Cofactors of mitochondrial enzymes attenuate copper-induced death in vitro and in vivo.

abstract：:Hereditary canine spinal muscular atrophy (HCSMA) features rapidly progressive muscle weakness that affects muscles in an apparent proximal-to-distal gradient. In the medial gastrocnemius (MG) muscle of homozygous HCSMA animals, motor unit tetanic failure is apparent before the appearance of muscle weakness and appear...

journal_title：Annals of neurology

authors： Balice-Gordon RJ,Smith DB,Goldman J,Cork LC,Shirley A,Cope TC,Pinter MJ

更新日期：2000-05-01 00:00:00

abstract：OBJECTIVE:The prevalence of mitochondrial disease has proven difficult to establish, predominantly as a result of clinical and genetic heterogeneity. The phenotypic spectrum of mitochondrial disease has expanded significantly since the original reports that associated classic clinical syndromes with mitochondrial DNA (...

journal_title：Annals of neurology

authors： Gorman GS,Schaefer AM,Ng Y,Gomez N,Blakely EL,Alston CL,Feeney C,Horvath R,Yu-Wai-Man P,Chinnery PF,Taylor RW,Turnbull DM,McFarland R

更新日期：2015-05-01 00:00:00

abstract：:An infant presented with massive hyperchylomicronemia and a severe encephalopathy. MRI showed marked lipid deposition throughout the brain. Despite the normalization of the biochemistry, there was little clinical improvement, and at 18 months of age she has severe developmental delay, a strikingly abnormal MRI. Apolip...

journal_title：Annals of neurology

authors： Wilson CJ,Priore Oliva C,Maggi F,Catapano AL,Calandra S

更新日期：2003-06-01 00:00:00

abstract：:A patient is described with the dermatological features of blue rubber bleb nevus syndrome (BRBNS), focal seizures, and lateralized neurological signs. CAT scan demonstrated a nonenhanced density in the region of the vein of Galen. Neuropathological examination showed that this density was a clot within a vein of Gale...

journal_title：Annals of neurology

authors： Waybright EA,Selhorst JB,Rosenblum WI,Suter CG

更新日期：1978-05-01 00:00:00

abstract：:Alzheimer's disease (AD) and Parkinson's disease (PD) are generally considered to be separate and distinct disease entities. However, a considerable amount of evidence demonstrates that these disorders share common clinical and neuropathologic features and that overlap between the two conditions is extensive. For exam...

journal_title：Annals of neurology

authors： Perl DP,Olanow CW,Calne D

更新日期：1998-09-01 00:00:00

abstract：:We studied patients with partial and primary generalized seizures using fluorine-18-labeled 2-fluorodeoxyglucose and positron emission tomography. Interictal studies of patients with partial seizures showed regions of focal or lateralized hypometabolism in 15 of 17 patients with unilateral electroencephalographic foci...

journal_title：Annals of neurology

authors： Theodore WH,Brooks R,Sato S,Patronas N,Margolin R,Di Chiro G,Porter RJ

更新日期：1984-01-01 00:00:00

abstract：:A 51-year-old man with non-HLA-DR2 histocompatibility developed classic signs and symptoms of the narcoleptic tetrad soon after recovering from an episode of cardiopulmonary insufficiency, which occurred during induction of surgical anesthesia. Symptoms included excessive daytime sleepiness, hypnagogic hallucinations,...

journal_title：Annals of neurology

authors： Rivera VM,Meyer JS,Hata T,Ishikawa Y,Imai A

更新日期：1986-05-01 00:00:00

abstract：OBJECTIVE:Right-handedness and left-sided language lateralization is an unresolved mystery with unknown cause/effect relations. Most studies suggest that the language lateralization is related to a fundamental brain asymmetry: right-handedness may be secondary. We analyzed the possibility of an opposite cause/effect re...

journal_title：Annals of neurology

authors： Auer T,Pinter S,Kovacs N,Kalmar Z,Nagy F,Horvath RA,Koszo B,Kotek G,Perlaki G,Koves M,Kalman B,Komoly S,Schwarcz A,Woermann FG,Janszky J

