Quantifying physical decline in juvenile neuronal ceroid lipofuscinosis (Batten disease).

abstract：:Both mevalonic aciduria, characterized by psychomotor retardation, cerebellar ataxia, recurrent fever attacks, and death in early childhood, and hyper-immunoglobulin D (hyper-IgD) syndrome, with recurrent fever attacks without neurologic symptoms, are caused by a functional deficiency of mevalonate kinase. In a system...

journal_title：Neurology

authors： Simon A,Kremer HP,Wevers RA,Scheffer H,De Jong JG,Van Der Meer JW,Drenth JP

更新日期：2004-03-23 00:00:00

abstract：:Spontaneous arm levitation (SAL) is well-recognized in cortical-basal ganglionic degeneration, but not in other neurodegenerative diseases. We report six cases of progressive supranuclear palsy (PSP) with SAL. Pathologic examination in four revealed the classic features of PSP with additional areas of focal cortical d...

journal_title：Neurology

authors： Barclay CL,Bergeron C,Lang AE

更新日期：1999-03-10 00:00:00

abstract：:We reviewed retrospectively 114 prednisone dose reduction attempts in 63 myasthenic patients. Dose reduction was considered successful if a patient remained asymptomatic for more than 1 year on no prednisone or a stable low dose of prednisone. Successful dose reduction attempts were more common in patients taking azat...

journal_title：Neurology

authors： Miano MA,Bosley TM,Heiman-Patterson TD,Reed J,Sergott RC,Savino PJ,Schatz NJ

更新日期：1991-06-01 00:00:00

abstract：:Subarachnoid neurolysis using ethanol to destroy selectively the posterior roots of the spinal cord is a method for providing pain relief in patients with advanced cancer. Weakness of the extremities is a complication of the procedure that has been attributed to spread of the neurolytic agent to the anterior roots. Th...

journal_title：Neurology

authors： McGarvey ML,Ferrante FM,Patel RS,Maljian JA,Stecker M

更新日期：2000-04-11 00:00:00

abstract：OBJECTIVE:To determine possible effects of prolonged marijuana use on the cerebrovascular system during a month of monitored abstinence and to assess how the intensity of current use might have influenced cerebrovascular perfusion in these marijuana users. METHOD:The authors recorded blood flow velocity in the anterio...

journal_title：Neurology

authors： Herning RI,Better WE,Tate K,Cadet JL

更新日期：2005-02-08 00:00:00

abstract：:Immunomodulating agents have beneficial effects in the treatment of multiple sclerosis (MS), decreasing the frequency of relapses, the progression of disability, and MRI measures of disease activity. Despite the efficacy of these agents, many patients continue to show progression of disability, breakthrough relapses, ...

journal_title：Neurology

authors： Jeffery DR

更新日期：2004-12-28 00:00:00

abstract：:A group of 204 muscular dystrophy patients were screened for immunohistochemical and biochemical alpha-sarcoglycan defect and their DNA was analyzed for pathogenetic mutation in the four sarcoglycan genes. We identified 21 patients with alpha-, beta-, or gamma-sarcoglycan gene mutations. Patients with alpha-sarcoglyca...

journal_title：Neurology

authors： Angelini C,Fanin M,Freda MP,Duggan DJ,Siciliano G,Hoffman EP

更新日期：1999-01-01 00:00:00

abstract：OBJECTIVE:To determine the prevalence of developmental venous anomaly in adult patients with diffuse glioma. METHODS:We performed a retrospective cohort study (2010-2016) of consecutive adult patients harboring a supratentorial diffuse glioma in 2 centers: Sainte-Anne Hospital (experimental and control sets) and Pitié...

journal_title：Neurology

authors： Roux A,Edjlali M,Porelli S,Tauziede-Espariat A,Zanello M,Dezamis E,Zah-Bi G,Sanson M,Puget S,Capelle L,Varlet P,Oppenheim C,Pallud J

更新日期：2019-01-01 00:00:00

abstract：:We performed a quantitative immunocytochemical study using a polyclonal antibody directed against choline acetyltransferase (ChAT) in the lower pontine reticular formation in four control subjects and three patients with progressive supranuclear palsy (PSP). In the normal brains, there was detectable ChAT-like immunor...

journal_title：Neurology

authors： Malessa S,Hirsch EC,Cervera P,Javoy-Agid F,Duyckaerts C,Hauw JJ,Agid Y

更新日期：1991-10-01 00:00:00

abstract：OBJECTIVE:To reassess the evidence for management issues related to the care of women with epilepsy (WWE) during pregnancy, including preconceptional folic acid use, prenatal vitamin K use, risk of hemorrhagic disease of the newborn, clinical implications of placental and breast milk transfer of antiepileptic drugs (AE...

