Efficient three-drug cocktail for disease induced by mutant superoxide dismutase.

abstract：OBJECTIVE:To examine the functional neuroanatomy that could account for pure Gerstmann syndrome, which is the selective association of acalculia, finger agnosia, left-right disorientation, and agraphia. METHODS:We used structural and functional neuroimaging at high spatial resolution in healthy subjects to seek a shar...

journal_title：Annals of neurology

authors： Rusconi E,Pinel P,Eger E,LeBihan D,Thirion B,Dehaene S,Kleinschmidt A

更新日期：2009-11-01 00:00:00

abstract：:Homocarnosine, a dipeptide of gamma-aminobutyric acid (GABA) and histidine, is thought to be an inhibitory neuromodulator synthesized in subclasses of GABAergic neurons. Homocarnosine is present in human brain in greater amounts (0.4-1.0 micromol/g) than in other animals. The antiepileptic drug vigabatrin increases hu...

journal_title：Annals of neurology

authors： Petroff OA,Mattson RH,Behar KL,Hyder F,Rothman DL

更新日期：1998-12-01 00:00:00

abstract：:We studied the in vitro electrical activity of rat neostriatal neurons following chronic neuroleptic treatment. In haloperidol-treated rats, unlike naive animals, activation of neostriatal D2 dopamine receptors induced a potent presynaptic inhibition of glutamate-mediated excitatory synaptic potentials. Haloperidol tr...

journal_title：Annals of neurology

authors： Calabresi P,De Murtas M,Mercuri NB,Bernardi G

更新日期：1992-04-01 00:00:00

abstract：:Isolated vertigo with horizontal positional nystagmus as an impending sign of a central lesion has rarely been reported. Here we present neuro-otologic findings of patients with these clinical signs. Lesion overlays from 6 patients with ageotropic positional nystagmus revealed that the nodulus and vermis are common ar...

journal_title：Annals of neurology

authors： Lee HJ,Kim ES,Kim M,Chu H,Ma HI,Lee JS,Koo JW,Kim HJ,Hong SK

更新日期：2014-12-01 00:00:00

abstract：OBJECTIVES:Amyloid-beta(42) (Abeta(42)) appears central to Alzheimer's disease (AD) pathogenesis and is a major component of amyloid plaques. Mean cerebrospinal fluid (CSF) Abeta(42) is decreased in dementia of the Alzheimer's type. This decrease may reflect plaques acting as an Abeta(42) "sink," hindering transport of...

journal_title：Annals of neurology

authors： Fagan AM,Mintun MA,Mach RH,Lee SY,Dence CS,Shah AR,LaRossa GN,Spinner ML,Klunk WE,Mathis CA,DeKosky ST,Morris JC,Holtzman DM

更新日期：2006-03-01 00:00:00

abstract：:Axonal degeneration is a major cause of permanent neurological deficit in multiple sclerosis (MS), but no current therapies for the disease are known to be effective at axonal protection. Here, we examine the ability of a sodium channel-blocking agent, flecainide, to reduce axonal degeneration in an experimental model...

journal_title：Annals of neurology

authors： Bechtold DA,Kapoor R,Smith KJ

更新日期：2004-05-01 00:00:00

abstract：OBJECTIVE:To determine the clinical consequences of the PMP22 point mutation, T118M, which has been previously considered to either cause an autosomal recessive form of Charcot-Marie-Tooth (CMT) disease or be a benign polymorphism. METHODS:We analyzed patients from five separate kindreds and characterized their periph...

journal_title：Annals of neurology

authors： Shy ME,Scavina MT,Clark A,Krajewski KM,Li J,Kamholz J,Kolodny E,Szigeti K,Fischer RA,Saifi GM,Scherer SS,Lupski JR

更新日期：2006-02-01 00:00:00

abstract：OBJECTIVE:A study was undertaken to establish an enzyme-linked immunosorbent assay (ELISA) to detect JC virus (JCV)-specific antibodies in multiple sclerosis (MS) patients, and to evaluate its potential utility for identifying patients at higher or lower risk (ie, risk stratification) of developing progressive multifoc...

journal_title：Annals of neurology

authors： Gorelik L,Lerner M,Bixler S,Crossman M,Schlain B,Simon K,Pace A,Cheung A,Chen LL,Berman M,Zein F,Wilson E,Yednock T,Sandrock A,Goelz SE,Subramanyam M

更新日期：2010-09-01 00:00:00

abstract：:To explore the concept that dystonia may result from dysfunction of the sensory system, 14 patients with focal hand dystonia were tested during two somatosensory discrimination tasks. Compared with controls, patients had a higher threshold in a task involving discrimination of two electric stimuli closely related temp...

