Aminopyridines for the treatment of neurologic disorders

Michael Strupp, Julian Teufel, Andreas Zwergal, Roman Schniepp, Kamran Khodakhah, Katharina Feil

Research output: Contribution to journalReview article

16 Citations (Scopus)

Abstract

Purpose of review: To identify the different indications for the treatment of neurologic disorders with the potassium channel blockers 4-aminopyridine (4-AP) and 3,4-diaminopyridine (3,4-DAP). Recent findings: 4-AP is an effective symptomatic treatment for downbeat nystagmus (DBN), episodic ataxia type 2 (EA2) (5-10 mg TID), and impaired gait in multiple sclerosis (MS) (10 mg BID). 3,4-DAP (5 mg/d-20 mg TID) improves symptoms in Lambert-Eaton myasthenic syndrome (LEMS) (randomized placebo-controlled trials for all 4 entities). 4-AP may also be effective in cerebellar gait ataxia of different etiologies (2 case series), upbeat nystagmus, and limb ataxia in MS (single cases). In the recommended dosages, they are well tolerated. The assumed mode of action is a blockade of mainly Kv 1.5: in DBN, this increases the excitability of Purkinje cells (PC), and in EA2, restores the precision of resting discharge of PC. In MS, 4-AP improves the conduction of action potentials in demyelinated axons, and in LEMS, 3,4-DAP facilitates the transmission at the neuromuscular endplate by prolonging the action potential duration. Summary: There is sufficient evidence that APs are indicated for the symptomatic treatment of DBN, EA2, gait ataxia due to MS and cerebellar disorders, and LEMS with a reasonable risk-benefit profile.

Original languageEnglish (US)
Pages (from-to)65-76
Number of pages12
JournalNeurology: Clinical Practice
Volume7
Issue number1
DOIs
StatePublished - Feb 1 2017

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Aminopyridines
4-Aminopyridine
Gait Ataxia
Lambert-Eaton Myasthenic Syndrome
Nervous System Diseases
Multiple Sclerosis
Purkinje Cells
Action Potentials
Potassium Channel Blockers
Cerebellar Diseases
Ataxia
Therapeutics
Gait
Axons
Randomized Controlled Trials
Placebos
3,4-diaminopyridine

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Aminopyridines for the treatment of neurologic disorders. / Strupp, Michael; Teufel, Julian; Zwergal, Andreas; Schniepp, Roman; Khodakhah, Kamran; Feil, Katharina.

In: Neurology: Clinical Practice, Vol. 7, No. 1, 01.02.2017, p. 65-76.

Research output: Contribution to journalReview article

Strupp, Michael ; Teufel, Julian ; Zwergal, Andreas ; Schniepp, Roman ; Khodakhah, Kamran ; Feil, Katharina. / Aminopyridines for the treatment of neurologic disorders. In: Neurology: Clinical Practice. 2017 ; Vol. 7, No. 1. pp. 65-76.
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