Peripheral neuropathy in classic Niemann-Pick disease: ultrastructure of nerves and skeletal muscles.

abstract：:The presence of an APOE epsilon4 allele may be a risk factor for neuropathy severity in diabetes. The authors assessed the frequency of APOE epsilon4 in patients presenting with sensory predominant neuropathy. APOE epsilon4 frequency among patients with early diabetic neuropathy and impaired glucose tolerance-associat...

journal_title：Neurology

authors： Zhou Z,Hoke A,Cornblath DR,Griffin JW,Polydefkis M

更新日期：2005-01-11 00:00:00

abstract：BACKGROUND:Although hospital-outcome relationships have been explored for a variety of procedures and interventions, little is known about the association between annual stroke admission volumes and stroke mortality. Our aim was to determine whether facility type and hospital volume was associated with stroke mortality...

journal_title：Neurology

authors： Saposnik G,Baibergenova A,O'Donnell M,Hill MD,Kapral MK,Hachinski V,Stroke Outcome Research Canada (SORCan) Working Group.

更新日期：2007-09-11 00:00:00

abstract：OBJECTIVE:To investigate the feasibility, safety, and efficacy of a ketogenic diet (KD) for superrefractory status epilepticus (SRSE) in adults. METHODS:We performed a prospective multicenter study of patients 18 to 80 years of age with SRSE treated with a KD treatment algorithm. The primary outcome measure was signif...

journal_title：Neurology

authors： Cervenka MC,Hocker S,Koenig M,Bar B,Henry-Barron B,Kossoff EH,Hartman AL,Probasco JC,Benavides DR,Venkatesan A,Hagen EC,Dittrich D,Stern T,Radzik B,Depew M,Caserta FM,Nyquist P,Kaplan PW,Geocadin RG

更新日期：2017-03-07 00:00:00

abstract：OBJECTIVE:To assess the effects of the modified amino acid acetyl-dl-leucine (AL) on cerebellar ataxia, eye movements, and quality of life of patients with Niemann-Pick type C (NP-C) disease. METHODS:Twelve patients with NP-C disease were treated with AL 3 g/d for 1 week and then with 5 g/d for 3 weeks with a subseque...

journal_title：Neurology

authors： Bremova T,Malinová V,Amraoui Y,Mengel E,Reinke J,Kolníková M,Strupp M

更新日期：2015-10-20 00:00:00

abstract：:Stuttering is an action-induced speech disorder with involuntary, audible, or silent repetitions or prolongations in the utterance of short speech elements (sounds, syllables) and words. Symptomatic treatment programs frequently have initial success; persistent benefit is variable and many patients remain disabled. St...

journal_title：Neurology

authors： Brin MF,Stewart C,Blitzer A,Diamond B

更新日期：1994-12-01 00:00:00

abstract：OBJECTIVE:To determine whether groups of surrogates for patients with severe acute brain injury (SABI) with poor prognosis can be identified based on their prioritization of goals-of-care (GOC) decisional concerns, an online survey of 1,588 adults recruited via a probability-based panel representative of the US populat...

journal_title：Neurology

authors： Hwang DY,Knies AK,Mampre D,Kolenikov S,Schalk M,Hammer H,White DB,Holloway RG,Sheth KN,Fraenkel L

更新日期：2020-05-12 00:00:00

abstract：:Zinc is essential for normal embryogenesis and may have particular importance for closure of the human neural tube. Compared to 258 controls, we found increased zinc content in umbilical cord serum in eight of nine newborn anencephalics (p less than 0.02) and three infants with spina bifida (p less than 0.001). Increa...

journal_title：Neurology

authors： Zimmerman AW

更新日期：1984-04-01 00:00:00

abstract：OBJECTIVES:Determine the frequency of and the predictive factors for abnormal ocular fundus findings among emergency department (ED) headache patients. METHODS:Cross-sectional study of prospectively enrolled adult patients presenting to our ED with a chief complaint of headache. Ocular fundus photographs were obtained...

journal_title：Neurology

authors： Thulasi P,Fraser CL,Biousse V,Wright DW,Newman NJ,Bruce BB

更新日期：2013-01-29 00:00:00

abstract：INTRODUCTION:As clinical trials move toward earlier intervention, we sought to redefine the β-amyloid (Aβ)-PET threshold based on the lowest point in a baseline distribution that robustly predicts future Aβ accumulation and cognitive decline in 3 independent samples of clinically normal individuals. METHODS:Sequential...

journal_title：Neurology

authors： Farrell ME,Jiang S,Schultz AP,Properzi MJ,Price JC,Becker JA,Jacobs HIL,Hanseeuw BJ,Rentz DM,Villemagne VL,Papp KV,Mormino EC,Betensky RA,Johnson KA,Sperling RA,Buckley RF,Alzheimer's Disease Neuroimaging Initiative and t

