Ullrich disease: collagen VI deficiency: EM suggests a new basis for muscular weakness.

abstract：:It is generally believed that a diet high in carbohydrate improves exercise tolerance in patients with carnitine palmitoyltransferase II (CPT II) deficiency, but it has never been systematically investigated. The authors investigated the effect of a high- vs low-carbohydrate diet on exercise tolerance in four patients...

journal_title：Neurology

authors： Ørngreen MC,Ejstrup R,Vissing J

更新日期：2003-08-26 00:00:00

abstract：:Intracranial EEG recording is often required to identify an area of the brain for resective surgery for intractable epilepsy. We simultaneously compared bilaterally placed depth and limited subdural electrode EEG to determine the most effective method of recording seizures from the temporal lobes. Localized complex pa...

journal_title：Neurology

authors： Sperling MR,O'Connor MJ

更新日期：1989-11-01 00:00:00

abstract：OBJECTIVE:In children with sickle cell disease (SCD), silent cerebral infarcts are the most frequent cause of neurologic injury. We determined the sensitivity and specificity of selective neurocognitive measures when separating children with silent cerebral infarcts and SCD from sibling controls. Additionally, we teste...

journal_title：Neurology

authors： DeBaun MR,Schatz J,Siegel MJ,Koby M,Craft S,Resar L,Chu JY,Launius G,Dadash-Zadeh M,Lee RB,Noetzel M

更新日期：1998-06-01 00:00:00

abstract：:To identify clinical and electrophysiologic features related to IV immunoglobulin (IVIg) responsiveness in chronic inflammatory demyelinating polyneuropathy (CIDP), the authors conducted a multicenter study on 312 patients with CIDP (199 responders and 113 nonresponders). Muscle atrophy and decreased compound muscle a...

journal_title：Neurology

authors： Iijima M,Yamamoto M,Hirayama M,Tanaka F,Katsuno M,Mori K,Koike H,Hattori N,Arimura K,Nakagawa M,Yoshikawa H,Hayasaka K,Onodera O,Baba M,Yasuda H,Saito T,Nakazato M,Nakashima K,Kira J,Kaji R,Oka N,Sobue G

更新日期：2005-04-26 00:00:00

abstract：:We tested serum specimens from patients with HAM/TSP and asymptomatic HTLV-I carriers from endemic areas of Japan, Jamaica, Colombia, and Chile for neutralizing antibodies against HTLV-I. The data suggest a trend for neutralizing activity to be found more frequently in the sera from HAM/TSP patients than in sera from ...

journal_title：Neurology

authors： Minagawa H,Yoshida T,Itoyama Y,Mora CA,Albert P,Mori R

更新日期：1992-11-01 00:00:00

abstract：:The forearm segment of the ulnar nerve is difficult to assess if the nerve has been injured severely at the wrist. A new, simple nerve conduction technique of recording from the sensory dorsal branch of the nerve localizes the lesion and enables easier evaluation of that segment than do other techniques. ...

journal_title：Neurology

authors： Jabre JF

更新日期：1980-08-01 00:00:00

abstract：BACKGROUND:Abeta42-immunization reduces plaque burden and improves cognition in transgenic mouse models of Alzheimer disease (AD). This phase 1 study evaluated the safety, tolerability, and immunogenicity of AN1792 (human aggregated Abeta42) in patients with mild to moderate AD. METHODS:Twenty patients were enrolled i...

journal_title：Neurology

authors： Bayer AJ,Bullock R,Jones RW,Wilkinson D,Paterson KR,Jenkins L,Millais SB,Donoghue S

更新日期：2005-01-11 00:00:00

abstract：OBJECTIVES:To estimate patient-acceptable symptom state (PASS) cut points for myasthenia gravis (MG) health scales. METHODS:We conducted an electronic survey that included the Myasthenia Gravis Impairment Index (MGII), EuroQol 5-Dimension (EQ5D), and a simple PASS question. PASS-anchored thresholds were estimated for ...

journal_title：Neurology

authors： Mendoza M,Tran C,Bril V,Katzberg HD,Barnett C

更新日期：2020-09-22 00:00:00

abstract：:Clinicians have long suspected an association of classic amyotrophic lateral sclerosis (ALS) with Parkinson's disease (PD), dementia, or both. If proven, this would raise the possibility of a shared genetic susceptibility to the three disorders. To investigate this hypothesis, we compared 151 newly diagnosed ALS patie...

journal_title：Neurology

authors： Majoor-Krakauer D,Ottman R,Johnson WG,Rowland LP

更新日期：1994-10-01 00:00:00

abstract：OBJECTIVE:To investigate the feasibility and clinical utility of using passive electrocorticography (ECoG) for online spatial-temporal functional mapping (STFM) of language cortex in patients being monitored for epilepsy surgery. METHODS:We developed and tested an online system that exploits ECoG's temporal resolution...

