Abstract:
:Because the symptomatic treatments for multiple sclerosis (MS) are limited, new approaches have been sought. Anatomical studies of MS lesions show a relative preservation of axons, and clinical studies suggest that some of the neurological impairment in patients with MS is physiological. Electrophysiological studies suggest that demyelination exposes axonal potassium channels that decrease action-potential duration and amplitude, hindering action-potential propagation. Potassium channel blockers, including aminopyridines, have been shown to improve nerve conduction in experimentally demyelinated nerves. Two potassium channel blockers, 4-aminopyridine (AP) and 3,4 diaminopyridine (DAP) have been tested in patients with MS. Preliminary studies of AP demonstrated benefit in many temperature-sensitive patients with MS, and improvement of function was found in a large randomized double-blind, placebo-controlled crossover trial of 3 months of oral treatment in 68 patients with MS. An open-label trial of DAP showed improvement in some deficits, and a double-blind placebo-controlled trial showed significant improvements in prospectively defined neurological deficits. A crossover comparison of the two agents suggested that AP produces more central nervous system side effects (dizziness and confusion), whereas DAP produces more peripheral side effects (paresthesias and abdominal pain). Both agents have rarely caused seizures. These studies suggest that aminopyridines may provide a new approach to the symptomatic treatment of MS.
journal_name
Ann Neuroljournal_title
Annals of neurologyauthors
Bever CT Jrdoi
10.1002/ana.410360728subject
Has Abstractpub_date
1994-01-01 00:00:00pages
S118-21eissn
0364-5134issn
1531-8249journal_volume
36 Supplpub_type
杂志文章,评审abstract::Homocarnosine, a dipeptide of gamma-aminobutyric acid (GABA) and histidine, is thought to be an inhibitory neuromodulator synthesized in subclasses of GABAergic neurons. Homocarnosine is present in human brain in greater amounts (0.4-1.0 micromol/g) than in other animals. The antiepileptic drug vigabatrin increases hu...
journal_title:Annals of neurology
pub_type: 临床试验,杂志文章
doi:10.1002/ana.410440614
更新日期:1998-12-01 00:00:00
abstract::We performed a population-based clinical and molecular genetic study of spinocerebellar ataxia type 6 (SCA6) in the northeast of England. The minimum point prevalence of SCA6 was 1.59 in 100,000 (95% confidence interval [CI], 1.04-2.14), and the number of individuals who either had SCA6 or are at risk of developing SC...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.20110
更新日期:2004-05-01 00:00:00
abstract:OBJECTIVE:Guillain-Barré syndrome (GBS) is an acute postinfectious immune-mediated polyneuropathy. Although preceding respiratory tract infections with Mycoplasma pneumoniae have been reported in some cases, the role of M. pneumoniae in the pathogenesis of GBS remains unclear. We here cultured, for the first time, M. p...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24755
更新日期:2016-10-01 00:00:00
abstract::The published information on glutamate levels in cerebrospinal fluid (CSF) and modifications in neurological disorders is controversial. In the present study, we demonstrated a metabolic instability of glutamate in untreated CSF and a spurious elevation of its levels by the current methods of CSF acidification. These ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410330316
更新日期:1993-03-01 00:00:00
abstract::We describe two new clinical syndromes, mirror agnosia and mirror ataxia, both characterized by the deficit of reaching for an object through a mirror in association with a lesion of either parietal lobe. Clinical investigation of 13 patients demonstrated that the impairments affected both sides of the body. In mirror...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/1531-8249(199907)46:1<51::aid-ana9>3.0.co;
更新日期:1999-07-01 00:00:00
abstract::Inborn errors of urea synthesis can present in the newborn period as a catastrophic illness or later in childhood or adulthood with an indolent course punctuated by hyperammonemic episodes. Because symptoms mimic other neuropsychiatric disorders, it is common for there to be a delay in diagnosis, often with dire conse...
journal_title:Annals of neurology
pub_type: 杂志文章,评审
doi:10.1002/ana.410350204
更新日期:1994-02-01 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:The polyglutamine diseases, including Huntington's disease (HD) and multiple spinocerebellar ataxias (SCAs), are among the commonest hereditary neurodegenerative diseases. They are caused by expanded CAG tracts, encoding glutamine, in different genes. Longer CAG repeat tracts are associated with earlier ages ...
