Abstract:
:Adult GM2 gangliosidosis is a rare disorder that often presents with both neurological and psychiatric syndromes. Effective treatment of the psychotic and affective symptoms associated with this disorder has been complicated by poor treatment response and the concern that many psychotropic agents may worsen the underlying gangliosidosis. We report the successful use of electroconvulsive therapy for treatment of severe depression in a young man with adult GM2 gangliosidosis.
journal_name
Ann Neuroljournal_title
Annals of neurologyauthors
Renshaw PF,Stern TA,Welch C,Schouten R,Kolodny EHdoi
10.1002/ana.410310320keywords:
subject
Has Abstractpub_date
1992-03-01 00:00:00pages
342-4issue
3eissn
0364-5134issn
1531-8249journal_volume
31pub_type
杂志文章,评审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
pub_type: 杂志文章
doi:10.1002/ana.21846
更新日期:2010-02-01 00:00:00
abstract::Mitochondria are cellular organelles crucial for energy supply and calcium homeostasis in neuronal cells, and their dysfunction causes seizure activity in some rare human epilepsies. To directly test whether mitochondrial respiratory chain enzymes are abnormal in the most common form of chronic epilepsy, temporal lobe...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:
更新日期:2000-11-01 00:00:00
abstract:OBJECTIVE:Recent studies carried out on amyotrophic lateral sclerosis patients suggest that the disease might initiate in the motor cortex and spread to its targets along the corticofugal tracts. In this study, we aimed to test the corticofugal hypothesis of amyotrophic lateral sclerosis experimentally. METHODS:Sod1G8...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.25833
更新日期:2020-10-01 00:00:00
abstract:OBJECTIVE:To evaluate the ability of mesenchymal stem cells (MSCs), a subset of adult stem cells from bone marrow, to cure experimental autoimmune encephalomyelitis. METHODS:The outcome of the injection of MSCs, in mice immunized with the peptide 139-151 of the proteolipid protein (PLP), was studied analyzing clinical...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.21076
更新日期:2007-03-01 00:00:00
abstract:OBJECTIVE:The Veterans Administration Cooperative Studies Program #468, a multicenter study that randomized Parkinson's disease (PD) patients to either subthalamic nucleus (STN) or globus pallidus internus (GPi) deep brain stimulation (DBS), found that stimulation at either target provided similar overall motoric benef...
journal_title:Annals of neurology
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1002/ana.24374
更新日期:2015-04-01 00:00:00
abstract:OBJECTIVE:Cortical spreading depolarizations (CSDs) are intense and ubiquitous depolarization waves relevant for the pathophysiology of migraine and brain injury. CSDs disrupt the blood-brain barrier (BBB), but the mechanisms are unknown. METHODS:A total of six CSDs were evoked over 1 hour by topical application of 30...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.25298
更新日期:2018-09-01 00:00:00
abstract::Passive joint position sense was tested in 10 subjects after unilateral total hip replacement surgery (which included capsulectomy). Varied initial limb position, amplitude, and velocity of movement were used. The unoperated side was used as a control. Statistically barely significant errors (p less than 0.025) on the...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410130612
更新日期:1983-06-01 00:00:00
abstract:OBJECTIVE:Nonketotic hyperglycinemia is a neurometabolic disorder characterized by intellectual disability, seizures, and spasticity. Patients with attenuated nonketotic hyperglycinemia make variable developmental progress. Predictive factors have not been systematically assessed. METHODS:We reviewed 124 patients stra...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24485
更新日期:2015-10-01 00:00:00
abstract::Plasma exchange (PE) is the standard treatment in Guillain-Barré syndrome (GBS) patients who have lost the ability to walk. The effect of exchanges before this stage and the optimal number of exchanges for the other patients are still unknown. We randomized 556 GBS patients according to severity and number of exchange...
journal_title:Annals of neurology
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.1002/ana.410410304
更新日期:1997-03-01 00:00:00
abstract:OBJECTIVE:The UCHL-1 gene is widely cited as a susceptibility factor for sporadic Parkinson's disease (PD). The strongest evidence comes from a meta-analysis of small studies that reported the S18Y polymorphism as protective against PD, after pooling studies of white and Asian subjects. Here, we present data that chall...
