Cardiac involvement in genetically confirmed facioscapulohumeral muscular dystrophy.

abstract：:The presence of the APOE epsilon 4 allele encoding apolipoprotein E4 (apoE4) is the major genetic risk factor for late-onset Alzheimer's disease (AD). However, the molecular and cellular mechanisms by which APOE epsilon 4 renders AD risk are unclear. In this report, we present genetic evidence that an apoE receptor, L...

journal_title：Neurology

authors： Kang DE,Saitoh T,Chen X,Xia Y,Masliah E,Hansen LA,Thomas RG,Thal LJ,Katzman R

更新日期：1997-07-01 00:00:00

abstract：:In vivo proton magnetic resonance spectroscopy (MRS) may be used to quantify brainstem neuronal degeneration in ALS because of the neuronal localization of N-acetylaspartate and N-acetylaspartylglutamate, together termed NA, which are estimated with this technique. We measured the ratio of NA to creatine/phosphocreati...

journal_title：Neurology

authors： Cwik VA,Hanstock CC,Allen PS,Martin WR

更新日期：1998-01-01 00:00:00

abstract：OBJECTIVE:To study the safety of off-label IV thrombolysis in patients with very severe stroke (NIH Stroke Scale [NIHSS] scores >25) compared with severe stroke (NIHSS scores 15-25), where treatment is within European regulations. METHODS:Data were analyzed from 57,247 patients with acute ischemic stroke receiving IV ...

journal_title：Neurology

authors： Mazya MV,Lees KR,Collas D,Rand VM,Mikulik R,Toni D,Wahlgren N,Ahmed N

更新日期：2015-12-15 00:00:00

abstract：:The major glycoprotein P0 from human and bovine peripheral nerves carries the L2/HNK-1 and L3 carbohydrate epitopes and is recognized by serum from patients with IgM gammopathy and polyneuropathy. Only serum from patients with reactivity toward the myelin-associated glycoprotein (MAG) was reactive with P0, while serum...

journal_title：Neurology

authors： Bollensen E,Steck AJ,Schachner M

更新日期：1988-08-01 00:00:00

abstract：OBJECTIVE:To identify phenotypic features of febrile seizures that can be used to reduce heterogeneity and thereby increase power in linkage analysis. BACKGROUND:Despite exciting discoveries in several rare pedigrees, the genetic basis of common febrile seizures remains a mystery. The major drawback of studying common...

journal_title：Neurology

authors： Pal DK,Kugler SL,Mandelbaum DE,Durner M

更新日期：2003-02-11 00:00:00

abstract：OBJECTIVES:To investigate the role of beta oscillations in urinary voiding and their association with lower urinary tract symptoms in Parkinson disease (PD). METHODS:We used surgically implanted deep brain stimulation electrodes to record local field potential signals from the subthalamic nucleus (STN) and globus pall...

journal_title：Neurology

authors： Roy HA,Aziz TZ,Fitzgerald JJ,Green AL

更新日期：2018-04-24 00:00:00

abstract：:Degenerative diseases of the CNS, such as stiff-person syndrome (SPS), progressive cerebellar ataxia, and Rasmussen encephalitis, have been characterized by the presence of autoantibodies. Recent findings in individuals with Batten disease and in animal models for the disorder indicate that this condition may be assoc...

journal_title：Neurology

authors： Pearce DA,Atkinson M,Tagle DA

更新日期：2004-12-14 00:00:00

abstract：:Self-reported versus informant-reported memory problems in nondemented elderly adults and in individuals with very mild and mild dementia of the Alzheimer type (DAT) were correlated with cognitive outcomes. No significant correlations were found between self-reported memory complaints and cognitive performance or (in ...

journal_title：Neurology

authors： Carr DB,Gray S,Baty J,Morris JC

更新日期：2000-12-12 00:00:00

abstract：OBJECTIVE:There is a high risk of readmission within 30 days of index acute ischemic stroke (AIS), but effect of readmission to a different hospital is not known. We performed a retrospective cohort study to assess our hypothesis that 30-day readmission outcomes after AIS are worse for those readmitted to another hospi...

journal_title：Neurology

authors： Stein LK,Agarwal P,Thaler A,Kwon CS,Jette N,Dhamoon MS

更新日期：2019-11-12 00:00:00

abstract：OBJECTIVE:To investigate the temporal relationship between inflammation and cerebral atrophy in a longitudinal study of 19 patients with relapsing-remitting multiple sclerosis (RRMS) using serial monthly contrast enhanced MRI examinations and monthly measurements of brain fractional volume (BFV) for an average of 4 (ra...

