Dichloroacetate exerts therapeutic effects in transgenic mouse models of Huntington's disease.

abstract：OBJECTIVE:The objective of this study was to investigate whether cytosolic phospholipase A2 (cPLA2 ), an important isoform of PLA2 that mediates the release of arachidonic acid, plays a role in the pathogenesis of spinal cord injury (SCI). METHODS:A combination of molecular, histological, immunohistochemical, and beha...

journal_title：Annals of neurology

authors： Liu NK,Deng LX,Zhang YP,Lu QB,Wang XF,Hu JG,Oakes E,Bonventre JV,Shields CB,Xu XM

更新日期：2014-05-01 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：:Electroencephalographic (EEG) and evoked potential data were recorded during behavioral testing from 8 dyslexic and 10 normal boys aged 9 to 11 years. Topographic mapping of their brain electrical activity revealed four discrete regions of difference between the two groups involving both hemispheres, left more than ri...

journal_title：Annals of neurology

authors： Duffy FH,Denckla MB,Bartels PH,Sandini G

更新日期：1980-05-01 00:00:00

abstract：:The characteristic pathological features of Huntington's disease (HD) are neostriatal atrophy and neuronal loss. Although neuroradiological studies often show caudate atrophy in patients with moderate HD, frequently no caudate atrophy is found early in the illness. There have been no quantitative reports to date on in...

journal_title：Annals of neurology

authors： Harris GJ,Pearlson GD,Peyser CE,Aylward EH,Roberts J,Barta PE,Chase GA,Folstein SE

更新日期：1992-01-01 00:00:00

abstract：:A nested polymerase chain reaction was used for the detection of Epstein-Barr virus DNA in 1 patient with encephalitis, and in 1 patient with myelitis. Epstein-Barr virus DNA was detected in cerebrospinal fluid samples obtained at the onset of neurological symptoms in both patients, and serological findings indicated ...

journal_title：Annals of neurology

authors： Landgren M,Kyllerman M,Bergström T,Dotevall L,Ljungström L,Ricksten A

更新日期：1994-05-01 00:00:00

abstract：:We studied neuronal activity and its relation to the accumulation of neurofibrillary tangles in Alzheimer's disease (AD) neurons by in situ hybridization to cytochrome oxidase subunit III messenger RNA, a marker of mitochondrial energy metabolism. In AD midtemporal cortex, levels of cytochrome oxidase subunit III mess...

journal_title：Annals of neurology

authors： Hatanpää K,Brady DR,Stoll J,Rapoport SI,Chandrasekaran K

更新日期：1996-09-01 00:00:00

abstract：:We describe a 50-year-old male patient with hyponatremia (serum sodium level, 128 mEq/L) discovered during routine follow-up for Henoch-Schönlein nephritis. The patient was known to have a generalized idiopathic epilepsy and was on 2,000 mg/day of sodium valproate. After exclusion of other causes such as hypothyroidis...

journal_title：Annals of neurology

authors： Branten AJ,Wetzels JF,Weber AM,Koene RA

更新日期：1998-02-01 00:00:00

abstract：:The frequency with which patients presenting with acute or chronic noncompressive cord syndromes subsequently develop multiple sclerosis is uncertain. Magnetic resonance imaging (MRI) was performed on 121 patients with such syndromes to determine the frequency of asymptomatic brain lesions and to assess the sensitivit...

journal_title：Annals of neurology

authors： Miller DH,McDonald WI,Blumhardt LD,du Boulay GH,Halliday AM,Johnson G,Kendall BE,Kingsley DP,MacManus DG,Moseley IF

更新日期：1987-12-01 00:00:00

abstract：OBJECTIVE:Reducing health and economic burdens from diagnostic delay of psychogenic nonepileptic seizures (PNES) requires prompt referral for video electroencephalography (VEEG) monitoring, the diagnostic gold standard. Practitioners make VEEG referrals when semiology suggests PNES, although few semiological signs are ...

journal_title：Annals of neurology

authors： Syed TU,LaFrance WC Jr,Kahriman ES,Hasan SN,Rajasekaran V,Gulati D,Borad S,Shahid A,Fernandez-Baca G,Garcia N,Pawlowski M,Loddenkemper T,Amina S,Koubeissi MZ

更新日期：2011-06-01 00:00:00

abstract：OBJECTIVE:To analyze the cumulative efficacy and safety of everolimus in treating subependymal giant cell astrocytomas (SEGA) associated with tuberous sclerosis complex (TSC) from an open-label phase II study (NCT00411619). Updated data became available from the conclusion of the extension phase and are presented in th...

journal_title：Annals of neurology

authors： Franz DN,Agricola K,Mays M,Tudor C,Care MM,Holland-Bouley K,Berkowitz N,Miao S,Peyrard S,Krueger DA

