Lambert-Eaton syndrome IgG inhibits transmitter release via P/Q Ca2+ channels.

Abstract:

OBJECTIVE:To determine whether immunoglobulin G (IgG) from patients with Lambert-Eaton myasthenic syndrome (LEMS) decreases action potential–evoked synaptic vesicle exocytosis,and whether the effect is mediated by P/Q-type voltage-gated calcium channels (VGCCs). METHODS:IgG was obtained from 4 patients with LEMS (3 males, 1 female), including 2 patients with lung malignancy. Antibodies against P/Q-type VGCCs were detected in all 4 patients, and against N-type VGCCs in 2. We incubated neuronal cultures with LEMS IgG and determined the size of the total recycling pool of synaptic vesicles and the rate of action potential–evoked exocytosis using fluorescence imaging of the amphiphilic dye SynaptoRed C1. Pooled IgG from healthy volunteers was used as a control. We repeated the experiments on synapses lacking P/Q-type calcium channels from a Cacna1a knockout mouse to determine whether these channels account for the pathogenic effect of LEMS IgG. RESULTS:LEMS IgG had no effect on the total recycling pool size but significantly reduced the rate of action potential–evoked synaptic exocytosis in wild-type neurons when compared with neurons treated with control IgG. In contrast, LEMS IgG had no effect on the rate of synaptic vesicle exocytosis in neurons lacking P/Q-type channels. CONCLUSIONS:These data provide direct evidence that LEMS IgG inhibits neurotransmitter release by acting on P/Q-type VGCCs.

journal_name

Neurology

journal_title

Neurology

authors

Spillane J,Ermolyuk Y,Cano-Jaimez M,Lang B,Vincent A,Volynski KE,Kullmann DM

doi

10.1212/WNL.0000000000001225

subject

Has Abstract

pub_date

2015-02-10 00:00:00

pages

575-9

issue

6

eissn

0028-3878

issn

1526-632X

pii

WNL.0000000000001225

journal_volume

84

pub_type

杂志文章
  • How experienced community neurologists make diagnoses during clinical encounters.

    abstract::Study of diagnostic practice is necessary to optimize neurologists' clinical performance and ensure patient safety. To our knowledge, this report is the first set of systematic observations of diagnostic practices of community neurologists in their clinics. The study consisted of six 2-week periods of in situ observat...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0b013e3182a840c7

    authors: Dhand A,Engstrom J,Dhaliwal G

    更新日期:2013-10-15 00:00:00

  • Distinct brain networks underlie cognitive dysfunction in Parkinson and Alzheimer diseases.

    abstract:OBJECTIVE:To determine whether cognitive impairment in Parkinson disease (PD) and Alzheimer disease (AD) derives from the same network pathology. METHODS:We analyzed 18F-fluorodeoxyglucose PET scans from 40 patients with AD and 40 age-matched healthy controls from the Alzheimer's Disease Neuroimaging Initiative and sc...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000003285

    authors: Mattis PJ,Niethammer M,Sako W,Tang CC,Nazem A,Gordon ML,Brandt V,Dhawan V,Eidelberg D

    更新日期:2016-11-01 00:00:00

  • Severe neurologic complications after hematopoietic stem cell transplantation in children.

    abstract:OBJECTIVE:To describe and evaluate the incidence and risk factors of severe neurologic events (SNE) in pediatric recipients of allogeneic or autologous hematopoietic stem cell transplantation (HSCT) for hematologic or nonhematologic diseases. METHODS:Retrospective analysis of 272 consecutive children admitted to the G...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.wnl.0000036608.42104.b9

    authors: Faraci M,Lanino E,Dini G,Fondelli MP,Morreale G,Dallorso S,Manzitti C,Calevo MG,Gaggero R,Castagnola E,Haupt R

    更新日期:2002-12-24 00:00:00

  • Dementia and dependence: do modifiable risk factors delay disability?

