Prevalence and patterns of cognitive impairment in sporadic ALS.

abstract：OBJECTIVE:To describe a clinical syndrome of cerebellar ataxia associated with muscle coenzyme Q10 (CoQ10) deficiency. BACKGROUND:Muscle CoQ10 deficiency has been reported only in a few patients with a mitochondrial encephalomyopathy characterized by 1) recurrent myoglobinuria; 2) brain involvement (seizures, ataxia, ...

journal_title：Neurology

authors： Musumeci O,Naini A,Slonim AE,Skavin N,Hadjigeorgiou GL,Krawiecki N,Weissman BM,Tsao CY,Mendell JR,Shanske S,De Vivo DC,Hirano M,DiMauro S

更新日期：2001-04-10 00:00:00

abstract：OBJECTIVE:To describe the clinical features of cardiac injury associated with neurogenic pulmonary edema (NPE) in patients with acute subarachnoid hemorrhage (SAH). BACKGROUND:NPE is generally viewed as a form of noncardiogenic pulmonary edema related to massive sympathetic discharge. METHODS:Case series. RESULTS:We...

journal_title：Neurology

authors： Mayer SA,Fink ME,Homma S,Sherman D,LiMandri G,Lennihan L,Solomon RA,Klebanoff LM,Beckford A,Raps EC

更新日期：1994-05-01 00:00:00

abstract：:Subacute necrotizing encephalopathy (SNE) or Leigh's disease is associated with various defects in oxidative phosphorylation (OXPHOS). However, the relationships between these OXPHOS defects and nuclear DNA or mitochondrial DNA (mtDNA) mutations is still unclear. We evaluated three SNE pedigrees (two singleton cases a...

journal_title：Neurology

authors： Shoffner JM,Fernhoff PM,Krawiecki NS,Caplan DB,Holt PJ,Koontz DA,Takei Y,Newman NJ,Ortiz RG,Polak M

更新日期：1992-11-01 00:00:00

abstract：OBJECTIVE:Spontaneous spinal CSF leaks are an important cause of new-onset headaches. Such leaks are reported to be particularly common at the cervico-thoracic junction. The authors undertook a study to determine the significance of these cervico-thoracic CSF leaks. METHODS:The patient population consisted of a consec...

journal_title：Neurology

authors： Schievink WI,Maya MM,Chu RM,Moser FG

更新日期：2015-06-16 00:00:00

abstract：:We evaluated the effects of isoniazid on tremor in 13 patients with MS. Patients were evaluated before treatment, after 1 month of therapy (1,000 mg daily), and 1 month after the last dose. Ten patients improved on at least one of three methods of evaluation. Transient side effects were common, possibly because there ...

journal_title：Neurology

authors： Duquette P,Pleines J,du Souich P

更新日期：1985-12-01 00:00:00

abstract：:From 536 patients with the CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasis), seven were identified as having peripheral neuropathy not attributable to another cause. Peripheral neuropathy developed 0 to 25 years after their first symptoms of scleroderma. Unex...

journal_title：Neurology

authors： Dyck PJ,Hunder GG,Dyck PJ

更新日期：1997-12-01 00:00:00

abstract：:We analyzed our experience with cough, exertional, and vascular sexual headaches, evaluated the interrelationships among them, and examined the possible symptomatic cases. Seventy-two patients consulted us because of headaches precipitated by coughing (n = 30), physical exercise (n = 28), or sexual excitement (n = 14)...

journal_title：Neurology

authors： Pascual J,Iglesias F,Oterino A,Vázquez-Barquero A,Berciano J

更新日期：1996-06-01 00:00:00

abstract：OBJECTIVE:To address the role of cell death regulatory genes of the bcl-2 and caspase families in the neuropathology of human epilepsy using tissue extracted from patients undergoing temporal lobectomy for intractable seizures. METHODS:Using Western blotting and immunohistochemistry, the authors investigated the expre...

journal_title：Neurology

authors： Henshall DC,Clark RS,Adelson PD,Chen M,Watkins SC,Simon RP

更新日期：2000-07-25 00:00:00

abstract：:A 25-year-old man with the skin lesions of malignant atrophic papulosis had clinical and electrodiagnostic evidence of a multifocal asymmetric myelomalacia or polyradiculopathy in association with elevated CSF protein and hypoglycorrhachia. Autopsy findings included widespread infarctions and necrosis of brain, brains...

journal_title：Neurology

authors： Label LS,Tandan R,Albers JW

更新日期：1983-07-01 00:00:00

abstract：OBJECTIVE:To understand the experience and identify drivers and mitigating factors of burnout and well-being among US neurologists. METHODS:Inductive data analysis was applied to free text comments (n = 676) from the 2016 American Academy of Neurology survey of burnout, career satisfaction, and well-being. RESULTS:Re...

journal_title：Neurology

authors： Miyasaki JM,Rheaume C,Gulya L,Ellenstein A,Schwarz HB,Vidic TR,Shanafelt TD,Cascino TL,Keran CM,Busis NA

