Abstract:
:Ganaxolone (3alpha-hydroxy-3beta-methyl-5alpha-pregnan-20-one) is a novel neurosteroid which has anticonvulsant properties in a number of seizure models as well as the ability to enhance function of the gamma-aminobutyric acid-A (GABA(A)) receptor complex via a neurosteroid binding site. The object of these experiments was to ascertain the efficacy of ganaxolone against absence seizures. Ganaxolone was assessed in the low-dose pentylenetetrazol (PTZ) and the gamma-hydroxybutyric acid (GHB) model of absence seizures in rats. Ganaxolone pretreatment resulted in a significant prolongation of absence seizure in both the PTZ and GHB models. Further, ganaxolone in doses above 20 mg/kg alone produced bilaterally synchronous spike wave discharges (SWDs) associated with behavioral arrest. These data suggest that augmentation of GABA(A) receptor complex function by neurosteroids has the potential to result in or exacerbate absence seizures.
journal_name
Ann Neuroljournal_title
Annals of neurologyauthors
Snead OC 3rddoi
10.1002/ana.410440417subject
Has Abstractpub_date
1998-10-01 00:00:00pages
688-91issue
4eissn
0364-5134issn
1531-8249journal_volume
44pub_type
杂志文章abstract::A human immunodeficiency virus (HIV-I) was isolated from the brain of a patient with progressive dementia but no obvious immunosuppression. This isolate, designated as HIV-IBR, was molecularly cloned and sequenced, and its long terminal repeat (LTR) and envelope sequences were compared with those of other HIV isolates...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410230717
更新日期:1988-01-01 00:00:00
abstract::We report increased modification of proteins by 4-hydroxynonenal (HNE), a product of membrane lipid peroxidation, in the lumbar spinal cord of sporadic amyotrophic lateral sclerosis (ALS) patients versus that of neurologically normal controls. By immunohistochemistry, HNE-protein modification was detected in ventral h...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410440518
更新日期:1998-11-01 00:00:00
abstract:OBJECTIVE:To determine whether cerebrovascular risk factors are associated with subsequent diagnoses of Parkinson disease, and whether these associations are similar in magnitude to those with subsequent diagnoses of Alzheimer disease. METHODS:This was a retrospective cohort study using claims data from a 5% random sa...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.25564
更新日期:2019-10-01 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::Most neurodegenerative disorders are thought to result primarily from the accumulation of misfolded proteins, which interfere with protein homeostasis in neurons. For a subset of diseases, however, noncoding regions of RNAs assume a primary toxic gain-of-function, leading to degeneration in many tissues, including the...
journal_title:Annals of neurology
pub_type: 杂志文章,评审
doi:10.1002/ana.21948
更新日期:2010-03-01 00:00:00
abstract::Intracytoplasmic aggregation of alpha-synuclein protein as Lewy bodies in the brainstem neurons is diagnostic for Parkinson's disease, whereas if this process also occurs in the cortical neurons, it is considered pathognomonic for dementia with Lewy bodies. However, the link between alpha-synuclein incorporation into ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.20321
更新日期:2005-01-01 00:00:00
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
pub_type: 杂志文章
doi:10.1002/ana.410020414
更新日期:1977-10-01 00:00:00
abstract::Using placebo data from 3 randomized multiple sclerosis (MS) trials with uniform inclusion criteria, we investigated heterogeneity of Expanded Disability Status Scale (EDSS) progression by geographical areas. Our analysis revealed a significantly lower EDSS progression in Eastern European countries (10.8%) compared wi...
journal_title:Annals of neurology
pub_type: 杂志文章,meta分析
doi:10.1002/ana.25323
更新日期:2018-10-01 00:00:00
abstract::Central motor conduction times for the adductor pollicis muscle, the twitch force of that muscle to scalp magnetic motor cortex stimulation, and the maximum force of phasic voluntary contraction of the same muscle were measured in 15 patients with multiple sclerosis. Two tests of manual dexterity of the same hand also...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410290104
更新日期:1991-01-01 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
pub_type: 杂志文章
doi:10.1002/ana.410150722
更新日期:1984-01-01 00:00:00
abstract:OBJECTIVE:Ischemic injury of axons is a feature of periventricular leukomalacia, a pathological correlate of cerebral palsy. Recent evidence suggests that axons are damaged before they receive the first layer of compact myelin. Here we examine the cellular mechanisms underlying ischemic-type injury of premyelinated cen...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.21767
更新日期:2009-11-01 00:00:00
abstract::We prepared RNA probes from cloned segments of human and murine enteroviruses (EVs) for in situ hybridization of skeletal muscle biopsies from patients with dermatomyositis (DM), polymyositis, other inflammatory myopathies, and noninflammatory muscle diseases, and from normal control subjects. A probe derived from The...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410260204
更新日期:1989-08-01 00:00:00
abstract::A 30-year-old man presented with decreased vision in the right eye of three weeks' duration. Examination indicated a chiasmal syndrome and evidence of subarachnoid hemorrhage. CAT scan showed a large suprasellar mass. Surgical intervention confirmed the presence of a globular lesion filled with blood clots involving m...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410040515
更新日期:1978-11-01 00:00:00
abstract:OBJECTIVE:Case series with (11)C-PK11195 and positron emission tomography (PET) in stroke patients suggest that activated microglia may be detected in remote brain regions with fiber tract connections to the lesion site as an indicator of poststroke neuroinflammation. However, the specificity of these imaging findings ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.21796
更新日期:2009-12-01 00:00:00