journal_title：Annals of neurology

authors： Bara-Jimenez W,Shelton P,Sanger TD,Hallett M

更新日期：2000-03-01 00:00:00

abstract：:The long-term effects of brief but repetitive febrile seizures (FS) on memory have not been as thoroughly investigated as the impact of single and prolonged seizure in the developing brain. Using a heated-air FS paradigm, we subjected male rat pups to one, three, or nine episodes of brief FS on days 10 to 12 postpartu...

journal_title：Annals of neurology

authors： Chang YC,Huang AM,Kuo YM,Wang ST,Chang YY,Huang CC

更新日期：2003-12-01 00:00:00

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:A study was undertaken to describe the clinical spectrum, voltage-gated potassium channel (VGKC) complex antibody specificities, and central nervous system localization of antibody binding in 29 patients diagnosed with Morvan syndrome (MoS). METHODS:Clinical data were collected using questionnaires. Radioimm...

journal_title：Annals of neurology

authors： Irani SR,Pettingill P,Kleopa KA,Schiza N,Waters P,Mazia C,Zuliani L,Watanabe O,Lang B,Buckley C,Vincent A

更新日期：2012-08-01 00:00:00

abstract：:The etiology of sudden death in patients with epilepsy remains unclear. Previous studies in a well-established sheep model of status epilepticus showed that more than one-third of the unsedated animals died within 5 minutes of seizure onset due to hypoventilation. The relative contributions of airway obstruction and c...

journal_title：Annals of neurology

authors： Johnston SC,Siedenberg R,Min JK,Jerome EH,Laxer KD

更新日期：1997-10-01 00:00:00

abstract：:The effect of focal ischemia on tissue pH was studied at various times up to 6 hours after permanent middle cerebral artery occlusion in rats. Tissue pH was imaged by using umbelliferone fluorescence and correlated with cerebral blood flow, ATP content, and recordings of the steady potential. Circumscribed foci of all...

journal_title：Annals of neurology

authors： Back T,Hoehn M,Mies G,Busch E,Schmitz B,Kohno K,Hossmann KA

更新日期：2000-04-01 00:00:00

abstract：OBJECTIVE:The relationship between genetic variation in the T-type calcium channel gene CACNA1H and childhood absence epilepsy is well established. The purpose of this study was to investigate the range of epilepsy syndromes for which CACNA1H variants may contribute to the genetic susceptibility architecture and determ...

journal_title：Annals of neurology

authors： Heron SE,Khosravani H,Varela D,Bladen C,Williams TC,Newman MR,Scheffer IE,Berkovic SF,Mulley JC,Zamponi GW

更新日期：2007-12-01 00:00:00

abstract：:In a double-blinded, placebo-controlled, crossover study in seven mitochondrial myopathy patients (MM), we investigated whether lowering of lactate with dichloroacetate (DCA) can improve exercise tolerance and oxidative capacity in MM. DCA lowered plasma lactate at rest and during exercise (from 10.5 +/- 2.0 to 5.0 +/...

journal_title：Annals of neurology

authors： Vissing J,Gansted U,Quistorff B

更新日期：2001-05-01 00:00:00

abstract：:We report the clinical and neuropathological findings in an immunocompetent 19-year-old patient with a fatal acute Epstein-Barr virus (EBV) meningoencephalitis and a lymphoma-like B-lymphocyte response. Our results suggest that an immunotoxic rather than direct viral neuronal invasion mediates brain damage in EBV ence...

journal_title：Annals of neurology

authors： Schellinger PD,Sommer C,Leithäuser F,Schwab S,Storch-Hagenlocher B,Hacke W,Kiessling M

更新日期：1999-05-01 00:00:00

abstract：:Calmodulin is a major Ca2+ -binding protein that may mediate many Ca2+ -regulated processes in neuronal function. Calmodulin is present in the presynaptic nerve terminal in association with synaptic vesicles and in postsynaptic density fractions. Several calmodulin-regulated synaptic biochemical processes have been id...

journal_title：Annals of neurology

authors： DeLorenzo RJ

更新日期：1984-01-01 00:00:00

abstract：:Ten laminin alpha2-deficient patients were identified by both immunofluorescence and immunoblotting (30% of congenital muscular dystrophy patients tested). Three of the laminin alpha2-deficient patients were carrying a diagnosis of infantile polymyositis prior to immunostaining studies. The clinical features in the 10...

journal_title：Annals of neurology

authors： Pegoraro E,Mancias P,Swerdlow SH,Raikow RB,Garcia C,Marks H,Crawford T,Carver V,Di Cianno B,Hoffman EP

更新日期：1996-11-01 00:00:00

abstract：:Analysis of mitochondrial DNA (mtDNA) in muscle and blood from 72 patients with mitochondrial myopathy showed that 30 had major deletions of a variable proportion of muscle mtDNA. All of these 30 patients presented with progressive external ophthalmoplegia and limb weakness, and 8 had the additional features of the Ke...

