Sequence-dependent and independent inhibition specific for mutant ataxin-3 by small interfering RNA.

abstract：:This review discusses the etiology and pathogenesis of Parkinson's disease (PD). Mitochondrial respiratory failure and oxidative stress appear to be two major contributors to nigral neuronal death in PD. Complex I deficiency has been reported by several groups and appears to be one of the basic abnormalities responsib...

journal_title：Annals of neurology

authors： Mizuno Y,Yoshino H,Ikebe S,Hattori N,Kobayashi T,Shimoda-Matsubayashi S,Matsumine H,Kondo T

更新日期：1998-09-01 00:00:00

abstract：:From a population-based sample of 15,504 patients attending Canadian multiple sclerosis (MS) clinics, we have determined the frequency of conjugal MS and have estimated the recurrence risk in offspring of such matings. Twenty-three MS cases were found among 13,550 spouses of study probands for a crude conjugal rate of...

journal_title：Annals of neurology

authors： Ebers GC,Yee IM,Sadovnick AD,Duquette P

更新日期：2000-12-01 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

abstract：:Light and electron microscopic findings from two sural nerve biopsies obtained at a one-year interval from a patient with the clinical features of Seitelberger's disease are described. Ballooned axons with accumulations of membranous profiles, vesicles, mitochondria, and a homogeneous center were present, and there we...

journal_title：Annals of neurology

authors： Begeer JH,Houthoff HJ,van Weerden TW,de Groot CJ,Blaauw EH,le Coultre R

更新日期：1979-12-01 00:00:00

abstract：:Discussing the problem of multiple sclerosis and viruses should not be limited to reviewing the epidemiological evidence in favor, or against, a particular candidate, such as Epstein-Barr virus or human herpes virus 6. In this text, I discuss the difficulty of going from association to causation in human epidemiology;...

journal_title：Annals of neurology

authors： Brahic M

更新日期：2010-07-01 00:00:00

abstract：:Only 6 patients with intracranial hypertension associated with unruptured cerebral arteriovenous malformations have been reported. We report 6 additional patients seen at the Cleveland Clinic during the past 10 years. The average age was 28 years (range, 19-44 years); 4 were women. Symptoms and signs included papilled...

journal_title：Annals of neurology

authors： Chimowitz MI,Little JR,Awad IA,Sila CA,Kosmorsky G,Furlan AJ

更新日期：1990-05-01 00:00:00

abstract：:A patient with recessive generalized congenital myotonia and severe, disabling weakness underwent various forms of treatment while being monitored electrophysiologically. Phenytoin, verapamil, and acetazolamide were ineffective, but tocainide yielded good results. Improvement was dose-dependent, and was limited by irr...

journal_title：Annals of neurology

authors： Streib EW

更新日期：1986-05-01 00:00:00

abstract：:Two extended haplotypes of the tau gene (H1 and H2) have been described. The frequency of H1 haplotype is increased in progressive supranuclear palsy (PSP). PSP is associated with filamentous tau lesions in neurons and glia, which are reportedly composed exclusively of tau isoforms with four repeats in the microtubule...

journal_title：Annals of neurology

authors： Liu WK,Le TV,Adamson J,Baker M,Cookson N,Hardy J,Hutton M,Yen SH,Dickson DW

更新日期：2001-10-01 00:00:00

abstract：:To elucidate the mechanism by which carbamazepine lowers somatostatin concentration in cerebrospinal fluid of humans, the effect of carbamazepine on secretion of this peptide was studied in rat cerebral cell cultures. Concentrations of carbamazepine within the therapeutic range (4 x 10(-5) M) inhibited spontaneous rel...

journal_title：Annals of neurology

authors： Reichlin S,Mothon S

更新日期：1991-04-01 00:00:00

abstract：OBJECTIVES:African Americans are at greater risk for developing Alzheimer's disease (AD) dementia than non-Hispanic whites. In addition to biological considerations (eg, genetic influences and comorbid disorders), social and environmental factors may increase the risk of AD dementia. This paper (1) assesses neuroimagin...

journal_title：Annals of neurology

authors： Meeker KL,Wisch JK,Hudson D,Coble D,Xiong C,Babulal GM,Gordon BA,Schindler SE,Cruchaga C,Flores S,Dincer A,Benzinger TL,Morris JC,Ances BM

