Pyruvate dehydrogenase deficiency in spinocerebellar degenerations.

abstract：:Videotape analysis of 76 attacks in 14 epileptics showed two electro-clinical types of psychomotor attacks. The first and most common type had three clinical phases, consisting of an initial motionless stare, stereotyped movements, and reactive automatisms during impaired consciousness. The second and less common type...

journal_title：Neurology

authors： Escueta AV,Kunze U,Waddell G,Boxley J,Nadel A

更新日期：1977-02-01 00:00:00

abstract：:The clinical and genetic features of three non-Portuguese and non-Japanese patients with aprataxin gene mutations are reported. Patient 1 came from Italy and presented with typical ataxia with ocular motor apraxia (OMA). She was homozygous for the W279X nonsense mutation, which is associated with the Portuguese foundi...

journal_title：Neurology

authors： Tranchant C,Fleury M,Moreira MC,Koenig M,Warter JM

更新日期：2003-03-11 00:00:00

abstract：OBJECTIVE:To determine the latency, magnitude, and duration of the long-duration response (LDR) to levodopa in PD in relationship to the administration of levodopa at different interdose intervals. METHODS:In six patients with PD, two different 15-day treatment regimens were used in which the drug was administered wit...

journal_title：Neurology

authors： Zappia M,Bosco D,Plastino M,Nicoletti G,Branca D,Oliveri RL,Aguglia U,Gambardella A,Quattrone A

更新日期：1999-08-11 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：OBJECTIVES:To determine the prognostic role of late auditory (N100) and cognitive evoked potentials (MMN) for awakening in a cohort of comatose patients categorized by etiology. METHODS:The authors prospectively studied a series of 346 comatose patients. Coma was caused by stroke (n = 125), trauma (n = 96), anoxia (n ...

journal_title：Neurology

authors： Fischer C,Luauté J,Adeleine P,Morlet D

更新日期：2004-08-24 00:00:00

abstract：OBJECTIVES:To evaluate basal ganglia changes along the amyotrophic lateral sclerosis (ALS)-ALS-frontotemporal dementia (FTD) continuum using multiple, complementary imaging techniques. METHODS:Sixty-seven C9orf72-negative patients with ALS and 39 healthy controls were included in a cross-sectional quantitative MRI stu...

journal_title：Neurology

authors： Machts J,Loewe K,Kaufmann J,Jakubiczka S,Abdulla S,Petri S,Dengler R,Heinze HJ,Vielhaber S,Schoenfeld MA,Bede P

更新日期：2015-10-13 00:00:00

abstract：OBJECTIVE:To evaluate the production rate of CSF in patients with differing disease states. METHODS:The authors measured the production rate of CSF in three groups of patients: five patients with PD below age 60 (aged 51 +/- 4 years, mean +/- SD), nine with PD over age 60 (aged 69 +/- 6 years, mean +/- SD), and seven ...

journal_title：Neurology

authors： Silverberg GD,Heit G,Huhn S,Jaffe RA,Chang SD,Bronte-Stewart H,Rubenstein E,Possin K,Saul TA

更新日期：2001-11-27 00:00:00

abstract：:Although a neuromuscular syndrome has been induced experimentally by vitamin E deficiency, a human syndrome has not yet been documented. This report describes a 7-year-old boy with severe malabsorption since birth who presented with progressive external ophthalmoplegia, proximal muscle weakness, peripheral neuropathy,...

journal_title：Neurology

authors： Tomasi LG

更新日期：1979-08-01 00:00:00

abstract：:A two-octapeptide repeat deletion of the prion protein gene has been recently observed in a patient with a 2-year history of dementia and a clinical diagnosis of possible Creutzfeldt-Jakob disease (CJD). The authors report a similar deletion in a patient with a definitive diagnosis of CJD. Since the two-repeat deletio...

journal_title：Neurology

authors： Capellari S,Parchi P,Wolff BD,Campbell J,Atkinson R,Posey DM,Petersen RB,Gambetti P

更新日期：2002-11-26 00:00:00

abstract：:We report on the prevalence of dementia in Canadians age 85 years and older. The purpose of this study was to determine whether the prevalence of dementia continued to increase in the very old, and to define the types of dementia and their relative proportions in this age group. We collected data as part of the Canadi...

journal_title：Neurology

authors： Ebly EM,Parhad IM,Hogan DB,Fung TS

更新日期：1994-09-01 00:00:00

abstract：:The authors evaluated response, time to progression (TTP), survival, prognostic factors, and toxicity in 63 patients with a recurrent glioblastoma multiforme treated with procarbazine, lomustine, and vincristine (PCV) chemotherapy. Complete and partial response was observed in two (3%) and five patients (8%). In 16 pa...

