The pathophysiology of myotonia produced by aromatic carboxylic acids.

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 Neurol

journal_title

Annals of neurology

authors

Furman RE,Barchi RL

doi

10.1002/ana.410040411

subject

Has Abstract

pub_date

1978-10-01 00:00:00

pages

357-65

issue

4

eissn

0364-5134

issn

1531-8249

journal_volume

4

pub_type

杂志文章
  • Clinical trial of plasma exchange and high-dose intravenous immunoglobulin in myasthenia gravis. Myasthenia Gravis Clinical Study Group.

    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

    authors: Gajdos P,Chevret S,Clair B,Tranchant C,Chastang C

    更新日期:1997-06-01 00:00:00

  • Heat opens axon initial segment sodium channels: a febrile seizure mechanism?

    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

    authors: Thomas EA,Hawkins RJ,Richards KL,Xu R,Gazina EV,Petrou S

    更新日期:2009-08-01 00:00:00

  • Selective reduction of blood flow to white matter during hypotension in newborn dogs: a possible mechanism of periventricular leukomalacia.

    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

    authors: Young RS,Hernandez MJ,Yagel SK

    更新日期:1982-11-01 00:00:00

  • Devic's neuromyelitis optica: a clinicopathological study of 8 patients.

    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

    authors: Mandler RN,Davis LE,Jeffery DR,Kornfeld M

    更新日期:1993-08-01 00:00:00

  • Simvastatin enhances learning and memory independent of amyloid load in mice.

    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

    authors: Li L,Cao D,Kim H,Lester R,Fukuchi K

    更新日期:2006-12-01 00:00:00

  • Olig1 is required for noggin-induced neonatal myelin repair.

    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

    authors: Sabo JK,Heine V,Silbereis JC,Schirmer L,Levison SW,Rowitch DH

    更新日期:2017-04-01 00:00:00

  • Total dream loss: a distinct neuropsychological dysfunction after bilateral PCA stroke.

    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

    authors: Bischof M,Bassetti CL

    更新日期:2004-10-01 00:00:00

  • A novel role of phospholipase A2 in mediating spinal cord secondary injury.

    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

    authors: Liu NK,Zhang YP,Titsworth WL,Jiang X,Han S,Lu PH,Shields CB,Xu XM

    更新日期:2006-04-01 00:00:00

  • A comparison of somatosensory evoked and motor evoked potentials in stroke.

    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

    authors: Macdonell RA,Donnan GA,Bladin PF

    更新日期:1989-01-01 00:00:00

  • Defining neurodegeneration on Guam by targeted genomic sequencing.

    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

    authors: Steele JC,Guella I,Szu-Tu C,Lin MK,Thompson C,Evans DM,Sherman HE,Vilariño-Güell C,Gwinn K,Morris H,Dickson DW,Farrer MJ

    更新日期:2015-03-01 00:00:00

  • Antibodies to alpha-synuclein detect Lewy bodies in many Down's syndrome brains with Alzheimer's disease.

    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

    authors: Lippa CF,Schmidt ML,Lee VM,Trojanowski JQ

    更新日期:1999-03-01 00:00:00

  • Aging, energy, and oxidative stress in neurodegenerative diseases.

    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

    authors: Beal MF

    更新日期:1995-09-01 00:00:00

  • Strategic Considerations for Applying the Flipped Classroom to Neurology Education.

    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

    authors: Sandrone S,Berthaud JV,Carlson C,Cios J,Dixit N,Farheen A,Kraker J,Owens JWM,Patino G,Sarva H,Weber D,Schneider LD

    更新日期:2020-01-01 00:00:00

  • Hippocampal sclerosis after febrile status epilepticus: the FEBSTAT study.

    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

    authors: Lewis DV,Shinnar S,Hesdorffer DC,Bagiella E,Bello JA,Chan S,Xu Y,MacFall J,Gomes WA,Moshé SL,Mathern GW,Pellock JM,Nordli DR Jr,Frank LM,Provenzale J,Shinnar RC,Epstein LG,Masur D,Litherland C,Sun S,FEBSTAT Study

    更新日期:2014-02-01 00:00:00

  • No cerebrocervical venous congestion in patients with multiple sclerosis.

