3,4-Diaminopyridine in Childhood Myasthenia: Double-Blind, Placebo-Controlled Trial

Anlar, Banu; Varlı, Kubilay; Ozdirim, E; Ertan, Mevlüt

doi:10.1177/088307389601100608

Cited by 26 publications

(12 citation statements)

References 6 publications

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“…23 A favorable response to 3,4-DAP combined with pyridostigmine in unselected CMS patients was previously reported, 24 but another clinical trial of CMS patients with predominantly ocular symptoms found little response to 3,4-DAP. 25 In our experience, 3,4-DAP increases endurance and reduces lid ptosis, but external ocular muscles respond less than limb muscles and in some patients the medication becomes less effective with continued use. Patients with low-expressor or null mutations in each allele of the AChR ⑀ subunit can partially substitute the fetal ␥ for the ⑀ subunit.…”

Section: Management Of Different Types Of Cmss Endplate Achr Deficienmentioning

confidence: 81%

The Therapy of Congenital Myasthenic Syndromes

Engel

2007

Neurotherapeutics

104

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Section: Management Of Different Types Of Cmss Endplate Achr Deficienmentioning

confidence: 81%

The Therapy of Congenital Myasthenic Syndromes

Engel

2007

Neurotherapeutics

104

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“…1-6 was combined with the terms: 7. treatment, 8. medication, 9. therapy, 10. controlled clinical trial, 11. randomized controlled trial, 12. clinical trial, 13. multicenter study, 14. meta-analysis, 15. cross-over studies, 16. thymectomy and 17. immunosuppression.…”

Section: Methodsmentioning

confidence: 99%

“…In a double‐blind, placebo‐controlled trial, the drug seemed effective in congenital (hereditary and non‐immune) myasthenia patients. Juvenile patients with MG did not respond [16] (class III evidence). The drug is not recommended in autoimmune MG, although it may prove useful in some forms of congenital myasthenia (level C recommendation).…”

Section: Myasthenia Gravis (Mg)mentioning

confidence: 99%

Guidelines for treatment of autoimmune neuromuscular transmission disorders

Skeie

Apostolski

Evoli

et al. 2010

Euro J of Neurology

406

258

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Background: Important progress has been made in our understanding of the autoimmune neuromuscular transmission (NMT) disorders; myasthenia gravis (MG), Lambert–Eaton myasthenic syndrome (LEMS) and neuromyotonia (Isaacs’ syndrome). Methods: To prepare consensus guidelines for the treatment of the autoimmune NMT disorders, references retrieved from MEDLINE, EMBASE and the Cochrane Library were considered and statements prepared and agreed on by disease experts. Conclusions: Anticholinesterase drugs should be given first in the management of MG, but with some caution in patients with MuSK antibodies (good practice point). Plasma exchange is recommended in severe cases to induce remission and in preparation for surgery (recommendation level B). IvIg and plasma exchange are effective for the treatment of MG exacerbations (recommendation level A). For patients with non‐thymomatous MG, thymectomy is recommended as an option to increase the probability of remission or improvement (recommendation level B). Once thymoma is diagnosed, thymectomy is indicated irrespective of MG severity (recommendation level A). Oral corticosteroids are first choice drugs when immunosuppressive drugs are necessary (good practice point). When long‐term immunosuppression is necessary, azathioprine is recommended to allow tapering the steroids to the lowest possible dose whilst maintaining azathioprine (recommendation level A). 3,4‐Diaminopyridine is recommended as symptomatic treatment and IvIG has a positive short‐term effect in LEMS (good practice point). Neuromyotonia patients should be treated with an antiepileptic drug that reduces peripheral nerve hyperexcitability (good practice point). For paraneoplastic LEMS and neuromyotonia optimal treatment of the underlying tumour is essential (good practice point). Immunosuppressive treatment of LEMS and neuromyotonia should be similar to MG (good practice point).

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“…In a double‐blind, placebo‐controlled trial the drug seemed effective in congenital (hereditary and non‐immune) myasthenia patients. Juvenile MG patients did not respond [13] (class III evidence). The drug is not recommended in autoimmune MG although it may prove useful in some forms of congenital myasthenia (level C recommendation).…”

Section: Myasthenia Gravismentioning

confidence: 99%

Guidelines for the treatment of autoimmune neuromuscular transmission disorders

Skeie

Apostolski

Evoli

et al. 2006

Euro J of Neurology

170

184

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Important progress has been made in our understanding of the cellular and molecular processes underlying the autoimmune neuromuscular transmission (NMT) disorders; myasthenia gravis (MG), Lambert-Eaton myasthenic syndrome (LEMS) and neuromyotonia (peripheral nerve hyperexcitability; Isaacs syndrome). To prepare consensus guidelines for the treatment of the autoimmune NMT disorders. References retrieved from MEDLINE, EMBASE and the Cochrane Library were considered and statements prepared and agreed on by disease experts and a patient representative. The proposed practical treatment guidelines are agreed upon by the Task Force: (i) Anticholinesterase drugs should be the first drug to be given in the management of MG (good practice point).(ii) Plasma exchange is recommended as a short-term treatment in MG, especially in severe cases to induce remission and in preparation for surgery (level B recommendation). Background and objectives

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3,4-Diaminopyridine in Childhood Myasthenia: Double-Blind, Placebo-Controlled Trial

Cited by 26 publications

References 6 publications

The Therapy of Congenital Myasthenic Syndromes

The Therapy of Congenital Myasthenic Syndromes

Guidelines for treatment of autoimmune neuromuscular transmission disorders

Guidelines for the treatment of autoimmune neuromuscular transmission disorders

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