A family with different clinical forms of acid maltase deficiency (glycogenosis type II): biochemical and genetic studies.

abstract：:We attempted to determine whether separable components underlie normal subjects' upward bias in bisecting vertical and radial lines under visual guidance. Twelve normal subjects indicated the midpoint of visually presented lines oriented vertically, radially, and horizontally. We placed directional labels ("TOP" and "...

journal_title：Neurology

authors： Jeerakathil TJ,Kirk A

更新日期：1994-04-01 00:00:00

abstract：OBJECTIVES:To describe the frequency of cervical arterial abnormalities in children with acute arterial ischemic stroke (AIS), to examine predictors of this, and to characterize observed abnormalities in terms of specific diagnoses. METHODS:Review of case notes of children with AIS (2002-2009) and analysis of their ne...

journal_title：Neurology

authors： Ganesan V,Cox TC,Gunny R

更新日期：2011-01-11 00:00:00

abstract：:Evaluating rehabilitation requires rating scales that detect change. The authors examined Barthel Index (BI) data from 1,495 patients at a neurorehabilitation unit to determine whether total scale responsiveness accurately reflects item responsiveness. Total score effect sizes were moderate to large (0.47 to 1.09). It...

journal_title：Neurology

authors： O'Connor RJ,Cano SJ,Thompson AJ,Hobart JC

更新日期：2004-05-25 00:00:00

abstract：OBJECTIVE:To investigate recovery of medical decision-making capacity (MDC) over the first year following traumatic brain injury (TBI). METHODS:A total of 177 participants (111 persons with TBI and 66 healthy controls) were recruited from an inpatient/outpatient TBI rehabilitation unit and outpatient neurology departm...

journal_title：Neurology

authors： Steward KA,Gerstenecker A,Triebel KL,Kennedy R,Novack TA,Dreer LE,Marson DC

更新日期：2016-09-06 00:00:00

abstract：OBJECTIVE:To determine the diagnostic value of high-resolution ultrasound (HRUS) for detection of chronic inflammatory demyelinating polyneuropathy (CIDP), Lewis-Sumner syndrome (LSS), and multifocal motor neuropathy (MMN). METHODS:Between January 2013 and January 2015, we enrolled 75 consecutive treatment-naive patie...

journal_title：Neurology

authors： Goedee HS,van der Pol WL,van Asseldonk JH,Franssen H,Notermans NC,Vrancken AJ,van Es MA,Nikolakopoulos S,Visser LH,van den Berg LH

更新日期：2017-01-10 00:00:00

abstract：OBJECTIVE:Dominant, left anteromedial temporal lobe resection (AMTLR) for seizure control carries risks to verbal episodic memory and visual object naming. Consistent with traditional thinking, verbal memory decline is considered a consequence of hippocampal removal and naming decline has been attributed to lateral tem...

journal_title：Neurology

authors： Hamberger MJ,Seidel WT,McKhann GM 2nd,Goodman RR

更新日期：2010-05-11 00:00:00

abstract：:Fasciculations and cramps may occur in motor neuron disease or as part of a more benign syndrome. A man with apparently benign fasciculations and cramps for 4 years developed progressive muscle weakness and wasting. Such a previously undocumented evolution of benign fasciculations and cramps to motor neuron disease ma...

journal_title：Neurology

authors： Fleet WS,Watson RT

更新日期：1986-07-01 00:00:00

abstract：OBJECTIVE:To investigate whether recurrent seizures cause hippocampal damage in temporal lobe epilepsy (TLE). PATIENTS:Eighteen patients with newly diagnosed cryptogenic TLE, 14 patients with chronic well-controlled cryptogenic TLE, 32 patients with chronic drug-resistant cryptogenic TLE, and 25 healthy subjects were ...

journal_title：Neurology

authors： Kälviäinen R,Salmenperä T,Partanen K,Vainio P,Riekkinen P,Pitkänen A

更新日期：1998-05-01 00:00:00

abstract：:The authors estimated the sample sizes needed for exploratory trials of MS assessing the efficacy of new treatments in reducing the number of new enhancing lesions vs those of interferon-beta or glatiramer acetate. The sample sizes per arm ranged from 868 (effect: 20%) to 94 (effect: 50%) for patients with relapsing-r...

journal_title：Neurology

authors： Sormani MP,Rovaris M,Bagnato F,Molyneux P,Bruzzi P,Pozzilli C,Miller DH,Comi G,Filippi M