journal_title:Annals of neurology
pub_type: 杂志文章,多中心研究
doi:10.1002/ana.20757
更新日期:2006-04-01 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
pub_type: 杂志文章
doi:10.1002/ana.410350522
更新日期:1994-05-01 00:00:00
abstract::White matter degeneration is one of the pathological conditions of dentatorubral-pallidoluysian atrophy. Autopsy brains exhibited a reduced number of glial cells in the lesions and an involvement of oligodendrocytes in nuclear inclusion formation, which previously has been recognized only as a pathological hallmark in...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.10352
更新日期:2002-11-01 00:00:00
abstract:OBJECTIVE:Loss of function mutations in PINK1 typically lead to early onset Parkinson disease (PD). Zebrafish (Danio rerio) are emerging as a powerful new vertebrate model to study neurodegenerative diseases. We used a pink1 mutant (pink(-/-) ) zebrafish line with a premature stop mutation (Y431*) in the PINK1 kinase d...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.23999
更新日期:2013-12-01 00:00:00
abstract::Tetrabenazine is considered to act in a manner similar to reserpine to reduce the involuntary movements of tardive dyskinesia or Huntington's disease and to improve psychoses. We determined that tetrabenazine also has properties of a dopamine receptor antagonist by testing the ability of tetrabenazine to block the inh...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410120308
更新日期:1982-09-01 00:00:00
abstract:OBJECTIVE:Intracellular amyloid β-protein (Aβ) contributes to neurodegeneration in Alzheimer disease (AD). Apomorphine (APO) is a dopamine receptor agonist for Parkinson disease and also protects against oxidative stress. Efficacy of APO for an AD mouse model and effects of APO on cell cultures are studied. METHODS:Th...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.22319
更新日期:2011-02-01 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
pub_type: 杂志文章
doi:10.1002/ana.24151
更新日期:2014-05-01 00:00:00
abstract::In patients with the frontal variant of frontotemporal lobar degeneration (fv-FTLD), behavioral abnormalities may vary from apathy with motor slowness (apathetic form) to disinhibition with agitation (disinhibited form). These clinical presentations may be related to specific regional cerebral dysfunction and to defic...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.20365
更新日期:2005-02-01 00:00:00
abstract::Copper toxicity contributes to neuronal death in Wilson's disease and has been speculatively linked to the pathogenesis of Alzheimer's and prion diseases. We examined copper-induced neuronal death with the goal of developing neuroprotective strategies. Copper catalyzed an increase in hydroxyl radical generation in sol...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.10276
更新日期:2002-08-01 00:00:00
abstract:OBJECTIVE:Reducing levels of the microtubule-associated protein tau has shown promise as a potential treatment strategy for diseases with secondary epileptic features such as Alzheimer disease. We wanted to determine whether tau reduction may also be of benefit in intractable genetic epilepsies. METHODS:We studied a m...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24230
更新日期:2014-09-01 00:00:00
abstract::We analyzed the prion protein gene (PRNP) region in patients with transmissible spongiform encephalopathy associated with the PRNP D178N mutation. The results suggest that the D178N chromosomes had independent origins in each affected pedigree or apparently sporadic case. A de novo spontaneous PRNP mutation was observ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.10267
更新日期:2002-09-01 00:00:00
abstract::Orally administered levodopa remains the most effective symptomatic treatment for Parkinson's disease (PD). The introduction of levodopa therapy is often delayed, however, because of the fear that it might be toxic for the remaining dopaminergic neurons and, thus, accelerate the deterioration of patients. However, in ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410430504
更新日期:1998-05-01 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
pub_type: 杂志文章
doi:10.1002/ana.410260603
更新日期:1989-12-01 00:00:00
abstract::In adult brain, during insulin-induced hypoglycemia, striatal extracellular fluid concentrations of the excitatory amino acids glutamate and aspartate rise markedly (fourfold to tenfold). In this study, we used in vivo microdialysis to determine if insulin-induced hypoglycemia altered striatal amino acid efflux in sim...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410280408
更新日期:1990-10-01 00:00:00
abstract:OBJECTIVE:Faster time from onset to recanalization (OTR) in acute ischemic stroke using endovascular therapy (ET) has been associated with better outcome. However, previous studies were based on less-effective first-generation devices, and analyzed only dichotomized disability outcomes, which may underestimate the full...
journal_title:Annals of neurology
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1002/ana.24474
更新日期:2015-10-01 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
pub_type: 杂志文章
doi:10.1002/ana.20977
更新日期:2006-10-01 00:00:00
abstract::Persons affected with tuberous sclerosis complex (TSC) develop a wide range of neurological abnormalities including aberrant neuronal migration and seizures. In an effort to model TSC-associated central nervous system abnormalities in mice, we generated two independent lines of astrocyte-specific Tsc1 conditional knoc...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.10283
更新日期:2002-09-01 00:00:00
abstract:OBJECTIVE:The Ts65Dn (Ts) mouse model of Down syndrome (DS) is exquisitely sensitive to an infantile spasms phenotype induced by γ-aminobutyric acidB receptor (GABAB R) agonists. The Ts mouse contains the core genomic triplication of the DS critical region, which includes 3 copies of the Kcnj6 gene that encodes the GAB...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24749
更新日期:2016-10-01 00:00:00
abstract::Actin filaments in skin fibroblasts from patients with Huntington's disease (HD) were examined using immunofluorescent methods. Actin filaments were seen along the axis of cell elongation (stress or sheath filaments) as well as in areas of membrane ruffling (lattice filaments). In some cases, filaments appeared to rad...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410040614
更新日期:1978-12-01 00:00:00
abstract::We evaluated the role of positron emission tomography (PET) with [18F]deoxyglucose (FDG) (FDG-PET) for planning surgery in 53 patients who had temporal lobectomy for uncontrolled seizures at National Institutes of Health from 1981 to 1990. Investigators blinded to PET data used results of telemetered video-electroence...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410320613
更新日期:1992-12-01 00:00:00
abstract:OBJECTIVE:Multiple sclerosis (MS) is a chronic neuroinflammatory and neurodegenerative disease of unknown etiology. Although the prevalent view regards a CD4+ -lymphocyte autoimmune reaction against myelin at the root of the disease, recent studies propose autoimmunity as a secondary reaction to idiopathic brain damage...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.26014
更新日期:2021-01-06 00:00:00