更新日期：2021-01-26 00:00:00

abstract：:An emerging issue in stroke prevention and stroke treatment is that therapies shown to be effective in clinical trials are not being used or are being used suboptimally. One of many explanations relates to cost, ranging from concerns about inappropriate use of societal resources to more immediate concerns of organizat...

journal_title：Neurology

authors： Matchar DB

更新日期：1998-09-01 00:00:00

abstract：:We studied lymphocyte subsets and major histocompatibility complex (MHC) class II antigens in frozen brain tissues from three patients with subacute sclerosing panencephalitis (SSPE) using immunocytochemical techniques. Perivascular cuffs consisted predominantly of T cells and MHC class II-positive cells. In the paren...

journal_title：Neurology

authors： Nagano I,Nakamura S,Yoshioka M,Kogure K

更新日期：1991-10-01 00:00:00

abstract：OBJECTIVE:To develop a scientifically sound and clinically relevant evidence-based guideline for the treatment of painful diabetic neuropathy (PDN). METHODS:We performed a systematic review of the literature from 1960 to August 2008 and classified the studies according to the American Academy of Neurology classificati...

journal_title：Neurology

authors： Bril V,England J,Franklin GM,Backonja M,Cohen J,Del Toro D,Feldman E,Iverson DJ,Perkins B,Russell JW,Zochodne D,American Academy of Neurology.,American Association of Neuromuscular and Electrodiagnostic Medicine.,American Acade

更新日期：2011-05-17 00:00:00

abstract：:Hirayama disease is a nonprogressive, asymmetric amyotrophy of the hands and forearms, possibly caused by compression of the lower cervical cord on neck flexion. The authors used phase-contrast MR angiography to study a patient with this disorder and observed abnormal spinal epidural venous dilation on neck flexion. I...

journal_title：Neurology

authors： Baba Y,Nakajima M,Utsunomiya H,Tsuboi Y,Fujiki F,Kusuhara T,Yamada T

更新日期：2004-04-27 00:00:00

abstract：OBJECTIVE:To evaluate patients with early HIV-cognitive motor complex (HIV-CMC) for possible regional cerebral blood flow (rCBF) abnormalities on perfusion MRI (pMRI). BACKGROUND:Nuclear medicine techniques have demonstrated global and focal cerebral perfusion abnormalities in patients with HIV dementia. Ultrafast pMR...

journal_title：Neurology

authors： Chang L,Ernst T,Leonido-Yee M,Speck O

更新日期：2000-01-25 00:00:00

abstract：:Extracranial and intracranial angioplasty and stenting of the cerebral vessels are being performed more frequently. One clinical trial demonstrated equivalent outcomes between extracranial carotid angioplasty and carotid endarterectomy, but the results in both groups were suboptimal. Concerns remain about the iatrogen...

journal_title：Neurology

authors： Chaturvedi S,Fessler R

更新日期：2002-09-10 00:00:00

abstract：:Primary deficiency of merosin is the cause of the classic form of congenital muscular dystrophy (CMD) accompanied by brain white matter abnormalities. We report a female infant with dystrophinopathy who was deficient in merosin in skeletal muscle. The patient had a phenotype of typical CMD and white matter abnormaliti...

journal_title：Neurology

authors： Tachi N,Ohya K,Chiba S,Matsuo M,Patria SY,Matsumura K

更新日期：1997-08-01 00:00:00

abstract：OBJECTIVE:To quantify the effects of cognitive training (CT) on cognitive and behavioral outcome measures in patients with Parkinson disease (PD). METHODS:We systematically searched 5 databases for randomized controlled trials (RCTs) of CT in patients with PD reporting cognitive or behavioral outcomes. Efficacy was me...

journal_title：Neurology

authors： Leung IH,Walton CC,Hallock H,Lewis SJ,Valenzuela M,Lampit A

更新日期：2015-11-24 00:00:00

abstract：:Gamma hydroxybutyrate was administered to adult cats by intravenous infusion at varying dosages while an electroencephalogram (EEG) was recorded from electrodes placed stereotactically in the right and left hippocampus and thalamic intralaminar nucleus and from cortical electrodes. Blood cerebrospinal fluid samples we...

journal_title：Neurology

authors： Snead OC 3rd,Yu RK,Huttenlocher PR

更新日期：1976-01-01 00:00:00

abstract：:In the same family, the generalized or infantile form of acid maltase deficiency (glycogenosis type II, Pompe disease) and the muscular or adult-onset form affected different individuals. Autosomal-recessive inheritance for the two clinical forms was demonstrated in this family by assay of acid alpha-glucosidase in mu...

journal_title：Neurology

authors： Loonen MC,Busch HF,Koster JF,Martin JJ,Niermeijer MF,Schram AW,Brouwer-Kelder B,Mekes W,Slee RG,Tager JM

更新日期：1981-10-01 00:00:00

abstract：:Neurologic complications of both the central and peripheral nervous systems occur frequently in patients with primary Sjögren's syndrome (primary SS), but the underlying cause of these complications is unknown. We studied the presence of antineuronal antibodies in relation to neurologic complications in a consecutive ...