更新日期：2009-01-01 00:00:00

abstract：OBJECTIVE:It is estimated that one of four ischemic strokes are noticed upon awakening and are not candidates for intravenous recombinant tissue plasminogen activator (rtPA) because their symptoms are >3 hours from last seen normal (LSN). We tested the safety of rtPA in a multicenter, single-arm, prospective, open-labe...

journal_title：Annals of neurology

authors： Barreto AD,Fanale CV,Alexandrov AV,Gaffney KC,Vahidy FS,Nguyen CB,Sarraj A,Rahbar M,Grotta JC,Savitz SI,Wake-Up Stroke Investigators.

更新日期：2016-08-01 00:00:00

abstract：:The prevalence and titer of serum antibodies to several Epstein-Barr virus (EBV) antigens were compared among patients with multiple sclerosis, healthy siblings of multiple sclerosis patients, patients with other neurological diseases, and healthy non-blood-related subjects. Serum-cerebrospinal fluid (serum-CSF) pairs...

journal_title：Annals of neurology

authors： Sumaya CV,Myers LW,Ellison GW,Ench Y

更新日期：1985-04-01 00:00:00

abstract：:We prepared RNA probes from cloned segments of human and murine enteroviruses (EVs) for in situ hybridization of skeletal muscle biopsies from patients with dermatomyositis (DM), polymyositis, other inflammatory myopathies, and noninflammatory muscle diseases, and from normal control subjects. A probe derived from The...

journal_title：Annals of neurology

authors： Rosenberg NL,Rotbart HA,Abzug MJ,Ringel SP,Levin MJ

更新日期：1989-08-01 00:00:00

abstract：:The term Charcot-Wilbrand syndrome (CWS) denotes dream loss following focal brain damage. We report the first case of CWS, in whom neuropsychological functions, extension of the underlying lesion, and sleep architecture changes were assessed. A 73-year-old woman reported a total dream loss after acute, bilateral occip...

journal_title：Annals of neurology

authors： Bischof M,Bassetti CL

更新日期：2004-10-01 00:00:00

abstract：:To study the distribution of myelin-associated glycoprotein (MAG) in human nervous tissue and in multiple sclerosis (MS) lesions, we used paraffin sections and our modification of the peroxidase-antiperoxidase technique. Sections of MS lesions also were treated with antiserum to basic protein (BP) and with histologica...

journal_title：Annals of neurology

authors： Itoyama Y,Sternberger NH,Webster HD,Quarles RH,Cohen SR,Richardson EP Jr

更新日期：1980-02-01 00:00:00

abstract：:Intracytoplasmic aggregation of alpha-synuclein protein as Lewy bodies in the brainstem neurons is diagnostic for Parkinson's disease, whereas if this process also occurs in the cortical neurons, it is considered pathognomonic for dementia with Lewy bodies. However, the link between alpha-synuclein incorporation into ...

journal_title：Annals of neurology

authors： Parkkinen L,Kauppinen T,Pirttilä T,Autere JM,Alafuzoff I

更新日期：2005-01-01 00:00:00

abstract：:Four infants had bilateral hippocampal sclerosis by magnetic resonance scans, including oblique coronal fast spin echo images of the temporal lobes; [18F]fluorodeoxyglucose-positron emission tomographic scans, done in 2 infants, showed isolated bilateral anterior temporal lobe hypometabolism. All had epilepsy with epi...

journal_title：Annals of neurology

authors： DeLong GR,Heinz ER

更新日期：1997-07-01 00:00:00

abstract：:In diabetic nerves, the activation of the polyol pathway and a resulting decrease in Na(+)-K(+) ATPase activity lead to intra-axonal Na(+) accumulation and a smaller Na(+) gradient across the axolemma than normal. To investigate whether glycemic control is associated with acutely reversible changes in axonal excitabil...