    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

    authors: Doepp F,Paul F,Valdueza JM,Schmierer K,Schreiber SJ

    更新日期:2010-08-01 00:00:00

  • Regional variation in brain lactate in Leigh syndrome by localized 1H magnetic resonance spectroscopy.

    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

    authors: Detre JA,Wang ZY,Bogdan AR,Gusnard DA,Bay CA,Bingham PM,Zimmerman RA

    更新日期:1991-02-01 00:00:00

  • Peripheral nerve function and metabolic control in diabetes mellitus.

    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

    authors: Troni W,Carta Q,Cantello R,Caselle MT,Rainero I

    更新日期:1984-08-01 00:00:00

  • Vascular adrenergic receptor responses in skeletal muscle in myotonic dystrophy.

    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

    authors: Mechler F,Mastaglia FL

    更新日期:1981-02-01 00:00:00

  • Effects of treatment on long-term survivors with malignant astrocytomas.

    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

    authors: Imperato JP,Paleologos NA,Vick NA

    更新日期:1990-12-01 00:00:00

  • The authorship lottery: an impediment to research collaboration?

    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

    authors: Kaufmann P,Annis C,Griggs RC,Muscle Study Group Executive Committee.

    更新日期:2010-12-01 00:00:00

  • Recurrent attacks of amaurosis fugax treated with calcium channel blocker.

    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

    authors: Winterkorn JM,Teman AJ

    更新日期:1991-09-01 00:00:00

  • Assessment of reliability and biological significance of glutamate levels in cerebrospinal fluid.

    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

    authors: Ferrarese C,Pecora N,Frigo M,Appollonio I,Frattola L

    更新日期:1993-03-01 00:00:00

  • Is epileptogenic cortex truly hypometabolic on interictal positron emission tomography?

    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:

    authors: Juhász C,Chugani DC,Muzik O,Watson C,Shah J,Shah A,Chugani HT

    更新日期:2000-07-01 00:00:00

  • Blood biomarkers of carbohydrate, lipid, and apolipoprotein metabolisms and risk of amyotrophic lateral sclerosis: A more than 20-year follow-up of the Swedish AMORIS cohort.

    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

    authors: Mariosa D,Hammar N,Malmström H,Ingre C,Jungner I,Ye W,Fang F,Walldius G

    更新日期:2017-05-01 00:00:00

  • Dysfunctional sarcomere contractility contributes to muscle weakness in ACTA1-related nemaline myopathy (NEM3).

    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

    authors: Joureau B,de Winter JM,Conijn S,Bogaards SJP,Kovacevic I,Kalganov A,Persson M,Lindqvist J,Stienen GJM,Irving TC,Ma W,Yuen M,Clarke NF,Rassier DE,Malfatti E,Romero NB,Beggs AH,Ottenheijm CAC

    更新日期:2018-02-01 00:00:00

  • The acute effects of glycemic control on axonal excitability in human diabetics.

    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

    authors: Kitano Y,Kuwabara S,Misawa S,Ogawara K,Kanai K,Kikkawa Y,Yagui K,Hattori T

    更新日期:2004-10-01 00:00:00

  • Accelerating progress in induced pluripotent stem cell research for neurological diseases.

    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

    authors: Ito D,Okano H,Suzuki N

    更新日期:2012-08-01 00:00:00

  • Brain microemboli during cardiac surgery or aortography.

    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

    authors: Moody DM,Bell MA,Challa VR,Johnston WE,Prough DS

    更新日期:1990-10-01 00:00:00

  • Abnormal B-cell cytokine responses a trigger of T-cell-mediated disease in MS?

    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

    authors: Bar-Or A,Fawaz L,Fan B,Darlington PJ,Rieger A,Ghorayeb C,Calabresi PA,Waubant E,Hauser SL,Zhang J,Smith CH

    更新日期:2010-04-01 00:00:00

  • Anti-GM1 IgG antibodies induce leukocyte effector functions via Fcgamma receptors.

    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

    authors: van Sorge NM,van den Berg LH,Geleijns K,van Strijp JA,Jacobs BC,van Doorn PA,Wokke JH,van de Winkel JG,Leusen JH,van der Pol WL

    更新日期:2003-05-01 00:00:00