Skin nerve pathology: Biomarkers of premanifest and manifest amyloid neuropathy.

abstract：:To define the molecular mechanisms underlying amphetamine (AMPH) neurotoxicity, primary cultures of dopaminergic neurons were examined for drug-induced changes in dopamine (DA) distribution, oxidative stress, protein damage, and cell death. As in earlier studies, AMPH rapidly redistributed vesicular DA to the cytoplas...

journal_title：Annals of neurology

authors： Lotharius J,O'Malley KL

更新日期：2001-01-01 00:00:00

abstract：:Neurotrophic factors have been demonstrated to prevent the development of peripheral neuropathy in animal models, but the therapeutic use of these factors in human disease has been limited by the short serum half-life and dose-limiting side effects of these potent peptides. We used peripheral subcutaneous inoculation ...

journal_title：Annals of neurology

authors： Chattopadhyay M,Wolfe D,Huang S,Goss J,Glorioso JC,Mata M,Fink DJ

更新日期：2002-01-01 00:00:00

abstract：:Mutations in DJ-1 (PARK7) have been reported in two consanguineous families with young-onset Parkinson's disease (YOPD). This study aims to confirm the presence of pathogenic DJ-1 mutations and determine their contribution in young-onset and more typical later onset Parkinson's disease (PD). The entire open reading fr...

journal_title：Annals of neurology

authors： Abou-Sleiman PM,Healy DG,Quinn N,Lees AJ,Wood NW

更新日期：2003-09-01 00:00:00

abstract：:We recorded horizontal smooth-pursuit responses to sinusoidal and step-ramp stimuli in 7 patients with unilateral frontal lobe lesions. Five patients had directional smooth-pursuit deficits, all with impairment toward the side of cerebral damage. Ipsidirectional pursuit defects involved pursuit maintenance to sinusoid...

journal_title：Annals of neurology

authors： Morrow MJ,Sharpe JA

更新日期：1995-04-01 00:00:00

abstract：:The hypothalamus was systematically examined in 30 patients with pathologically documented idiopathic parkinsonism. Using Lewy body formation as a marker for nerve cell degeneration, we observed abnormalities in every hypothalamus examined. Of the thirteen hypothalamic nuclei that could be individually identified, non...

journal_title：Annals of neurology

authors： Langston JW,Forno LS

更新日期：1978-02-01 00:00:00

abstract：:Systems for automatic assessment of cutaneous touch-pressure, vibratory, and thermal sensation have been developed. These systems use stimuli which are quantified and reproducible, a two-alternative forced-choice technique, and programmed steps to test, score, and report. If normal responses from series of healthy per...

journal_title：Annals of neurology

authors： Dyck PJ,Zimmerman IR,O'Brien PC,Ness A,Caskey PE,Karnes J,Bushek W

更新日期：1978-12-01 00:00:00

abstract：OBJECTIVE:Migraine is among the most common and debilitating neurological conditions. Familial hemiplegic migraine type 1 (FHM1), a monogenic migraine subtype, is caused by gain-of-function of voltage-gated CaV 2.1 calcium channels. FHM1 mice carry human pathogenic mutations in the α1A subunit of CaV 2.1 channels and a...

journal_title：Annals of neurology

authors： Eikermann-Haerter K,Arbel-Ornath M,Yalcin N,Yu ES,Kuchibhotla KV,Yuzawa I,Hudry E,Willard CR,Climov M,Keles F,Belcher AM,Sengul B,Negro A,Rosen IA,Arreguin A,Ferrari MD,van den Maagdenberg AM,Bacskai BJ,Ayata C

更新日期：2015-08-01 00:00:00

abstract：:Basis pontis lacunes cause contralateral but rarely ipsilateral ataxia. We explored this phenomenon with isotope tract tracing in the rhesus monkey. Labeled pontocerebellar fibers cross midline and disperse widely in the opposite hemipons before coalescing in the brachium pontis. This anatomical arrangement suggests t...

journal_title：Annals of neurology

authors： Schmahmann JD,Rosene DL,Pandya DN

更新日期：2004-04-01 00:00:00

abstract：:To elucidate the role of D-serine in human central nervous system, we analyzed D-serine, L-serine, and glycine concentrations in cerebrospinal fluid of healthy children and children with a defective L-serine biosynthesis (3-phosphoglycerate dehydrogenase deficiency). Healthy children showed high D-serine concentration...

journal_title：Annals of neurology

authors： Fuchs SA,Dorland L,de Sain-van der Velden MG,Hendriks M,Klomp LW,Berger R,de Koning TJ

更新日期：2006-10-01 00:00:00

abstract：OBJECTIVE:To assess the associations of blood biomarkers of carbohydrate, lipid, and apolipoprotein metabolisms with the future risk of amyotrophic lateral sclerosis (ALS). METHODS:In the Apolipoprotein-related MOrtality RISk study, we enrolled 636,132 men and women during 1985-1996 in Stockholm, Sweden, with measurem...