journal_title：Annals of neurology

authors： Kitano Y,Kuwabara S,Misawa S,Ogawara K,Kanai K,Kikkawa Y,Yagui K,Hattori T

更新日期：2004-10-01 00:00:00

abstract：OBJECTIVE:Accurate identification of the ischemic penumbra, the therapeutic target in acute clinical stroke, is of critical importance to identify patients who might benefit from reperfusion therapies beyond the established time windows. Therefore, we aimed to validate magnetic resonance imaging (MRI) mismatch-based pe...

journal_title：Annals of neurology

authors： Zaro-Weber O,Fleischer H,Reiblich L,Schuster A,Moeller-Hartmann W,Heiss WD

更新日期：2019-06-01 00:00:00

abstract：OBJECTIVE:To investigate the frequency and subtypes of mild cognitive impairment (MCI) in idiopathic rapid eye movement sleep behavior disorder (RBD) and Parkinson's disease (PD) in association with RBD. METHODS:One hundred and twelve subjects without dementia or major depression including 32 idiopathic RBD patients, ...

journal_title：Annals of neurology

authors： Gagnon JF,Vendette M,Postuma RB,Desjardins C,Massicotte-Marquez J,Panisset M,Montplaisir J

更新日期：2009-07-01 00:00:00

abstract：:Saturated very long-chain fatty acids in erythrocyte membranes, blood plasma, and mononuclear cells were studied in 4 patients with childhood-adolescent adrenoleukodystrophy and 4 patients with adult adrenoleukodystrophy and 19 normal control subjects by using high-performance liquid chromatography. Ratios of C26:0 to...

journal_title：Annals of neurology

authors： Antoku Y,Koike F,Ohtsuka Y,Sakai T,Tsukamoto K,Nagara H,Iwashita H,Goto I

更新日期：1991-07-01 00:00:00

abstract：OBJECTIVE:Nemaline myopathy (NM) is one of the most common congenital nondystrophic myopathies and is characterized by muscle weakness, often from birth. Mutations in ACTA1 are a frequent cause of NM (ie, NEM3). ACTA1 encodes alpha-actin 1, the main constituent of the sarcomeric thin filament. The mechanisms by which m...

journal_title：Annals of neurology

authors： Joureau B,de Winter JM,Conijn S,Bogaards SJP,Kovacevic I,Kalganov A,Persson M,Lindqvist J,Stienen GJM,Irving TC,Ma W,Yuen M,Clarke NF,Rassier DE,Malfatti E,Romero NB,Beggs AH,Ottenheijm CAC

更新日期：2018-02-01 00:00:00

abstract：:Hyperekplexia is a rare, autosomal dominant neurological disorder characterized by hypertonia, especially in infancy, and by an exaggerated startle response. This disorder is caused by mutations in the alpha 1 subunit of the inhibitory glycine receptor (GLRA1). We previously reported two GLRA1 point mutations detected...

journal_title：Annals of neurology

authors： Shiang R,Ryan SG,Zhu YZ,Fielder TJ,Allen RJ,Fryer A,Yamashita S,O'Connell P,Wasmuth JJ

更新日期：1995-07-01 00:00:00

abstract：:The etiology of neurodegenerative diseases remains enigmatic; however, evidence for defects in energy metabolism, excitotoxicity, and for oxidative damage is increasingly compelling. It is likely that there is a complex interplay between these mechanisms. A defect in energy metabolism may lead to neuronal depolarizati...

journal_title：Annals of neurology

authors： Beal MF

更新日期：1995-09-01 00:00:00

abstract：:Based on prior reports of free light chains of immunoglobulin G (IgG) in the cerebrospinal fluid (CSF) of patients with multiple sclerosis (MS), we quantitated free kappa and lambda chains and whole IgG concentrations using sensitive and specific radioimmunoassays (RIAs). The RIA for free kappa chains had a sensitivit...

