Oral phenylalanine loading in dopa-responsive dystonia: a possible diagnostic test.

abstract：:We studied the nature and causes of delusions after infarction of the right cerebral hemisphere. Delusions involved orientation in time and place, events in the recent past, and the identities of familiar individuals. Some were transient and disorganized, and others were more fixed. No particular site in the right hem...

journal_title：Neurology

authors： Levine DN,Grek A

更新日期：1984-05-01 00:00:00

abstract：:Dementia occurs infrequently in patients with giant cell (temporal) arteritis (GCA). Three elderly women with biopsy-proven GCA showed abrupt cognitive decline during periods of clinically active GCA, 1 to 6 months after diagnostic temporal artery biopsy, during periods of corticosteroid taper. One patient had additio...

journal_title：Neurology

authors： Caselli RJ

更新日期：1990-05-01 00:00:00

abstract：BACKGROUND:Although phenylketonuria is a treatable disease, patients with late or nonoptimal phenylalanine-restricted diet may experience brain damage. The authors used tridimensional MRI and a voxelwise analysis method to investigate possible volume changes in the brain parenchyma of patients with phenylketonuria. ME...

journal_title：Neurology

authors： Pérez-Dueñas B,Pujol J,Soriano-Mas C,Ortiz H,Artuch R,Vilaseca MA,Campistol J

更新日期：2006-04-11 00:00:00

abstract：:In clinical trials, copolymer I (Cop 1) appears to reduce the number of exacerbations in early relapsing-remitting multiple sclerosis. The mechanism of this effect is uncertain, but Cop 1 also reduces the severity of experimental allergic encephalomyelitis and inhibits the response of murine myelin basic protein (MBP)...

journal_title：Neurology

authors： Burns J,Littlefield K

更新日期：1991-08-01 00:00:00

abstract：:Alzheimer's disease is characterized by relative sparing of primary sensory and motor cortex and a lack of sensory or motor symptomatology. We report a case of presenile onset dementia accompanied by a slowly progressive hemiparesis. Autopsy examination showed severe pathologic involvement of somatosensory cortex with...

journal_title：Neurology

authors： Jagust WJ,Davies P,Tiller-Borcich JK,Reed BR

更新日期：1990-01-01 00:00:00

abstract：OBJECTIVE:To determine the relationship between hippocampal, amygdalar, and entorhinal cortex atrophy and duration of epilepsy, presence of secondary generalized seizures, and prolonged childhood febrile convulsions in patients with pharmacologically intractable temporal lobe epilepsy (TLE). METHODS:Volumetric MRI of ...

journal_title：Neurology

authors： Bernasconi N,Natsume J,Bernasconi A

更新日期：2005-07-26 00:00:00

abstract：BACKGROUND:Charcot Marie Tooth type 1a (CMT1a) is a primarily demyelinating neuropathy, characterized by slowly progressive muscle weakness, atrophy, and sensory loss, and is most pronounced in both feet and hands. There is increasing evidence that muscle weakness is determined by motor axonal dysfunction. OBJECTIVE:T...

journal_title：Neurology

authors： Videler AJ,van Dijk JP,Beelen A,de Visser M,Nollet F,van Schaik IN

更新日期：2008-10-14 00:00:00

abstract：BACKGROUND:The epsilon 4 allele of the APOE gene (APOE) is more frequent in patients with AD than in the general population, but studies are inconclusive as to whether it affects rate of progression or survival. Because survival in AD is generally longer in women than in men, the authors investigated whether APOE affec...

journal_title：Neurology

authors： Dal Forno G,Carson KA,Brookmeyer R,Troncoso J,Kawas CH,Brandt J

更新日期：2002-04-09 00:00:00

abstract：OBJECTIVE:To determine whether changes in brain biometals in Alzheimer disease (AD) and in normal brain tissue are tandemly associated with amyloid beta-peptide (Abeta) burden and dementia severity. METHODS:The authors measured zinc, copper, iron, manganese, and aluminum and Abeta levels in postmortem neocortical tiss...

journal_title：Neurology

authors： Religa D,Strozyk D,Cherny RA,Volitakis I,Haroutunian V,Winblad B,Naslund J,Bush AI

更新日期：2006-07-11 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：:We describe a 66-year-old woman with long-standing HTLV-I-associated myelopathy who developed multiple parenchymal hemorrhages and whose angiogram suggested cerebral vasculitis. After cyclophosphamide and glucocorticoid therapy, both her acute deficits and chronic paraparesis improved. HTLV-I may be an etiologic agent...