    abstract:OBJECTIVE:To identify modifying factors that preserve functional independence among individuals at high dementia risk. METHODS:Health and Retirement Study participants aged 65 years or older without baseline activities of daily living (ADL) limitations (n = 4,922) were interviewed biennially for up to 12 years. Dement...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000000357

    authors: Rist PM,Capistrant BD,Wu Q,Marden JR,Glymour MM

    更新日期:2014-04-29 00:00:00

  • Acute naming deficits following dominant temporal lobectomy: prediction by age at 1st risk for seizures.

    abstract::Age at 1st risk for seizures may predict anomia following dominant anterior temporal lobectomy. We assessed confrontation naming before and 2 to 3 weeks after surgery in 45 right-handed patients grouped by side of focus and presence or absence of early (less than or equal to 5 years) risk factors. After left lobectomy...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.40.10.1509

    authors: Stafiniak P,Saykin AJ,Sperling MR,Kester DB,Robinson LJ,O'Connor MJ,Gur RC

    更新日期:1990-10-01 00:00:00

  • Does hypothermia influence the predictive value of bilateral absent N20 after cardiac arrest?

    abstract:BACKGROUND:Bilateral absent N20 responses of median nerve somatosensory evoked potentials (SEPs) reliably predict poor prognosis after cardiac arrest. However, the studies supporting this fact were carried out before hypothermia was established as standard treatment. Recent evidence suggests that hypothermia treatment ...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0b013e3181d5a631

    authors: Leithner C,Ploner CJ,Hasper D,Storm C

    更新日期:2010-03-23 00:00:00

  • Mapping brain recovery after concussion: From acute injury to 1 year after medical clearance.

    abstract:OBJECTIVE:To test the hypothesis that concussion-related brain alterations seen at symptomatic injury and medical clearance to return to play (RTP) will have dissipated by 1 year after RTP. METHODS:For this observational study, 24 athletes with concussion were scanned longitudinally within 1 week after injury, at RTP,...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000008523

    authors: Churchill NW,Hutchison MG,Graham SJ,Schweizer TA

    更新日期:2019-11-19 00:00:00

  • The risks and costs of multiple-generic substitution of topiramate.

    abstract:OBJECTIVE:To investigate clinical and economic consequences following generic substitution of one vs multiple generics of topiramate (Topamax; Ortho-McNeil Neurologics, Titusville, NJ). METHODS:Medical and pharmacy claims data of Régie de l'Assurance-Maladie du Québec from January 2006 to October 2007 were used. Patie...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0b013e3181aa5300

    authors: Duh MS,Paradis PE,Latrémouille-Viau D,Greenberg PE,Lee SP,Durkin MB,Wan GJ,Rupnow MF,LeLorier J

    更新日期:2009-06-16 00:00:00

  • Arterial wall properties in carotid artery dissection: an ultrasound study.

    abstract:BACKGROUND:Cervical artery dissection is often attributed to an underlying arteriopathy related to a generalized extracellular matrix defect. OBJECTIVE:The authors compared the hemodynamic and morphologic properties of the carotid artery, as assessed noninvasively by ultrasonography, in patients with spontaneous inter...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.55.5.663

    authors: Guillon B,Tzourio C,Biousse V,Adraï V,Bousser MG,Touboul PJ

    更新日期:2000-09-12 00:00:00

  • Distribution of partial seizures during the sleep--wake cycle: differences by seizure onset site.

    abstract:OBJECTIVE:To evaluate the effects of sleep on partial seizures arising from various brain regions. METHODS:The authors prospectively studied 133 patients with localization-related epilepsy undergoing video-EEG monitoring over a 2-year period. Seizure type, site of onset, sleep/wake state at onset, duration, and epilep...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.56.11.1453

    authors: Herman ST,Walczak TS,Bazil CW

    更新日期:2001-06-12 00:00:00

  • The thymus gland in elderly patients with myasthenia gravis.