abstract::Regional cerebral blood flow (rCBF) was measured in human subjects during saccadic eye movements by a 254-channel dynamic gamma camera. Focal rCBF increases were repeatedly observed in an area within the middle precentral and premotor regions which corresponds to the frontal eye field in humans. Our findings suggest t...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410050112
更新日期:1979-01-01 00:00:00
abstract::A 71-year-old woman presented with a short history of episodes of severe left-sided orbital and temporal pain in paroxysms lasting 60 to 90 seconds, and accompanied by ipsilateral lacrimation of the eye, rhinorrhea, and conjunctival injection. Results of clinical examination and structural imaging were normal and a cl...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/1531-8249(199911)46:5<791::aid-ana18>3.0.c
更新日期:1999-11-01 00:00:00
abstract::The Pilot Stroke Data Bank obtained information on 94 patients with intracerebral hemorrhage. These data were used to identify factors predictive of 30-day outcome from among 85 demographic, historical, clinical, and laboratory variables generally available to clinicians on the day of admission. The 9 univariate facto...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410240213
更新日期:1988-08-01 00:00:00
abstract:OBJECTIVE:Charcot-Marie-Tooth type 4J (CMT4J) is a rare autosomal recessive neuropathy caused by mutations in FIG4 that result in loss of FIG4 protein. This study investigates the natural history and mechanisms of segmental demyelination in CMT4J. METHODS:Over the past 9 years, we have enrolled and studied a cohort of...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.25198
更新日期:2018-04-01 00:00:00
abstract::The past decade has seen advances in the management of patients with epilepsy. The development of practical long-term electroencephalographic techniques, with or without simultaneous video recording, has increased the accuracy of diagnosis of seizure types. The technique also provides clinicians and investigators with...
journal_title:Annals of neurology
pub_type: 杂志文章,评审
doi:10.1002/ana.410090103
更新日期:1981-01-01 00:00:00
abstract::Reductions in cerebral metabolism sufficient to impair cognition in normal individuals also occur in Alzheimer's disease (AD). The degree of clinical disability in AD correlates closely to the magnitude of the reduction in brain metabolism. Therefore, we tested whether impairments in tricarboxylic acid (TCA) cycle enz...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.20474
更新日期:2005-05-01 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
pub_type: 杂志文章
doi:10.1002/ana.410410416
更新日期:1997-04-01 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
pub_type: 杂志文章
doi:10.1002/ana.10675
更新日期:2003-09-01 00:00:00
abstract::The oculocerebrorenal syndrome (OCRS), Lowe's syndrome, is an X-linked, recessive disease characterized by mental retardation, congenital corneal abnormalities and cataracts, growth failure, rickets, osseous abnormalities, renal dysfunction with periodic acidosis, hypotonia, and areflexia. Ultrastructural studies of s...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410160109
更新日期:1984-07-01 00:00:00
abstract::Two recent case-control studies have suggested a strong association of a missense polymorphism in exon 2 of the cathepsin D gene (CTSD) and Alzheimer disease (AD). However, these findings were not confirmed in another independent study. We analyzed this polymorphism in two large and independent AD study populations an...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/1531-8249(200101)49:1<114::aid-ana18>3.0.c
更新日期:2001-01-01 00:00:00
abstract::Eight patients with recent cerebral hemispheric infarction were studied with positron emission tomography and the oxygen-15 steady-state inhalation and [18F]deoxyglucose techniques to obtain values of regional cerebral blood flow, oxygen consumption, and glucose metabolism. The Sokoloff equation, used to calculate glu...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410140605
更新日期:1983-12-01 00:00:00
abstract::There is currently no effective pharmacological treatment for amyotrophic lateral sclerosis (ALS). Because evidence suggests that multiple pathways may contribute to ALS pathogenesis, we tested in a mouse model of ALS (SOD1(G37R) mice) a combination approach consisting of three drugs for distinct targets in the comple...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.10500
更新日期:2003-04-01 00:00:00
abstract:OBJECTIVE:To evaluate the outcomes 1 year and longer following stereotactic laser amygdalohippocampotomy for mesial temporal lobe epilepsy in a large series of patients treated over a 5-year period since introduction of this novel technique. METHODS:Surgical outcomes of a consecutive series of 58 patients with mesial ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.25180
更新日期:2018-03-01 00:00:00
abstract::We have recently reported homozygous mutations in the PINK1 gene in three consanguineous families with early-onset parkinsonism (EOP) linked to the PARK6 locus. To further evaluate the pathogenic role of PINK1 in EOP and to draw genotype-phenotype correlates, we performed PINK1 mutation analysis in a cohort of Italian...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.20256
更新日期:2004-09-01 00:00:00
abstract::Recently, the mutation causing early-onset generalized torsion dystonia has been identified as a GAG deletion in the gene for an adenosine triphosphate-binding protein named torsinA. We describe a German family with 5 clinically affected individuals carrying this mutation. In at least 4 of the 5 patients, the disease ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410440119
更新日期:1998-07-01 00:00:00
abstract:OBJECTIVE:Right-handedness and left-sided language lateralization is an unresolved mystery with unknown cause/effect relations. Most studies suggest that the language lateralization is related to a fundamental brain asymmetry: right-handedness may be secondary. We analyzed the possibility of an opposite cause/effect re...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.21538
更新日期:2009-01-01 00:00:00