Lack of apoptosis in mitochondrial encephalomyopathies.

abstract：OBJECTIVE:To assess the natural progression of muscle weakness in spinal muscular atrophy (SMA) IIIb. METHODS:Ten patients with SMA IIIb were followed for at least 10 years. Age at disease onset varied between 9 and 18 years. Patients were initially seen 2 to 10 years after disease onset. They were evaluated at approx...

journal_title：Neurology

authors： Deymeer F,Serdaroglu P,Parman Y,Poda M

更新日期：2008-08-26 00:00:00

abstract：:We measured monoamine metabolites and biopterin in the CSF of 37 patients with dementia of the Alzheimer type (DAT), with or without extrapyramidal signs, and in 14 age-matched healthy controls. Compared with concentrations in DAT and controls, the concentrations of homovanillic acid (HVA) and biopterin were significa...

journal_title：Neurology

authors： Kaye JA,May C,Daly E,Atack JR,Sweeney DJ,Luxenberg JS,Kay AD,Kaufman S,Milstien S,Friedland RP

更新日期：1988-04-01 00:00:00

abstract：:We studied the effects of bromocriptine on periodic limb movements in sleep (PLMS) of narcoleptic patients. Bromocriptine significantly reduced the number and index of PLMS without improving the nocturnal sleep disruption in that disorder. These results support the hypothesis that dopaminergic mechanisms are involved ...

journal_title：Neurology

authors： Boivin DB,Lorrain D,Montplaisir J

更新日期：1993-10-01 00:00:00

abstract：:A 20-year-old mentally retarded woman, who had a history of intractable epileptic seizures since early childhood, had prolonged episodes of confusion, decreased responsiveness and automatic behavior lasting as long as 2 days. These are believed to represent epileptic status of the complex partial (psychomotor) type be...

journal_title：Neurology

authors： Markand ON,Wheeler GL,Pollack SL

更新日期：1978-02-01 00:00:00

abstract：:The authors evaluated how history of concussion affects symptom reporting prior to and after sustaining a concussion. At baseline, athletes with a positive concussion history reported more current symptomatology than athletes who had never been concussed. At 2 hours postinjury, concussed athletes with a history of pre...

journal_title：Neurology

authors： Bruce JM,Echemendia RJ

更新日期：2004-10-26 00:00:00

abstract：OBJECTIVE:To model the temporal trajectory of β-amyloid accumulation using serial amyloid PET imaging. METHODS:Participants, aged 70-92 years, were enrolled in either the Mayo Clinic Study of Aging (n = 246) or the Mayo Alzheimer's Disease Research Center (n = 14). All underwent 2 or more serial amyloid PET examinatio...

journal_title：Neurology

authors： Jack CR Jr,Wiste HJ,Lesnick TG,Weigand SD,Knopman DS,Vemuri P,Pankratz VS,Senjem ML,Gunter JL,Mielke MM,Lowe VJ,Boeve BF,Petersen RC

更新日期：2013-03-05 00:00:00

abstract：OBJECTIVE:To validate, examine the internal validity, and adapt to children the electronic version of the composite cerebellar functional severity (CCFS) score. METHODS:In this multicenter study, we compared the validated manual device with the new electronic version (n = 46) and analyzed its kinetics in 146 patients ...

journal_title：Neurology

authors： Filipovic Pierucci A,Mariotti C,Panzeri M,Giunti P,Boesch S,Schulz JB,Pandolfo M,Durr A,Tezenas du Montcel S,EFACTS Study Group.

更新日期：2015-03-24 00:00:00

abstract：:Long-term hypothalamic stimulation is effective in improving drug-resistant chronic cluster headache (CH). We assessed acute hypothalamic stimulation to resolve ongoing CH attacks in 16 patients implanted to prevent chronic CH, investigating 136 attacks. A pain intensity reduction of > or =50% occurred in 25 of 108 ev...

journal_title：Neurology

authors： Leone M,Franzini A,Broggi G,Mea E,Cecchini AP,Bussone G

更新日期：2006-11-28 00:00:00

abstract：:Neurologic abnormalities were seen in 13 of 20 patients with a history of chronic solvent vapor (primarily toluene) abuse for 2 or more years. The patients were evaluated after an abstinence period of at least 4 weeks, to avoid neurologic effects of acute intoxication. Neurologic signs included cognitive (60%), pyrami...

journal_title：Neurology

authors： Hormes JT,Filley CM,Rosenberg NL

更新日期：1986-05-01 00:00:00

abstract：:Plasma exchange (PE) was shown in a previous double-blind randomized controlled study to confer significant additional benefit at 1 year upon patients with chronic progressive multiple sclerosis (CPMS) treated with immunosuppressive drug therapy (ISDT). Efficacy over an extended term, indications for retreatment, and ...