    abstract::This study was undertaken to determine the amount and histologic appearance of the persistent thymic tissue removed post mortem from 20 patients over 60 years of age with myasthenia gravis. One patient died several days after thymectomy. No recognizable thymic tissue on gross examination was seen in any patient. On mi...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.25.3.294

    authors: Perlo VP,Arnason B,Castleman B

    更新日期:1975-03-01 00:00:00

  • Multifocal motor neuropathy. Serum IgM anti-GM1 ganglioside antibodies in most patients detected using covalent linkage of GM1 to ELISA plates.

    abstract::IgM anti-GM1 antibodies occur with increased frequency in the serum of patients with multifocal motor neuropathy (MMN). We tested the ability of serum IgM from patients with MMN to bind to GM1 ganglioside covalently bound to secondary amino groups on ELISA plates (Co-GM1). The Co-GM1 technique detected high titer (> 1...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.49.5.1289

    authors: Pestronk A,Choksi R

    更新日期:1997-11-01 00:00:00

  • Lacunar lesions are independently associated with disability and cognitive impairment in CADASIL.

    abstract:OBJECTIVE:Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary arteriopathy caused by mutations of the Notch3 gene. The disease is characterized by T2-hyperintense lesions (subcortical white matter lesions), T1-hypointense lesions (lacunar lesions), and T2...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.wnl.0000265221.05610.70

    authors: Viswanathan A,Gschwendtner A,Guichard JP,Buffon F,Cumurciuc R,O'Sullivan M,Holtmannspötter M,Pachai C,Bousser MG,Dichgans M,Chabriat H

    更新日期:2007-07-10 00:00:00

  • Defective auditory interhemispheric transfer in a patient with a PAX6 mutation.

    abstract::Heterozygous PAX6 mutation is associated with an absent or hypoplastic anterior commissure and a reduction in the area of the corpus callosum. The authors found deficient auditory interhemispheric transfer in a 53-year-old woman with a PAX6 mutation who had an absent anterior commissure but normal callosal volume. ...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.wnl.0000106950.74155.0a

    authors: Bamiou DE,Musiek FE,Sisodiya SM,Free SL,Mitchell TN,Davies RA

    更新日期:2004-02-10 00:00:00

  • A double-blind study comparing carbamazepine with phenytoin as initial seizure therapy in adults.

    abstract::Carbamazepine was compared with phenytoin in a double-blind study. Of 87 patients, data on 70 patients were complete and used for analysis. Thirty-five patients were treated with each drug. The incidence of major side effects, minor side effects, and complete control (85%) was the same in both groups. A mild but signi...

    journal_title:Neurology

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:10.1212/wnl.33.7.904

    authors: Ramsay RE,Wilder BJ,Berger JR,Bruni J

    更新日期:1983-07-01 00:00:00

  • Neuropathological findings in Sneddon's syndrome.

    abstract::The authors report the neuropathologic findings in a case of Sneddon's syndrome. There were multiple small, predominantly cortical, infarcts, with focal hyperplasia and fibrotic occlusion of arterial vessels in the superficial white matter, cortex, and leptomeninges. A very occasional arterial thrombus was seen. These...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.wnl.0000055931.66009.d3

    authors: Hilton DA,Footitt D

    更新日期:2003-04-08 00:00:00

  • Brain reserve and cognitive reserve protect against cognitive decline over 4.5 years in MS.

    abstract:OBJECTIVE:Based on the theories of brain reserve and cognitive reserve, we investigated whether larger maximal lifetime brain growth (MLBG) and/or greater lifetime intellectual enrichment protect against cognitive decline over time. METHODS:Forty patients with multiple sclerosis (MS) underwent baseline and 4.5-year fo...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000000433

    authors: Sumowski JF,Rocca MA,Leavitt VM,Dackovic J,Mesaros S,Drulovic J,DeLuca J,Filippi M

    更新日期:2014-05-20 00:00:00

  • Antisaccade task reflects cortical involvement in mild cognitive impairment.

