Friedreich's ataxia: clinical pilot trial with recombinant human erythropoietin.

abstract：:The blood-brain barrier is a natural diffusion barrier, which expresses active carriers extruding drugs on their way to the brain back into the blood against concentration gradients. Whereas these so-called adenosine triphosphate-binding cassette (ABC) transporters prevent the brain entry of toxic compounds under phys...

journal_title：Annals of neurology

authors： Hermann DM,Kilic E,Spudich A,Krämer SD,Wunderli-Allenspach H,Bassetti CL

更新日期：2006-11-01 00:00:00

abstract：:Several mutations in the amyloid precursor protein (APP) gene have been found to associate with pathologic deposition of the beta-amyloid peptide (Abeta) in neuritic plaques or in the walls of cerebral vessels. We report a mutation at a novel site in APP in a three-generation Iowa family with autosomal dominant dement...

journal_title：Annals of neurology

authors： Grabowski TJ,Cho HS,Vonsattel JP,Rebeck GW,Greenberg SM

更新日期：2001-06-01 00:00:00

abstract：:To elucidate the role of D-serine in human central nervous system, we analyzed D-serine, L-serine, and glycine concentrations in cerebrospinal fluid of healthy children and children with a defective L-serine biosynthesis (3-phosphoglycerate dehydrogenase deficiency). Healthy children showed high D-serine concentration...

journal_title：Annals of neurology

authors： Fuchs SA,Dorland L,de Sain-van der Velden MG,Hendriks M,Klomp LW,Berger R,de Koning TJ

更新日期：2006-10-01 00:00:00

abstract：:We prepared RNA probes from cloned segments of human and murine enteroviruses (EVs) for in situ hybridization of skeletal muscle biopsies from patients with dermatomyositis (DM), polymyositis, other inflammatory myopathies, and noninflammatory muscle diseases, and from normal control subjects. A probe derived from The...

journal_title：Annals of neurology

authors： Rosenberg NL,Rotbart HA,Abzug MJ,Ringel SP,Levin MJ

更新日期：1989-08-01 00:00:00

abstract：:The activities of complex I and complex II/III in platelet mitochondria are reduced in patients with early, untreated Parkinson's disease. Coenzyme Q10 is the electron acceptor for complex I and complex II. We found that the level of coenzyme Q10 was significantly lower in mitochondria from parkinsonian patients than ...

journal_title：Annals of neurology

authors： Shults CW,Haas RH,Passov D,Beal MF

更新日期：1997-08-01 00:00:00

abstract：:This study quantitatively explored the dendritic/spine extent of supragranular pyramidal neurons across several cortical areas in two adult male subjects who had undergone a callosotomy several decades before death. In all cortical areas, there were numerous atypical, supragranular pyramidal neurons with elongated "ta...

journal_title：Annals of neurology

authors： Jacobs B,Creswell J,Britt JP,Ford KL,Bogen JE,Zaidel E

更新日期：2003-07-01 00:00:00

abstract：OBJECTIVE:Accurate identification of the ischemic penumbra, the therapeutic target in acute clinical stroke, is of critical importance to identify patients who might benefit from reperfusion therapies beyond the established time windows. Therefore, we aimed to validate magnetic resonance imaging (MRI) mismatch-based pe...

journal_title：Annals of neurology

authors： Zaro-Weber O,Fleischer H,Reiblich L,Schuster A,Moeller-Hartmann W,Heiss WD

更新日期：2019-06-01 00:00:00

abstract：:Fourteen children with brain tumors received endocrine evaluations at least one year following completion of cranial irradiation. Treatment consisted of operation (13 patients), craniospinal irradiation (6), whole brain irradiation (5), posterior fossa irradiation (3), and chemotherapy (10). Endocrine evaluation inclu...

journal_title：Annals of neurology

authors： Duffner PK,Cohen ME,Anderson SW,Voorhess ML,MacGillivray MH,Panahon A,Brecher ML

更新日期：1983-11-01 00:00:00

abstract：OBJECTIVE:Normal aging is often associated with a decline in learning and memory functions. This decline is manifested to a much greater extent in Alzheimer's disease. Recent studies have indicated statins, a class of cholesterol-lowering drugs, as a potential therapy for Alzheimer's disease. Our objective was to deter...

journal_title：Annals of neurology

authors： Li L,Cao D,Kim H,Lester R,Fukuchi K

更新日期：2006-12-01 00:00:00

abstract：:The unloading reflex was measured in the intrinsic muscles of the hand. By removing various fractions of the load on the muscles, we determined the size of the electromyographic response as a function of the change in the external force. This technique was applied to both hands of a patient with a pontine lesion who h...

journal_title：Annals of neurology

authors： Angel RW,Goldstein M

更新日期：1983-03-01 00:00:00

abstract：:Progressive multifocal leukoencephalopathy is the most common clinical presentation of JC virus (JCV)-associated central nervous system (CNS) disease and has emerged as a major safety concern in multiple sclerosis patients treated with the monoclonal antibody natalizumab. Here we report clinical, radiological, and his...