journal_title：Annals of neurology

authors： Mariosa D,Hammar N,Malmström H,Ingre C,Jungner I,Ye W,Fang F,Walldius G

更新日期：2017-05-01 00:00:00

abstract：OBJECTIVE:Amyotrophic lateral sclerosis (ALS) is a motor neuron disease whose pathophysiological deficits, causing impairment in motor function, are largely unknown. Here we propose that hydrogen sulfide (H2 S), as a glial-released inflammatory factor, contributes to ALS-mediated motor neuron death. METHODS:H2 S conce...

journal_title：Annals of neurology

authors： Davoli A,Greco V,Spalloni A,Guatteo E,Neri C,Rizzo GR,Cordella A,Romigi A,Cortese C,Bernardini S,Sarchielli P,Cardaioli G,Calabresi P,Mercuri NB,Urbani A,Longone P

更新日期：2015-04-01 00:00:00

abstract：:The mechanism whereby dystrophin deficiency leads to excessive fibrosis and muscle degeneration is not known. The absence of dystrophin in skeletal muscle is associated with reduced plasma membrane stability as evidenced by elevated serum levels of the cytoplasmic enzyme creatine kinase. Basic fibroblast growth factor...

journal_title：Annals of neurology

authors： D'Amore PA,Brown RH Jr,Ku PT,Hoffman EP,Watanabe H,Arahata K,Ishihara T,Folkman J

更新日期：1994-03-01 00:00:00

abstract：:Mutations of SURF-1, a gene located on chromosome 9q34, have recently been identified in patients affected by Leigh syndrome (LS), associated with deficiency of cytochrome c oxidase (COX), the terminal component of the mitochondrial respiratory chain. To investigate to what extent SURF-1 is responsible for human disor...

journal_title：Annals of neurology

authors： Tiranti V,Jaksch M,Hofmann S,Galimberti C,Hoertnagel K,Lulli L,Freisinger P,Bindoff L,Gerbitz KD,Comi GP,Uziel G,Zeviani M,Meitinger T

更新日期：1999-08-01 00:00:00

abstract：:We investigated the relationship between cerebral activity (measured with positron emission tomography) and word rate in normal subjects and aphasic patients listening to monosyllabic words at rates up to those encountered in normal speech. By measuring the slope of the regression of the individual activity-word rate ...

journal_title：Annals of neurology

authors： Leff A,Crinion J,Scott S,Turkheimer F,Howard D,Wise R

更新日期：2002-05-01 00:00:00

abstract：OBJECTIVE:The aim of the study was to evaluate whether the visual analysis of muscle magnetic resonance imaging scans can identify specific patterns of muscle involvement. METHODS:We assessed scans from 83 patients with muscle disorders characterized by rigidity of the spine secondary to mutations in 4 different genes...

journal_title：Annals of neurology

authors： Mercuri E,Clements E,Offiah A,Pichiecchio A,Vasco G,Bianco F,Berardinelli A,Manzur A,Pane M,Messina S,Gualandi F,Ricci E,Rutherford M,Muntoni F

更新日期：2010-02-01 00:00:00

abstract：:The blood-brain barrier is a natural diffusion barrier, which expresses active carriers extruding drugs on their way to the brain back into the blood against concentration gradients. Whereas these so-called adenosine triphosphate-binding cassette (ABC) transporters prevent the brain entry of toxic compounds under phys...

journal_title：Annals of neurology

authors： Hermann DM,Kilic E,Spudich A,Krämer SD,Wunderli-Allenspach H,Bassetti CL

更新日期：2006-11-01 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：:We demonstrate that the 3-hydroxy-3-methyl-glutaryl-coenzyme A (HMG-CoA) reductase inhibitors atorvastatin and simvastatin enhance functional outcome and induce brain plasticity when administered after stroke to rats. With atorvastatin treatment initiated 1 day after stroke, animals exhibited significant increases in ...

journal_title：Annals of neurology

authors： Chen J,Zhang ZG,Li Y,Wang Y,Wang L,Jiang H,Zhang C,Lu M,Katakowski M,Feldkamp CS,Chopp M

更新日期：2003-06-01 00:00:00

abstract：OBJECTIVE:Metrics of diffusion tensor imaging (DTI) and magnetization transfer imaging (MTI) can detect diffuse axonal injury in traumatic brain injury (TBI). The relationship between the changes in these imaging measures and the underlying pathologies is still relatively unknown. This study investigated the radiologic...

journal_title：Annals of neurology

authors： Tu TW,Williams RA,Lescher JD,Jikaria N,Turtzo LC,Frank JA

更新日期：2016-06-01 00:00:00

abstract：:Excessive astrocytosis in cortical tubers in tuberous sclerosis complex (TSC) suggests that astrocytes may be important for epileptogenesis in TSC. We previously demonstrated that astrocyte-specific Tsc1 gene inactivation in mice (Tsc1 cKO mice) results in progressive epilepsy. Here, we report that glutamate transport...