更新日期：2021-02-01 00:00:00

abstract：:Solitary neoplastic proliferation of plasma cells (plasmacytoma) rarely occurs in the central nervous system. The longest follow-Up in nine previously reported patients was 5 years. We have followed two patients with solitary intracranial plasmacytoma for 8 and 13 years, respectively. At the time of biopsy diagnosis, ...

journal_title：Annals of neurology

authors： Krumholz A,Weiss HD,Jiji VH,Bakal D,Kirsh MB

更新日期：1982-05-01 00:00:00

abstract：OBJECTIVE:Central nervous system pathology in multiple sclerosis includes both focal inflammatory perivascular injury and injury to superficial structures, including the subpial region of the cortex, which reportedly exhibits a gradient of damage from the surface inward. We assessed how early in the multiple sclerosis ...

journal_title：Annals of neurology

authors： Fadda G,Brown RA,Magliozzi R,Aubert-Broche B,O'Mahony J,Shinohara RT,Banwell B,Marrie RA,Yeh EA,Collins DL,Arnold DL,Bar-Or A,Canadian Pediatric Demyelinating Disease Network.

更新日期：2019-03-01 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：:Infection by the human immunodeficiency virus type 1 (HIV-1) often results in neurological dysfunction including HIV dementia (HIVD). Alterations in cytokine and redox balance are thought to play important roles in the pathogenesis of HIVD, but the specific mechanisms underlying neuronal dysfunction and death are unkn...

journal_title：Annals of neurology

authors： Haughey NJ,Cutler RG,Tamara A,McArthur JC,Vargas DL,Pardo CA,Turchan J,Nath A,Mattson MP

更新日期：2004-02-01 00:00:00

abstract：:The worldwide epidemiology of Creutzfeldt-Jakob disease (CJD) is presented from an analysis of 1,435 patients. In the United States, the average annual mortality rate is at least 0.26 deaths per million. Temporal-spatial clustering of cases was not found in the United States, but reports from other countries indicate ...

journal_title：Annals of neurology

authors： Masters CL,Harris JO,Gajdusek DC,Gibbs CJ Jr,Bernoulli C,Asher DM

更新日期：1979-02-01 00:00:00

abstract：:Unexpected sudden death is a common event in otherwise healthy epileptics, though its etiology has remained unclear. Many authors have suggested cardiac arrhythmias as the cause, and limited data in humans and animal studies have supported this. However, autopsy series in humans have shown pulmonary edema, a phenomeno...

journal_title：Annals of neurology

authors： Johnston SC,Horn JK,Valente J,Simon RP

更新日期：1995-04-01 00:00:00

abstract：:We describe a new cause of spinal cord ischemia illustrated by two cases--compression by the diaphragmatic crus of a lumbar artery giving rise to a spinal artery. The diagnosis has been established by dynamic spinal angiography, showing complete occlusion of the lumbar artery. Surgical section of the diaphragmatic cru...

journal_title：Annals of neurology

authors： Rogopoulos A,Benchimol D,Paquis P,Mahagne MH,Bourgeon A

更新日期：2000-08-01 00:00:00

abstract：:Neuromuscular disorders reported in association with human immunodeficiency virus (HIV) infection include several forms of peripheral neuropathy and polymyositis. We report 11 patients with HIV-associated myopathy. Five patients with acquired immunodeficiency syndrome (AIDS), 2 with AIDS-related complex, and 4 otherwi...

journal_title：Annals of neurology

authors： Simpson DM,Bender AN

更新日期：1988-07-01 00:00:00

abstract：:Regular, evidence-based assignment of patients to etiologic stroke categories is essential to enable valid comparison among studies. We designed an algorithm (SSS-TOAST) that incorporated recent advances in stroke imaging and epidemiology to identify the most probable TOAST category in the presence of evidence for mul...

journal_title：Annals of neurology

authors： Ay H,Furie KL,Singhal A,Smith WS,Sorensen AG,Koroshetz WJ

更新日期：2005-11-01 00:00:00

abstract：:We studied 23 patients with clinically defined mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS), 25 oligosymptomatic or asymptomatic maternal relatives, and 50 mitochondrial disease control subjects for the presence of a previously reported heteroplasmic point mutation at nt 3,243 in ...