journal_title：Neurology

authors： Harden CL,Pennell PB,Koppel BS,Hovinga CA,Gidal B,Meador KJ,Hopp J,Ting TY,Hauser WA,Thurman D,Kaplan PW,Robinson JN,French JA,Wiebe S,Wilner AN,Vazquez B,Holmes L,Krumholz A,Finnell R,Shafer PO,Le Guen C,Americ

更新日期：2009-07-14 00:00:00

abstract：:Electroencephalography has traditionally been considered a useful adjunct to the clinical evaluation of headache. We performed a literature review to determine whether evidence supports the routine use of the electroencephalogram (EEG) for patients with headache. After excluding case reports, case series in which subj...

journal_title：Neurology

authors： Gronseth GS,Greenberg MK

更新日期：1995-07-01 00:00:00

abstract：:Six members of a family had a neurologic disease with autosomal dominant transmission and adolescent onset of progressive wasting of predominantly distal axial muscles and muscles innervated by cranial nerves V, VII, X, and XII, leading to severe morbidity in adulthood. Muscle biopsy of the propositus revealed "ragged...

journal_title：Neurology

authors： Dobkin BH,Verity MA

更新日期：1976-08-01 00:00:00

abstract：:Restless legs syndrome (RLS) is a common neurologic disorder that affects 5 to 10% of the population and increases in prevalence with aging. The clinical hallmarks of RLS include dysesthesias or paresthesias in the legs and sometimes the arms, occurring primarily at rest, which are usually worse in the evening and are...

journal_title：Neurology

authors： Comella CL

更新日期：2002-02-26 00:00:00

abstract：BACKGROUND:Most cavernous sinus dural arteriovenous malformations (CSdAVM) have a benign clinical course. Those CSdAVM that drain into cortical veins have an increased risk for neurologic complications. OBJECTIVE:To find whether a specific clinical sign predicts cortical venous drainage (CVD) in CSdAVM. METHODS:The r...

journal_title：Neurology

authors： Stiebel-Kalish H,Setton A,Berenstein A,Kalish Y,Nimii Y,Kupersmith MJ

更新日期：2002-05-28 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：:The authors recruited 19 nonambulant patients with Guillain-Barré syndrome into a pilot, double-blind, randomized, placebo-controlled safety trial of interferon beta 1a (IFN[beta]-1a) (Rebif). Participants received IFN[beta]-1a or placebo subcutaneously three times weekly, 22 microg for the first week and then 44 micr...

journal_title：Neurology

authors： Pritchard J,Gray IA,Idrissova ZR,Lecky BR,Sutton IJ,Swan AV,Willison HJ,Winer JB,Hughes RA

更新日期：2003-11-11 00:00:00

abstract：BACKGROUND:Paroxysmal nonkinesigenic dyskinesia (PNKD) is a rare disorder characterized by episodic hyperkinetic movement attacks. We have recently identified mutations in the MR-1 gene causing familial PNKD. METHODS:We reviewed the clinical features of 14 kindreds with familial dyskinesia that was not clearly induced...

journal_title：Neurology

authors： Bruno MK,Lee HY,Auburger GW,Friedman A,Nielsen JE,Lang AE,Bertini E,Van Bogaert P,Averyanov Y,Hallett M,Gwinn-Hardy K,Sorenson B,Pandolfo M,Kwiecinski H,Servidei S,Fu YH,Ptácek L

更新日期：2007-05-22 00:00:00

abstract：:The aim of this randomized, double-blind, placebo-controlled cross-over study was to test whether valproic acid relieves painful polyneuropathy. Thirty-one patients completed two treatment phases of 4 weeks' duration with valproic acid (1,500 mg daily) and placebo. There was no significant difference between valproic ...

journal_title：Neurology

authors： Otto M,Bach FW,Jensen TS,Sindrup SH

更新日期：2004-01-27 00:00:00

abstract：OBJECTIVE:To examine the long-term cognitive trajectories of individuals with normal cognition at baseline and distinct amyloid/tau/neurodegeneration (ATN) profiles. METHODS:Pooling data across 4 cohort studies, 814 cognitively normal participants (mean baseline age = 59.6 years) were classified into 8 ATN groups usin...

journal_title：Neurology

authors： Soldan A,Pettigrew C,Fagan AM,Schindler SE,Moghekar A,Fowler C,Li QX,Collins SJ,Carlsson C,Asthana S,Masters CL,Johnson S,Morris JC,Albert M,Gross AL

更新日期：2019-04-02 00:00:00

abstract：:We evaluated four formulas for estimating the intra-blood-brain barrier (IBBB) synthesis of IgG in a computer model of two types of BBB damage and in 1,629 consecutively received pairs of CSF and serum. These four formulas were the IgG synthesis rate (SR), IgG(loc) (ie, local), IgG index, and IgG extended index. Resul...