    abstract:OBJECTIVE:The aims of this study were to examine executive dysfunction using an antisaccade (AS) task in normal elderly (NE) and patients with mild cognitive impairment (MCI) and Alzheimer disease (AD) as well as to evaluate the relationship between AS performance and cortical thinning within AD-associated regions. ME...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0b013e3182a6cbfe

    authors: Heuer HW,Mirsky JB,Kong EL,Dickerson BC,Miller BL,Kramer JH,Boxer AL

    更新日期:2013-10-01 00:00:00

  • Effect of white matter lesions on manual dexterity in healthy middle-aged persons.

    abstract:OBJECTIVES:We hypothesized that integrated motor-visual functions measured by manipulative manual dexterity are affected by white matter lesion (WML) burden as measured on cranial MRI across relevant brain regions in subjects at risk of preclinical occult vascular disease. METHODS:A real-time cross-sectional study of ...

    journal_title:Neurology

    pub_type: 杂志文章,随机对照试验

    doi:10.1212/WNL.0000000000001557

    authors: Nyquist PA,Yanek LR,Bilgel M,Cuzzocreo JL,Becker LC,Chevalier-Davis K,Yousem D,Prince J,Kral BG,Vaidya D,Becker DM

    更新日期:2015-05-12 00:00:00

  • Benefits of rivastigmine on attention in dementia associated with Parkinson disease.

    abstract::In a 24-week, randomized, double-blind, placebo-controlled, multicenter study of rivastigmine, 487 patients with dementia associated with Parkinson disease underwent assessment of attention on the Cognitive Drug Research computerized cognitive assessment system before dosing and 16 and 24 weeks later. Significant bene...

    journal_title:Neurology

    pub_type: 杂志文章,多中心研究,随机对照试验

    doi:10.1212/01.wnl.0000184517.69816.e9

    authors: Wesnes KA,McKeith I,Edgar C,Emre M,Lane R

    更新日期:2005-11-22 00:00:00

  • Effect of age at onset on progression and mortality in Parkinson's disease.

    abstract::We examined longitudinal disability scores in 54 patients with Parkinson's disease followed for 6 years at UCLA. We sorted data into 3 groups based on age at onset of symptoms: group A, onset under 50 years; group B, 50 to 59 years; group C, 60 years or older. There were no significant differences between groups initi...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.39.9.1187

    authors: Diamond SG,Markham CH,Hoehn MM,McDowell FH,Muenter MD

    更新日期:1989-09-01 00:00:00

  • Detection of subtle gait disturbance and future fall risk in early multiple sclerosis.

    abstract:OBJECTIVE:To test the hypothesis that higher-challenge gait and balance tasks are more sensitive than traditional metrics to subtle patient-reported gait dysfunction and future fall risk in early multiple sclerosis (MS). METHODS:Persons with early MS (n = 185; ≤5 years diagnosed) reported gait function (MS Walking Sca...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000008938

    authors: Brandstadter R,Ayeni O,Krieger SC,Harel NY,Escalon MX,Katz Sand I,Leavitt VM,Fabian MT,Buyukturkoglu K,Klineova S,Riley CS,Lublin FD,Miller AE,Sumowski JF

    更新日期:2020-03-31 00:00:00

  • Association between waking EEG slowing and REM sleep behavior disorder in PD without dementia.

    abstract:OBJECTIVE:To compare nondemented patients with Parkinson's disease (PD) with and without REM sleep behavior disorder (RBD) to healthy controls on quantitative EEG characteristics for both wakefulness and REM sleep. METHODS:Fifteen patients with PD (7 patients with polysomnographic-confirmed RBD [PD-RBD] and 8 patients...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.wnl.0000106460.34682.e9

    authors: Gagnon JF,Fantini ML,Bédard MA,Petit D,Carrier J,Rompré S,Décary A,Panisset M,Montplaisir J

    更新日期:2004-02-10 00:00:00

  • Pediatric cavernous sinus thrombosis: A case series and review of the literature.