journal_title：Annals of neurology

authors： Wong M,Ess KC,Uhlmann EJ,Jansen LA,Li W,Crino PB,Mennerick S,Yamada KA,Gutmann DH

更新日期：2003-08-01 00:00:00

abstract：:Multiple sclerosis patients who become pregnant experience a significant decrease in relapses that may be mediated by a shift in immune responses from T helper 1 to T helper 2. Animal models of multiple sclerosis have shown that the pregnancy hormone, estriol, can ameliorate disease and can cause an immune shift. We t...

journal_title：Annals of neurology

authors： Sicotte NL,Liva SM,Klutch R,Pfeiffer P,Bouvier S,Odesa S,Wu TC,Voskuhl RR

更新日期：2002-10-01 00:00:00

abstract：:A young man developed pathological thirst and hyperdipsia, hyperphagia, disordered temperature regulation, a lowered threshold for aggressive behavior, apathy, impaired memory, and seizures following encephalitis. He had marked hyponatremia. Bouts of water drinking produced water intoxication and precipitated status e...

journal_title：Annals of neurology

authors： Howe JG,Penney MD,Currie S,Morgan D

更新日期：1983-02-01 00:00:00

abstract：:Cerebral blood flow (CBF) velocity is decreased in patients with Alzheimer's disease. It is being debated whether this reflects diminished demand because of advanced neurodegeneration or that cerebral hypoperfusion contributes to dementia. We examined the relation of CBF velocity as measured with transcranial Doppler ...

journal_title：Annals of neurology

authors： Ruitenberg A,den Heijer T,Bakker SL,van Swieten JC,Koudstaal PJ,Hofman A,Breteler MM

更新日期：2005-06-01 00:00:00

abstract：:A 42-year-old homosexual man without evidence of immune deficiency developed cerebral granulomatous angiitis in association with the isolation of human T-lymphotropic virus type III (HTLV-III) from brain tissue and cerebrospinal fluid. This syndrome may be an additional neurological sequela of HTLV-III infection. ...

journal_title：Annals of neurology

authors： Yankner BA,Skolnik PR,Shoukimas GM,Gabuzda DH,Sobel RA,Ho DD

更新日期：1986-09-01 00:00:00

abstract：:In cases of Alzheimer's presenile and senile dementia, Alzheimer's disease (AD) and senile dementia of the Alzheimer type (SDAT), respectively, we have observed, in addition to the gray matter degeneration, a lesion that has the character of an incomplete infarction confined to the white matter. It is encountered in 6...

journal_title：Annals of neurology

authors： Brun A,Englund E

更新日期：1986-03-01 00:00:00

abstract：:The value of various enhancement techniques as opposed to nonenhanced CT scanning was compared in 15 baboons with cerebral infarction secondary to embolization of the left middle cerebral artery. The most prominent CT findings in infarction included an area of low absorption in the opercula--basal ganglia--centrum sem...

journal_title：Annals of neurology

authors： Drayer BP,Dujovny M,Wolfson SK Jr,Boehnke M,Cook EE,Rosenbaum AE

更新日期：1979-01-01 00:00:00

abstract：:Dentatorubral-pallidoluysian atrophy is an autosomal dominant neurodegenerative disease characterized by various combinations of ataxia, choreoathetosis, myoclonus, epilepsy, and dementia as well as a wide range of ages at onset. A specific unstable trinucleotide repeat expansion in a gene on the short arm of chromoso...

journal_title：Annals of neurology

authors： Ikeuchi T,Koide R,Tanaka H,Onodera O,Igarashi S,Takahashi H,Kondo R,Ishikawa A,Tomoda A,Miike T

更新日期：1995-06-01 00:00:00

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：OBJECTIVE:To evaluate the efficacy and safety of BHT-3009 in relapsing-remitting multiple sclerosis (MS) and to confirm that BHT-3009 causes immune tolerance. METHODS:BHT-3009 is a tolerizing DNA vaccine for MS, encoding full-length human myelin basic protein. Relapsing-remitting MS patients were randomized 1:1:1 into...

journal_title：Annals of neurology

authors： Garren H,Robinson WH,Krasulová E,Havrdová E,Nadj C,Selmaj K,Losy J,Nadj I,Radue EW,Kidd BA,Gianettoni J,Tersini K,Utz PJ,Valone F,Steinman L,BHT-3009 Study Group.

更新日期：2008-05-01 00:00:00

abstract：:Despite many years of intensive research, multiple sclerosis (MS) defies understanding and treatment remains suboptimal. The prevailing hypothesis is that MS is immune mediated and that experimental allergic encephalomyelitis (EAE) is a suitable model to elucidate pathogenesis and devise therapy. This review examines ...

journal_title：Annals of neurology

authors： Sriram S,Steiner I

更新日期：2005-12-01 00:00:00