journal_title：Annals of neurology

authors： Rudick RA,Pallant A,Bidlack JM,Herndon RM

更新日期：1986-07-01 00:00:00

abstract：:Axonal loss is thought to be a likely cause of persistent disability after a multiple sclerosis relapse; therefore, noninvasive in vivo markers specific for axonal loss are needed. We used optic neuritis as a model of multiple sclerosis relapse to quantify axonal loss of the retinal nerve fiber layer (RNFL) and second...

journal_title：Annals of neurology

authors： Trip SA,Schlottmann PG,Jones SJ,Altmann DR,Garway-Heath DF,Thompson AJ,Plant GT,Miller DH

更新日期：2005-09-01 00:00:00

abstract：OBJECTIVE:We compared outcomes after treatment with direct oral anticoagulants (DOACs) and vitamin K antagonists (VKAs) in patients with atrial fibrillation (AF) and a recent cerebral ischemia. METHODS:We conducted an individual patient data analysis of seven prospective cohort studies. We included patients with AF an...

journal_title：Annals of neurology

authors： Seiffge DJ,Paciaroni M,Wilson D,Koga M,Macha K,Cappellari M,Schaedelin S,Shakeshaft C,Takagi M,Tsivgoulis G,Bonetti B,Kallmünzer B,Arihiro S,Alberti A,Polymeris AA,Ambler G,Yoshimura S,Venti M,Bonati LH,Muir KW,Ya

更新日期：2019-06-01 00:00:00

abstract：:Urinary urgency and frequency are common in α-synucleinopathies such as Parkinson disease, Lewy body dementia, and multiple system atrophy. These symptoms cannot be managed with dopamine therapy, and their underlying pathophysiology is unclear. We show that in individuals with Parkinson disease, Lewy body dementia, or...

journal_title：Annals of neurology

authors： VanderHorst VG,Samardzic T,Saper CB,Anderson MP,Nag S,Schneider JA,Bennett DA,Buchman AS

更新日期：2015-07-01 00:00:00

abstract：:Although Alzheimer's and Parkinson's diseases predominately affect elderly adults, the proteins that play a role in the pathogenesis of these diseases are expressed throughout life. In fact, many of the proteins hypothesized to be important in the progression of neurodegeneration play direct or indirect roles in the d...

journal_title：Annals of neurology

authors： Rogers D,Schor NF

更新日期：2010-02-01 00:00:00

abstract：OBJECTIVE:To develop RNA splicing biomarkers of disease severity and therapeutic response in myotonic dystrophy type 1 (DM1) and type 2 (DM2). METHODS:In a discovery cohort, we used microarrays to perform global analysis of alternative splicing in DM1 and DM2. The newly identified splicing changes were combined with p...

journal_title：Annals of neurology

authors： Nakamori M,Sobczak K,Puwanant A,Welle S,Eichinger K,Pandya S,Dekdebrun J,Heatwole CR,McDermott MP,Chen T,Cline M,Tawil R,Osborne RJ,Wheeler TM,Swanson MS,Moxley RT 3rd,Thornton CA

更新日期：2013-12-01 00:00:00

abstract：:The purpose of this study was to explore the feasibility of obtaining magnetic resonance-measured cerebral metabolic rate of oxygen utilization (MR-CMRO(2)) in acute ischemic stroke patients. Seven stroke patients were serially imaged: 4.5 +/- 0.9 hours (tp1), 3 to 5 days (tp2), and 1 to 3 months (tp3) after symptom o...

journal_title：Annals of neurology

authors： Lee JM,Vo KD,An H,Celik A,Lee Y,Hsu CY,Lin W

更新日期：2003-02-01 00:00:00

abstract：:We have developed a radioimmunoassay to measure a specific neurological component, the basic protein of myelin, and have used this test for assessing this component in spinal fluid. The levels of basic protein in spinal fluid correlate closely with the clinical activity of multiple sclerosis; therefore the test can be...

journal_title：Annals of neurology

authors： Cohen SR,Brooks BR,Herndon RM,McKhann GM

更新日期：1980-07-01 00:00:00