications due to pandemic influenza A (H1N1) 2009 infection (pH1N1'09) in children hospitalized with influenza. METHODS:Active hospital-based surveillance in 6 Australian tertiary pediatric referral centers between June 1 and September 30, 2009, f...

journal_title：Neurology

authors： Khandaker G,Zurynski Y,Buttery J,Marshall H,Richmond PC,Dale RC,Royle J,Gold M,Snelling T,Whitehead B,Jones C,Heron L,McCaskill M,Macartney K,Elliott EJ,Booy R

更新日期：2012-10-02 00:00:00

abstract：OBJECTIVE:To examine the relationship between carotid artery stenosis, other risk factors, and lacunar stroke. BACKGROUND:Carotid artery stenosis in patients presenting with lacune stroke may be coincidental or causal. The distinction by risk factor profile is uncertain. The risk and cause of subsequent stroke, and be...

journal_title：Neurology

authors： Inzitari D,Eliasziw M,Sharpe BL,Fox AJ,Barnett HJ

更新日期：2000-02-08 00:00:00

abstract：OBJECTIVES:Assuming the involvement of homocysteine in a generalized small-vessel disease, we investigated the association of homocysteine levels with progression of white matter lesions, lacunar infarcts, and kidney disease. METHODS:Within the SMART-MR (Second Manifestations of ARTerial disease-Magnetic Resonance) St...

journal_title：Neurology

authors： Kloppenborg RP,Geerlings MI,Visseren FL,Mali WP,Vermeulen M,van der Graaf Y,Nederkoorn PJ,SMART Study Group.

更新日期：2014-03-04 00:00:00

abstract：BACKGROUND:Several lines of evidence suggest that inflammatory mechanisms contribute to AD. OBJECTIVE:To examine whether several markers of inflammation are associated with cognitive decline in African-American and white well-functioning elders. METHODS:The authors studied 3,031 African-American and white men and wom...

journal_title：Neurology

authors： Yaffe K,Lindquist K,Penninx BW,Simonsick EM,Pahor M,Kritchevsky S,Launer L,Kuller L,Rubin S,Harris T

更新日期：2003-07-08 00:00:00

abstract：:We used [18F]-2-fluoro-2-deoxy-D-glucose (FDG) and PET to study regional cerebral glucose utilization in seven patients with fatal familial insomnia (FFI), an inherited prion disease with a mutation at codon 178 of the prion protein gene. Four patients were methionine/methionine homozygotes at codon 129 (symptom durat...

journal_title：Neurology

authors： Cortelli P,Perani D,Parchi P,Grassi F,Montagna P,De Martin M,Castellani R,Tinuper P,Gambetti P,Lugaresi E,Fazio F

更新日期：1997-07-01 00:00:00

abstract：BACKGROUND:In cerebral small vessel disease, the core MRI findings include white matter lesions (WML) and lacunar infarcts. While the clinical significance of WML is better understood, the contribution of lacunes to the rate of cognitive decline has not been established. This study investigated whether incident lacunes...

journal_title：Neurology

authors： Jokinen H,Gouw AA,Madureira S,Ylikoski R,van Straaten EC,van der Flier WM,Barkhof F,Scheltens P,Fazekas F,Schmidt R,Verdelho A,Ferro JM,Pantoni L,Inzitari D,Erkinjuntti T,LADIS Study Group.

更新日期：2011-05-31 00:00:00

abstract：:To assess reversibility of focal cerebral ischemia, we performed a neurologic and pathologic study of 27 monkeys subjected to temporary middle cerebral artery occlusion. An implanted snare ligature occluded the artery in awake monkeys for 30 minutes, 4 hours, 8 hours, 16 hours, 24 hours, or permanently. Serial neurolo...

journal_title：Neurology

authors： Crowell RM,Marcoux FW,DeGirolami U

更新日期：1981-10-01 00:00:00

abstract：OBJECTIVE:Development of an easy to administer, low-cost test of vestibular function. METHODS:Members of the NIH Toolbox Sensory Domain Vestibular, Vision, and Motor subdomain teams collaborated to identify 2 tests: 1) Dynamic Visual Acuity (DVA), and 2) the Balance Accelerometry Measure (BAM). Extensive work was comp...

journal_title：Neurology

authors： Rine RM,Schubert MC,Whitney SL,Roberts D,Redfern MS,Musolino MC,Roche JL,Steed DP,Corbin B,Lin CC,Marchetti GF,Beaumont J,Carey JP,Shepard NP,Jacobson GP,Wrisley DM,Hoffman HJ,Furman G,Slotkin J

更新日期：2013-03-12 00:00:00

abstract：:The authors report results of SPECT cerebral perfusion studies in two patients with familial dysautonomia (FD) during dysautonomic crises and when clinically stable. SPECT imaging studies used 99mTc ethylene cysteine dimer. During dysautonomic crises, regions in the temporoparietal and frontal lobes had increased upta...

journal_title：Neurology

authors： Axelrod FB,Zupanc M,Hilz MJ,Kramer EL

更新日期：2000-07-12 00:00:00

abstract：:Human subjects normally have miniature eye movements during tasks that require steady visual fixation. These eye movements were compared in 12 control subjects and 4 patients with degenerative cerebellar disorders. Slow drifts, small fixation saccades, square waves, saccadic oscillations, flutter, and vertical nystagm...

journal_title：Neurology

authors： Hotson JR

更新日期：1982-01-01 00:00:00

abstract：:A term neonate was being treated with intravenous phenytoin. To maintain a serum level above 10 micrograms per milliliter and abolish seizure activity, it was necessary to carry out repeated serum concentration measurements, administer several loading doses, and administer an unusually large maintenance dose (25 mg pe...

journal_title：Neurology

authors： Whelan HT,Hendeles L,Haberkern CM,Neims AH

更新日期：1983-01-01 00:00:00

abstract：OBJECTIVE:To assess the safety, tolerability, and biological activity of glial cell line-derived neurotrophic factor (GDNF) administered by an implanted intracerebroventricular (ICV) catheter and access port in advanced PD. BACKGROUND:GDNF is a peptide that promotes survival of dopamine neurons. It improved 6-OHDA- or...

journal_title：Neurology

authors： Nutt JG,Burchiel KJ,Comella CL,Jankovic J,Lang AE,Laws ER Jr,Lozano AM,Penn RD,Simpson RK Jr,Stacy M,Wooten GF,ICV GDNF Study Group. Implanted intracerebroventricular. Glial cell line-derived neurotrophic factor.