journal_title：Neurology

authors： Wang Y,Fifer MS,Flinker A,Korzeniewska A,Cervenka MC,Anderson WS,Boatman-Reich DF,Crone NE

更新日期：2016-03-29 00:00:00

abstract：OBJECTIVE:To evaluate the clinical, electrophysiologic, and treatment outcome features of orthostatic tremor (OT) in a large case series. METHODS:We performed medical record review of 184 patients who met clinical and electrodiagnostic criteria for OT from 1976 to 2013 at the Mayo Clinic. Demographic, clinical, electr...

journal_title：Neurology

authors： Hassan A,Ahlskog JE,Matsumoto JY,Milber JM,Bower JH,Wilkinson JR

更新日期：2016-02-02 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：:A 79-year-old woman had rheumatic chorea that persisted after age 5 years and increased in severity at age 73. The continued chorea and late decompensation could have been due to incomplete functional resolution in the basal ganglia and decreasing reserve of striatal neurons with age. Sydenham's chorea persisting into...

journal_title：Neurology

authors： Gibb WR,Lees AJ,Scadding JW

更新日期：1985-01-01 00:00:00

abstract：:We studied 13 patients with severe drug-induced parkinsonism (DIP) using L-6-[18F]fluorodopa positron emission tomography (18F-dopa PET) to investigate the integrity of their dopaminergic nigrostriatal projections. Twelve of these patients were subsequently followed clinically for a median of 23.5 months (range, 6 to ...

journal_title：Neurology

authors： Burn DJ,Brooks DJ

更新日期：1993-03-01 00:00:00

abstract：BACKGROUND:Krabbe disease (globoid cell leukodystrophy [GLD]) is an autosomal recessive lysosomal disorder affecting the central and peripheral nervous system. The authors performed MRS to characterize metabolic alterations and their regional variation in brain tissue in GLD in vivo. METHODS:Abnormalities of cerebral ...

journal_title：Neurology

authors： Brockmann K,Dechent P,Wilken B,Rusch O,Frahm J,Hanefeld F

更新日期：2003-03-11 00:00:00

abstract：OBJECTIVE:To investigate to what extent subjective memory complaints and APOE-epsilon4 allele carriage predict future cognitive decline in cognitively intact elderly persons, by evaluating both their separate and combined effects. METHODS:We selected 1,168 subjects from the population-based Longitudinal Aging Study Am...

journal_title：Neurology

authors： Dik MG,Jonker C,Comijs HC,Bouter LM,Twisk JW,van Kamp GJ,Deeg DJ

更新日期：2001-12-26 00:00:00

abstract：:An autoimmune reaction to the endplate acetylcholine receptor (AChR) is thought to be responsible for the muscular weakness of myasthenia gravis (MG). However, the significance of antithymic antibodies and the thymic AChR-like protein is still uncertain. We transferred immunoglobulins (Igs) from patients with MG to mi...

journal_title：Neurology

authors： Dalkara T,Onur R,Ruacan SA,Topaktaş S

更新日期：1983-10-01 00:00:00

abstract：:Our understanding of the pathogenesis of Alzheimer's disease (AD) had advanced rapidly, particularly in the area of genetics. Clinical trials of agents that offer the promise of going beyond symptomatic treatment to actually slowing the progression of disease, perhaps by enhancing the viability of neurons, are under w...

journal_title：Neurology

authors： Whitehouse PJ

更新日期：1997-05-01 00:00:00

abstract：:Magnetization transfer imaging (MTI) is a magnetic resonance imaging (MRI) technique that is now used in multiple sclerosis (MS) studies, and is thought to have a higher pathological specificity than conventional T2-weighted imaging. This review outlines the correlations between magnetization transfer (MT) metrics and...

journal_title：Neurology

authors： Rovaris M,Horsfield MA,Filippi M

更新日期：1999-01-01 00:00:00

abstract：OBJECTIVE:To describe the clinical features, neuropathology, and genetic studies in a family with autosomal dominant frontotemporal dementia (FTD). BACKGROUND:Clinical Pick's disease, or FTD with parkinsonism, has been described in several families linked to chromosome 17 (FTDP-17). Most of these have shown tau protei...

journal_title：Neurology

authors： Kertesz A,Kawarai T,Rogaeva E,St George-Hyslop P,Poorkaj P,Bird TD,Munoz DG

更新日期：2000-02-22 00:00:00

abstract：OBJECTIVES:To determine whether TMEM106B single nucleotide polymorphisms (SNPs) are associated with frontotemporal lobar degeneration (FTLD) in patients with and without mutations in progranulin (GRN) and to determine whether TMEM106B modulates GRN expression. METHODS:We performed a case-control study of 3 SNPs in TME...

