In vivo gene therapy for pyridoxine-induced neuropathy by herpes simplex virus-mediated gene transfer of neurotrophin-3.

abstract：:A child with neurofibromatosis exhibited gigantism and acromegaly in association with a hypothalamic mass lesion. Bromocriptine, 5 mg daily, reduced somatic growth rate and restored biochemical homeostasis but had no effect on tumor growth. Radiation therapy arrested tumor enlargement and stabilized deteriorating visu...

journal_title：Annals of neurology

authors： Duchowny MS,Katz R,Bejar RL

更新日期：1984-03-01 00:00:00

abstract：:Antibody against acetylcholine receptor (AChR) of human skeletal muscle was measured using enzyme-linked immunosorbent assay and found in 23 (74%) of 31 Japanese patients with generalized myasthenia gravis. In 15 patients with generalized myasthenia gravis who had not undergone thymectomy and who were not receiving ad...

journal_title：Annals of neurology

authors： Kawanami S,Tsuji R,Oda K

更新日期：1984-02-01 00:00:00

abstract：OBJECTIVE:Recanalization of arterial obstruction is associated with improved clinical outcomes. There are no controlled data demonstrating whether arterial obstruction status predicts the treatment effect of intravenous (IV) tissue plasminogen activator (tPA). We aimed to determine if the presence of arterial obstructi...

journal_title：Annals of neurology

authors： De Silva DA,Churilov L,Olivot JM,Christensen S,Lansberg MG,Mlynash M,Campbell BC,Desmond P,Straka M,Bammer R,Albers GW,Davis SM,Donnan GA,EPITHET-DEFUSE Investigators.

更新日期：2011-10-01 00:00:00

abstract：OBJECTIVE:To determine the presence and kinetics of antibodies against synaptic proteins in patients with herpes simplex virus encephalitis (HSE). METHODS:Retrospective analysis of 44 patients with polymerase chain reaction-proven HSE for the presence of a large panel of onconeuronal and synaptic receptor antibodies. ...

journal_title：Annals of neurology

authors： Prüss H,Finke C,Höltje M,Hofmann J,Klingbeil C,Probst C,Borowski K,Ahnert-Hilger G,Harms L,Schwab JM,Ploner CJ,Komorowski L,Stoecker W,Dalmau J,Wandinger KP

更新日期：2012-12-01 00:00:00

abstract：:The frequency with which patients presenting with acute or chronic noncompressive cord syndromes subsequently develop multiple sclerosis is uncertain. Magnetic resonance imaging (MRI) was performed on 121 patients with such syndromes to determine the frequency of asymptomatic brain lesions and to assess the sensitivit...

journal_title：Annals of neurology

authors： Miller DH,McDonald WI,Blumhardt LD,du Boulay GH,Halliday AM,Johnson G,Kendall BE,Kingsley DP,MacManus DG,Moseley IF

更新日期：1987-12-01 00:00:00

abstract：:Although the static encephalopathies of childhood are common, little is known of the underlying alterations in the neurochemical anatomy of the brain. Using quantitative morphological techniques, we examined the effect of perinatal hypoxia-ischemia on the number and distribution of cholinergic (ACh) neurons in a unila...

journal_title：Annals of neurology

authors： Burke RE,Karanas AL

更新日期：1990-01-01 00:00:00

abstract：OBJECTIVE:Neonatal white matter injury (NWMI) is a lesion found in preterm infants that can lead to cerebral palsy. Although antagonists of bone morphogenetic protein (BMP) signaling, such as Noggin, promote oligodendrocyte precursor cell (OPC) production after hypoxic-ischemic (HI) injury, the downstream functional ta...

journal_title：Annals of neurology

authors： Sabo JK,Heine V,Silbereis JC,Schirmer L,Levison SW,Rowitch DH

更新日期：2017-04-01 00:00:00

abstract：:Juvenile myoclonic epilepsy is a common type of idiopathic generalized epilepsy characterized by myoclonic, generalized tonic-clonic, and in 30% of patients, absence seizures. We studied a three-generation pedigree of 33 members, 10 of whom were clinically affected with juvenile myoclonic epilepsy or presented with su...

journal_title：Annals of neurology

authors： Serratosa JM,Delgado-Escueta AV,Medina MT,Zhang Q,Iranmanesh R,Sparkes RS

更新日期：1996-02-01 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：:Noradrenergic fibers were identified in rat cervical lymph nodes by fluorescence histochemistry and confirmed by radioenzymatic determination of norepinephrine. After superior cervical ganglionectomy, lymph node norepinephrine and noradrenergic fluorescence were strikingly decreased. The alpha-adrenergic radioligand [...

journal_title：Annals of neurology

authors： Giron LT Jr,Crutcher KA,Davis JN

更新日期：1980-11-01 00:00:00

abstract：OBJECTIVE:We compared outcomes after treatment with direct oral anticoagulants (DOACs) and vitamin K antagonists (VKAs) in patients with atrial fibrillation (AF) and a recent cerebral ischemia. METHODS:We conducted an individual patient data analysis of seven prospective cohort studies. We included patients with AF an...