    abstract:OBJECTIVE:To describe clinical characteristics, imaging findings, morbidity, and mortality in a single-center cohort of 12 pediatric cavernous sinus thrombosis cases and to review all cases available in recent English literature. METHODS:Clinical data and radiographic studies on 12 cases from our institution were anal...

    journal_title:Neurology

    pub_type: 杂志文章,评审

    doi:10.1212/WNL.0000000000001886

    authors: Smith DM,Vossough A,Vorona GA,Beslow LA,Ichord RN,Licht DJ

    更新日期:2015-09-01 00:00:00

  • Intrinsic epileptogenicity of cortical tubers revealed by intracranial EEG monitoring.

    abstract:OBJECTIVE:We sought to identify intracranial EEG patterns characteristic of epileptogenic tubers and to understand the contribution of perituberal cortex. METHODS:Twenty-three intracranial EEG monitoring studies were reviewed from 17 children aged 1.3-7.7 years with tuberous sclerosis complex and intractable multifoca...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0b013e3182768923

    authors: Mohamed AR,Bailey CA,Freeman JL,Maixner W,Jackson GD,Harvey AS

    更新日期:2012-12-04 00:00:00

  • Crossed cerebellar and uncrossed basal ganglia and thalamic diaschisis in Alzheimer's disease.

    abstract::We detected crossed cerebellar as well as uncrossed basal ganglia and thalamic diaschisis in Alzheimer's disease by positron emission tomography (PET) using 18F-fluorodeoxyglucose. We studied a series of 26 consecutive, clinically diagnosed Alzheimer cases, including 6 proven by later autopsy, and compared them with 9...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.39.4.541

    authors: Akiyama H,Harrop R,McGeer PL,Peppard R,McGeer EG

    更新日期:1989-04-01 00:00:00

  • Depression and symptoms affect quality of life in psychogenic nonepileptic seizures.

    abstract:BACKGROUND:In patients with active epilepsy, adverse medication effects and severity of depression are correlated with health-related quality of life, but seizure frequency is not. We sought to examine if the same pattern exists in patients with psychogenic nonepileptic seizures (PNES). METHODS:We administered seizure...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0b013e3181b04c83

    authors: LaFrance WC Jr,Syc S

    更新日期:2009-08-04 00:00:00

  • Highly fatal fast-channel syndrome caused by AChR ε subunit mutation at the agonist binding site.

    abstract:OBJECTIVE:To characterize the molecular basis of a novel fast-channel congenital myasthenic syndrome. METHODS:We used the candidate gene approach to identify the pathogenic mutation in the acetylcholine receptor (AChR) ε subunit, genetically engineered the mutant AChR into HEK cells, and evaluated the level of express...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0b013e31825b5bda

    authors: Shen XM,Brengman JM,Edvardson S,Sine SM,Engel AG

    更新日期:2012-07-31 00:00:00

  • Relative utility of 1H spectroscopic imaging and hippocampal volumetry in the lateralization of mesial temporal lobe epilepsy.

    abstract:OBJECTIVES:To determine the relative utility of 1H MRSI and hippocampal volumetry for the lateralization of mesial temporal lobe epilepsy (MTLE) in patients with intractable epilepsy. BACKGROUND:MTLE is the most common partial-onset seizure disorder in patients undergoing temporal lobe epilepsy surgery. MR volumetry a...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.51.1.66

    authors: Kuzniecky R,Hugg JW,Hetherington H,Butterworth E,Bilir E,Faught E,Gilliam F

    更新日期:1998-07-01 00:00:00

  • Computed tomographic angiography for diagnosis of brain death.

    abstract::The authors report two patients with suspected brain death who required confirmatory tests other than clinical examination because of prolonged barbiturate administration for intracranial hypertension. Absence of intracranial blood flow was documented on CT angiography and confirmed by CT perfusion images. Cerebral an...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.62.4.652

    authors: Qureshi AI,Kirmani JF,Xavier AR,Siddiqui AM

    更新日期:2004-02-24 00:00:00