Acute hydrocephalus in nonketotic hyperglycinemia.

abstract：:The authors identified a novel mtDNA mutation (T9176G) in the ATPase 6 gene in a family in which a 10-year-old girl had a severe neurodegenerative disorder, her elder sister had died of Leigh syndrome (LS), and a maternal uncle had a spinocerebellar disorder. Biochemical studies disclosed a reduced rate of ATP synthes...

journal_title：Neurology

authors： Carrozzo R,Tessa A,Vázquez-Memije ME,Piemonte F,Patrono C,Malandrini A,Dionisi-Vici C,Vilarinho L,Villanova M,Schägger H,Federico A,Bertini E,Santorelli FM

更新日期：2001-03-13 00:00:00

abstract：:This review summarizes evidence, primarily from recent human studies, indirectly supporting a novel hypothesis: that the assessment of healthy individuals' responses to standardized noxious stimuli in a controlled laboratory environment has important implications for the later risk of developing a broad spectrum of ch...

journal_title：Neurology

authors： Edwards RR

更新日期：2005-08-09 00:00:00

abstract：OBJECTIVE:To determine the usefulness of the 14-3-3 test in patients with dementia of various causes. BACKGROUND:Recent reports have suggested that the detection of the 14-3-3 protein in the CSF of patients with Creutzfeldt--Jakob disease is a highly sensitive and specific marker of the disease that might be used as a...

journal_title：Neurology

authors： Burkhard PR,Sanchez JC,Landis T,Hochstrasser DF

更新日期：2001-06-12 00:00:00

abstract：OBJECTIVES:SCN1A mutations were recently reported in several patients with severe myoclonic epilepsy in infancy (SMEI). The authors analyzed SCN1A mutations in 93 patients with SMEI and made genotype-phenotype correlation to clarify the role of this gene in the etiology of SMEI. METHODS:All patients fulfilled the crit...

journal_title：Neurology

authors： Nabbout R,Gennaro E,Dalla Bernardina B,Dulac O,Madia F,Bertini E,Capovilla G,Chiron C,Cristofori G,Elia M,Fontana E,Gaggero R,Granata T,Guerrini R,Loi M,La Selva L,Lispi ML,Matricardi A,Romeo A,Tzolas V,Valseriati

更新日期：2003-06-24 00:00:00

abstract：:We followed 21 patients with sensory neuronopathy without evidence of cancer for up to 23 years. All were seronegative for type 1 antineuronal nuclear antibodies (ANNA-1, also called "anti-Hu"). We additionally studied 67 seropositive patients with sensory neuropathy or a related neurologic syndrome. Ninety-one percen...

journal_title：Neurology

authors： Chalk CH,Lennon VA,Stevens JC,Windebank AJ

更新日期：1993-11-01 00:00:00

abstract：OBJECTIVE:To determine the prevalence and incidence of epilepsy among U.S. Medicare beneficiaries aged 65 years old and over, and to compare rates across demographic groups. METHODS:We performed a retrospective analysis of Medicare administrative claims for 2001-2005, defining prevalent cases as persons with ≥1 claim ...

journal_title：Neurology

authors： Faught E,Richman J,Martin R,Funkhouser E,Foushee R,Kratt P,Kim Y,Clements K,Cohen N,Adoboe D,Knowlton R,Pisu M

更新日期：2012-02-14 00:00:00

abstract：OBJECTIVE:To investigate the role of intragenic deletions of ALDH7A1 in patients with clinical and biochemical evidence of pyridoxine-dependent epilepsy but only a single identifiable mutation in ALDH7A1. METHODS:We designed a custom oligonucleotide array with high-density probe coverage across the ALDH7A1 gene. We pe...

journal_title：Neurology

authors： Mefford HC,Zemel M,Geraghty E,Cook J,Clayton PT,Paul K,Plecko B,Mills PB,Nordli DR Jr,Gospe SM Jr

更新日期：2015-09-01 00:00:00

abstract：:We studied the interrater variability among three neurologists of the diagnosis of a seizure in 100 patients evaluated for a possible "first seizure." We found that use of simple descriptive diagnostic criteria and discussion among the neurologists themselves improved the diagnostic agreement. ...

journal_title：Neurology

authors： van Donselaar CA,Geerts AT,Meulstee J,Habbema JD,Staal A

更新日期：1989-02-01 00:00:00

abstract：OBJECTIVE:To investigate whether tactile extinction alters the cortical somatosensory activations induced by hand vibration. BACKGROUND:Tactile extinction occurs mainly after right-brain lesions and consists of the inability to perceive a contralesional cutaneous stimulation when a similar stimulus is applied to the m...

journal_title：Neurology

authors： Remy P,Zilbovicius M,Degos JD,Bachoud-Lévi AC,Rancurel G,Cesaro P,Samson Y

更新日期：1999-02-01 00:00:00

abstract：OBJECTIVE:To assess the frequency of mutations in C19orf12 in the greater neurodegeneration with brain iron accumulation (NBIA) population and further characterize the associated phenotype. METHODS:Samples from 161 individuals with idiopathic NBIA were screened, and C19orf12 mutations were identified in 23 subjects. D...

