Cognitive profiles differ in autopsy-confirmed frontotemporal dementia and AD.

abstract：OBJECTIVE:We developed the Recurrence and Recovery Questionnaire (RRQ) by converting the Pediatric Stroke Outcome Measure (PSOM) to a questionnaire for telephone interview and sought to validate the RRQ in a large cohort. METHOD:We analyzed parents' RRQ responses and same-day PSOM assessments for 232 children who had ...

journal_title：Neurology

authors： Lo WD,Ichord RN,Dowling MM,Rafay M,Templeton J,Halperin A,Smith SE,Licht DJ,Moharir M,Askalan R,Deveber G,International Pediatric Stroke Study (IPSS) Investigators.

更新日期：2012-08-28 00:00:00

abstract：:A marked decrease in the amplitude of the evoked thenar compound muscle action potential followed brief exercise at room temperature in two patients with paramyotonia congenita. This decline was more rapid and pronounced in our patient with coexistent thyrotoxicosis. The prolonged recovery period in paramyotonia conge...

journal_title：Neurology

authors： Gutmann L,Riggs JE,Brick JF

更新日期：1986-01-01 00:00:00

abstract：:Three children with cerebral oligodendrogliomas causing partial complex or generalized seizures presented with completely normal neurologic examinations. CT showed low-density, nonenhancing surface lesions. Although these CT features are usually associated with infarcts or cysts, neoplasm was suspected because of irre...

journal_title：Neurology

authors： Varma RR,Crumrine PK,Bergman I,Latchaw RE,Price RA,Vries J,Painter MJ

更新日期：1983-06-01 00:00:00

abstract：OBJECTIVE:To determine the incidence and clinical effects of antibodies that develop during treatment with natalizumab. METHODS:In two randomized, double-blind, placebo-controlled studies (natalizumab safety and efficacy in relapsing remitting multiple sclerosis [MS, AFFIRM] and safety and efficacy of natalizumab in c...

journal_title：Neurology

authors： Calabresi PA,Giovannoni G,Confavreux C,Galetta SL,Havrdova E,Hutchinson M,Kappos L,Miller DH,O'Connor PW,Phillips JT,Polman CH,Radue EW,Rudick RA,Stuart WH,Lublin FD,Wajgt A,Weinstock-Guttman B,Wynn DR,Lynn F,Panzar

更新日期：2007-10-02 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：:In children, seizures associated with status epilepticus (SE) include a number of types that are age-related. These types of seizures are not associated with SE in older patients. Likewise, etiologies of SE in children are also unique to this patient population, in particular those responsible for SE in the neonate. C...

journal_title：Neurology

authors： Lockman LA

更新日期：1990-05-01 00:00:00

abstract：OBJECTIVES:We used functional MRI (fMRI), transcranial Doppler ultrasound, and visual evoked potentials (VEPs) to determine the nature of blood flow responses to functional brain activity and carbon dioxide (CO2) inhalation in patients with cerebral amyloid angiopathy (CAA), and their association with markers of CAA se...

journal_title：Neurology

authors： Peca S,McCreary CR,Donaldson E,Kumarpillai G,Shobha N,Sanchez K,Charlton A,Steinback CD,Beaudin AE,Flück D,Pillay N,Fick GH,Poulin MJ,Frayne R,Goodyear BG,Smith EE

更新日期：2013-11-05 00:00:00

abstract：:Restless legs syndrome (RLS) is characterized by an urge to move the legs. The symptoms are more intense in the evening and during periods of inactivity. RLS symptoms are relieved by movement. It is classified as a sleep disorder, but in many ways it is actually a disorder of wakefulness that seems to intrude upon the...

journal_title：Neurology

authors： Auerbach S,Walters AS

更新日期：2014-04-08 00:00:00

abstract：:Central nervous system disorders may cause obstructive sleep apnea, but this sleep-related breathing disorder is rarely associated with a structural abnormality of the medulla. We present a patient with obstructive apnea resulting from autopsy-proven syringobulbia-myelia. ...

journal_title：Neurology

authors： Haponik EF,Givens D,Angelo J

更新日期：1983-08-01 00:00:00

abstract：:To study the incidence of abnormalities of the pyruvate (PDH) or ketoglutarate (KGDH) dehydrogenase complexes in patients with spinocerebellar degenerations, we measured the activities of PDH and KGDH in platelet-enriched preparations from the blood of 14 patients. Low PDH was found in 6 of the 14 patients; low KGDH w...

journal_title：Neurology

authors： Kark RA,Rodriguez-Budelli M

更新日期：1979-01-01 00:00:00

abstract：OBJECTIVE:To identify the genetic and physiologic basis for recessive myasthenic congenital myopathy in 2 families, suggestive of a channelopathy involving the sodium channel gene, SCN4A. METHODS:A combination of whole exome sequencing and targeted mutation analysis, followed by voltage-clamp studies of mutant sodium ...