更新日期：2001-11-27 00:00:00

abstract：OBJECTIVE:To study activin signaling and its blockade in sporadic inclusion body myositis (sIBM) through translational studies and a randomized controlled trial. METHODS:We measured transforming growth factor β signaling by SMAD2/3 phosphorylation in muscle biopsies of 50 patients with neuromuscular disease (17 with s...

journal_title：Neurology

authors： Amato AA,Sivakumar K,Goyal N,David WS,Salajegheh M,Praestgaard J,Lach-Trifilieff E,Trendelenburg AU,Laurent D,Glass DJ,Roubenoff R,Tseng BS,Greenberg SA

更新日期：2014-12-09 00:00:00

abstract：:A clinical procedure for determining contrast sensitivity was performed on patients with suspected sella area masses. The test consisted of a series of six plates, each containing a bar pattern of fixed sinusoidal spatial frequency in which contrast varies along the length of the bar. Patients with compressive lesions...

journal_title：Neurology

authors： Kupersmith MJ,Siegel IM,Carr RE

更新日期：1981-05-01 00:00:00

abstract：:We investigated the possible role of potassium deficiency, a recognized cause of myopathy, in the pathogenesis of experimental acute alcoholic myopathy (EAM) in the rat. Alcohol-treated animals receiving the same dietary potassium as controls developed mild hypokalemia; however, muscle potassium was preserved or eleva...

journal_title：Neurology

authors： Haller RG,Carter NW,Ferguson E,Knochel JP

更新日期：1984-04-01 00:00:00

abstract：:4-Aminopyridine (4-AP) has a favorable effect on the disability of certain patients with MS. We investigated the effect of 4-AP on neuropsychological performance in 20 MS patients using a randomized, double-blind, placebo-controlled, crossover design. Although there was a trend for improved performance with 4-AP for t...

journal_title：Neurology

authors： Smits RC,Emmen HH,Bertelsmann FW,Kulig BM,van Loenen AC,Polman CH

更新日期：1994-09-01 00:00:00

abstract：OBJECTIVE:The NIH Stroke Scale (NIHSS) may not appropriately assess the spectrum of posterior circulation (PC)-related neurologic deficits. We determined the cutoff baseline NIHSS score that predicts independent daily life activity during the chronic stage in anterior circulation (AC) vs PC ischemic strokes. METHODS:A...

journal_title：Neurology

authors： Sato S,Toyoda K,Uehara T,Toratani N,Yokota C,Moriwaki H,Naritomi H,Minematsu K

更新日期：2008-06-10 00:00:00

abstract：:A man with onset of obsessive-compulsive disorder (OCD) at age 62 had a large right posterior frontal infarct and occlusion of the ipsilateral internal carotid artery. We review additional cases from the literature with obsessive or compulsive behaviors and structural lesions. OCD may have a structural correlate, and ...

journal_title：Neurology

authors： Swoboda KJ,Jenike MA

更新日期：1995-12-01 00:00:00

abstract：:A striking behavioral abnormality is described in three individuals who had severe head trauma. At a point when general mnestic capabilities had returned to a near normal level, the patients persistently relocated the hospital at another geographical site, even in the face of compelling counter-evidence. The strong pa...

journal_title：Neurology

authors： Benson DF,Gardner H,Meadows JC

更新日期：1976-02-01 00:00:00

abstract：:We studied two patients with Meige syndrome who developed alpha-methyldopa-induced parkinsonism. Opposite responses of parkinsonian and dystonic symptoms to antiparkinson drugs in some cases suggest a functionally reciprocal relationship between these disorders. ...

journal_title：Neurology

authors： Sechi GP,Demontis G,Rosati G

更新日期：1985-11-01 00:00:00

abstract：:The scanning electronmicroscope was used to study the development of anencephaly in an experimental model. Anencephaly was produced consistently, using vitamin A as the teratogen. Embryos destined to become anencephalic displayed failure of opposing sides of the rostral neural tube to fuse. Subsequently, the neural fo...

journal_title：Neurology

authors： Smith MT,Wood LR,Honig SR

更新日期：1982-09-01 00:00:00

abstract：:Women were enrolled in our prospective cohort study of epilepsy and pregnancy to determine the developmental outcome of offspring and the immediate outcome of pregnancy. Women with epilepsy (case group) were enrolled before conception or during the first trimester, and women without epilepsy or chronic illness (contro...

journal_title：Neurology

authors： Leavitt AM,Yerby MS,Robinson N,Sells CJ,Erickson DM

更新日期：1992-04-01 00:00:00

abstract：:Prompted by the observation that a fellow medical student sneezed at about the same time in class every morning, we recorded the time of each of the 118 sneezes she made on 69 days over a 6.5-month period. Analysis of the sneeze times with the goodness-of-fit test revealed a highly nonuniform distribution (p < 0.0001)...