journal_title：Neurology

authors： Hogarth P,Gregory A,Kruer MC,Sanford L,Wagoner W,Natowicz MR,Egel RT,Subramony SH,Goldman JG,Berry-Kravis E,Foulds NC,Hammans SR,Desguerre I,Rodriguez D,Wilson C,Diedrich A,Green S,Tran H,Reese L,Woltjer RL,Hayfli

更新日期：2013-01-15 00:00:00

abstract：:To test the safety and efficacy of recombinant human insulin-like growth factor-I (rhIGF-I) in ALS, 183 patients from eight European centers were randomized to receive double-blind placebo (n = 59) or rhIGF-I 0.1 mg/kg/day (n = 124) subcutaneously for 9 months. At study completion, the primary efficacy outcome measure...

journal_title：Neurology

authors： Borasio GD,Robberecht W,Leigh PN,Emile J,Guiloff RJ,Jerusalem F,Silani V,Vos PE,Wokke JH,Dobbins T

更新日期：1998-08-01 00:00:00

abstract：OBJECTIVE:To determine the prevalence, incidence, and predictors of epilepsy among older adults in the Cardiovascular Health Study (CHS). METHODS:We analyzed data prospectively collected in CHS and merged with data from outpatient Medicare administrative claims. We identified cases with epilepsy using self-report, ant...

journal_title：Neurology

authors： Choi H,Pack A,Elkind MS,Longstreth WT Jr,Ton TG,Onchiri F

更新日期：2017-02-28 00:00:00

abstract：:In a double-blind, placebo-controlled, parallel-group, randomized clinical trial, we studied the efficacy of long-term (1-year) oral treatment with acetyl-L-carnitine in 130 patients with a clinical diagnosis of Alzheimer's disease. We employed 14 outcome measures to assess functional and cognitive impairment. After 1...

journal_title：Neurology

authors： Spagnoli A,Lucca U,Menasce G,Bandera L,Cizza G,Forloni G,Tettamanti M,Frattura L,Tiraboschi P,Comelli M

更新日期：1991-11-01 00:00:00

abstract：OBJECTIVE:Optical coherence tomography (OCT) noninvasively quantifies retinal nerve fiber layer (RNFL) thickness. Studies show RNFL thinning in multiple sclerosis (MS), and we assessed its association with brain atrophy. METHODS:RNFL thickness was measured in 40 patients with MS and 15 controls. Brain parenchymal frac...

journal_title：Neurology

authors： Gordon-Lipkin E,Chodkowski B,Reich DS,Smith SA,Pulicken M,Balcer LJ,Frohman EM,Cutter G,Calabresi PA

更新日期：2007-10-16 00:00:00

abstract：:Relating the onset of clinical attacks as recorded on a videotape to surface and depth electrographic events improves our ability to define the local or diffuse origin of seizures. In two patients with complex partial seizures, all 14 attacks appeared 3 to 6 seconds after focal discharges began in the amygdala or hipp...

journal_title：Neurology

authors： Delgado-Escueta AV,Nashold B,Freedman M,Keplinger C,Waddell G,Miller P,Carwille S

更新日期：1979-04-01 00:00:00

abstract：:Acute chorea in a child followed ingestion of pemoline mesylate. In guinea pigs, in an experimental model of chorea, chronic administration of pemoline induced behavioral supersensitivity to other dopaminergic agonists. Pemoline is similar to both d-amphetamine and methylphenidate in altering central dopaminergic sens...

journal_title：Neurology

authors： Nausieda PA,Koller WC,Weiner WJ,Klawans HL

更新日期：1981-03-01 00:00:00

abstract：BACKGROUND:Chronic cerebrospinal venous insufficiency (CCSVI) was proposed as the causal trigger for developing multiple sclerosis (MS). However, current data are contradictory and a gold standard for venous flow assessment is missing. OBJECTIVE:To compare structural magnetic resonance venography (MRV) and dynamic ext...

journal_title：Neurology

authors： Doepp F,Würfel JT,Pfueller CF,Valdueza JM,Petersen D,Paul F,Schreiber SJ

更新日期：2011-11-08 00:00:00

abstract：:The position of the auditory pathways in the human corpus callosum has not been defined by modern imaging techniques. We report a case with a discrete hemorrhagic lesion in the posterior body of the corpus callosum. The only signs of disturbed callosal function were limited to the auditory system--suppression of left ...

journal_title：Neurology

authors： Alexander MP,Warren RL

更新日期：1988-05-01 00:00:00

abstract：OBJECTIVES/METHODS:To examine evidence for effectiveness of anteromesial temporal lobe and localized neocortical resections for disabling complex partial seizures by systematic review and analysis of the literature since 1990. RESULTS:One intention-to-treat Class I randomized, controlled trial of surgery for mesial te...

journal_title：Neurology

authors： Engel J Jr,Wiebe S,French J,Sperling M,Williamson P,Spencer D,Gumnit R,Zahn C,Westbrook E,Enos B,Quality Standards Subcommittee of the American Academy of Neurology.,American Epilepsy Society.,American Association of Neurological