journal_title：Neurology

authors： Elia N,Palmio J,Castañeda MS,Shieh PB,Quinonez M,Suominen T,Hanna MG,Männikkö R,Udd B,Cannon SC

更新日期：2019-03-26 00:00:00

abstract：:Large prospective epidemiologic studies have linked the consumption of coffee and other caffeinated beverages to a reduced risk of subsequently developing PD. Caffeine as well as more specific antagonists of the adenosine A(2A) receptor have also now been found to attenuate neurotoxicity in a mouse model of PD. The co...

journal_title：Neurology

authors： Schwarzschild MA,Chen JF,Ascherio A

更新日期：2002-04-23 00:00:00

abstract：OBJECTIVE:To investigate the long-term outcome of patients with spinal cord infarct (SCI) and identify prognostic predictors. METHODS:We reviewed 115 patients with SCI treated between 1990 and 2007. Severity of impairment was defined using the American Spinal Injury Association (ASIA) scoring. Functional outcome endpo...

journal_title：Neurology

authors： Robertson CE,Brown RD Jr,Wijdicks EF,Rabinstein AA

更新日期：2012-01-10 00:00:00

abstract：:We found an antinuclear antibody highly restricted to nuclei of neurons in two patients with subacute sensory neuronopathy complicating oat cell carcinoma of the lung. Serum was tested by indirect immunofluorescence and immunoperoxidase staining. At low concentrations of antibody, only the nuclei of the neurons were s...

journal_title：Neurology

authors： Graus F,Cordon-Cardo C,Posner JB

更新日期：1985-04-01 00:00:00

abstract：OBJECTIVES:The Stroke-Thrombolytic Predictive Instrument (Stroke-TPI) predicts the probability of good and bad outcomes with and without recombinant tissue plasminogen activator (rtPA). We sought to rebuild and externally validate a simpler Stroke-TPI to support implementation in routine clinical care. METHODS:Using t...

journal_title：Neurology

authors： Kent DM,Ruthazer R,Decker C,Jones PG,Saver JL,Bluhmki E,Spertus JA

更新日期：2015-09-15 00:00:00

abstract：OBJECTIVE:To investigate the efficacy and safety of aceneuramic acid extended-release (Ace-ER), a treatment intended to replace deficient sialic acid, in patients with GNE myopathy. METHODS:UX001-CL301 was a phase 3, double-blind, placebo-controlled, randomized, international study evaluating the efficacy and safety o...

journal_title：Neurology

authors： Lochmüller H,Behin A,Caraco Y,Lau H,Mirabella M,Tournev I,Tarnopolsky M,Pogoryelova O,Woods C,Lai A,Shah J,Koutsoukos T,Skrinar A,Mansbach H,Kakkis E,Mozaffar T

更新日期：2019-04-30 00:00:00

abstract：OBJECTIVE:To determine the efficacy of acetyl-l-carnitine (ALCAR) on the rate of decline in early-onset AD patients. METHODS:A 1-year, multicenter, double-blind, placebo-controlled, randomized trial was conducted. Subjects were 45 to 65 years old, with a diagnosis of probable AD according to National Institute of Neur...

journal_title：Neurology

authors： Thal LJ,Calvani M,Amato A,Carta A

更新日期：2000-09-26 00:00:00

abstract：:Although referrals for narcolepsy are increasing, physicians continue to confront obstacles in diagnosing and treating this condition. Patients with narcolepsy also face challenges at work, at school, and at home. One of the more disturbing problems is the lack of public awareness of the disorder and its potential the...

journal_title：Neurology

authors： Douglas NJ

更新日期：1998-02-01 00:00:00

abstract：:We tested the effect of bovine cortex phosphatidylserine (BC-PS), a membrane phospholipid known to inhibit the release of the cytokine tumor necrosis factor (TNF), in SJL/J mice sensitized for adoptively transferred experimental autoimmune encephalomyelitis (EAE). Control, sensitized mice developed severe clinical and...

journal_title：Neurology

authors： Monastra G,Cross AH,Bruni A,Raine CS

更新日期：1993-01-01 00:00:00

abstract：OBJECTIVE:To determine whether patients with Alzheimer disease (AD) compensate for neuropathologic changes when performing a mnemonic task by recruiting 1) the same brain regions as age-matched, healthy controls, but to a greater extent; 2) additional brain regions not activated by controls; or 3) both. METHODS:Twelve...