journal_title：Neurology

authors： Peter JB,Bowman RL

更新日期：1992-03-01 00:00:00

abstract：OBJECTIVES:(1) To assess the diagnostic accuracy of EMG in radiculopathy. (2) To evaluate the intrarater reliability and interrater reliability of EMG in radiculopathy. (3) To assess the presence of confirmation bias in EMG. METHODS:Three experienced academic electromyographers interpreted 3 compact discs with 20 EMG ...

journal_title：Neurology

authors： Narayanaswami P,Geisbush T,Jones L,Weiss M,Mozaffar T,Gronseth G,Rutkove SB

更新日期：2016-01-19 00:00:00

abstract：OBJECTIVES:To learn caregivers' preferences for the treatment of AD with a disease-slowing therapy, and to identify relationships between these preferences and the characteristics of caregivers and patients. METHODS:A structured interview with 40 caregivers of patients with AD. Preferences were measured for an AD-slow...

journal_title：Neurology

authors： Karlawish JH,Klocinski JL,Merz J,Clark CM,Asch DA

更新日期：2000-10-10 00:00:00

abstract：:Nodoparanodopathy is a recent concept in the field of peripheral neuropathy, corresponding to peripheral nerve disorders stemming from an autoimmune attack directed and limited to the nodal region. This concept was identified using modern techniques of electrophysiology, immunology, and pathology (including electron m...

journal_title：Neurology

authors： Mathis S,Le Masson G,Vallat JM

更新日期：2019-10-29 00:00:00

abstract：BACKGROUND:Lowering cholesterol is associated with reduced CNS amyloid deposition and increased dietary cholesterol increases amyloid accumulation in animal studies. Epidemiologic data suggest that use of 3-hydroxy-3-methylglutaryl coenzyme A (HMG-CoA) reductase inhibitors (statins) may decrease the risk of Alzheimer d...

journal_title：Neurology

authors： Sano M,Bell KL,Galasko D,Galvin JE,Thomas RG,van Dyck CH,Aisen PS

更新日期：2011-08-09 00:00:00

abstract：OBJECTIVE:To systematically review the evidence concerning the diagnostic yield of genetic and metabolic evaluation of children with global developmental delay or intellectual disability (GDD/ID). METHODS:Relevant literature was reviewed, abstracted, and classified according to the 4-tiered American Academy of Neurolo...

journal_title：Neurology

authors： Michelson DJ,Shevell MI,Sherr EH,Moeschler JB,Gropman AL,Ashwal S

更新日期：2011-10-25 00:00:00

abstract：BACKGROUND:Evidence linking APOE to myelin repair, neuronal plasticity, and cerebral inflammatory processes suggests that it may be relevant in multiple sclerosis (MS). The purpose of this study was to determine whether the epsilon4 allele of APOE is associated with cognitive deficits in patients with MS. METHOD:Using...

journal_title：Neurology

authors： Ghaffar O,Reis M,Pennell N,O'Connor P,Feinstein A

更新日期：2010-05-18 00:00:00

abstract：:The authors pooled four population-based European studies to determine the risk for institutionalization and death after stroke. Risk for institutionalization was assessed in 13,095 subjects. Survival was calculated for prevalent stroke in 7929 individuals and incident stroke in 8550 individuals. The adjusted risk of ...

journal_title：Neurology

authors： Schmidt R,Breteler MM,Inzitari D,Fratiglioni L,Hofman A,Launer LJ

更新日期：2000-01-01 00:00:00

abstract：BACKGROUND:Co-occurrence of Parkinson disease (PD) and melanoma has been reported in numerous studies. If this was due to common genetic mechanisms, a positive family history of melanoma would be associated with an excessive PD risk, independent of environmental risk factors for PD. METHODS:We prospectively examined a...

journal_title：Neurology

authors： Gao X,Simon KC,Han J,Schwarzschild MA,Ascherio A

更新日期：2009-10-20 00:00:00

abstract：OBJECTIVE:To estimate whole-brain microinfarct burden from microinfarct counts in routine postmortem examination. METHODS:We developed a simple mathematical method to estimate the total number of cerebral microinfarcts from counts obtained in the small amount of tissue routinely examined in brain autopsies. We derived...

journal_title：Neurology

authors： Westover MB,Bianchi MT,Yang C,Schneider JA,Greenberg SM

更新日期：2013-04-09 00:00:00

abstract：BACKGROUND:Several studies indicate semantic fluency more sensitively discriminates patients with Alzheimer disease (AD) from normal elderly persons, with disproportionate impairment of semantic over phonemic fluency. OBJECTIVE:To determine the ability of abbreviated fluency measures in the clinic setting (1-minute le...

journal_title：Neurology

authors： Canning SJ,Leach L,Stuss D,Ngo L,Black SE

更新日期：2004-02-24 00:00:00