A randomized trial of 3,4-diaminopyridine in Lambert-Eaton myasthenic syndrome.

Abstract:

OBJECTIVES:The authors report the results of a prospective, placebo-controlled, randomized study to evaluate the effectiveness of 3,4-diaminopyridine (DAP) in patients with Lambert-Eaton myasthenic syndrome (LEMS) and to determine the acute and long-term side effects of DAP. METHODS:Twenty-six patients with LEMS completed a two-arm parallel treatment protocol in which DAP, 20 mg three times daily, or placebo was given blindly for 6 days, and a quantitative examination of muscle strength (the quantitative myasthenia gravis [QMG] score) was used as the primary measure of efficacy. After the blinded study, patients were given open-label DAP and monitored for side effects as long as there was symptomatic improvement. RESULTS:Twelve patients took DAP, and 14 took placebo. There was no difference in the age of LEMS onset, gender distribution, incidence of lung cancer, or baseline muscle strength between the patients who were randomly assigned to receive placebo and those randomly assigned to DAP. Statistical analysis using the Wilcoxon's rank sum test demonstrated that patients who received DAP had a significantly greater improvement in the QMG score and in the summated amplitude of compound muscle action potentials recorded from three sentinel limb muscles. All but one LEMS patient had significant symptomatic improvement from subsequent open-label DAP. Side effects of DAP were negligible, consisting of perioral and digital paresthesia. Laboratory measurements demonstrated no evidence of toxicity affecting liver, renal, hematologic, endocrinologic, encephalographic, or electrocardiologic function acutely or after 6 months of open-label DAP. CONCLUSIONS:This study corroborates previous studies and many years of clinical experience showing that DAP is an effective and safe treatment for LEMS.

journal_name

Neurology

journal_title

Neurology

authors

Sanders DB,Massey JM,Sanders LL,Edwards LJ

doi

10.1212/wnl.54.3.603

subject

Has Abstract

pub_date

2000-02-08 00:00:00

pages

603-7

issue

3

eissn

0028-3878

issn

1526-632X

journal_volume

54

pub_type

临床试验,杂志文章,随机对照试验
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  • How experienced community neurologists make diagnoses during clinical encounters.

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    pub_type: 杂志文章

    doi:10.1212/WNL.0b013e3182a840c7

    authors: Dhand A,Engstrom J,Dhaliwal G

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  • Safety and long-term efficacy of ventro-oral thalamotomy for focal hand dystonia: A retrospective study of 171 patients.

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    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000006818

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    更新日期:2019-01-22 00:00:00

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    doi:10.1212/wnl.48.5.1185

    authors: Regard M,Landis T

    更新日期:1997-05-01 00:00:00

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    journal_title:Neurology

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:10.1212/wnl.50.3.645

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  • Multiple sclerosis among utility workers.

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    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.52.6.1279

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    更新日期:1999-04-12 00:00:00

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    pub_type: 杂志文章

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  • Fatal familial insomnia: a second kindred with mutation of prion protein gene at codon 178.

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    更新日期:1992-03-01 00:00:00

  • Neutralizing antibodies against HTLV-I in HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP) patients and asymptomatic carriers.

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    pub_type: 杂志文章

    doi:10.1212/wnl.27.12.1125

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    更新日期:1977-12-01 00:00:00

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    doi:10.1212/wnl.57.8.1499

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    abstract::Gilles de la Tourette's syndrome (TS) is a familial disorder that is often exacerbated by stress or fatigue. Here we present a family of a TS proband that has several members with obsessive-compulsive symptoms, a bleeding disorder, and an unusual sensitivity to heat. The proband, who is affected by all of these traits...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.41.12.1984

    authors: Lombroso PJ,Mack G,Scahill L,King RA,Leckman JF

    更新日期:1991-12-01 00:00:00

  • Progression in temporal lobe epilepsy: differential atrophy in mesial temporal structures.

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    doi:10.1212/01.wnl.0000169066.46912.fa

    authors: Bernasconi N,Natsume J,Bernasconi A

    更新日期:2005-07-26 00:00:00

  • Circulating cortisol and cognitive and structural brain measures: The Framingham Heart Study.

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    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/WNL.0000000000006549

    authors: Echouffo-Tcheugui JB,Conner SC,Himali JJ,Maillard P,DeCarli CS,Beiser AS,Vasan RS,Seshadri S

    更新日期:2018-11-20 00:00:00

  • Migraine in pregnancy.

    abstract::Migraine does not increase the risk for complications of pregnancy for the mother or for the fetus: the incidences of toxemia, miscarriages, abnormal labour, congenital anomalies, and stillbirths are comparable to those of the general population. Several retrospective studies have shown a tendency for migraine to impr...

    journal_title:Neurology

    pub_type: 杂志文章,评审

    doi:

    authors: Aubé M

    更新日期:1999-01-01 00:00:00

  • Reforms in academic neurology: needs of a rapidly changing India.

    abstract::"Healthy citizens are the greatest asset any country can have."-Sir Winston Churchill. ...

    journal_title:Neurology

    pub_type: 杂志文章

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    authors: Sharma M

    更新日期:2014-01-28 00:00:00

  • Glucose levels and outcome after subarachnoid hemorrhage.

    abstract::In a cohort of 337 patients with subarachnoid hemorrhage (SAH), we investigated the relationship between blood glucose levels, baseline characteristics, and outcome by means of Student's t-test and multivariate logistic regression. The mean glucose levels on admission and from day 1 to 10 were significantly higher in ...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/01.wnl.0000090466.68866.02

    authors: Dorhout Mees SM,van Dijk GW,Algra A,Kempink DR,Rinkel GJ

    更新日期:2003-10-28 00:00:00

  • Valproic acid has no effect on pain in polyneuropathy: a randomized, controlled trial.