journal_title：Neurology

authors： Grant AC,Roter EP

更新日期：1994-03-01 00:00:00

abstract：:We report the lst case of neuroleptic malignant syndrome occurring in a patient receiving desipramine, a tricyclic antidepressant, with no exposure to neuroleptics. Our case suggests that this syndrome may be caused by a central imbalance between norepinephrine and dopamine, and not by dopamine depletion alone. ...

journal_title：Neurology

authors： Baca L,Martinelli L

更新日期：1990-11-01 00:00:00

abstract：:Calcification in brainstem gliomas is an unusual histologic and and radiologic finding. To date, radiologic evidence of calcification in these tumors has not been reported. The two patients described here had evidence of calcification on either computerized tomography (CT) or plain skull films. Increasing availability...

journal_title：Neurology

authors： Duffner PK,Klein DM,Cohen ME

更新日期：1978-08-01 00:00:00

abstract：BACKGROUND:A large proportion of survivors of traumatic brain injury (TBI) have persistent cognitive impairments, the profile of which does not always correspond to the size and location of injuries. One possible explanation could be that TBI-induced damage extends beyond obvious lesion sites to affect remote brain net...

journal_title：Neurology

authors： Kasahara M,Menon DK,Salmond CH,Outtrim JG,Taylor Tavares JV,Carpenter TA,Pickard JD,Sahakian BJ,Stamatakis EA

更新日期：2010-07-13 00:00:00

abstract：OBJECTIVE:To investigate whether the presence of cerebral microbleeds, which present as focal lesions on imaging, is associated with a diffuse loss of white matter microstructural integrity in the brain. METHODS:In the prospective, population-based Rotterdam Scan Study, a total of 4,493 participants underwent brain MR...

journal_title：Neurology

authors： Akoudad S,de Groot M,Koudstaal PJ,van der Lugt A,Niessen WJ,Hofman A,Ikram MA,Vernooij MW

更新日期：2013-11-26 00:00:00

abstract：OBJECTIVE:We aimed to perform an observational study of age at loss of independent ambulation (LoA) and side-effect profiles associated with different glucocorticoid corticosteroid (GC) regimens in Duchenne muscular dystrophy (DMD). METHODS:We studied 340 participants in the Cooperative International Neuromuscular Res...

journal_title：Neurology

authors： Bello L,Gordish-Dressman H,Morgenroth LP,Henricson EK,Duong T,Hoffman EP,Cnaan A,McDonald CM,CINRG Investigators.

更新日期：2015-09-22 00:00:00

abstract：:Multiple chemical and coagulation determinations were undertaken on the subdural hematoma fluid from the reformed effusions of two patients. It was found that plasma or blood repeatedly reentered the subdural cavity. Coagulation studies compared the in vitro effects of subdural fluid with those of cerebrospinal fluid,...

journal_title：Neurology

authors： Labadie EL,Glover D

更新日期：1975-07-01 00:00:00

abstract：OBJECTIVE:To investigate the effect of age, sex, APOE4 genotype, and lifestyle enrichment (education/occupation, midlife cognitive activity, and midlife physical activity) on Alzheimer disease (AD) biomarker trajectories using longitudinal imaging data (brain β-amyloid load via Pittsburgh compound B PET and neurodegene...

journal_title：Neurology

authors： Vemuri P,Lesnick TG,Przybelski SA,Knopman DS,Machulda M,Lowe VJ,Mielke MM,Roberts RO,Gunter JL,Senjem ML,Geda YE,Rocca WA,Petersen RC,Jack CR Jr

更新日期：2016-03-22 00:00:00

abstract：:Dyskinesias are usually seen in Parkinson's disease (PD) patients after several years of L-dopa therapy. Their presence has been attributed to supersensitivity of striatal D1 and D2 receptors. We have used PET to assess striatal D2 receptor binding in untreated PD patients and striatal D1 and D2 binding in L-dopa-trea...

journal_title：Neurology

authors： Turjanski N,Lees AJ,Brooks DJ

更新日期：1997-09-01 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

abstract：OBJECTIVE:Cerebral cavernous malformations (CCM) occur in familial and sporadic forms that cannot be distinguished by phenotype. Mutations in Krit1, a gene located at the CCM1 locus on chromosome 7q21, account for the majority of familial CCM cases. The authors investigated the role that mutations at the CCM1 locus pla...

journal_title：Neurology

authors： Reich P,Winkler J,Straube A,Steiger HJ,Peraud A

更新日期：2003-04-08 00:00:00