更新日期：2017-10-17 00:00:00

abstract：OBJECTIVE:To determine the minimum incidence and minimum prevalence rates of small-fiber neuropathy (SFN) in a well-defined region in the southern part of the Netherlands. METHODS:In this cross-sectional study with retrospective data collection, we used data of patients diagnosed with pure SFN at our Small Fiber Neuro...

journal_title：Neurology

authors： Peters MJ,Bakkers M,Merkies IS,Hoeijmakers JG,van Raak EP,Faber CG

更新日期：2013-10-08 00:00:00

abstract：BACKGROUND:Congenital muscular dystrophies (CMD) with reduced glycosylation of alpha-dystroglycan (alpha-DG) are a heterogeneous group of conditions associated with mutations in six genes encoding proven or putative glycosyltransferases. OBJECTIVES:The aim of the study was to establish the prevalence of mutations in t...

journal_title：Neurology

authors： Mercuri E,Messina S,Bruno C,Mora M,Pegoraro E,Comi GP,D'Amico A,Aiello C,Biancheri R,Berardinelli A,Boffi P,Cassandrini D,Laverda A,Moggio M,Morandi L,Moroni I,Pane M,Pezzani R,Pichiecchio A,Pini A,Minetti C,Mon

更新日期：2009-05-26 00:00:00

abstract：OBJECTIVE:To demonstrate noninvasive localization of cognitive cortical areas involved in language processing with magnetoencephalography (MEG) interpreted by multiresolution FOCUSS (MR-FOCUSS), a current density imaging technique. METHOD:MEG data were collected during verb-generation and picture-naming tasks from 18 ...

journal_title：Neurology

authors： Bowyer SM,Moran JE,Mason KM,Constantinou JE,Smith BJ,Barkley GL,Tepley N

更新日期：2004-06-22 00:00:00

abstract：BACKGROUND:The pathogenesis of stroke-like episodes in patients with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) remains unknown. METHODS:Fourteen stroke-like episodes in six patients with MELAS were studied using clinical, neuroradiologic, and electrophysiologic approaches...

journal_title：Neurology

authors： Iizuka T,Sakai F,Suzuki N,Hata T,Tsukahara S,Fukuda M,Takiyama Y

更新日期：2002-09-24 00:00:00

abstract：OBJECTIVE:Quality of neurologic emergency management in an under-resourced country may be improved by standard operating procedures (SOPs). METHODS:Neurologic SOPs were implemented in a large urban (Banjul) and a small rural (Brikama) hospital in the Gambia. As quality indicators of neurologic emergency management, pe...

journal_title：Neurology

authors： Jaiteh LES,Helwig SA,Jagne A,Ragoschke-Schumm A,Sarr C,Walter S,Lesmeister M,Manitz M,Blaß S,Weis S,Schlund V,Bah N,Kauffmann J,Fousse M,Kangankan S,Ramos Cabrera A,Kronfeld K,Ruckes C,Liu Y,Nyan O,Fassbender K

更新日期：2017-07-11 00:00:00

abstract：:The occurrence of Creutzfeldt-Jakob disease (CJD) among American Indians and Alaska Natives in the United States was evaluated using national multiple cause-of-death data and medical information obtained from state health departments. Twelve CJD deaths were identified for 1981 through 2002, and the average annual age-...

journal_title：Neurology

authors： Maddox RA,Holman RC,Belay ED,Cheek JE,Yorita KL,Schonberger LB

更新日期：2006-02-14 00:00:00

abstract：:The prevalence and psychopathologic features of psychiatric adverse events (PAE) in 517 patients taking levetiracetam (LEV) were investigated. Fifty-three (10.1%) patients developed PAE. A significant association was found with previous psychiatric history, history of febrile convulsions, and history of status epilept...

journal_title：Neurology

authors： Mula M,Trimble MR,Yuen A,Liu RS,Sander JW

更新日期：2003-09-09 00:00:00

abstract：:We evaluated 85 patients with serologic evidence of Borrelia burgdorferi infection. Manifestations included encephalopathy (41), neuropathy (27), meningitis (2), multiple sclerosis (MS) (6), and psychiatric disorders (3). We performed lumbar punctures in 53, brain MRI in 33, and evoked potentials (EPs) in 33. Only pat...

journal_title：Neurology

authors： Halperin JJ,Luft BJ,Anand AK,Roque CT,Alvarez O,Volkman DJ,Dattwyler RJ

更新日期：1989-06-01 00:00:00

abstract：OBJECTIVE:To assess cortical inhibitory and excitatory mechanisms in obsessive-compulsive disorder (OCD). BACKGROUND:Transcranial magnetic stimulation (TMS) studies have found decreased neuronal inhibition and a reduced cortical silent period in the primary motor area in Tourette's syndrome, focal dystonia, and other ...

journal_title：Neurology

authors： Greenberg BD,Ziemann U,Corá-Locatelli G,Harmon A,Murphy DL,Keel JC,Wassermann EM

