Cerebrospinal fluid antibodies to myelin basic protein in acute idiopathic optic neuritis.

abstract：:Three patients with inferior branch palsies of the oculomotor nerve are described. Two were under 10 years of age and the third was 30 years old at the onset. All 3 presented with painless diplopia. The onset was sudden in 2 patients and progressive in the third. The palsy cleared within a short time in the 2 patients...

journal_title：Annals of neurology

authors： Susac JO,Hoyt WF

更新日期：1977-10-01 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：:Striatal particulate preparations, both from rats with lesion-induced striatal dopamine (DA) loss and from some striatal dopamine (DA) loss and from some patients with Parkinson's disease, exhibit increased 3H-neuroleptic binding, which is interpreted to be the mechanism of denervation-induced behavioral supersensitiv...

journal_title：Annals of neurology

authors： Bennett JP Jr,Wooten GF

更新日期：1986-04-01 00:00:00

abstract：OBJECTIVE:To understand the mechanisms of skeletal muscle destruction and resistance to enzyme replacement therapy in Pompe disease, a deficiency of lysosomal acid alpha-glucosidase (GAA), in which glycogen accumulates in lysosomes primarily in cardiac and skeletal muscles. METHODS:We have analyzed compartments of the...

journal_title：Annals of neurology

authors： Fukuda T,Ewan L,Bauer M,Mattaliano RJ,Zaal K,Ralston E,Plotz PH,Raben N

更新日期：2006-04-01 00:00:00

abstract：:Guillain-Barré syndrome (GBS) is a recognized entity for which the basis for diagnosis is descriptive in our present state of knowledge. Diagnosis rests upon pattern recognition of the clinical picture plus other features including elevated cerebrospinal fluid protein level, electrophysiological changes of marked slow...

journal_title：Annals of neurology

authors： Asbury AK

更新日期：1981-01-01 00:00:00

abstract：:This paper attempts to place into perspective the present state of clinical investigation with PET in neurology. To this end we briefly review the work of the neurology group of the MRC Cyclotron Unit at Hammersmith Hospital, by reference to studies performed in normal subjects and in patients with cerebrovascular dis...

journal_title：Annals of neurology

authors： Frackowiak RS,Wise RJ,Gibbs JM,Jones T

更新日期：1984-01-01 00:00:00

abstract：:Hemophilus influenzae is the most common cause of bacterial meningitis in children, and a high percentage of survivors are at risk for long-term sequelae. To explore the mechanisms responsible for these sequelae, a neonatal rat model was used to define the behavioral, electrophysiological, and biochemical changes foll...

journal_title：Annals of neurology

authors： Konkol RJ,Chapman L,Breese GR,Collier AM,Kilts C,Finley C,Vogel RR,Mailman RB,Bendeich EG

更新日期：1987-04-01 00:00:00

abstract：:Previous imaging studies using 11C-flumazenil in patients with mesial temporal lobe epilepsy and neocortical partial seizure disorders have found focal decreases in gamma-aminobutyric acid type A/benzodiazepine receptor binding. These studies used subjective visual assessment and a region of interest approach to quant...

journal_title：Annals of neurology

authors： Richardson MP,Koepp MJ,Brooks DJ,Fish DR,Duncan JS

更新日期：1996-08-01 00:00:00

abstract：OBJECTIVE:Infantile spasms are seizures associated with a severe epileptic encephalopathy presenting in the first 2 years of life, and optimal treatment continues to be debated. This study evaluates early and sustained response to initial treatments and addresses both clinical remission and electrographic resolution of...

journal_title：Annals of neurology

authors： Knupp KG,Coryell J,Nickels KC,Ryan N,Leister E,Loddenkemper T,Grinspan Z,Hartman AL,Kossoff EH,Gaillard WD,Mytinger JR,Joshi S,Shellhaas RA,Sullivan J,Dlugos D,Hamikawa L,Berg AT,Millichap J,Nordli DR Jr,Wirrell E,