journal_title：Annals of neurology

authors： Ciafaloni E,Ricci E,Shanske S,Moraes CT,Silvestri G,Hirano M,Simonetti S,Angelini C,Donati MA,Garcia C

更新日期：1992-04-01 00:00:00

abstract：:We demonstrated that an IgM M-protein from a patient with motor neuron syndrome had antibody activity against gangliosides GM1, GD1b, and asialo GM1. Studies with a sugar-binding lectin suggested that the epitope in the patient's M-IgM involved the Gal(beta 1-3) GalNAc moiety. Immunohistological techniques demonstrate...

journal_title：Annals of neurology

authors： Nardelli E,Steck AJ,Barkas T,Schluep M,Jerusalem F

更新日期：1988-05-01 00:00:00

abstract：:Sera from 6 of 12 patients with paraneoplastic cerebellar degeneration (PCD) contained anti-Purkinje cell antibodies, as determined by indirect immunofluorescence on frozen sections of normal human cerebellum. Samples of cerebrospinal fluid from 2 of the patients with serum antibodies were tested, and both specimens c...

journal_title：Annals of neurology

authors： Jaeckle KA,Graus F,Houghton A,Cardon-Cardo C,Nielsen SL,Posner JB

更新日期：1985-11-01 00:00:00

abstract：:Measurement of conduction velocity along the H reflex arc was used to study sensorimotor peripheral nerve function in diabetic patients during short- and long-term improvement of hyperglycemia. In ten type I diabetics a slight (p less than 0.05) conduction increase occurred after 6 hours of normal glycemia induced by ...

journal_title：Annals of neurology

authors： Troni W,Carta Q,Cantello R,Caselle MT,Rainero I

更新日期：1984-08-01 00:00:00

abstract：OBJECTIVE:Huntington's disease (HD) is a monogenic, fully penetrant neurodegenerative disorder, providing an ideal model for understanding brain changes occurring in the years prior to disease onset. Diffusion tensor imaging (DTI) studies show widespread white matter disorganization in the early premanifest stages (pre...

journal_title：Annals of neurology

authors： Zhang J,Gregory S,Scahill RI,Durr A,Thomas DL,Lehericy S,Rees G,Tabrizi SJ,Zhang H,TrackOn-HD investigators.

更新日期：2018-10-01 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：:Selective neuronal vulnerability is a key feature of the neuropathology of Huntington's disease. We used [3H]CP-55,940, a synthetic cannabinoid, to label cannabinoid receptors in tissue sections from individuals dying with Huntington's disease and from normal control subjects. The density of cannabinoid receptors in s...

journal_title：Annals of neurology

authors： Richfield EK,Herkenham M

更新日期：1994-10-01 00:00:00

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：:More than a decade ago myasthenic symptoms were observed in rabbits immunized with acetylcholine receptor (AChR) [119] and AChR deficiency was found at the neuromuscular junction in human myasthenia gravis (MG) [36]. By 1977 the autoimmune character of MG and the pathogenic role of AChR antibodies had been established...

journal_title：Annals of neurology

authors： Engel AG

更新日期：1984-11-01 00:00:00

abstract：:Angelman syndrome (AS) results from lack of genetic contribution from maternal chromosome 15q11-13. This region encompasses three GABAA receptor subunit genes (beta3, alpha5, and gamma3). The characteristic phenotype of AS is severe mental retardation, ataxic gait, tremulousness, and jerky movements. We studied the mo...

journal_title：Annals of neurology

authors： Guerrini R,De Lorey TM,Bonanni P,Moncla A,Dravet C,Suisse G,Livet MO,Bureau M,Malzac P,Genton P,Thomas P,Sartucci F,Simi P,Serratosa JM

更新日期：1996-07-01 00:00:00

abstract：OBJECTIVE:Rapid reversal of the anticoagulatory effect of vitamin K antagonists represents the primary emergency treatment for oral anticoagulant-related intracerebral hemorrhage (OAC-ICH). Predicting the amount of prothrombin complex concentrate (PCC) needed to reverse OAC in individual patients is difficult, and repe...

journal_title：Annals of neurology

authors： Rizos T,Jenetzky E,Herweh C,Hug A,Hacke W,Steiner T,Veltkamp R

更新日期：2010-06-01 00:00:00