journal_title：Neurology

authors： Khatri BO,McQuillen MP,Hoffmann RG,Harrington GJ,Schmoll D

更新日期：1991-03-01 00:00:00

abstract：:We investigated the activation of frontal and occipital cortical areas in 14 normal volunteers during voluntary saccades in light or dark and during imagined saccades using functional magnetic resonance imaging (FMRI) with electro-oculogram monitoring. Voluntary saccades in light or dark and imagined saccades led to a...

journal_title：Neurology

authors： Bodis-Wollner I,Bucher SF,Seelos KC,Paulus W,Reiser M,Oertel WH

更新日期：1997-08-01 00:00:00

abstract：BACKGROUND:Cross-sectional studies suggest gender differences in cognitive function and risk of AD in older persons. However, longitudinal studies comparing change in cognitive function and risk of AD in men and women have had mixed results. The authors investigated gender differences in rate of decline for different c...

journal_title：Neurology

authors： Barnes LL,Wilson RS,Schneider JA,Bienias JL,Evans DA,Bennett DA

更新日期：2003-06-10 00:00:00

abstract：BACKGROUND:Optimal treatment remains uncertain for patients with cognitive impairment that persists or returns after standard IV antibiotic therapy for Lyme disease. METHODS:Patients had well-documented Lyme disease, with at least 3 weeks of prior IV antibiotics, current positive IgG Western blot, and objective memory...

journal_title：Neurology

authors： Fallon BA,Keilp JG,Corbera KM,Petkova E,Britton CB,Dwyer E,Slavov I,Cheng J,Dobkin J,Nelson DR,Sackeim HA

更新日期：2008-03-25 00:00:00

abstract：:With the use of the newer antidepressants beyond the traditional tricyclics and monoamine oxidase inhibitors, newer options in headache prophylaxis are provided as well as the potential for undesirable and even potentially life-threatening interactions between medications. In this article, four patient reports of a sp...

journal_title：Neurology

authors： Diamond S,Pepper BJ,Diamond ML,Freitag FG,Urban GJ,Erdemoglu AK

更新日期：1998-07-01 00:00:00

abstract：:T, B, and "null" lymphocytes were determined in peripheral blood obtained from 24 control subjects and 15 multiple sclerosis patients. The total number of lymphocytes and T lymphocytes were reduced significantly in multiple sclerosis patients as compared with those of controls. Although the percentage of B lymphocytes...

journal_title：Neurology

authors： Reddy MM,Goh KO

更新日期：1976-10-01 00:00:00

abstract：:The biochemical hallmark of adult Refsum disease (ARD) is an isolated deficiency in the breakdown of phytanic acid. This usually results from a PHYH gene defect, although some cases have been found to carry a PEX7 defect. We describe the phenotype of such a patient, indistinguishable from that of classic ARD. Hence, w...

journal_title：Neurology

authors： Horn MA,van den Brink DM,Wanders RJ,Duran M,Poll-The BT,Tallaksen CM,Stokke OH,Moser H,Skjeldal OH

更新日期：2007-02-27 00:00:00

abstract：:An infant with Pena-Shokeir phenotype was born to a cocaine-using mother. The pathologic findings included polyhydramnios, facial anomalies, arthrogryposis, camptodactyly, pulmonary hypoplasia, and tetralogy of Fallot. The neuropathologic findings were diffuse brainstem and spinal cord neuronal degeneration and focal ...

journal_title：Neurology

authors： Lavi E,Montone KT,Rorke LB,Kliman HJ

更新日期：1991-09-01 00:00:00

abstract：:We review adjunctive ketogenic diet (KD) and modified Atkins diet (MAD) treatment of refractory epilepsy in adults. Only a few studies have been published, all open-label. Because of the disparate, uncontrolled nature of the studies, we analyzed all studies individually, without a meta-analysis. Across all studies, 32...

journal_title：Neurology

authors： Klein P,Tyrlikova I,Mathews GC

更新日期：2014-11-18 00:00:00

abstract：OBJECTIVE:To investigate if patterns of CSF biomarkers (T-tau, P-tau, and Abeta42) can predict cognitive progression, outcome of cholinesterase inhibitor (ChEI) treatment, and mortality in Alzheimer disease (AD). METHODS:We included outpatients with AD (n = 151) from a prospective treatment study with ChEI. At baselin...

journal_title：Neurology

authors： Wallin AK,Blennow K,Zetterberg H,Londos E,Minthon L,Hansson O

更新日期：2010-05-11 00:00:00

abstract：BACKGROUND:Galantamine is a reversible, competitive cholinesterase inhibitor that also allosterically modulates nicotinic acetylcholine receptors. These mechanisms of action provided the rationale for a therapeutic trial of galantamine in AD. METHODS:A 6-month, multicenter, double-blind trial was undertaken in 636 pat...

journal_title：Neurology

authors： Raskind MA,Peskind ER,Wessel T,Yuan W

更新日期：2000-06-27 00:00:00

abstract：OBJECTIVE:The gradual development of cutaneous allodynia during the course of a migraine attack is commonly detected by quantitative sensory testing (QST) in migraineurs seeking secondary and tertiary medical help. In this study, the authors developed a questionnaire that tested the recollection of the patients on thei...