journal_title：Neurology

authors： Smith D,Lucas S,Jacewicz M

更新日期：1993-02-01 00:00:00

abstract：:Progressive supranuclear palsy (PSP) is a relatively common neurodegenerative tauopathy clinically characterized by parkinsonism, axial rigidity, and supranuclear gaze palsy. Pathologic findings of PSP are neuronal loss, gliosis, and neurofibrillary tangles in basal ganglia, diencephalon, and brainstem; there is incre...

journal_title：Neurology

authors： Fujioka S,Algom AA,Murray ME,Strongosky A,Soto-Ortolaza AI,Rademakers R,Ross OA,Wszolek ZK,Dickson DW

更新日期：2013-05-28 00:00:00

abstract：OBJECTIVE:Identifying an intriguing mechanism for unmasking recessive hereditary spastic paraplegias. METHOD:Herein, we describe 4 novel homozygous FA2H mutations in 4 nonconsanguineous families detected by whole-exome sequencing or a targeted gene panel analysis providing high coverage of all known hereditary spastic...

journal_title：Neurology

authors： Soehn AS,Rattay TW,Beck-Wödl S,Schäferhoff K,Monk D,Döbler-Neumann M,Hörtnagel K,Schlüter A,Ruiz M,Pujol A,Züchner S,Riess O,Schüle R,Bauer P,Schöls L

更新日期：2016-07-12 00:00:00

abstract：:T lymphocytes may play a central role in MS. The search for more targeted immunosuppression than is currently available has led to recent clinical trials of novel therapeutics. We studied 29 patients in a double-blind placebo-controlled trial of the chimeric monoclonal anti-CD4 antibody, cM-T412 (Centocor, Leiden, Hol...

journal_title：Neurology

authors： Llewellyn-Smith N,Lai M,Miller DH,Rudge P,Thompson AJ,Cuzner ML

更新日期：1997-04-01 00:00:00

abstract：:Call-Fleming syndrome is a reversible segmental vasoconstriction of cerebral arteries manifested by a "thunderclap" headache and focal neurologic symptoms. Although of unknown etiology, it has been reported in association with vasoactive sympathomimetic drugs. The authors report Call-Fleming syndrome in two patients w...

journal_title：Neurology

authors： Noskin O,Jafarimojarrad E,Libman RB,Nelson JL

更新日期：2006-07-11 00:00:00

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：OBJECTIVE:To use punch skin biopsies to evaluate the loss of intra-epidermal nerve fibers in sensory neuropathies. BACKGROUND:Previous assessments of epidermal nerve fibers have been constrained by relatively insensitive staining techniques and variability in quantification. METHODS:Punch skin biopsies were performed...

journal_title：Neurology

authors： McCarthy BG,Hsieh ST,Stocks A,Hauer P,Macko C,Cornblath DR,Griffin JW,McArthur JC

更新日期：1995-10-01 00:00:00

abstract：OBJECTIVE:To determine whether cognitive impairment in Parkinson disease (PD) and Alzheimer disease (AD) derives from the same network pathology. METHODS:We analyzed 18F-fluorodeoxyglucose PET scans from 40 patients with AD and 40 age-matched healthy controls from the Alzheimer's Disease Neuroimaging Initiative and sc...

journal_title：Neurology

authors： Mattis PJ,Niethammer M,Sako W,Tang CC,Nazem A,Gordon ML,Brandt V,Dhawan V,Eidelberg D

更新日期：2016-11-01 00:00:00

abstract：OBJECTIVE:To investigate whether vitamin D, smoking, and anti-Epstein-Barr virus (EBV) antibody concentrations predict long-term cognitive status and neuroaxonal injury in multiple sclerosis (MS). METHODS:This study was conducted among 278 patients with clinically isolated syndrome who participated in the clinical tri...

journal_title：Neurology

authors： Cortese M,Munger KL,Martínez-Lapiscina EH,Barro C,Edan G,Freedman MS,Hartung HP,Montalbán X,Foley FW,Penner IK,Hemmer B,Fox EJ,Schippling S,Wicklein EM,Kappos L,Kuhle J,Ascherio A,BENEFIT Study Group.

更新日期：2020-05-05 00:00:00

abstract：OBJECTIVES:To survey US physicians involved in acute stroke care to determine the proportion of hospitals that currently meet the recommended Brain Attack Coalition (BAC) criteria for Primary Stroke Centers (PSC) and obtain opinions regarding the value of stroke centers. METHODS:A survey regarding the BAC guidelines f...

journal_title：Neurology

authors： Kidwell CS,Shephard T,Tonn S,Lawyer B,Murdock M,Koroshetz W,Alberts M,Hademenos GJ,Saver JL

更新日期：2003-05-13 00:00:00

abstract：OBJECTIVES:Observational and experimental studies suggest that nonsteroidal anti-inflammatory drugs (NSAIDs) may protect against Alzheimer disease (AD); however, clinical trials and other observational studies, including the Adult Changes in Thought (ACT) study, show no protection or promotion of AD. The objective of t...