更新日期：2003-01-14 00:00:00

abstract：OBJECTIVE:To describe the epidemiology, etiology, clinical characteristics, treatment, outcome, and prevention of zoonotic bacterial meningitis in human adults. METHODS:We identified 16 zoonotic bacteria causing meningitis in adults. RESULTS:Zoonotic bacterial meningitis is uncommon compared to bacterial meningitis c...

journal_title：Neurology

authors： van Samkar A,Brouwer MC,van der Ende A,van de Beek D

更新日期：2016-09-13 00:00:00

abstract：:The authors describe a case of syphilitic amyotrophy in a 37-year-old man presenting with subacute progressive painless weakness in the right arm. Syphilitic amyotrophy is still an existing differential diagnosis of painless and progressive weakness. The authors explore potential obstacles in the diagnostic workup and...

journal_title：Neurology

authors： Etgen T,Bischoff C,Resch M,Winbeck K,Conrad B,Sander D

更新日期：2003-02-11 00:00:00

abstract：:This double-blind, placebo-controlled, crossover study of the acute treatment of migraine investigated the efficacy and tolerability of oral sumatriptan 100 mg (Imitrex) administered for up to nine attacks compared with placebo administered for up to three attacks. Patients were randomized to receive oral sumatriptan ...

journal_title：Neurology

authors： Rederich G,Rapoport A,Cutler N,Hazelrigg R,Jamerson B

更新日期：1995-08-01 00:00:00

abstract：OBJECTIVE:To investigate the effect of optimal combination of evidence-based drug therapies including antihypertensive agents, lipid modifiers, and antithrombotic agents on risk of recurrent vascular events after stroke. METHODS:We analyzed the database of a multicenter trial involving 3,680 recent noncardioembolic st...

journal_title：Neurology

authors： Park JH,Ovbiagele B

更新日期：2015-01-06 00:00:00

abstract：:We compared the number of new gadolinium-enhancing and T2-weighted lesions on six monthly MRI scans in eight patients with relapsing multiple sclerosis before and during treatment with weekly intramuscular interferon beta-1a (Avonex; 30 microg). MRI activity was modestly reduced during treatment (p = 0.016). The treat...

journal_title：Neurology

authors： Waubant E,Goodkin DE,Sloan R,Andersson PB

更新日期：1999-09-11 00:00:00

abstract：OBJECTIVE:To determine the potential of detection in CSF of specific Borrelia burgdorferi antigen, OspA, as a marker of infection in neurologic Lyme disease and compare this with the detection of antibody. DESIGN:CSF from 83 neurologic patients in an area highly endemic for Lyme disease was examined prospectively for ...

journal_title：Neurology

authors： Coyle PK,Schutzer SE,Deng Z,Krupp LB,Belman AL,Benach JL,Luft BJ

更新日期：1995-11-01 00:00:00

abstract：:The prevalence of spontaneous lingual-facial-buccal movements was determined in 661 patients between the ages of 50 and 79 without neuroleptic exposure or known CNS disease. The prevalence was 0.8% between ages 50 and 59, 6% between ages 60 and 69, and 7.8% between ages 70 and 79. ...

journal_title：Neurology

authors： Klawans HL,Barr A

更新日期：1982-05-01 00:00:00

abstract：:A 47-year-old woman had myasthenia gravis with only moderate weakness and a consistently poor response to anticholinesterase medication. She was investigated with routine electrophysiologic studies and single-fiber electromyography. The studies showed a limited effect of anticholinesterase medication. There was a decr...

journal_title：Neurology

authors： Schwartz MS,Stålberg E

更新日期：1975-01-01 00:00:00

abstract：OBJECTIVE:To investigate the association of triglyceride (TG) principal component scores with Alzheimer disease (AD) and the amyloid, tau, neurodegeneration, and cerebrovascular disease (A/T/N/V) biomarkers for AD. METHODS:Serum levels of 84 TG species were measured with untargeted lipid profiling of 689 participants ...

journal_title：Neurology

authors： Bernath MM,Bhattacharyya S,Nho K,Barupal DK,Fiehn O,Baillie R,Risacher SL,Arnold M,Jacobson T,Trojanowski JQ,Shaw LM,Weiner MW,Doraiswamy PM,Kaddurah-Daouk R,Saykin AJ,Alzheimer's Disease Neuroimaging Initiative and Alzheim

更新日期：2020-05-19 00:00:00

abstract：:Rats with unilateral lesions of the substantia nigra were treated with L-dopa 25 mg/kg subcutaneously 30 minutes after treatment with carbidopa 25 mg/kg intraperitoneally. In the ipsilateral striatum, dopamine levels achieved a peak of 1.57 +/- 0.63 micrograms/g (control 0.19 +/- 0.05 micrograms/g) at 20 minutes and d...

journal_title：Neurology

authors： Spencer SE,Wooten GF

更新日期：1984-08-01 00:00:00