journal_title：Annals of neurology

authors： Schippling S,Kempf C,Büchele F,Jelcic I,Bozinov O,Bont A,Linnebank M,Sospedra M,Weller M,Budka H,Martin R

更新日期：2013-10-01 00:00:00

abstract：:This paper attempts to place into perspective the present state of clinical investigation with PET in neurology. To this end we briefly review the work of the neurology group of the MRC Cyclotron Unit at Hammersmith Hospital, by reference to studies performed in normal subjects and in patients with cerebrovascular dis...

journal_title：Annals of neurology

authors： Frackowiak RS,Wise RJ,Gibbs JM,Jones T

更新日期：1984-01-01 00:00:00

abstract：OBJECTIVE:To develop RNA splicing biomarkers of disease severity and therapeutic response in myotonic dystrophy type 1 (DM1) and type 2 (DM2). METHODS:In a discovery cohort, we used microarrays to perform global analysis of alternative splicing in DM1 and DM2. The newly identified splicing changes were combined with p...

journal_title：Annals of neurology

authors： Nakamori M,Sobczak K,Puwanant A,Welle S,Eichinger K,Pandya S,Dekdebrun J,Heatwole CR,McDermott MP,Chen T,Cline M,Tawil R,Osborne RJ,Wheeler TM,Swanson MS,Moxley RT 3rd,Thornton CA

更新日期：2013-12-01 00:00:00

abstract：:A 45-year-old woman was diagnosed as having the unclassified form of botulism. Her intestines may have been predisposed to colonization with Clostridium botulinum because of a jejunoileal bypass procedure that had been done several years earlier. One other similar case has been reported. ...

journal_title：Annals of neurology

authors： Freedman M,Armstrong RM,Killian JM,Boland D

更新日期：1986-11-01 00:00:00

abstract：:A prospective clinical and biochemical study on the effects of treatment with haloperidol has been performed in seven patients with Tourette syndrome. Pretreatment cerebrospinal fluid levels of homovanillic acid (CSF HVA) were significantly reduce in all patients, whereas 5-hydroxyindoleacetic acid was reduced in only...

journal_title：Annals of neurology

authors： Singer HS,Butler IJ,Tune LE,Seifert WE Jr,Coyle JT

更新日期：1982-10-01 00:00:00

abstract：OBJECTIVE:The risk for symptomatic intracerebral hemorrhage (sICH) associated with thrombolytic treatment has not been evaluated in large studies using diffusion-weighted imaging (DWI). Here, we investigated the relation between pretreatment DWI lesion size and the risk for sICH after thrombolysis. METHODS:In this ret...

journal_title：Annals of neurology

authors： Singer OC,Humpich MC,Fiehler J,Albers GW,Lansberg MG,Kastrup A,Rovira A,Liebeskind DS,Gass A,Rosso C,Derex L,Kim JS,Neumann-Haefelin T,MR Stroke Study Group Investigators.

更新日期：2008-01-01 00:00:00

abstract：:To explore the concept that dystonia may result from dysfunction of the sensory system, 14 patients with focal hand dystonia were tested during two somatosensory discrimination tasks. Compared with controls, patients had a higher threshold in a task involving discrimination of two electric stimuli closely related temp...

journal_title：Annals of neurology

authors： Bara-Jimenez W,Shelton P,Sanger TD,Hallett M

更新日期：2000-03-01 00:00:00

abstract：:Four examples of grossly mineralized lesions of the brain, causing seizures, are described; they include 2 hamartomas, a choristoma (novel associations), and an idiopathic "brain stone." Each was detectable with routine roentgenograms, and the seizures were ameliorated by surgical therapy. ...

journal_title：Annals of neurology

authors： Averback P

更新日期：1977-10-01 00:00:00

abstract：:Three patients with inferior branch palsies of the oculomotor nerve are described. Two were under 10 years of age and the third was 30 years old at the onset. All 3 presented with painless diplopia. The onset was sudden in 2 patients and progressive in the third. The palsy cleared within a short time in the 2 patients...

journal_title：Annals of neurology

authors： Susac JO,Hoyt WF

更新日期：1977-10-01 00:00:00

abstract：:Human prion diseases can occur as an idiopathic disorder (sporadic Creutzfeldt-Jakob disease) or can be acquired, as is the case for variant Creutzfeldt-Jakob disease. These disorders are characterized by the accumulation of a protease-resistant form of the host-encoded prion protein termed PrP(Sc) in the brains of af...

journal_title：Annals of neurology

authors： Head MW,Bunn TJ,Bishop MT,McLoughlin V,Lowrie S,McKimmie CS,Williams MC,McCardle L,MacKenzie J,Knight R,Will RG,Ironside JW