更新日期：2003-02-25 00:00:00

abstract：OBJECTIVE:To study the presenting features and value of routine diagnostic procedures in patients with leptomeningeal metastases (LMM) related to the primary malignancy to improve diagnostic assessment. METHODS:The authors studied the presenting features and value of routine diagnostic procedures in relation to the hi...

journal_title：Neurology

authors： van Oostenbrugge RJ,Twijnstra A

更新日期：1999-07-22 00:00:00

abstract：OBJECTIVE:To detect and quantify lesions of the small-caliber sural nerve (SN) in symptomatic and asymptomatic transthyretin familial amyloid polyneuropathy (TTR-FAP) by high-resolution magnetic resonance neurography (MRN) in correlation with electrophysiologic and histopathologic findings. METHODS:Twenty-five patient...

journal_title：Neurology

authors： Kollmer J,Sahm F,Hegenbart U,Purrucker JC,Kimmich C,Schönland SO,Hund E,Heiland S,Hayes JM,Kristen AV,Röcken C,Pham M,Bendszus M,Weiler M

更新日期：2017-08-01 00:00:00

abstract：:In the first half of the 19th century, European physicians-including Marshall Hall, Moritz Romberg, and Bernardus Brach-described loss of postural control in darkness of patients with severely compromised proprioception. Romberg and Brach emphasized the relationship between this sign and tabes dorsalis. Later, other n...

journal_title：Neurology

authors： Lanska DJ,Goetz CG

更新日期：2000-10-24 00:00:00

abstract：OBJECTIVE:To discuss the indications and timing for resective surgery in patients with Sturge-Weber syndrome (SWS) and medication-resistant epilepsy. BACKGROUND:SWS that causes epilepsy severe enough to merit surgery is rare. Because of the variable natural history of the disease, it is difficult to establish clear-cu...

journal_title：Neurology

authors： Arzimanoglou AA,Andermann F,Aicardi J,Sainte-Rose C,Beaulieu MA,Villemure JG,Olivier A,Rasmussen T

更新日期：2000-11-28 00:00:00

abstract：OBJECTIVE:To characterize patterns of cognitive functioning in a well-defined group of patients with MRI-identified coexisting left temporal lobe developmental malformations (TLDM) and mesial temporal sclerosis (MTS), and to examine neuropsychological outcome in this dual-pathology group following epilepsy surgery. ME...

journal_title：Neurology

authors： Martin R,Dowler R,Gilliam F,Faught E,Morawetz R,Kuzniecky R

更新日期：1999-09-11 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：:A subset of human B lymphocytes expresses Leu-1 (CD5), a pan-T cell marker, which is the equivalent of the murine Lyt-1 molecule. CD5+ B cells produce autoantibodies in vitro; therefore, they may play a role in the pathogenesis of autoimmune disorders. In myasthenia gravis (MG), autoantibodies are directed against the...

journal_title：Neurology

authors： Ragheb S,Lisak RP

更新日期：1990-07-01 00:00:00

abstract：BACKGROUND:Given that vagal afferents project to brainstem regions that promote alertness, the authors tested the hypothesis that vagus nerve stimulation (VNS) would improve daytime sleepiness in patients with epilepsy. METHODS:Sixteen subjects with medically refractory seizures underwent polysomnography and multiple ...

journal_title：Neurology

authors： Malow BA,Edwards J,Marzec M,Sagher O,Ross D,Fromes G

更新日期：2001-09-11 00:00:00

abstract：OBJECTIVES:To define CSF β-amyloid 1-42 (Aβ42) cutpoints to detect cortical amyloid deposition as assessed by 11C-Pittsburgh compound B ([11C]PiB)-PET and to compare these calculated cutpoints with cutpoints currently used in clinical practice. METHODS:We included 433 participants (57 controls, 99 with mild cognitive ...

journal_title：Neurology

authors： Zwan MD,Rinne JO,Hasselbalch SG,Nordberg A,Lleó A,Herukka SK,Soininen H,Law I,Bahl JM,Carter SF,Fortea J,Blesa R,Teunissen CE,Bouwman FH,van Berckel BN,Visser PJ

更新日期：2016-01-05 00:00:00

abstract：:Neurologic complications of brain tissue-derived rabies vaccine may be chronic or progressive. Lymphocytes and serum from three patients with this form of encephalitis were studied. Two patients had positive lymphoproliferation to purified myelin, two had antibody to white matter, and one had antibody to myelin basic ...

journal_title：Neurology

authors： Hemachudha T,Griffin DE,Johnson RT,Giffels JJ

更新日期：1988-01-01 00:00:00

abstract：OBJECTIVE:To compare nonadherence to statins in older black and white adults following an ischemic stroke. METHODS:We studied black and white adults ≥66 years of age with Medicare fee-for-service insurance coverage hospitalized for ischemic stroke from 2007 to 2012 who filled a statin prescription within 30 days follo...

journal_title：Neurology

authors： Albright KC,Zhao H,Blackburn J,Limdi NA,Beasley TM,Howard G,Bittner V,Howard VJ,Muntner P

更新日期：2017-05-09 00:00:00