journal_title：Neurology

authors： Jakubowski M,Silberstein S,Ashkenazi A,Burstein R

更新日期：2005-11-08 00:00:00

abstract：:Inclusion-body myositis (IBM) is an inflammatory muscle disease that has proven resistant to treatment. Tumor necrosis factor molecules have been detected in muscle biopsies from patients with IBM. Etanercept is a TNFalpha receptor fusion protein that binds and inactivates tumor necrosis factor. Nine patients were tre...

journal_title：Neurology

authors： Barohn RJ,Herbelin L,Kissel JT,King W,McVey AL,Saperstein DS,Mendell JR

更新日期：2006-01-24 00:00:00

abstract：BACKGROUND:Previous studies have suggested a variety of factors that may be associated with the presence of hippocampal formation (HF) atrophy in patients with complex partial seizures (CPS), including a history of complex or prolonged febrile seizures (FS), age at seizure onset, and epilepsy duration. OBJECTIVE:To de...

journal_title：Neurology

authors： Theodore WH,Bhatia S,Hatta J,Fazilat S,DeCarli C,Bookheimer SY,Gaillard WD

更新日期：1999-01-01 00:00:00

abstract：OBJECTIVE:To assess the effects of the modified amino acid acetyl-dl-leucine (AL) on cerebellar ataxia, eye movements, and quality of life of patients with Niemann-Pick type C (NP-C) disease. METHODS:Twelve patients with NP-C disease were treated with AL 3 g/d for 1 week and then with 5 g/d for 3 weeks with a subseque...

journal_title：Neurology

authors： Bremova T,Malinová V,Amraoui Y,Mengel E,Reinke J,Kolníková M,Strupp M

更新日期：2015-10-20 00:00:00

abstract：OBJECTIVE:To determine whether blood-based biomarkers can differentiate older veterans with and without traumatic brain injury (TBI) and cognitive impairment (CogI). METHODS:We enrolled 155 veterans from 2 veterans' retirement homes: 90 without TBI and 65 with TBI history. Participants were further separated into CogI...

journal_title：Neurology

authors： Peltz CB,Kenney K,Gill J,Diaz-Arrastia R,Gardner RC,Yaffe K

更新日期：2020-09-01 00:00:00

abstract：:Neurologic complications of brain tissue-derived rabies vaccine may be chronic or progressive. Lymphocytes and serum from three patients with this form of encephalitis were studied. Two patients had positive lymphoproliferation to purified myelin, two had antibody to white matter, and one had antibody to myelin basic ...

journal_title：Neurology

authors： Hemachudha T,Griffin DE,Johnson RT,Giffels JJ

更新日期：1988-01-01 00:00:00

abstract：OBJECTIVE:To assess the potential relationship of ultraviolet B radiation (UVB) and Epstein-Barr virus (EBV) exposure in explaining the period prevalence of multiple sclerosis (MS) in England. METHODS:English national Hospital Episode Statistics covering all admissions to National Health Service hospitals in England i...

journal_title：Neurology

authors： Ramagopalan SV,Handel AE,Giovannoni G,Rutherford Siegel S,Ebers GC,Chaplin G

更新日期：2011-04-19 00:00:00

abstract：OBJECTIVE:There is only limited evidence from adequately controlled clinical trials to support high-dose methylprednisolone therapy for attacks of multiple sclerosis (MS) and none supporting oral administration. We assessed the effect of oral high-dose methylprednisolone therapy in attacks of MS. METHODS:Twenty-five p...

journal_title：Neurology

authors： Sellebjerg F,Frederiksen JL,Nielsen PM,Olesen J

更新日期：1998-08-01 00:00:00

abstract：OBJECTIVE:To determine, in a cross-sectional evaluation of nondemented individuals, if age-related memory decline is influenced by apolipoprotein E (apoE) genotype. BACKGROUND:The apoE-4 allele is an important risk factor for AD. PET in cognitively normal apoE-4 carriers (mean age, 56 years) shows reduced cerebral met...

journal_title：Neurology

authors： Caselli RJ,Graff-Radford NR,Reiman EM,Weaver A,Osborne D,Lucas J,Uecker A,Thibodeau SN

更新日期：1999-07-13 00:00:00

abstract：OBJECTIVE:Idiopathic REM sleep behavior disorder is a parasomnia characterized by dream enactment and is commonly a prediagnostic sign of parkinsonism and dementia. Since risk factors have not been defined, we initiated a multicenter case-control study to assess environmental and lifestyle risk factors for REM sleep be...

journal_title：Neurology

authors： Postuma RB,Montplaisir JY,Pelletier A,Dauvilliers Y,Oertel W,Iranzo A,Ferini-Strambi L,Arnulf I,Hogl B,Manni R,Miyamoto T,Mayer G,Stiasny-Kolster K,Puligheddu M,Ju Y,Jennum P,Sonka K,Santamaria J,Fantini ML,Zucconi

更新日期：2012-07-31 00:00:00