journal_title：Neurology

authors： Moll JW,Markusse HM,Pijnenburg JJ,Vecht CJ,Henzen-Logmans SC

更新日期：1993-12-01 00:00:00

abstract：BACKGROUND:Krabbe disease (globoid cell leukodystrophy [GLD]) is an autosomal recessive lysosomal disorder affecting the central and peripheral nervous system. The authors performed MRS to characterize metabolic alterations and their regional variation in brain tissue in GLD in vivo. METHODS:Abnormalities of cerebral ...

journal_title：Neurology

authors： Brockmann K,Dechent P,Wilken B,Rusch O,Frahm J,Hanefeld F

更新日期：2003-03-11 00:00:00

abstract：OBJECTIVE:To examine metabolism during exercise in 2 patients with muscle phosphorylase kinase (PHK) deficiency and to further define the phenotype of this rare glycogen storage disease (GSD). METHODS:Patient 1 (39 years old) had mild exercise-induced forearm pain, and EMG showed a myopathic pattern. Patient 2 (69 yea...

journal_title：Neurology

authors： Preisler N,Orngreen MC,Echaniz-Laguna A,Laforet P,Lonsdorfer-Wolf E,Doutreleau S,Geny B,Akman HO,Dimauro S,Vissing J

更新日期：2012-01-24 00:00:00

abstract：:About one-third of patients with acoustic tumor (AT) initially seek medical attention for nonaudiologic complaints. The nonspecific early symptoms of AT require the inclusion of AT in many neurologic differential diagnoses. Advances in electrophysiologic and radiographic tests have allowed earlier diagnosis of AT at a...

journal_title：Neurology

authors： Hart RG,Gardner DP,Howieson J

更新日期：1983-02-01 00:00:00

abstract：OBJECTIVE:To conduct a systematic review on the association between tetanus vaccination and the risk of multiple sclerosis (MS). METHODS:The authors searched the databases Medline, LILACS, EMBASE, and Science Citation Index including the period 1966 to September 1, 2005. Eligible studies had to meet the following incl...

journal_title：Neurology

authors： Hernán MA,Alonso A,Hernández-Díaz S

更新日期：2006-07-25 00:00:00

abstract：:Although people with migraine are aware of their headaches they are often not aware that they have migraine. This can lead to inappropriate or ineffective use of treatments, delays in seeking appropriate care, and miscommunication during clinical encounters. This article assesses self-recognition of migraine and ident...

journal_title：Neurology

authors： Lipton RB,Stewart WF,Liberman JN

更新日期：2002-05-14 00:00:00

abstract：:The intracarotid amobarbital test (IAT) examines hemispheric memory and language. We set out to determine whether memory performance on the IAT correlated with seizure relief after anterior temporal lobectomy in 117 patients with refractory epilepsy. The IAT assessed recognition memory performance for nine items with ...

journal_title：Neurology

authors： Sperling MR,Saykin AJ,Glosser G,Moran M,French JA,Brooks M,O'Connor MJ

更新日期：1994-12-01 00:00:00

abstract：OBJECTIVE:To examine the diagnostic accuracy of nerve ultrasound in a prospective cohort of consecutive patients with a clinical suspicion of chronic inflammatory neuropathies, including chronic inflammatory demyelinating polyneuropathy, Lewis-Sumner syndrome, and multifocal motor neuropathy, and to determine the added...

journal_title：Neurology

authors： Herraets IJT,Goedee HS,Telleman JA,van Eijk RPA,van Asseldonk JT,Visser LH,van den Berg LH,van der Pol WL

更新日期：2020-04-07 00:00:00

abstract：:Current medical educational theory encourages the development of competency-based curricula. The Accreditation Council for Graduate Medical Education's 6 core competencies for resident education (medical knowledge, patient care, professionalism, interpersonal and communication skills, practice-based learning, and syst...

journal_title：Neurology

authors： Merlin LR,Horak HA,Milligan TA,Kraakevik JA,Ali II

更新日期：2014-07-29 00:00:00

abstract：:Ninety-seven chronic alcoholics, both impaired and unimpaired, without clinically evident liver disease, showed significantly more cerebral atrophy on computed tomography than age-matched neurologic controls. Age was the variable most highly correlated with cerebral atrophy measurements, and it accounted for most of t...

journal_title：Neurology

authors： Carlen PL,Wilkinson DA,Wortzman G,Holgate R,Cordingley J,Lee MA,Huszar L,Moddel G,Singh R,Kiraly L,Rankin JG

更新日期：1981-04-01 00:00:00

abstract：:We reviewed the clinical and laboratory features of 81 patients who had trigeminal sensory neuropathy (TSN) and a connective tissue disease (CTD). The neuropathy developed before the symptoms of CTD in 6/81 patients (7%), and in 38/81 patients (47%) TSN and CTD were diagnosed concurrently. The most frequently associat...

journal_title：Neurology

authors： Hagen NA,Stevens JC,Michet CJ Jr

更新日期：1990-06-01 00:00:00