Abstract:
:A series of nine related aromatic monocarboxylic acids (ACAs) previously shown to inhibit muscle membrane chloride conductance (GCl) selectively in the rat were studied for their ability to produce myotonia. All nine induced characteristic repetitive electrical activity and delayed relaxation in isolated muscle, although the concentrations required for this action varied widely. In each case, myotonia was observed at concentrations that correlated closely with previously determined half-maximal concentrations for inhibition of GCl. Intracellular recordings from muscle made myotonic with ACA revealed prolonged latencies at rheobase, multiple driven spikes, and self-sustaining repetitive activity similar to that previously reported in hereditary goat myotonia. Phase-plane diagrams of membrane action potentials recorded after exposure to the most effective of these compounds suggested little effect on the voltage-dependent sodium system. The changes seen could be duplicated by simple removal of chloride ion. The expression of repetitive electrical activity in the presence of low membrane GCl depends on ambient temperature and on the concentration of calcium ion. Increasing temperature and decreasing Ca++ predispose toward myotonic activity; converse conditions inhibit myotonia. Myotonia induced by ACA is inhibited by concentrations of diphenylhydantoin that are clinically effective in controlling hereditary myotonia in humans.
journal_name
Ann Neuroljournal_title
Annals of neurologyauthors
Furman RE,Barchi RLdoi
10.1002/ana.410040411subject
Has Abstractpub_date
1978-10-01 00:00:00pages
357-65issue
4eissn
0364-5134issn
1531-8249journal_volume
4pub_type
杂志文章abstract::We have conducted a trial to randomly assess the efficacy and tolerance of intravenous immunoglobulin (i.v.Ig) or plasma exchange (PE) in myasthenia gravis (MG) exacerbation and to compare two doses of i.v.Ig. Eighty-seven patients with MG exacerbation were randomized to receive either three PE (n = 41), or i.v.Ig (n ...
journal_title:Annals of neurology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1002/ana.410410615
更新日期:1997-06-01 00:00:00
abstract:OBJECTIVE:A number of hypotheses have been put forward as to why humans respond to fever by seizing. The current leading hypotheses are that respiratory alkalosis produces an as yet unidentified change in neural excitability or that inflammatory mediators potentiate excitatory synaptic transmission. However, it is well...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.21712
更新日期:2009-08-01 00:00:00
abstract::The cerebrovascular response of newborn animals to hypotension has not been defined on a regional basis. Using an autoradiographic technique employing 14C-iodoantipyrine as indicator, we studied the cerebral physiological responses of newborn dogs to hypotension induced by exsanguination or by administration of Escher...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410120506
更新日期:1982-11-01 00:00:00
abstract::We report the clinical, imaging, and laboratory features of 8 patients with Devic's neuromyelitis optica. All patients had severe myelopathy and optic neuritis. In no patient was the brain, the brainstem, or the cerebellum affected, even after several years of disease. Various immunosuppressive treatments failed to be...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410340211
更新日期:1993-08-01 00:00:00
abstract:OBJECTIVE:Normal aging is often associated with a decline in learning and memory functions. This decline is manifested to a much greater extent in Alzheimer's disease. Recent studies have indicated statins, a class of cholesterol-lowering drugs, as a potential therapy for Alzheimer's disease. Our objective was to deter...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.21053
更新日期:2006-12-01 00:00:00
abstract:OBJECTIVE:Neonatal white matter injury (NWMI) is a lesion found in preterm infants that can lead to cerebral palsy. Although antagonists of bone morphogenetic protein (BMP) signaling, such as Noggin, promote oligodendrocyte precursor cell (OPC) production after hypoxic-ischemic (HI) injury, the downstream functional ta...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24907
更新日期:2017-04-01 00:00:00
abstract::The term Charcot-Wilbrand syndrome (CWS) denotes dream loss following focal brain damage. We report the first case of CWS, in whom neuropsychological functions, extension of the underlying lesion, and sleep architecture changes were assessed. A 73-year-old woman reported a total dream loss after acute, bilateral occip...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.20246