journal_title：Neurology

authors： Finch N,Carrasquillo MM,Baker M,Rutherford NJ,Coppola G,Dejesus-Hernandez M,Crook R,Hunter T,Ghidoni R,Benussi L,Crook J,Finger E,Hantanpaa KJ,Karydas AM,Sengdy P,Gonzalez J,Seeley WW,Johnson N,Beach TG,Mesulam M,

更新日期：2011-02-01 00:00:00

abstract：:A rare case of solitary CNS chloroma in a patient with chronic granulocytic leukemia (CGL) is described. This appears to be the first reported case of intracranial chloroma in CGL without systemic blastic crisis. ...

journal_title：Neurology

authors： Lysy J,Globus M,Okon E,Polliack A,Chowers I

更新日期：1983-08-01 00:00:00

abstract：:The authors assessed the referral pattern and outcome in patients with Guillain-Barré syndrome (n = 266) after the introduction of intravenous immunoglobulin (IVIg). Fewer patients were transferred from small to large hospitals after the introduction of IVIg (p = 0.05). This did not result in a worse outcome in patien...

journal_title：Neurology

authors： Van Koningsveld R,Van Doorn PA,Schmitz PI,Van der Meché FG

更新日期：2001-02-27 00:00:00

abstract：OBJECTIVE:To report the development of CNS vasculitis in a patient with multiple sclerosis (MS) treated with daclizumab. METHODS:This report includes clinical, MRI, immunologic, and pathology data and CSF analysis. RESULTS:After completing a phase II daclizumab monotherapy study with an optimal response as evidenced ...

journal_title：Neurology

authors： Ohayon J,Oh U,Richert N,Martin J,Vortmeyer A,McFarland H,Bielekova B

更新日期：2013-01-29 00:00:00

abstract：:In the same family, the generalized or infantile form of acid maltase deficiency (glycogenosis type II, Pompe disease) and the muscular or adult-onset form affected different individuals. Autosomal-recessive inheritance for the two clinical forms was demonstrated in this family by assay of acid alpha-glucosidase in mu...

journal_title：Neurology

authors： Loonen MC,Busch HF,Koster JF,Martin JJ,Niermeijer MF,Schram AW,Brouwer-Kelder B,Mekes W,Slee RG,Tager JM

更新日期：1981-10-01 00:00:00

abstract：OBJECTIVE:To determine whether cardiac magnetic resonance imaging (CMR) could be useful in identifying previously undiagnosed cardiomyopathies in a cohort of patients with ischemic stroke who underwent standard etiologic investigation and to describe the type and frequency of these cardiomyopathies. METHODS:We perform...

journal_title：Neurology

authors： Fonseca AC,Marto JP,Pimenta D,Guimarães T,Alves PN,Inácio N,Viana-Baptista M,Pinho E Melo T,Pinto FJ,Ferro JM,Almeida AG

更新日期：2020-01-07 00:00:00

abstract：OBJECTIVE:To study the safety of off-label IV thrombolysis in patients with very severe stroke (NIH Stroke Scale [NIHSS] scores >25) compared with severe stroke (NIHSS scores 15-25), where treatment is within European regulations. METHODS:Data were analyzed from 57,247 patients with acute ischemic stroke receiving IV ...

journal_title：Neurology

authors： Mazya MV,Lees KR,Collas D,Rand VM,Mikulik R,Toni D,Wahlgren N,Ahmed N

更新日期：2015-12-15 00:00:00

abstract：OBJECTIVE:To evaluate the efficacy and safety of onabotulinumtoxinA 100 U in noncatheterizing patients with multiple sclerosis (MS) with urinary incontinence (UI) due to neurogenic detrusor overactivity (NDO). METHODS:In this randomized, double-blind phase III study, patients received onabotulinumtoxinA 100 U (n = 66)...

journal_title：Neurology

authors： Tullman M,Chartier-Kastler E,Kohan A,Keppenne V,Brucker BM,Egerdie B,Mandle M,Nicandro JP,Jenkins B,Denys P

更新日期：2018-08-14 00:00:00

abstract：:Patients with Parkinson's disease (PD) commonly complain of impaired visual function and difficulty reading, despite normal visual acuity. Although previous studies have evaluated contrast sensitivity, color vision, visuospatial processing, visual hallucinations, and ocular movements, none has systematically evaluated...

journal_title：Neurology

authors： Biousse V,Skibell BC,Watts RL,Loupe DN,Drews-Botsch C,Newman NJ

更新日期：2004-01-27 00:00:00

abstract：:We studied 20 patients with an acute parietal stroke with hemisensory disturbances but no visual field deficit and no or only slight motor weakness, without thalamic involvement on CT or MRI and found three main sensory syndromes. (1) The pseudothalamic sensory syndrome consists of a faciobrachiocrural impairment of e...

journal_title：Neurology

authors： Bassetti C,Bogousslavsky J,Regli F

更新日期：1993-10-01 00:00:00