更新日期：2015-12-01 00:00:00

abstract：:The phenotype of severe congenital hydrocephalus secondary to neural cell adhesion molecule L1 (L1CAM) gene mutations includes the distinct finding of brainstem corticospinal tract hypoplasia. Using diffusion-weighted imaging (DWI), we failed to demonstrate anisotropy in the corticospinal tracts of the basis pontis in...

journal_title：Annals of neurology

authors： Graf WD,Born DE,Shaw DW,Thomas JR,Holloway LW,Michaelis RC

更新日期：2000-01-01 00:00:00

abstract：:Twenty-five patients with recently diagnosed leptomeningeal metastases underwent a prospective clinical and electro-physiological study to assess the mode and extent of peripheral nerve and spinal root involvement. Motor conduction velocities and F wave latencies in the legs were found frequently to be disturbed compa...

journal_title：Annals of neurology

authors： Argov Z,Siegal T

更新日期：1985-06-01 00:00:00

abstract：:We measured the intrinsic mechanical properties and protein content of single skinned muscle fibers obtained from patients who had Duchenne muscular dystrophy. To check for possible nonspecific changes caused by muscle disease per se, we also studied the properties of muscle fibers obtained from patients exhibiting se...

journal_title：Annals of neurology

authors： Horowits R,Dalakas MC,Podolsky RJ

更新日期：1990-06-01 00:00:00

abstract：:Evidence implicating mitochondrial dysfunction in the central nervous system of patients with sporadic amyotrophic lateral sclerosis (SALS) has recently been accumulating. In contrast, data on mitochondrial function in skeletal muscle in SALS are scarce and controversial. We investigated the in situ properties of musc...

journal_title：Annals of neurology

authors： Echaniz-Laguna A,Zoll J,Ribera F,Tranchant C,Warter JM,Lonsdorfer J,Lampert E

更新日期：2002-11-01 00:00:00

abstract：OBJECTIVE:Intracranial electroencephalography (ICEEG) with chronically implanted electrodes is a costly invasive diagnostic procedure that remains necessary for a large proportion of patients who undergo evaluation for epilepsy surgery. This study was designed to evaluate whether magnetic source imaging (MSI), a noninv...

journal_title：Annals of neurology

authors： Knowlton RC,Razdan SN,Limdi N,Elgavish RA,Killen J,Blount J,Burneo JG,Ver Hoef L,Paige L,Faught E,Kankirawatana P,Bartolucci A,Riley K,Kuzniecky R

更新日期：2009-06-01 00:00:00

abstract：:A 37-year-old homosexual man with the acquired immune deficiency syndrome (AIDS) developed progressive, ultimately fatal, neurological deficits 12 weeks after a course of cutaneous zoster. Premortem radiological procedures and cerebrospinal fluid analyses were nondiagnostic. At postmortem examination, several opportun...

journal_title：Annals of neurology

authors： Ryder JW,Croen K,Kleinschmidt-DeMasters BK,Ostrove JM,Straus SE,Cohn DL

更新日期：1986-02-01 00:00:00

abstract：:Amyloid plaques in Alzheimer's disease contain beta-amyloid, encoded by portions of exons 16 and 17 of the amyloid precursor protein. The specific association of rare amyloid precursor protein mutations with some kindreds with early-onset familial Alzheimer's disease suggests that specific abnormalities in amyloid pre...

journal_title：Annals of neurology

authors： Peacock ML,Murman DL,Sima AA,Warren JT Jr,Roses AD,Fink JK

更新日期：1994-04-01 00:00:00

abstract：:Ultrasound scans of three newborn infants with cerebral ischemic lesions demonstrated on computed tomographic scan showed unexpected changes in echogenicity of brain parenchyma in addition to lateralized mass effect. Areas of increased echogenicity were present adjacent to regions of infarction identified on computed ...

journal_title：Annals of neurology

authors： Wilson-Davis SL,Lo W,Filly RA

更新日期：1983-08-01 00:00:00

abstract：:The regional metabolic rate for glucose (rMRglu) was studied in macaque monkeys after spinal cord transection at T10, both during spinal shock and after reflexes had returned. The rMRglu was measured in all Rexed layers in cord segments both caudal and rostral to the level of the transection utilizing the quantitative...

journal_title：Annals of neurology

authors： Schwartzman RJ,Eidelberg E,Alexander GM,Yu J

更新日期：1983-07-01 00:00:00

abstract：:Benign familial neonatal convulsions (BFNC) is a rare dominantly inherited epileptic syndrome characterized by frequent brief seizures within the first days of life. The disease is caused by mutations in one of two recently identified voltage-gated potassium channel genes, KCNQ2 or KCNQ3. Here, we describe a four-gene...