journal_title：Annals of neurology

authors： Seiffge DJ,Paciaroni M,Wilson D,Koga M,Macha K,Cappellari M,Schaedelin S,Shakeshaft C,Takagi M,Tsivgoulis G,Bonetti B,Kallmünzer B,Arihiro S,Alberti A,Polymeris AA,Ambler G,Yoshimura S,Venti M,Bonati LH,Muir KW,Ya

更新日期：2019-06-01 00:00:00

abstract：:A patient with longstanding Mollaret's meningitis had an increased number of circulating natural killer cells during an acute attack, as evidenced by 73% large granular lymphocytes and 80% Leu-11a-positive peripheral mononuclear cells. Numbers of large granular lymphocytes and Leu-11a-positive cells returned to normal...

journal_title：Annals of neurology

authors： Goldstein R,Guberman A,Izaguirre CA,Karsh J

更新日期：1986-09-01 00:00:00

abstract：:We describe the cases of eight patients with chronic idiopathic anhidrosis. These patients were heat intolerant and became hot, flushed, dizzy, dyspneic, and weak but did not sweat when the ambient temperature was high or when they exercised. Four patients had preganglionic sudomotor lesions and in the remaining 4 the...

journal_title：Annals of neurology

authors： Low PA,Fealey RD,Sheps SG,Su WP,Trautmann JC,Kuntz NL

更新日期：1985-09-01 00:00:00

abstract：:A primary haplotype (H1) of the microtubule-associated protein Tau (MAPT) gene is associated with Parkinson's disease (PD). However, the mechanism for disease susceptibility remains unknown. We examined the promoter region of MAPT and identified single nucleotide polymorphisms and insertions of 1 to 11 nucleotides. Th...

journal_title：Annals of neurology

authors： Kwok JB,Teber ET,Loy C,Hallupp M,Nicholson G,Mellick GD,Buchanan DD,Silburn PA,Schofield PR

更新日期：2004-03-01 00:00:00

abstract：OBJECTIVE:The discovery of a posture-dependent effect on the difference between intraocular pressure (IOP) and intracranial pressure (ICP) at the level of lamina cribrosa could have important implications for understanding glaucoma and idiopathic intracranial hypertension and could help explain visual impairments in as...

journal_title：Annals of neurology

authors： Eklund A,Jóhannesson G,Johansson E,Holmlund P,Qvarlander S,Ambarki K,Wåhlin A,Koskinen LO,Malm J

更新日期：2016-08-01 00:00:00

abstract：:In CAG repeat diseases, affected neurons possess many cytoplasmic granules immunopositive for expanded polyglutamine stretches. Electron microscopic immunohistochemistry showed that the granules corresponded to lysosomes of primitive type. The results suggest that, in addition to the ubiquitin/proteasome pathway, muta...

journal_title：Annals of neurology

authors： Yamada M,Tsuji S,Takahashi H

更新日期：2002-10-01 00:00:00

abstract：:There is increasing interest in the potential of dopamine agonists to provide a neuroprotective effect and to alter the natural course of levodopa-treated Parkinson's disease (PD). Theoretically, such a protective effect might derive from (a) a levodopa sparing effect, (b) stimulation dopamine autoreceptors resulting ...

journal_title：Annals of neurology

authors： Olanow CW,Jenner P,Brooks D

更新日期：1998-09-01 00:00:00

abstract：:Six polypeptides resolved by two-dimensional electrophoresis of homogenates from human skeletal muscle have been identified as tropomyosin by electrophoretic and immunochemical methods. The 6 proteins are consistently present in approximately the same abundance in normal biceps, deltoid, gastrocnemius, and quadriceps ...

journal_title：Annals of neurology

authors： Giometti CS,Danon MJ

更新日期：1985-08-01 00:00:00

abstract：:Microelectrode recording methods for stereotactic localization of the subthalamic nucleus (STN) and surrounding structures are described. These methods accurately define targets for chronic deep brain stimulation in the treatment of Parkinson's disease. Mean firing rates and a burst index were determined for all recor...

journal_title：Annals of neurology

authors： Hutchison WD,Allan RJ,Opitz H,Levy R,Dostrovsky JO,Lang AE,Lozano AM

更新日期：1998-10-01 00:00:00

abstract：:We present a patient with hemidystonia and an abnormality of the contralateral basal ganglion seen only with positron emission tomography. A 50-year-old sinistral man suffered minor trauma to the right side of his head and neck. Within 20 minutes he developed paroxysmal intermittent dystonic posturing of his right fac...

journal_title：Annals of neurology

authors： Perlmutter JS,Raichle ME

更新日期：1984-03-01 00:00:00

abstract：:Dichloroacetate (DCA) stimulates pyruvate dehydrogenase complex (PDHC) activity and lowers cerebral lactate concentrations. In the R6/2 and N171-82Q transgenic mouse models of Huntington's disease (HD), DCA significantly increased survival, improved motor function, delayed loss of body weight, attenuated the developme...