更新日期:2004-10-01 00:00:00
abstract:OBJECTIVE:To investigate whether phospholipase A2 (PLA2) plays a role in the pathogenesis of spinal cord injury (SCI). METHODS:Biochemical, Western blot, histological, immunohistochemical, electron microscopic, electrophysiological, and behavior assessments were performed to investigate (1) SCI-induced PLA2 activity, ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.20798
更新日期:2006-04-01 00:00:00
abstract::Nineteen patients with radiologically confirmed stroke, and varying degrees of hemiparesis, were studied using somatosensory evoked potentials and the recently developed technique of transcutaneous motor cortex stimulation. The functional deficit caused by stroke was assessed at the time of evoked potential testing an...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410250111
更新日期:1989-01-01 00:00:00
abstract:OBJECTIVE:Amyotrophic lateral sclerosis/parkinsonism-dementia complex has been described in Guam, Western Papua, and the Kii Peninsula of Japan. The etiology and pathogenesis of this complex neurodegenerative disease remains enigmatic. METHODS:In this study, we have used targeted genomic sequencing to evaluate the con...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24346
更新日期:2015-03-01 00:00:00
abstract::Immunohistochemical examination of 20 Down's syndrome brains, using antibodies to alpha-, beta-, and gamma-synuclein, demonstrated many alpha-synuclein-positive Lewy bodies and dystrophic neurites in 50% of amygdala samples from Down's syndrome brains with Alzheimer's disease. Similar lesions were less common in other...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/1531-8249(199903)45:3<353::aid-ana11>3.0.c
更新日期:1999-03-01 00:00:00
abstract::The etiology of neurodegenerative diseases remains enigmatic; however, evidence for defects in energy metabolism, excitotoxicity, and for oxidative damage is increasingly compelling. It is likely that there is a complex interplay between these mechanisms. A defect in energy metabolism may lead to neuronal depolarizati...
journal_title:Annals of neurology
pub_type: 杂志文章,评审
doi:10.1002/ana.410380304
更新日期:1995-09-01 00:00:00
abstract::Nowadays, the "flipped classroom" approach is taking the center stage within medical education. However, very few reports on the implementation of the flipped classroom in neurology have been published to date, and this educational model still represents a challenge for students and educators alike. In this article, n...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.25609
更新日期:2020-01-01 00:00:00
abstract:OBJECTIVE:Whether febrile status epilepticus (FSE) produces hippocampal sclerosis (HS) and temporal lobe epilepsy (TLE) has long been debated. Our objective is to determine whether FSE produces acute hippocampal injury that evolves to HS. METHODS:FEBSTAT and 2 affiliated studies prospectively recruited 226 children ag...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24081
更新日期:2014-02-01 00:00:00
abstract:OBJECTIVE:Multiple sclerosis (MS) is characterized by demyelination centered around cerebral veins. Recent studies suggested this topographic pattern may be caused by venous congestion, a condition termed chronic cerebrospinal venous insufficiency (CCSVI). Published sonographic criteria of CCSVI include reflux in the d...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.22085
更新日期:2010-08-01 00:00:00
abstract::Localized water-suppressed 1H magnetic resonance spectroscopy was performed in an 11-month-old infant with Leigh syndrome. Spectra obtained from the basal ganglia, occipital cortex, and brainstem showed elevations in lactate, which were most pronounced in regions where abnormalities were seen with routine T2-weighted ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410290219
更新日期:1991-02-01 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
pub_type: 杂志文章
doi:10.1002/ana.410160204
更新日期:1984-08-01 00:00:00
abstract::The pharmacological responses of vascular adrenergic receptors to intravenously administered epinephrine, phentolamine, and propranolol were assessed by measuring muscle blood flow (MBF) changes in the tibialis anterior muscle using the xenon 133 clearance technique and were compared in 8 normal subjects and 11 patien...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410090209
更新日期:1981-02-01 00:00:00