journal_title：Neurology

authors： Gould RL,Arroyo B,Brown RG,Owen AM,Bullmore ET,Howard RJ

更新日期：2006-09-26 00:00:00

abstract：OBJECTIVE:To demonstrate that progress has been made in unifying brain death determination guidelines in the last decade by directly comparing the policies of the US News and World Report's top 50 ranked neurologic institutions from 2006 and 2015. METHODS:We solicited official hospital guidelines in 2015 from these to...

journal_title：Neurology

authors： Wang HH,Varelas PN,Henderson GV,Wijdicks EF,Greer DM

更新日期：2017-02-07 00:00:00

abstract：:We investigated the possible role of potassium deficiency, a recognized cause of myopathy, in the pathogenesis of experimental acute alcoholic myopathy (EAM) in the rat. Alcohol-treated animals receiving the same dietary potassium as controls developed mild hypokalemia; however, muscle potassium was preserved or eleva...

journal_title：Neurology

authors： Haller RG,Carter NW,Ferguson E,Knochel JP

更新日期：1984-04-01 00:00:00

abstract：:We used the Mayo Clinic medical records linkage system to detect all cases of intracranial vascular malformation (IVM) among residents of Olmsted County, Minnesota from 1965 to 1992 and found 48 IVMs over the 27-year period, including four patients with IVM detected at autopsy, without prior symptoms. Twenty-nine of 4...

journal_title：Neurology

authors： Brown RD Jr,Wiebers DO,Torner JC,O'Fallon WM

更新日期：1996-04-01 00:00:00

abstract：OBJECTIVE:To estimate the prevalence and examine the course of mild cognitive impairment (MCI), amnestic type, using current criteria, within a representative community sample. METHODS:Retroactive application of MCI criteria to data collected during a prospective epidemiologic study was performed. The subjects were dr...

journal_title：Neurology

authors： Ganguli M,Dodge HH,Shen C,DeKosky ST

更新日期：2004-07-13 00:00:00

abstract：:Dyskinesias are usually seen in Parkinson's disease (PD) patients after several years of L-dopa therapy. Their presence has been attributed to supersensitivity of striatal D1 and D2 receptors. We have used PET to assess striatal D2 receptor binding in untreated PD patients and striatal D1 and D2 binding in L-dopa-trea...

journal_title：Neurology

authors： Turjanski N,Lees AJ,Brooks DJ

更新日期：1997-09-01 00:00:00

abstract：:Idiopathic trigeminal sensory neuropathy is a benign disorder. We report two patients with transient MRI abnormalities, suggesting transient inflammation of the trigeminal nerve caused temporary facial sensory symptoms. ...

journal_title：Neurology

authors： Rorick MB,Chandar K,Colombi BJ

更新日期：1996-05-01 00:00:00

abstract：OBJECTIVE:To clarify the clinical and neurophysiologic spectrum of myoclonus-dystonia patients with mutations of the SGCE gene. METHODS:We prospectively studied 41 consecutive patients from 22 families with documented mutations of the SGCE gene. The patients had a standardized interview, neurologic examination, and de...

journal_title：Neurology

authors： Roze E,Apartis E,Clot F,Dorison N,Thobois S,Guyant-Marechal L,Tranchant C,Damier P,Doummar D,Bahi-Buisson N,André-Obadia N,Maltete D,Echaniz-Laguna A,Pereon Y,Beaugendre Y,Dupont S,De Greslan T,Jedynak CP,Ponsot G,D

更新日期：2008-03-25 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：:Nigrostriatal dopaminergic function and cerebral energy metabolism were measured with PET in two brothers with early-onset parkinsonism caused by mutation of the parkin gene. Energy metabolism did not differ, but the nigrostriatal dopaminergic pattern was clearly different than that of sporadic PD. Thus parkinsonism i...

journal_title：Neurology

authors： Portman AT,Giladi N,Leenders KL,Maguire P,Veenma-van der Duin L,Swart J,Pruim J,Simon ES,Hassin-Baer S,Korczyn AD

更新日期：2001-06-26 00:00:00

abstract：OBJECTIVE:To examine whether prior statin use affects outcome and intracranial hemorrhage (ICH) rates in stroke patients receiving IV thrombolysis (IVT). METHODS:In a pooled observational study of 11 IVT databases, we compared outcomes between statin users and nonusers. Outcome measures were excellent 3-month outcome ...

journal_title：Neurology

authors： Engelter ST,Soinne L,Ringleb P,Sarikaya H,Bordet R,Berrouschot J,Odier C,Arnold M,Ford GA,Pezzini A,Zini A,Rantanen K,Rocco A,Bonati LH,Kellert L,Strbian D,Stoll A,Meier N,Michel P,Baumgartner RW,Leys D,Tatlisum

更新日期：2011-08-30 00:00:00