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    journal_title:Neurology

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:10.1212/wnl.62.2.285

    authors: Otto M,Bach FW,Jensen TS,Sindrup SH

    更新日期:2004-01-27 00:00:00

  • The long-term clinical outcome of myasthenia gravis in patients with thymoma.

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    journal_title:Neurology

    pub_type: 杂志文章,评审

    doi:10.1212/wnl.51.4.1198

    authors: Bril V,Kojic J,Dhanani A

    更新日期:1998-10-01 00:00:00

  • Differential effects of beta and gamma interferons on expression of major histocompatibility complex antigens and intercellular adhesion molecule-1 in cultured fetal human astrocytes.

    abstract::We investigated the modulatory effects of human interferon beta (IFN-beta) and gamma (IFN-gamma) on expression of class I and II major histocompatibility complex (MHC) antigens and intercellular adhesion molecule-1 (ICAM-1) in fetal human astrocytes in culture using flow cytometry and immunocytochemistry. Under the ba...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.45.2.367

    authors: Satoh J,Paty DW,Kim SU

    更新日期:1995-02-01 00:00:00

  • Incidence of seizures with phenytoin toxicity.

    abstract::Among 50 patients with phenytoin intoxication, 14 had seizures during the episode. Seizures in 9 of these 14 patients probably resulted from poor seizure control despite high phenytoin levels, but in 5 cases, attacks were attributed to phenytoin toxicity. The only factor that seemed to correlate with seizures was a se...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.35.12.1769

    authors: Stilman N,Masdeu JC

    更新日期:1985-12-01 00:00:00

  • Seronegativity for type 1 antineuronal nuclear antibodies ('anti-Hu') in subacute sensory neuronopathy patients without cancer.

    abstract::We followed 21 patients with sensory neuronopathy without evidence of cancer for up to 23 years. All were seronegative for type 1 antineuronal nuclear antibodies (ANNA-1, also called "anti-Hu"). We additionally studied 67 seropositive patients with sensory neuropathy or a related neurologic syndrome. Ninety-one percen...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.43.11.2209

    authors: Chalk CH,Lennon VA,Stevens JC,Windebank AJ

    更新日期:1993-11-01 00:00:00

  • Mutation in the caveolin-3 gene causes a peculiar form of distal myopathy.

    abstract::The authors describe a patient with sporadic distal myopathy associated with reduced caveolin-3 in muscle fibers in which the muscle atrophy was restricted to the small muscles of the hands and feet. Gene analysis disclosed a heterozygous 80 G-->A substitution in the caveolin-3 gene that was identical to that of repor...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.58.2.323

    authors: Tateyama M,Aoki M,Nishino I,Hayashi YK,Sekiguchi S,Shiga Y,Takahashi T,Onodera Y,Haginoya K,Kobayashi K,Iinuma K,Nonaka I,Arahata K,Itoyama Y

    更新日期:2002-01-22 00:00:00

  • Elevated risk of Alzheimer's disease among workers with likely electromagnetic field exposure.

    abstract::We conducted a case-control study of the possible association of occupations with likely exposure to electromagnetic fields and Alzheimer's disease (AD) with patients from the Alzheimer Disease Treatment and Diagnostic Center, Rancho Los Amigos Medical Center, Downey, CA. Patients with definite or probable AD were the...

    journal_title:Neurology

    pub_type: 杂志文章

    doi:10.1212/wnl.47.6.1477

    authors: Sobel E,Dunn M,Davanipour Z,Qian Z,Chui HC

    更新日期:1996-12-01 00:00:00

  • Deprenyl in Parkinson disease.

    abstract::(-)Deprenyl, a specific monoamine oxidase subtype B inhibitor (MAOI-B), has been reported to be a safe and valuable adjunct to conventional treatment of parkinsonism. A double-blind, clinical comparison of (-)deprenyl with placebo was undertaken in 11 parkinsonian patients; the efficacy of 10 mg daily was studied over...

    journal_title:Neurology

    pub_type: 临床试验,杂志文章

    doi:10.1212/wnl.31.1.19

    authors: Eisler T,Teräväinen H,Nelson R,Krebs H,Weise V,Lake CR,Ebert MH,Whetzel N,Murphy DL,Kopin IJ,Calne DB

    更新日期:1981-01-01 00:00:00

  • A case-control study of HIV-1-related dementia and co-infection with HHV-8.

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    pub_type: 杂志文章,多中心研究

    doi:10.1212/wnl.59.6.950

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    更新日期:2002-09-24 00:00:00

  • Neurologic complications of influenza A(H1N1)pdm09: surveillance in 6 pediatric hospitals.

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    journal_title:Neurology

    pub_type: 杂志文章,多中心研究

    doi:10.1212/WNL.0b013e31826d5ea7

    authors: Khandaker G,Zurynski Y,Buttery J,Marshall H,Richmond PC,Dale RC,Royle J,Gold M,Snelling T,Whitehead B,Jones C,Heron L,McCaskill M,Macartney K,Elliott EJ,Booy R

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  • Alterations in bcl-2 and caspase gene family protein expression in human temporal lobe epilepsy.

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    pub_type: 杂志文章

    doi:10.1212/wnl.55.2.250

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    更新日期:2000-07-25 00:00:00