journal_title：Neurology

authors： Kappelle AC,Postma TJ,Taphoorn MJ,Groeneveld GJ,van den Bent MJ,van Groeningen CJ,Zonnenberg BA,Sneeuw KC,Heimans JJ

更新日期：2001-01-09 00:00:00

abstract：:Previous brain infarctions seen on CT are common in the absence of history of stroke. Eleven percent of patients (135/1,203) without stroke history had ischemic lesions on their first CT, unrelated to the presenting stroke. Stroke Data Bank files were reviewed to determine whether the occurrence, location, and CT char...

journal_title：Neurology

authors： Chodosh EH,Foulkes MA,Kase CS,Wolf PA,Mohr JP,Hier DB,Price TR,Furtado JG Jr

更新日期：1988-11-01 00:00:00

abstract：:We report a patient with unusual MRI abnormalities that had the physical characteristics of ferromagnetic artifact. We believe that the MRI artifacts were due to microscopic embolic metal fragments, most likely from a mechanical heart valve prosthesis. Potential sources of metal emboli should be considered in patients...

journal_title：Neurology

authors： Wingerchuk DM,Krecke KN,Fulgham JR

更新日期：1997-11-01 00:00:00

abstract：:Two patients with similar courses of neurologic impairment and subsequent recovery after cerebral air embolism complicating cardiac ablation procedures are described. Hyperbaric oxygen therapy, combined with aggressive resuscitative efforts, appears to have contributed to each patient's recovery. ...

journal_title：Neurology

authors： Hinkle DA,Raizen DM,McGarvey ML,Liu GT

更新日期：2001-03-27 00:00:00

abstract：OBJECTIVE:To assess cerebrovascular reactivity in response to a visual task in participants with cerebral amyloid angiopathy (CAA), Alzheimer disease (AD), and mild cognitive impairment (MCI) using fMRI. METHODS:This prospective cohort study included 40 patients with CAA, 22 with AD, 27 with MCI, and 25 healthy contro...

journal_title：Neurology

authors： Switzer AR,Cheema I,McCreary CR,Zwiers A,Charlton A,Alvarez-Veronesi A,Sekhon R,Zerna C,Stafford RB,Frayne R,Goodyear BG,Smith EE

更新日期：2020-09-08 00:00:00

abstract：:Butyrylcholinesterase is implicated in the pathology of AD. Selective inhibitors increase acetylcholine and improve cognitive function in animal models. In dementia with Lewy bodies, cholinergic activities are more affected than in AD. The authors report a highly significant association between temporal cortex butyryl...

journal_title：Neurology

authors： Perry E,McKeith I,Ballard C

更新日期：2003-06-10 00:00:00

abstract：:The association of the human leukocyte antigen (HLA)-DR2 allele with brain MRI signal abnormalities and with the development of MS was assessed in 178 patients enrolled in the Optic Neuritis Treatment Trial. HLA haplotype DR2 was present in 85 (48%) of the 178 patients. Its presence was associated with increased odds ...

journal_title：Neurology

authors： Hauser SL,Oksenberg JR,Lincoln R,Garovoy J,Beck RW,Cole SR,Moke PS,Kip KE,Gal RL,Long DT

更新日期：2000-05-09 00:00:00

abstract：:There have been few descriptions of spontaneous epidural hematoma of the spinal cord in children. Duration of symptoms in previous cases has varied from hours to days. We recently evaluated a 6-year-old girl with cord compression from an extradural clot, who complained of thoracic back pain for 3 months before becomin...

journal_title：Neurology

authors： Robertson WC Jr,Lee YE,Edmonson MB

更新日期：1979-01-01 00:00:00

abstract：:Fatal familial insomnia (FFI), a condition characterized by inability to sleep, dysautonomia, motor disturbances, and selective thalamic atrophy is a prion disease linked to a GAC----AAC mutation at codon 178 of the prion gene. These data were obtained from one kindred. We now report a second kindred affected by FFI a...

journal_title：Neurology

authors： Medori R,Montagna P,Tritschler HJ,LeBlanc A,Cortelli P,Tinuper P,Lugaresi E,Gambetti P

更新日期：1992-03-01 00:00:00

abstract：:In two patients, a sensory neuronopathy was the initial presentation of Sjögren's syndrome. There was no systemic or peripheral nerve vasculitis. Immunochemical studies failed to demonstrate specific antibodies against the nervous system. These features suggest that the damage to the sensory neurons might not be media...

journal_title：Neurology

authors： Graus F,Pou A,Kanterewicz E,Anderson NE

更新日期：1988-10-01 00:00:00

abstract：:Spontaneous circadian fluctuations of motor symptoms in Parkinson's disease (PD) often occur, with dysfunction typically less severe in the early morning than in the afternoon. In 23 PD patients with or without a history of circadian motor fluctuations, we studied contrast sensitivity (CS), a non-motor function, consi...

journal_title：Neurology

authors： Struck LK,Rodnitzky RL,Dobson JK

更新日期：1990-03-01 00:00:00

abstract：BACKGROUND:The annual relapse rate has been commonly used as a primary efficacy endpoint in phase III multiple sclerosis (MS) clinical trials. The aim of this study was to determine the relative contribution of different possible prognostic factors available at baseline to the on-study relapse rate in MS. METHODS:A to...

journal_title：Neurology

authors： Held U,Heigenhauser L,Shang C,Kappos L,Polman C,Sylvia Lawry Centre for MS Research.