更新日期：2016-03-01 00:00:00

abstract：:As more small peptidergic components of the central nervous system are isolated, their role in disease states is being investigated. Several of these neuropeptides, especially the opioidlike peptides, adrenocorticotropic hormone, and some hypothalamic releasing factors, have been found to alter neuronal excitability. ...

journal_title：Annals of neurology

authors： Bajorek JG,Lee RJ,Lomax P

更新日期：1984-01-01 00:00:00

abstract：:A family with Gerstmann-Sträussler-Scheinker disease had coincidental clinical onset in three members of two generations, a phenomenon suggesting a common source of a transmissible agent. A regular dietary supplement in this family was home-bred rabbit. The clinical picture, although generally similar to that in previ...

journal_title：Annals of neurology

authors： Hudson AJ,Farrell MA,Kalnins R,Kaufmann JC

更新日期：1983-12-01 00:00:00

abstract：OBJECTIVE:Autosomal-dominant lateral temporal epilepsy (ADLTE) is a genetic focal epilepsy characterized by auditory symptoms. Two genes, LGI1 and RELN, encoding secreted proteins, are implicated in the etiology of ADLTE, but half of the affected families remain genetically unsolved, and the underlying molecular mechan...

journal_title：Annals of neurology

authors： Dazzo E,Rehberg K,Michelucci R,Passarelli D,Boniver C,Vianello Dri V,Striano P,Striano S,Pasterkamp RJ,Nobile C

更新日期：2018-03-01 00:00:00

abstract：:Identifying asymptomatic older individuals at elevated risk for developing Alzheimer disease (AD) is of clinical importance. Among 1,081 asymptomatic older adults, a recently validated polygenic hazard score (PHS) significantly predicted time to AD dementia and steeper longitudinal cognitive decline, even after contro...

journal_title：Annals of neurology

authors： Tan CH,Hyman BT,Tan JJX,Hess CP,Dillon WP,Schellenberg GD,Besser LM,Kukull WA,Kauppi K,McEvoy LK,Andreassen OA,Dale AM,Fan CC,Desikan RS

更新日期：2017-09-01 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：:It has been known for some time that patients with Parkinson's disease (PD) have difficulty executing simultaneous motor acts, yet the pathophysiology underlying this impairment remains uncertain. We examined the possibility that these dual-task deficits stem from defective sensorimotor mechanisms producing interferen...

journal_title：Annals of neurology

authors： Caligiuri MP,Heindel WC,Lohr JB

更新日期：1992-01-01 00:00:00

abstract：:PINK1 was recently found to be associated with PARK6 as the causative gene. We performed mutation analysis in eight inbred families whose haplotypes link to the PARK6 region. We identified six pathogenic mutations (R246X, H271Q, E417G, L347P, and Q239X/R492X) in six unrelated families. All sites of mutations were nove...

journal_title：Annals of neurology

authors： Hatano Y,Li Y,Sato K,Asakawa S,Yamamura Y,Tomiyama H,Yoshino H,Asahina M,Kobayashi S,Hassin-Baer S,Lu CS,Ng AR,Rosales RL,Shimizu N,Toda T,Mizuno Y,Hattori N

更新日期：2004-09-01 00:00:00

abstract：:Friedreich's ataxia (FA) is the most frequently inherited ataxia. To test the hypothesis that iron is increased in the cerebellum of patients with FA, we developed a multigradient echo magnetic resonance sequence for the three-dimensional imaging of brain iron-induced contrast. Relaxation rate (R2*) values in the unaf...

journal_title：Annals of neurology

authors： Waldvogel D,van Gelderen P,Hallett M

更新日期：1999-07-01 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：:The pathogenetic role of anti-GM1 in chronic acquired demyelinating polyneuropathy (CADP) is uncertain. An association between antibodies and disease activity has not yet been established. In 8 patients with CADP followed longitudinally, anti-GM1 antibodies were monitored with a standardized enzyme-linked immunosorben...

journal_title：Annals of neurology

authors： Bech E,Andersen H,Orntoft TF,Jakobsen J

更新日期：1998-01-01 00:00:00

abstract：:Interpretation of biochemical measurements in the human brain after death is complicated by a variety of premortem, perimortem, and postmortem factors. The activity of glutamic acid decarboxylase (GAD) in particular has been found to vary considerably among human brains. In contrast to neurotransmitter-associated enzy...