journal_title：Annals of neurology

authors： Holt IJ,Harding AE,Cooper JM,Schapira AH,Toscano A,Clark JB,Morgan-Hughes JA

更新日期：1989-12-01 00:00:00

abstract：:This study provides preliminary evidence that computer-derived parameters of interictal spike waveforms can be used to assess seizure hazard in a quantitative manner. Thirteen children with spike foci and simple or complex partial seizures were studied before and during treatment with carbamazepine or phenobarbital; s...

journal_title：Annals of neurology

authors： Frost JD Jr,Kellaway P,Hrachovy RA,Glaze DG,Mizrahi EM

更新日期：1986-12-01 00:00:00

abstract：:We report a patient with a meningioma presenting with Cushing's syndrome, an unprecedented paraneoplastic presentation. Molecular studies, bioassay, and high-performance liquid chromatography of tumor specimens demonstrated the synthesis and secretion of bioactive corticotropin-releasing hormone. To our knowledge, thi...

journal_title：Annals of neurology

authors： Pecori Giraldi F,Terreni MR,Andreotti C,Losa M,Lanzi R,Pontiroli AE,Cavagnini F

更新日期：2003-01-01 00:00:00

abstract：:Recent studies have indicated a normal gene dose for the amyloid precursor protein (APP) in Alzheimer's disease (AD). These findings leave open the possibility that elevated levels of messenger RNA (mRNA) for this protein may contribute to the pathogenesis of AD. Using Northern analysis, we compared the levels of mRNA...

journal_title：Annals of neurology

authors： Clark AW,Krekoski CA,Parhad IM,Liston D,Julien JP,Hoar DI

更新日期：1989-04-01 00:00:00

abstract：OBJECTIVE:A study was undertaken to examine the association between overall cardiovascular health as recently defined by the American Heart Association in young adulthood to middle age and cognitive function in midlife. Overall ideal cardiovascular health incorporates 7 metrics, including the avoidance of overweight or...

journal_title：Annals of neurology

authors： Reis JP,Loria CM,Launer LJ,Sidney S,Liu K,Jacobs DR Jr,Zhu N,Lloyd-Jones DM,He K,Yaffe K

更新日期：2013-02-01 00:00:00

abstract：:Parkinson's disease evolves slowly, and there is current interest in exploring the earliest stages of the disorder, because of new approaches to studying pathogenesis and developing potential neuroprotective treatment. Recognizing early Parkinson's disease is not easy. The certainty of diagnosis increases as the disea...

journal_title：Annals of neurology

authors： Calne DB,Snow BJ,Lee C

更新日期：1992-01-01 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：OBJECTIVE:The objective of this study was to evaluate patients with ganglionic acetylcholine receptor antibody (gAChR-Ab) positive autoimmune autonomic ganglionopathy using a multimodal testing protocol to characterize their full clinical phenotype and explore biomarkers to quantify immunotherapy response. METHODS:We ...

journal_title：Annals of neurology

authors： Koay S,Vichayanrat E,Bremner F,Panicker JN,Lang B,Lunn MP,Watson L,Ingle GT,Hagen EM,McNamara P,Jacobson L,Provitera V,Nolano M,Vincent A,Mathias CJ,Iodice V

更新日期：2021-01-13 00:00:00

abstract：:Primary and secondary conditions leading to thiamine deficiency have overlapping features in children, presenting with acute episodes of encephalopathy, bilateral symmetric brain lesions, and high excretion of organic acids that are specific of thiamine-dependent mitochondrial enzymes, mainly lactate, alpha-ketoglutar...

journal_title：Annals of neurology

authors： Ortigoza-Escobar JD,Alfadhel M,Molero-Luis M,Darin N,Spiegel R,de Coo IF,Gerards M,Taylor RW,Artuch R,Nashabat M,Rodríguez-Pombo P,Tabarki B,Pérez-Dueñas B,Thiamine Deficiency Study Group.

更新日期：2017-09-01 00:00:00

abstract：:To characterize the protein composition of degenerating neurons in Alzheimer disease, enriched fractions of isolated cortical neurons from postmortem Alzheimer brain were compared by ultrastructural and biochemical techniques to neuronal isolates from aged normal controls and from patients with the nonfibrillary degen...

journal_title：Annals of neurology

authors： Selkoe DJ

更新日期：1980-11-01 00:00:00

abstract：:Eighty-three patients with epidural spinal cord compression, from metastatic cancer were treated with high-dose adrenocorticosteroids and a new radiation fractionation protocol. Only those patients were included who had complete or almost complete block on myelography and who had not received prior radiation therapy t...

journal_title：Annals of neurology

authors： Greenberg HS,Kim JH,Posner JB

更新日期：1980-10-01 00:00:00