journal_title：Neurology

authors： Richert ND,Howard T,Frank JA,Stone R,Ostuni J,Ohayon J,Bash C,McFarland HF

更新日期：2006-02-28 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：:Fluctuations in motor response eventually affect most patients with Parkinson's disease who have been exposed to chronic, intermittent levodopa administration for longer than 5 years. Response oscillations are often related to the timing of levodopa doses (predictable "wearing-off"), but random "on-off" effects also o...

journal_title：Neurology

authors： Poewe WH

更新日期：1994-07-01 00:00:00

abstract：OBJECTIVES:The aim of this study was to identify risk factors for rupture of unruptured cerebral aneurysms (UCAs) in elderly Japanese patients aged 70 years or older. METHODS:The participants included all patients 70 years of age or older in 3 prospective studies in Japan (the Unruptured Cerebral Aneurysm Study of Jap...

journal_title：Neurology

authors： Hishikawa T,Date I,Tokunaga K,Tominari S,Nozaki K,Shiokawa Y,Houkin K,Murayama Y,Ishibashi T,Takao H,Kimura T,Nakayama T,Morita A,For UCAS Japan and UCAS II Investigators.

更新日期：2015-11-24 00:00:00

abstract：OBJECTIVE:To identify independent predictors of clinical or cerebral lesion progression in a large sample of adult patients with moyamoya angiopathy (MMA) prior to decisions regarding revascularization surgery. METHODS:Ninety participants (median age, 37.5 years) were assessed at baseline and followed for a median tim...

journal_title：Neurology

authors： Hervé D,Ibos-Augé N,Calvière L,Rogan C,Labeyrie MA,Guichard JP,Godin O,Kossorotoff M,Habert MO,Lasserve ET,Chevret S,Chabriat H

更新日期：2019-07-23 00:00:00

abstract：:Vasospasm is the leading cause of death and disability in patients with aneurysmal subarachnoid hemorrhage (SAH). Transcranial Doppler ultrasonography (TCD) can detect the arterial narrowing noninvasively, but the sensitivity and specificity of this technique have not been reported in a population of patients with a h...

journal_title：Neurology

authors： Sloan MA,Haley EC Jr,Kassell NF,Henry ML,Stewart SR,Beskin RR,Sevilla EA,Torner JC

更新日期：1989-11-01 00:00:00

abstract：:Dopa-responsive dystonia (DRD) is an autosomal dominant disorder typically presenting as dystonia with diurnal variability. Described is an 8-year-old boy who had had waddling gait, generalized hypotonia, and proximal weakness since early childhood. He responded well to low-dose L-dopa. He had a point mutation of the ...

journal_title：Neurology

authors： Kong CK,Ko CH,Tong SF,Lam CW

更新日期：2001-09-25 00:00:00

abstract：OBJECTIVE:We sought to examine brain white matter abnormalities based on MRI in adults with Lesch-Nyhan disease (LND) or an attenuated variant (LNV) of this rare, X-linked neurodevelopmental disorder of purine metabolism. METHODS:In this observational study, we compared 21 adults with LND, 17 with LNV, and 33 age-, se...

journal_title：Neurology

authors： Schretlen DJ,Varvaris M,Vannorsdall TD,Gordon B,Harris JC,Jinnah HA

更新日期：2015-01-13 00:00:00

abstract：:We report a family with Machado-Joseph disease (MJD) that was previously diagnosed with dentatorubral-pallidoluysian atrophy (DRPLA), on the basis of the neuropathologic findings. Because the clinical and pathologic reevaluation strongly suggested a diagnosis of MJD, we conducted a genetic study in the family. Two pat...

journal_title：Neurology

authors： Sakai T,Antoku Y,Kawakami H,Maruyama H,Nakamura S,Tanaka K

更新日期：1996-04-01 00:00:00

abstract：:To assess reversibility of focal cerebral ischemia, we performed a neurologic and pathologic study of 27 monkeys subjected to temporary middle cerebral artery occlusion. An implanted snare ligature occluded the artery in awake monkeys for 30 minutes, 4 hours, 8 hours, 16 hours, 24 hours, or permanently. Serial neurolo...

journal_title：Neurology

authors： Crowell RM,Marcoux FW,DeGirolami U

更新日期：1981-10-01 00:00:00

abstract：OBJECTIVE:The objective of this study was to evaluate the association between spinal cord injury (SCI) and type 2 diabetes in a large representative sample and to determine whether an association exists irrespective of known risk factors for type 2 diabetes. METHODS:Data were obtained on 60,678 respondents to the Stat...