abstract::We reviewed the records of 160 consecutive patients with glioblastoma and anaplastic astrocytoma to evaluate the long-term consequences of radiation therapy and chemotherapy. We defined long-term survivors as those patients with glioblastoma or anaplastic astrocytoma who lived at least 100% longer than median survival...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410280614
更新日期:1990-12-01 00:00:00
abstract::Authorship of scientific publications holds great importance for basic and clinical researchers. Academic appointments and promotions, grant funding, and salary support depend to some extent on published recognition through authorship. Peer-recognition and personal satisfaction are additional incentives for authorship...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.22232
更新日期:2010-12-01 00:00:00
abstract::Vasospasm may be one of the causes of amaurosis fugax. A patient is reported who daily experienced multiple brief episodes of amaurosis fugax. The absence of physical, laboratory, or radiographic evidence for thromboembolism, hemodynamic compromise, or vasculitis, suggested that the amaurosis might be caused by vasosp...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410300317
更新日期:1991-09-01 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::Positron emission tomography (PET) of glucose metabolism is often applied for the localization of epileptogenic brain regions, but hypometabolic areas are often larger than or can miss epileptogenic cortex in nonlesional neocortical epilepsy. The present study is a three-dimensional brain surface analysis designed to ...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:
更新日期:2000-07-01 00:00:00
abstract:OBJECTIVE:To assess the associations of blood biomarkers of carbohydrate, lipid, and apolipoprotein metabolisms with the future risk of amyotrophic lateral sclerosis (ALS). METHODS:In the Apolipoprotein-related MOrtality RISk study, we enrolled 636,132 men and women during 1985-1996 in Stockholm, Sweden, with measurem...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.24936
更新日期:2017-05-01 00:00:00
abstract:OBJECTIVE:Nemaline myopathy (NM) is one of the most common congenital nondystrophic myopathies and is characterized by muscle weakness, often from birth. Mutations in ACTA1 are a frequent cause of NM (ie, NEM3). ACTA1 encodes alpha-actin 1, the main constituent of the sarcomeric thin filament. The mechanisms by which m...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.25144
更新日期:2018-02-01 00:00:00
abstract::In diabetic nerves, the activation of the polyol pathway and a resulting decrease in Na(+)-K(+) ATPase activity lead to intra-axonal Na(+) accumulation and a smaller Na(+) gradient across the axolemma than normal. To investigate whether glycemic control is associated with acutely reversible changes in axonal excitabil...
journal_title:Annals of neurology
pub_type: 临床试验,杂志文章
doi:10.1002/ana.20232
更新日期:2004-10-01 00:00:00
abstract::In 2006, Yamanaka's group pioneered a method for reprogramming somatic cells by introducing definite transcription factors, which enabled the generation of induced pluripotent stem cells (iPSCs) with pluripotency comparable to that of embryonic stem cells. These iPSCs are attracting considerable attention for their po...
journal_title:Annals of neurology
pub_type: 杂志文章,评审
doi:10.1002/ana.23596
更新日期:2012-08-01 00:00:00
abstract::We have observed many focal dilatations or very small aneurysms in terminal arterioles and capillaries of 4 of 5 patients and 6 dogs who had recently undergone cardiopulmonary bypass. A smaller number of sausagelike dilatations distended medium-sized arterioles. Two other patients had a small number of the same microv...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.410280403
更新日期:1990-10-01 00:00:00
abstract:OBJECTIVE:To study antibody-independent contributions of B cells to inflammatory disease activity, and the immune consequences of B-cell depletion with rituximab, in patients with multiple sclerosis (MS). METHODS:B-Cell effector-cytokine responses were compared between MS patients and matched controls using a 3-signal...
journal_title:Annals of neurology
pub_type: 杂志文章,随机对照试验
doi:10.1002/ana.21939
更新日期:2010-04-01 00:00:00
abstract::Guillain-Barré syndrome (GBS) is an immune-mediated neuropathy, in which leukocytes and humoral components of the immune system proposedly initiate localized inflammation. An important pathogenic role for anti-GM1 ganglioside antibodies has been suggested. Therefore, we evaluated anti-GM1 IgG antibody-induced leukocyt...
journal_title:Annals of neurology
pub_type: 杂志文章
doi:10.1002/ana.10503
更新日期:2003-05-01 00:00:00