更新日期：2004-06-01 00:00:00

abstract：:Intracytoplasmic aggregation of alpha-synuclein protein as Lewy bodies in the brainstem neurons is diagnostic for Parkinson's disease, whereas if this process also occurs in the cortical neurons, it is considered pathognomonic for dementia with Lewy bodies. However, the link between alpha-synuclein incorporation into ...

journal_title：Annals of neurology

authors： Parkkinen L,Kauppinen T,Pirttilä T,Autere JM,Alafuzoff I

更新日期：2005-01-01 00:00:00

abstract：:The authors report on a series of spontaneous intracranial hemorrhages associated with vein of Galen aneurysmal malformations (VGAMs). Thirty-four children with VGAMs have been treated at this institution since 1986. Eight children (24%) harbored the mural-type malformation, and 26 (76%) had the choroidal-type lesion....

journal_title：Annals of neurology

authors： Meyers PM,Halbach VV,Phatouros CP,Dowd CF,Malek AM,Lempert TE,Lefler JE,Higashida RT

更新日期：2000-06-01 00:00:00

abstract：OBJECTIVE:Gray matter (GM) damage and meningeal inflammation have been associated with early disease onset and a more aggressive disease course in multiple sclerosis (MS), but can these changes be identified in the patient early in the disease course? METHODS:To identify possible biomarkers linking meningeal inflammat...

journal_title：Annals of neurology

authors： Magliozzi R,Howell OW,Nicholas R,Cruciani C,Castellaro M,Romualdi C,Rossi S,Pitteri M,Benedetti MD,Gajofatto A,Pizzini FB,Montemezzi S,Rasia S,Capra R,Bertoldo A,Facchiano F,Monaco S,Reynolds R,Calabrese M

更新日期：2018-04-01 00:00:00

abstract：OBJECTIVE:Hemodynamic impairment in one hemisphere has been shown to trigger ipsilateral motor activation in the opposite hemisphere on functional imaging. We hypothesized that reversing the hypoperfusion would normalize the motor activation pattern. METHODS:We studied four patients with high-grade stenosis and impair...

journal_title：Annals of neurology

authors： Chmayssani M,Lazar RM,Hirsch J,Marshall RS

更新日期：2009-02-01 00:00:00

abstract：:Although the carbonic anhydrase inhibitors have been used in the treatment of the primary periodic paralyses (PPs), their efficacy has not been demonstrated in double-blind, placebo-controlled trials. Therefore, we tested the efficacy of dichlorphenamide (DCP; Daranide), a potent carbonic anhydrase inhibitor, in the t...

journal_title：Annals of neurology

authors： Tawil R,McDermott MP,Brown R Jr,Shapiro BC,Ptacek LJ,McManis PG,Dalakas MC,Spector SA,Mendell JR,Hahn AF,Griggs RC

更新日期：2000-01-01 00:00:00

abstract：:This review discusses the etiology and pathogenesis of Parkinson's disease (PD). Mitochondrial respiratory failure and oxidative stress appear to be two major contributors to nigral neuronal death in PD. Complex I deficiency has been reported by several groups and appears to be one of the basic abnormalities responsib...

journal_title：Annals of neurology

authors： Mizuno Y,Yoshino H,Ikebe S,Hattori N,Kobayashi T,Shimoda-Matsubayashi S,Matsumine H,Kondo T

更新日期：1998-09-01 00:00:00

abstract：:To characterize the protein composition of degenerating neurons in Alzheimer disease, enriched fractions of isolated cortical neurons from postmortem Alzheimer brain were compared by ultrastructural and biochemical techniques to neuronal isolates from aged normal controls and from patients with the nonfibrillary degen...

journal_title：Annals of neurology

authors： Selkoe DJ

更新日期：1980-11-01 00:00:00

abstract：:Although the basal ganglia have been implicated in the development of movement disorders since the 1940s, the exact role played by these structures has remained elusive. The development of the 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine-monkey model of parkinsonism, and the recent resurgence of surgical therapy for t...

journal_title：Annals of neurology

authors： Vitek JL,Giroux M

更新日期：2000-04-01 00:00:00

abstract：:Layer II of the entorhinal cortex contains the cells of origin for the perforant path, plays a critical role in memory processing, and consistently degenerates in end-stage Alzheimer's disease. The extent to which neuron loss in layer II of entorhinal cortex is related to mild cognitive impairment without dementia has...

journal_title：Annals of neurology

authors： Kordower JH,Chu Y,Stebbins GT,DeKosky ST,Cochran EJ,Bennett D,Mufson EJ

更新日期：2001-02-01 00:00:00

abstract：:Hereditary canine spinal muscular atrophy (HCSMA) features rapidly progressive muscle weakness that affects muscles in an apparent proximal-to-distal gradient. In the medial gastrocnemius (MG) muscle of homozygous HCSMA animals, motor unit tetanic failure is apparent before the appearance of muscle weakness and appear...

journal_title：Annals of neurology

authors： Balice-Gordon RJ,Smith DB,Goldman J,Cork LC,Shirley A,Cope TC,Pinter MJ

更新日期：2000-05-01 00:00:00