更新日期：2000-01-11 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：OBJECTIVE:To analyze the relationship between retinal thinning and nigral dopaminergic loss in de novo Parkinson disease (PD). METHODS:Forty-nine patients with PD and 54 age-matched controls were analyzed. Ophthalmologic examination and macula optical coherence tomography scans were performed with additional microperi...

journal_title：Neurology

authors： Ahn J,Lee JY,Kim TW,Yoon EJ,Oh S,Kim YK,Kim JM,Woo SJ,Kim KW,Jeon B

更新日期：2018-09-11 00:00:00

abstract：:A 60-year-old man presented with progressive large fiber sensory loss in the right first three fingers and, to a lesser extent, in both fourth and fifth fingers. Electrophysiologic studies were characteristic of chronic sensory demyelinating polyneuropathy, a variant of chronic inflammatory demyelinating polyneuropath...

journal_title：Neurology

authors： Thonnard JL,Detrembleur C,Van den Bergh PY

更新日期：1997-07-01 00:00:00

abstract：:Presenilin (PSEN)1 mutations are responsible for many cases of autosomal dominant Alzheimer disease (AD), although the clinical spectrum has not been fully defined. The authors describe two members of a kindred with a novel PSEN1 mutation (R278I) presenting with language impairment and relative preservation of memory....

journal_title：Neurology

authors： Godbolt AK,Beck JA,Collinge J,Garrard P,Warren JD,Fox NC,Rossor MN

更新日期：2004-11-09 00:00:00

abstract：:Using (18)F-fluorodopa PET and a constant 0.0062 influx rate in the putamen, dementia with Lewy bodies (n = 7) was distinguished from AD (n = 10) with a sensitivity of 86% and a specificity of 100%. A constant 0.0071 influx rate in the caudate yielded a sensitivity of 71% and a specificity of 100% for distinction of t...

journal_title：Neurology

authors： Hu XS,Okamura N,Arai H,Higuchi M,Matsui T,Tashiro M,Shinkawa M,Itoh M,Ido T,Sasaki H

更新日期：2000-11-28 00:00:00

abstract：BACKGROUND:There are no published MRI studies comparing interferon beta 1b (IFNbeta-1b) and glatiramer acetate (GA) for treatment of relapsing multiple sclerosis (MS). OBJECTIVE:To compare the efficacy of IFNbeta-1b and GA for suppression of MS disease activity as evidenced on frequent brain MRI. METHODS:A total of 7...

journal_title：Neurology

authors： Cadavid D,Wolansky LJ,Skurnick J,Lincoln J,Cheriyan J,Szczepanowski K,Kamin SS,Pachner AR,Halper J,Cook SD

更新日期：2009-06-09 00:00:00

abstract：:To test interphysician agreement on the diagnosis of subtype of ischemic stroke, we sent subtype definitions and 18 case summaries (clinical features and pertinent laboratory data) to 24 neurologists who have a special interest in stroke, and asked them to determine the most likely subtype diagnosis. The overall agree...

journal_title：Neurology

authors： Gordon DL,Bendixen BH,Adams HP Jr,Clarke W,Kappelle LJ,Woolson RF

更新日期：1993-05-01 00:00:00

abstract：:A 52-year-old steelworker displayed a consistent defect in leftward voluntary gaze across the midline. Both fast (saccadic) and slow (pursuit) eye movements were affected. Neuro-ophthalmologic evaluation revealed no additional neurologic or ophthalmologic deficit. Pupilary miosis occurred during leftward gaze attempt,...

journal_title：Neurology

authors： Troost BT,Troost EG

更新日期：1979-01-01 00:00:00

abstract：:The childhood type fo dermatomyositis, which occurs in children and young adults, shows a specific constellation of pathologic changes in muscle. Capillary necrosis leads to capillary loss, generally starting on the periphery of muscle fascicles. Electron microscopy discloses undulating tubules in endothelial cells, l...

journal_title：Neurology

authors： Carpenter S,Karpati G,Rothman S,Watters G

更新日期：1976-10-01 00:00:00

abstract：OBJECTIVE:We aimed to investigate whether cognitive deficits and structural and functional connectivity changes can be detected before symptom onset in a large cohort of carriers of microtubule-associated protein tau and progranulin mutations. METHODS:In this case-control study, 75 healthy individuals (aged 20-70 year...

journal_title：Neurology

authors： Dopper EG,Rombouts SA,Jiskoot LC,Heijer Td,de Graaf JR,Koning Id,Hammerschlag AR,Seelaar H,Seeley WW,Veer IM,van Buchem MA,Rizzu P,van Swieten JC

更新日期：2013-02-26 00:00:00

abstract：OBJECTIVE:To study the accuracy of the ABCD2 score in predicting early stroke risk following TIA and to model post-test probability of stroke for varying cutoff scores and baseline stroke risk. METHODS:Medline, PubMed, Embase, conference proceedings, and manuscript references up to October 2010 were searched for studi...

journal_title：Neurology

authors： Sanders LM,Srikanth VK,Blacker DJ,Jolley DJ,Cooper KA,Phan TG

更新日期：2012-09-04 00:00:00