journal_title：Annals of neurology

authors： Lerche H,Biervert C,Alekov AK,Schleithoff L,Lindner M,Klinger W,Bretschneider F,Mitrovic N,Jurkat-Rott K,Bode H,Lehmann-Horn F,Steinlein OK

更新日期：1999-09-01 00:00:00

abstract：:Direct DNA tests for mutations in neurogenetic disorders are establishing a new era in diagnostic neurology. These highly accurate and specific tests will greatly improve diagnosis and genetic counseling, lead to more accurate assessment of prognosis, and reduce the cost of diagnostic evaluations. However, the use and...

journal_title：Annals of neurology

authors： Bird TD,Bennett RL

更新日期：1995-08-01 00:00:00

abstract：OBJECTIVE:Blunted tachycardia during hypotension is a characteristic feature of patients with autonomic failure, but the range has not been defined. This study reports the range of orthostatic heart rate (HR) changes in patients with autonomic failure caused by neurodegenerative synucleinopathies. METHODS:Patients eva...

journal_title：Annals of neurology

authors： Norcliffe-Kaufmann L,Kaufmann H,Palma JA,Shibao CA,Biaggioni I,Peltier AC,Singer W,Low PA,Goldstein DS,Gibbons CH,Freeman R,Robertson D,Autonomic Disorders Consortium.

更新日期：2018-03-01 00:00:00

abstract：:Tropical spastic paraparesis (TSP) is a common myeloneuropathy with primary and predominant involvement of the pyramidal tract and minimal sensory loss. The epidemic form of TSP is related to toxic nutritional factors, but the endemic form occurs in clusters in tropical areas, especially in India, Africa, the Seychell...

journal_title：Annals of neurology

authors： Vernant JC,Maurs L,Gessain A,Barin F,Gout O,Delaporte JM,Sanhadji K,Buisson G,de-Thé G

更新日期：1987-02-01 00:00:00

abstract：:A persistent vegetative state (severe dementia) developed in a 30-year-old man following hypoglycemic coma. Despite the poor clinical outcome, sensory evoked response recovered between 6 and 34 months after the insult. The cerebral blood flow level at rest after 34 months was slightly above the normal range. This find...

journal_title：Annals of neurology

authors： Agardh CD,Rosén I,Ryding E

更新日期：1983-10-01 00:00:00

abstract：:Free and bound levels of anti-myelin basic protein (anti-MBP) antibodies were measured by radioimmunoassay in the cerebrospinal fluid of 20 patients with acute idiopathic optic neuritis, 133 patients with multiple sclerosis (MS) divided into three clinical subgroups, and 76 normal control subjects. Patients with idiop...

journal_title：Annals of neurology

authors： Warren KG,Catz I,Bauer C

更新日期：1988-03-01 00:00:00

abstract：:We report the first molecular defect in an NADH-dehydrogenase gene presenting as isolated myopathy. The proband had lifelong exercise intolerance but no weakness. A muscle biopsy showed cytochrome c oxidase (COX)-positive ragged-red fibers (RRFs), and analysis of the mitochondrial enzymes revealed complex I deficiency...

journal_title：Annals of neurology

authors： Andreu AL,Tanji K,Bruno C,Hadjigeorgiou GM,Sue CM,Jay C,Ohnishi T,Shanske S,Bonilla E,DiMauro S

更新日期：1999-06-01 00:00:00

abstract：:Intraneural perineuriomas are benign peripheral nerve sheath tumors that cause progressive debilitating focal extremity weakness. The etiology of perineuriomas is largely unknown. We utilized whole exome sequencing, copy number algorithm evaluation, and high-resolution whole genome microarray to investigate for a gene...

journal_title：Annals of neurology

authors： Klein CJ,Wu Y,Jentoft ME,Mer G,Spinner RJ,Dyck PJ,Dyck PJ,Mauermann ML

更新日期：2017-02-01 00:00:00

abstract：OBJECTIVE:Myasthenia gravis (MG), a neuromuscular disease mediated by anti-acetylcholine receptor (AChR) autoantibodies, is associated with thymic hyperplasia characterized by ectopic germinal centers that contain pathogenic antibody-producing B cells. Our thymic transcriptome study demonstrated increased expression of...

journal_title：Annals of neurology

authors： Berrih-Aknin S,Ruhlmann N,Bismuth J,Cizeron-Clairac G,Zelman E,Shachar I,Dartevelle P,de Rosbo NK,Le Panse R

更新日期：2009-10-01 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：:In view of the favorable claims of Cook and others, an open trial of chronic dorsal column stimulation was undertaken in 9 patients who had moderately advanced multiple sclerosis (MS) but were still ambulatory. No objective improvement was noted by either author during the postoperative observation period of 6 to 37 m...

journal_title：Annals of neurology

authors： Rosen JA,Barsoum AH

更新日期：1979-07-01 00:00:00