journal_title：Annals of neurology

authors： Maker HS,Weiss C,Weissbarth S,Silides DJ,Whetsell W

更新日期：1981-10-01 00:00:00

abstract：:The etiopathogenesis of Parkinson's disease (PD) has been elusive. Recently, several lines of evidence have converged to suggest that defects in the ubiquitin-proteasome system and proteolytic stress underlie nigral pathology in both familial and sporadic forms of the illness. In support of this concept, mutations in ...

journal_title：Annals of neurology

authors： McNaught KS,Olanow CW

更新日期：2003-01-01 00:00:00

abstract：:Hypoxic-ischemic brain injury in survivors of perinatal asphyxia is a frequently encountered clinical problem for which there is currently no effective therapy. Neurotrophins, such as brain-derived neurotrophic factor (BDNF), can protect responsive neurons against cell death in some injury paradigms. While the role of...

journal_title：Annals of neurology

authors： Cheng Y,Gidday JM,Yan Q,Shah AR,Holtzman DM

更新日期：1997-04-01 00:00:00

abstract：:Life table methods were used to determine the relative risk of Alzheimer's disease (AD) in relatives of index cases with AD. Risk of AD was assessed in 967 first-degree relatives of 128 probands with clinically diagnosed AD and 572 first-degree relatives of a control group consisting of 84 subjects with Parkinson's di...

journal_title：Annals of neurology

authors： Farrer LA,O'Sullivan DM,Cupples LA,Growdon JH,Myers RH

更新日期：1989-05-01 00:00:00

abstract：:Malignant tumors are known to have a "remote" or nonmetastatic effect on the central and peripheral nervous systems. Eight patients were seen with proximal muscle weakness in association with bronchogenic carcinoma (5), carcinoma of breast (2), and leukemia (1). Electromyography demonstrated small polyphasic motor uni...

journal_title：Annals of neurology

authors： Barron SA,Heffner RR Jr

更新日期：1978-09-01 00:00:00

abstract：OBJECTIVE:Our goal was to define the genetic cause of the profound hypomyelination in the taiep rat model and determine its relevance to human white matter disease. METHODS:Based on previous localization of the taiep mutation to rat chromosome 9, we tested whether the mutation resided within the Tubb4a (β-tubulin 4A) ...

journal_title：Annals of neurology

authors： Duncan ID,Bugiani M,Radcliff AB,Moran JJ,Lopez-Anido C,Duong P,August BK,Wolf NI,van der Knaap MS,Svaren J

更新日期：2017-05-01 00:00:00

abstract：:To determine the role of recombinant human erythropoietin as a possible treatment option in Friedreich's ataxia, we performed an open-label clinical pilot study. Primary outcome measure was the change of frataxin levels at week 8 versus baseline. Twelve Friedreich's ataxia patients received 5,000 units recombinant hum...

journal_title：Annals of neurology

authors： Boesch S,Sturm B,Hering S,Goldenberg H,Poewe W,Scheiber-Mojdehkar B

更新日期：2007-11-01 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：OBJECTIVE:Chronic seizures in women can have adverse effects on reproductive function, such as polycystic ovarian syndrome, but it has been difficult to dissociate the effects of epilepsy from the role of antiepileptic drugs. To distinguish the effects of chronic seizures from medication, we used the laboratory rat, be...

journal_title：Annals of neurology

authors： Scharfman HE,Kim M,Hintz TM,MacLusky NJ

更新日期：2008-12-01 00:00:00

abstract：OBJECTIVE:Abnormal cortical excitability is evident in various movement disorders that compromise fine motor control. Here we tested whether skilled finger movements can be restored in musicians with focal hand dystonia through behavioral training assisted by transcranial direct current stimulation to the motor cortex ...

journal_title：Annals of neurology

authors： Furuya S,Nitsche MA,Paulus W,Altenmüller E

更新日期：2014-05-01 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