journal_title：Neurology

authors： Sonnen JA,Larson EB,Walker RL,Haneuse S,Crane PK,Gray SL,Breitner JC,Montine TJ

更新日期：2010-09-28 00:00:00

abstract：OBJECTIVE:Using interictal alpha-[11C]methyl-l-tryptophan ([11C]AMT) PET scan, the authors have undertaken a quantitative analysis of all tubers visible on MRI or 2-deoxy-2-[18F]fluoro-d-glucose ([18F]FDG) PET, to determine the relationship between [11C]AMT uptake and epileptic activity on EEG. BACKGROUND:Tuberous scl...

journal_title：Neurology

authors： Asano E,Chugani DC,Muzik O,Shen C,Juhász C,Janisse J,Ager J,Canady A,Shah JR,Shah AK,Watson C,Chugani HT

更新日期：2000-05-23 00:00:00

abstract：BACKGROUND:Hematopoietic stem cell transplantation (HSCT) may slow the progression of Krabbe disease (KD) if performed early in the disease. The authors' studies indicate that more than 90% of patients with KD have severe abnormalities in peripheral nerve conduction. OBJECTIVE:To assess the effect of HSCT on nerve con...

journal_title：Neurology

authors： Siddiqi ZA,Sanders DB,Massey JM

更新日期：2006-07-25 00:00:00

abstract：:Immunomodulating agents have beneficial effects in the treatment of multiple sclerosis (MS), decreasing the frequency of relapses, the progression of disability, and MRI measures of disease activity. Despite the efficacy of these agents, many patients continue to show progression of disability, breakthrough relapses, ...

journal_title：Neurology

authors： Jeffery DR

更新日期：2004-12-28 00:00:00

abstract：:We present a clinical and morphological study concerning a kindred with slowly progressive X-linked muscular dystrophy (Becker type). Five males were affected; one died of heart failure at age 16. Severe and early cardiac disorder is unusual in this type of muscular dystrophy, and death at such an early age had not be...

journal_title：Neurology

authors： Kuhn E,Fiehn W,Schröder JM,Assmus H,Wagner A

更新日期：1979-08-01 00:00:00

abstract：:We have studied one family of a pedigree in which bulbospinal muscular atrophy and protanopia were segregated. Genotypes for these disorders were obtained on three generations, and the maximum likelihood estimate of recombination fraction was 0.4 with the lod score method. The results indicated that the two loci are n...

journal_title：Neurology

authors： Mukai E,Yasuma T

更新日期：1987-06-01 00:00:00

abstract：OBJECTIVE:To investigate the relationship between visual hallucinations in Parkinson disease (PD) and levels of γ-aminobutyric acid (GABA) in the primary visual cortex. METHODS:We utilized magnetic resonance spectroscopy to investigate occipital GABA levels in 36 participants with PD, 19 with and 17 without complex vi...

journal_title：Neurology

authors： Firbank MJ,Parikh J,Murphy N,Killen A,Allan CL,Collerton D,Blamire AM,Taylor JP

更新日期：2018-08-14 00:00:00

abstract：OBJECTIVE:To describe the clinical features of cardiac injury associated with neurogenic pulmonary edema (NPE) in patients with acute subarachnoid hemorrhage (SAH). BACKGROUND:NPE is generally viewed as a form of noncardiogenic pulmonary edema related to massive sympathetic discharge. METHODS:Case series. RESULTS:We...

journal_title：Neurology

authors： Mayer SA,Fink ME,Homma S,Sherman D,LiMandri G,Lennihan L,Solomon RA,Klebanoff LM,Beckford A,Raps EC

更新日期：1994-05-01 00:00:00

abstract：OBJECTIVE:To determine whether metformin is associated with a lower incidence of dementia than sulfonylureas. METHODS:This was a retrospective cohort study of US veterans ≥65 years of age with type 2 diabetes who were new users of metformin or a sulfonylurea and had no dementia. Follow-up began after 2 years of therap...

journal_title：Neurology

authors： Orkaby AR,Cho K,Cormack J,Gagnon DR,Driver JA

更新日期：2017-10-31 00:00:00

abstract：:The clinical and genetic features of three non-Portuguese and non-Japanese patients with aprataxin gene mutations are reported. Patient 1 came from Italy and presented with typical ataxia with ocular motor apraxia (OMA). She was homozygous for the W279X nonsense mutation, which is associated with the Portuguese foundi...

journal_title：Neurology

authors： Tranchant C,Fleury M,Moreira MC,Koenig M,Warter JM

更新日期：2003-03-11 00:00:00