journal_title：Neurology

authors： Cragg JJ,Noonan VK,Dvorak M,Krassioukov A,Mancini GB,Borisoff JF

更新日期：2013-11-19 00:00:00

abstract：:Glucocorticoid therapy slows the progression of Duchenne muscular dystrophy. In muscle cultures, the addition of the glucocorticoid methylprednisolone increases myogenesis in most normal mixed and clonal cultures. Conversely, in some normal clonal and most dystrophic cultures, methylprednisolone inhibits fusion. Howev...

journal_title：Neurology

authors： Hardiman O,Sklar RM,Brown RH Jr

更新日期：1993-02-01 00:00:00

abstract：:A randomized trial was completed to assess the feasibility of a 2-year education rehabilitation program for students with sickle cell disease and memory deficits. Eleven students were assigned to tutoring with or without memory training for 2 years. Eighty-two percent completed the program. Evidence of improvement in ...

journal_title：Neurology

authors： King AA,White DA,McKinstry RC,Noetzel M,Debaun MR

更新日期：2007-06-05 00:00:00

abstract：OBJECTIVE:To determine the efficacy and tolerability of unilateral thalamic deep brain stimulation (DBS) for patients with medically refractory essential tremor (ET) and the tremor associated with Parkinson's disease (PD). BACKGROUND:The tremor of ET and PD may produce functional disability despite optimal medical the...

journal_title：Neurology

authors： Ondo W,Jankovic J,Schwartz K,Almaguer M,Simpson RK

更新日期：1998-10-01 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：:The administration of lecithin has failed to improve memory in aged or demented individuals. We studied the effect of lecithin on another cholinergic function, REM sleep latency. Intravenous doses of physostigmine shortened the latency to the first REM sleep period, but oral lecithin did not. In contrast to physostigm...

journal_title：Neurology

authors： Kapen S,Fleming PD,Drachman DA

更新日期：1986-08-01 00:00:00

abstract：:A specific form of deep cerebral hemisphere infarction was identified in 11 of 1,600 stroke register patients. Despite the CT finding of a deep striatocapsular lesion, these patients showed evidence of both cortical and capsular abnormalities. Angiography showed significant proximal carotid artery lesions as well as e...

journal_title：Neurology

authors： Bladin PF,Berkovic SF

更新日期：1984-11-01 00:00:00

abstract：:Previous research has shown that the Mini-Mental State Examination (MMS) is biased as a measure of cognitive impairment in minority and low-education patients. The purpose of this study was to (1) develop a statistical correction for effects of age and education and (2) test the efficacy of the statistically adjusted ...

journal_title：Neurology

authors： Mungas D,Marshall SC,Weldon M,Haan M,Reed BR

更新日期：1996-03-01 00:00:00

abstract：:The association of APOE genotypes with cerebral microbleeds (CMBs) was examined on the basis of the location of CMBs in 414 patients who were admitted primarily because of stroke. With respect to possession of the epsilon2 or epsilon4 allele, the adjusted odds ratio was 1.94 (1.05 to 3.58) for lobar CMBs but 1.21 (0.6...

journal_title：Neurology

authors： Kim M,Bae HJ,Lee J,Kang L,Lee S,Kim S,Lee JE,Lee KM,Yoon BW,Kwon O,Koo JS,Kim BK

更新日期：2005-11-08 00:00:00

abstract：BACKGROUND:Sleep-disordered breathing (SDB) and respiratory failure (RF) are complications of acid maltase deficiency (AMD), a rare hereditary myopathy. OBJECTIVE:To define the relationship between lung and respiratory muscle function, to establish incidence and patterns of SDB, and to determine daytime predictors of ...

journal_title：Neurology

authors： Mellies U,Ragette R,Schwake C,Baethmann M,Voit T,Teschler H

更新日期：2001-10-09 00:00:00

abstract：:All Olmsted County, Minnesota, residents who experienced brain injury from 1935 through 1979 were identified and their medical records reviewed for survival and neurologic outcome. Minimum inclusion criteria included loss of consciousness or post-traumatic amnesia or neurologic evidence of brain injury or skull fractu...

journal_title：Neurology

authors： Wiederholt WC,Melton LJ 3rd,Annegers JF,Grabow JD,Laws ER Jr,Ilstrup DM

更新日期：1989-01-01 00:00:00