journal_title:Annals of neurology
pub_type: 杂志文章,多中心研究
doi:10.1002/ana.24656
更新日期:2016-06-01 00:00:00
abstract::A young man presented with apparent transient ischemic attacks following a motorcycle accident in which he sustained minor injuries only. Computerized axial tomography demonstrated a small right frontal infarct, and angiography revealed an aneurysm of the right internal carotid artery in its extracranial course. This ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410040118
更新日期:1978-07-01 00:00:00
abstract:OBJECTIVE:GM2 gangliosidoses are lysosomal diseases due to biallelic mutations in the HEXA (Tay-Sachs disease [TS]) or HEXB (Sandhoff disease [SD]) genes, with subsequent low hexosaminidase(s) activity. Most patients have childhood onset, but some experience the first symptoms during adolescence/adulthood. This study a...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.25689
更新日期:2020-04-01 00:00:00
abstract::Unverricht-Lundborg disease is a clinically recognizable form of progressive myoclonus epilepsy. Recently, in several families of both Finnish and Mediterranean extraction segregating Unverricht-Lundborg disease, the gene for this disease was linked to the same region of the long arm of chromosome 21. We performed lin...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410340519
更新日期:1993-11-01 00:00:00
abstract:OBJECTIVE:A workgroup commissioned by the Alzheimer's Association (AA) and the National Institute on Aging (NIA) recently published research criteria for preclinical Alzheimer disease (AD). We performed a preliminary assessment of these guidelines. METHODS:We employed Pittsburgh compound B positron emission tomography...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.22628
更新日期:2012-06-01 00:00:00
abstract::We describe a 33-year-old homosexual man with a steroid-responsive, remitting and relapsing leukoencephalopathy associated with recent human immunodeficiency virus type 1 (HIV-1) seroconversion. Biopsy of a parieto-occipital lesion revealed demyelination and astrogliosis with focal necrosis. Detailed investigations de...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410310107
更新日期:1992-01-01 00:00:00
abstract::Huntington's disease is an inherited disorder caused by expansion of a CAG trinucleotide repeat in the IT15 gene, which leads to expansion of a polyglutamine tract within the protein called huntingtin. Despite the characterization of the IT15 gene and the mutation involved in the disease, the normal function of huntin...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410420507
更新日期:1997-11-01 00:00:00
abstract:OBJECTIVE:Centronuclear myopathy (CNM) is a rare congenital myopathy characterized by prominence of central nuclei on muscle biopsy. CNM has been associated with mutations in MTM1, DNM2, and BIN1 but many cases remain genetically unresolved. RYR1 encodes the principal sarcoplasmic reticulum calcium release channel and ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.22119
更新日期:2010-11-01 00:00:00
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 underl...