更新日期：2005-12-13 00:00:00

abstract：OBJECTIVE:To describe the clinical features and outcome of anti-NMDA receptor (NMDAR) encephalitis in patients ≥45 years old. METHOD:Observational cohort study. RESULTS:In a cohort of 661 patients with anti-NMDAR encephalitis, we identified 31 patients ≥45 years old. Compared with younger adults (18-44 years), older ...

journal_title：Neurology

authors： Titulaer MJ,McCracken L,Gabilondo I,Iizuka T,Kawachi I,Bataller L,Torrents A,Rosenfeld MR,Balice-Gordon R,Graus F,Dalmau J

更新日期：2013-09-17 00:00:00

abstract：OBJECTIVE:To evaluate the occurrence, treatment, and outcome of subdural empyema complicating community-acquired bacterial meningitis in adults. METHODS:Case series from a prospective nationwide cohort study from Dutch hospitals from 2006 to 2011. RESULTS:Subdural empyema was diagnosed in 28 of 1,034 episodes (2.7%),...

journal_title：Neurology

authors： Jim KK,Brouwer MC,van der Ende A,van de Beek D

更新日期：2012-11-20 00:00:00

abstract：OBJECTIVES:To investigate clinical, imaging, and pathologic associations of the cingulate island sign (CIS) in dementia with Lewy bodies (DLB). METHODS:We retrospectively identified and compared patients with a clinical diagnosis of DLB (n=39); patients with Alzheimer disease (AD) matched by age, sex, and education (n...

journal_title：Neurology

authors： Graff-Radford J,Murray ME,Lowe VJ,Boeve BF,Ferman TJ,Przybelski SA,Lesnick TG,Senjem ML,Gunter JL,Smith GE,Knopman DS,Jack CR Jr,Dickson DW,Petersen RC,Kantarci K

更新日期：2014-08-26 00:00:00

abstract：:We report a patient with meningeal gliomatosis who had signs of meningitis, hypoglycorrhachia, and an intracranial mass. Despite suspicion of a primary intracranial neoplasm, repeated CSF cytologies were not diagnostic and led to a brain biopsy for diagnosis. Immunoperoxidase staining for glial fibrillary acidic prote...

journal_title：Neurology

authors： Wechsler LR,Gross RA,Miller DC

更新日期：1984-12-01 00:00:00

abstract：:A 12-year-old girl who had zoster ophthalmicus 10 months earlier presented with hemiparesis and corresponding basal ganglionic infarction related to middle cerebral artery branch thrombosis ipsilateral to the zoster. Hematologic evaluation disclosed protein C deficiency. This represents the first zoster-associated str...

journal_title：Neurology

authors： Cadavid D,Pearl PL,Dubovsky EC,Angiolillo A,Vezina LG

更新日期：1999-09-22 00:00:00

abstract：BACKGROUND:UK veterans who were deployed to the Gulf in 1990 to 1991 reported higher prevalence of neuromuscular symptoms. OBJECTIVE:To investigate whether these Gulf War-related symptoms were associated with objective evidence of neuromuscular dysfunction. METHODS:Forty-nine Gulf War veterans with more than four neu...

journal_title：Neurology

authors： Sharief MK,Priddin J,Delamont RS,Unwin C,Rose MR,David A,Wessely S

更新日期：2002-11-26 00:00:00

abstract：:The neurofilament is the major cytoskeletal structure of myelinated axons. In this study, CSF levels of the light subunit of the neurofilament protein (NFL) were increased in patients with vascular dementia (VAD), AD, and frontotemporal dementia (FTD) compared with neurologically healthy individuals. Because NFL is lo...

journal_title：Neurology

authors： Rosengren LE,Karlsson JE,Sjögren M,Blennow K,Wallin A

更新日期：1999-03-23 00:00:00

abstract：:Epilepsy is associated with a high rate of premature mortality from direct and indirect effects of seizures, epilepsy, and antiseizure therapies. Sudden unexpected death in epilepsy (SUDEP) is the second leading neurologic cause of total lost potential life-years after stroke, yet SUDEP may account for less than half ...

journal_title：Neurology

authors： Devinsky O,Spruill T,Thurman D,Friedman D

更新日期：2016-02-23 00:00:00