journal_title：Annals of neurology

authors： Andreassen OA,Ferrante RJ,Huang HM,Dedeoglu A,Park L,Ferrante KL,Kwon J,Borchelt DR,Ross CA,Gibson GE,Beal MF

更新日期：2001-07-01 00:00:00

abstract：OBJECTIVE:Newer magnetic resonance imaging (MRI) techniques have shown promise in capturing early Parkinson disease (PD)-related changes in the substantia nigra pars compacta (SNc), the key pathological loci. Their translational value, however, is hindered by technical complexity and inconsistent results. METHODS:A no...

journal_title：Annals of neurology

authors： Du G,Lewis MM,Sica C,Kong L,Huang X

更新日期：2019-01-01 00:00:00

abstract：:Dentatorubral-pallidoluysian atrophy is an autosomal dominant neurodegenerative disease characterized by various combinations of ataxia, choreoathetosis, myoclonus, epilepsy, and dementia as well as a wide range of ages at onset. A specific unstable trinucleotide repeat expansion in a gene on the short arm of chromoso...

journal_title：Annals of neurology

authors： Ikeuchi T,Koide R,Tanaka H,Onodera O,Igarashi S,Takahashi H,Kondo R,Ishikawa A,Tomoda A,Miike T

更新日期：1995-06-01 00:00:00

abstract：:This study reports a case of type VII mucopolysaccharidosis (beta-glucuronidase deficiency) presenting as lethal hydrops fetalis. Skin fibroblast cultures established postmortem revealed deficient beta-glucuronidase activity. Mucopolysaccharides were stored in various cells of the brain, heart, kidney, liver, and sple...

journal_title：Annals of neurology

authors： Irani D,Kim HS,El-Hibri H,Dutton RV,Beaudet A,Armstrong D

更新日期：1983-10-01 00:00:00

abstract：:During passive extension of the elbow, the triceps muscle normally shows a burst of electromyographic activity. This shortening reaction (SR) is known to be exaggerated in extrapyramidal disease states, but the effects of cerebellar disease are unknown. The SR was measured in both arms of a patient with hemiataxia, th...

journal_title：Annals of neurology

authors： Angel RW

更新日期：1982-03-01 00:00:00

abstract：:In a patient with acquired immunodeficiency disease syndrome (AIDS) and muscle weakness, a muscle biopsy specimen disclosed degeneration of muscle fibers, regeneration, and focal endomysial mononuclear inflammation. A conspicuous feature was the presence of perivascular macrophages within the endomysium that showed po...

journal_title：Annals of neurology

authors： Chad DA,Smith TW,Blumenfeld A,Fairchild PG,DeGirolami U

更新日期：1990-10-01 00:00:00

abstract：:Light and electron microscopic findings from two sural nerve biopsies obtained at a one-year interval from a patient with the clinical features of Seitelberger's disease are described. Ballooned axons with accumulations of membranous profiles, vesicles, mitochondria, and a homogeneous center were present, and there we...

journal_title：Annals of neurology

authors： Begeer JH,Houthoff HJ,van Weerden TW,de Groot CJ,Blaauw EH,le Coultre R

更新日期：1979-12-01 00:00:00

abstract：:Evidence implicating mitochondrial dysfunction in the central nervous system of patients with sporadic amyotrophic lateral sclerosis (SALS) has recently been accumulating. In contrast, data on mitochondrial function in skeletal muscle in SALS are scarce and controversial. We investigated the in situ properties of musc...

journal_title：Annals of neurology

authors： Echaniz-Laguna A,Zoll J,Ribera F,Tranchant C,Warter JM,Lonsdorfer J,Lampert E

更新日期：2002-11-01 00:00:00

abstract：OBJECTIVE:Mounting evidence links neurodegenerative disorders such as Parkinson disease and Alzheimer disease with mitochondrial dysfunction, and recent emphasis has focused on mitochondrial dynamics and quality control. Mitochondrial dynamics and mtDNA maintenance is another link recently emerged, implicating mutation...

journal_title：Annals of neurology

authors： Carelli V,Musumeci O,Caporali L,Zanna C,La Morgia C,Del Dotto V,Porcelli AM,Rugolo M,Valentino ML,Iommarini L,Maresca A,Barboni P,Carbonelli M,Trombetta C,Valente EM,Patergnani S,Giorgi C,Pinton P,Rizzo G,Tonon C,

更新日期：2015-07-01 00:00:00

abstract：OBJECTIVE:Paroxysmal hemicrania (PH) is a severe, strictly unilateral headache that lasts 2 to 30 minutes, occurs more than five times daily, is associated with trigeminal autonomic symptoms, and is exquisitely responsive to indomethacin. The purpose of the study was to determine the brain structures active in PH. MET...

journal_title：Annals of neurology

authors： Matharu MS,Cohen AS,Frackowiak RS,Goadsby PJ

更新日期：2006-03-01 00:00:00