journal_title:Annals of neurology
pub_type: 杂志文章,评审
doi:10.1002/ana.410310320
更新日期:1992-03-01 00:00:00
abstract::We describe a French amyotrophic lateral sclerosis (ALS) family with two distinct mutations in the Cu/Zn superoxide dismutase (SOD1) gene. The D90A mutation has been well described and clearly shown to cause recessive ALS. In this family, affected individuals are heterozygous for the D90A mutation and also carry a sin...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/1531-8249(20010201)49:2<267::aid-ana51>3.0
更新日期:2001-02-01 00:00:00
abstract:OBJECTIVE:There are no validated methods for predicting the timing of seizures. Using machine learning, we sought to forecast 24-hour risk of self-reported seizure from e-diaries. METHODS:Data from 5,419 patients on SeizureTracker.com (including seizure count, type, and duration) were split into training (3,806 patien...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.25812
更新日期:2020-09-01 00:00:00
abstract::Nine patients who had suffered strokes were examined between 10 and 34 days after onset using positron emission tomography. DMO labeled with carbon 11 was used to evaluate brain acid-base balance, and the oxygen-15 inhalation technique was used to measure regional cerebral blood flow, the oxygen extraction fraction, a...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410140405
更新日期:1983-10-01 00:00:00
abstract::A 65-year-old woman with normal pressure hydrocephalus experienced improved gait and hydrocephalus following surgical resection of a cauda equina neurinoma. Fibrinogen, detected in the cerebrospinal fluid, may be involved in the pathophysiological process of normal pressure hydrocephalus. ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410270414
更新日期:1990-04-01 00:00:00
abstract::Indirect evidence suggests that an autoimmune response to myelin basic protein (MBP) may be involved in the pathogenesis of multiple sclerosis (MS). In MS, several reports have suggested that restricted T-cell populations respond to MPB, as in inbred rodents with the MS disease model experimental allergic encephalomye...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410340313
更新日期:1993-09-01 00:00:00
abstract::Recently, variant mRNA transcripts for the astroglial glutamate transporter EAAT2 have been detected in brain tissues of 60% of patients with sporadic amyotrophic lateral sclerosis (SALS). We have tested the hypothesis that the gene for EAAT2 may be defective in some ALS cases. In 16 familial ALS (FALS) pedigrees with...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410430514
更新日期:1998-05-01 00:00:00
abstract::Recent neuroepidemiological studies of endemic tropical spastic paraparesis (TSP) have confirmed the existence of high-prevalence foci in several tropical islands, including Jamaica and Martinique in the Caribbean, Tumaco off the Pacific coast of Colombia, and the Seychelles in the Indian Ocean. There is a net prepond...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410230728
更新日期:1988-01-01 00:00:00
abstract:OBJECTIVE:To develop and validate a questionnaire for assessing cutaneous allodynia (CA), and to estimate the prevalence and severity of CA in the migraine population. METHODS:Migraineurs (n = 11,388) completed the Allodynia Symptom Checklist, assessing the frequency of allodynia symptoms during headache. Response opt...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.21211
更新日期:2008-02-01 00:00:00
abstract::Three-dimensional (volume) magnetic resonance angiography is a noninvasive technique that images the intracranial and cervical arterial vasculature without contrast agents. Twenty-four children with strokes had combined parenchymal magnetic resonance imaging and magnetic resonance angiography 1 day to 4 years after ac...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410290603
更新日期:1991-06-01 00:00:00
abstract:OBJECTIVE:Mutations in the genes encoding the extracellular matrix protein collagen VI (ColVI) cause a spectrum of disorders with variable inheritance including Ullrich congenital muscular dystrophy, Bethlem myopathy, and intermediate phenotypes. We extensively characterized, at the clinical, cellular, and molecular le...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.22087
更新日期:2010-10-01 00:00:00
abstract::One hundred fifty autopsy brains from patients with clinically diagnosed Alzheimer's disease (AD) were examined pathologically. The brains were received consecutively over a 3-year period from numerous sources as part of a research program in which one brain half was frozen for biochemical studies and the other half w...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410240110
更新日期:1988-07-01 00:00:00
abstract::Chronic progressive myelopathy (CPM) is a difficult clinical problem. Many patients who present with CPM turn out to have a spinal form of multiple sclerosis (MS), but until there is clear lesion dissemination, a definite clinical diagnosis cannot be made. We have looked for MS-related abnormalities in 72 patients wit...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410060508
更新日期:1979-11-01 00:00:00
abstract::We performed magnetic resonance imaging and magnetic resonance spectroscopic imaging on 28 patients with multiple sclerosis stratified for disability and clinical course (relapsing with at least partial remissions or secondary progressive disease). Lesions were segmented on the conventional proton density and T2-weigh...
journal_title:Annals of neurology
pub_type: 临床试验,杂志文章
doi:10.1002/ana.410410314
更新日期:1997-03-01 00:00:00
abstract::We demonstrate that the 3-hydroxy-3-methyl-glutaryl-coenzyme A (HMG-CoA) reductase inhibitors atorvastatin and simvastatin enhance functional outcome and induce brain plasticity when administered after stroke to rats. With atorvastatin treatment initiated 1 day after stroke, animals exhibited significant increases in ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.10555
更新日期:2003-06-01 00:00:00