3,4-Diaminopyridine (3,4-DAP) (2024)

Why have I been prescribed this medication?

3,4-Diaminopyridine (3,4-DAP) is used in the treatment of Lambert-Eaton Myasthenic Syndrome (LEMS) and some congenital myasthenic syndromes (CMS). It is used to improve muscle strength.

How it works

Aminopyridines, such as 3,4-DAP, are types of medication that are able to block voltage-gated potassium channels. Potassium channels act as tunnels within our cells and allow the passage of potassium in and out. Potassium, and other elements in cells such as calcium, are responsible for a number of different roles, but in the case of myasthenia, the most important one is their ability to help nerves to function and muscles to contract.

In myasthenia, and specifically in the case of LEMS, the calcium channels in nerve cells are blocked, preventing passage of calcium, and reducing the amount of acetylcholine released in the space between nerve and muscle cells (a site called the neuromuscular junction). Without acetylcholine, muscles do not receive the message to contract and this can cause weakness at the joints. What 3,4-DAP does is to essentially extend the interval during which the calcium channel is open, and it does this by blocking the potassium channel.

How it is delivered

3,4-DAP is administered as a tablet. It can also be prepared as a suspension if you have difficulty swallowing if your dose is less than 5 mg.

When you have it and how long you will be taking it

3,4-DAP should be taken as and when your muscles will need it most. Your doctor may provide detailed instructions for when you should take it, and this should be followed. The length of time you will be taking 3,4-DAP for depends on your needs. Do not stop taking your medication unless your consultant neurologist tells you to, even if you feel unwell.

Side effects

All medicines can cause unwanted side effects, which usually improve as your body adjusts to the new medicine. 3,4-DAP is usually very well tolerated. In the early weeks of treatment, 3,4-DAP may cause tingling around the mouth and in the fingers and toes, but this usually disappears about an hour after taking the drug. Other side effects can include:

  • Diarrhoea
  • Blurred vision
  • Fatigue

Please check the patient information sheet supplied with your medication for a more detailed list of possible side effects. If any side effects disclosed concern you, seek reassurance from your doctor. If these symptoms persist, please contact your doctor. If you are worried about possible side effects and would like to talk to someone, you can also contact your doctor or the team at myaware who can help reassure you.

Known UK suppliers

Ascot Laboratories

FAQ (what else do I need to know?)

Before taking 3,4-DAP it is important to tell your doctor if you are pregnant or plan to become pregnant, as side effects for unborn babies is as yet unknown. This also applies if you are breastfeeding or planning to breastfeed.

Caution needs to be taken for people who have epilepsy and are being treated with 3,4-DAP. In addition, certain heart conditions can be affected by 3,4-DAP, and asthma can be exacerbated. If this concerns you, please discuss with your doctor the best route of treatment.

Treatments

3,4-Diaminopyridine (3,4-DAP) (2024)

FAQs

What is 3,4-diaminopyridine used for? ›

3,4-Diaminopyridine (3,4-DAP) is used in the treatment of Lambert-Eaton Myasthenic Syndrome (LEMS) and some congenital myasthenic syndromes (CMS). It is used to improve muscle strength.

What is 3,4-diaminopyridine drugbank? ›

Amifampridine, or 3,4-diaminopyridine (3,4-DAP), is a quaternary ammonium compound that blocks presynaptic potassium channels, and subsequently prolongs the action potential and increases presynaptic calcium concentrations 1.

Is amifampridine the same as 3,4-DAP? ›

First-line medical therapy involves the use of 3,4-diaminopyridine/amifampridine. Both names refer to the same drug. There are currently 2 slightly different preparations of this drug made by different manufacturers, both of which have received FDA approval for the treatment of LEMS.

What is the drug amifampridine used for? ›

Amifampridine is used to treat Lambert-Eaton myasthenic syndrome (LEMS), a rare autoimmune disease, wherein the immune system attacks the connection between the nerve and muscle (neuromuscular junction) and blocks the ability of the nerve cells to send signals to the muscle cells, leading to muscle weakness of the arms ...

What prescription is DAP? ›

DAPSONE (DAP sone) is an antiinfective. It is used to treat a skin problem called Dermatitis herpetiformis. This medicine is also used to treat leprosy. This medicine may be used for other purposes; ask your health care provider or pharmacist if you have questions.

How does 3,4-diaminopyridine affect the action potential? ›

Specifically, 4-aminopryridine (4-AP) and 3,4-diaminopyridine [3,4-DAP], which block (juxta-)paranodal F and I K+ currents in the peripheral nerve [see above], improve conduction in demyelinated axons by prolonging the duration of the action potential and thereby improving the safety factor of impulse transmission ( ...

What are the effects of 3,4-diaminopyridine on myasthenia gravis preliminary results of an open label study? ›

3,4-DAP had a significant effect on the QMG score (p = 0.0251), on repetitive nerve stimulation (p = 0.0251), and on the forced vital capacity (p = 0.03), thus indicating that it may reduce the level of disability and the decremental muscle response.

How much does amifampridine cost? ›

Amifampridine phosphate costs between $11,990 and $63,948 per patient each year.

What is the use of Diaminopyrimidine drugs? ›

Diaminopyrimidine oxide is a compound similar to minoxidil which is used to aid in hair growth.

How does 3,4-diaminopyridine and pyridostigmine treat Lambert-Eaton syndrome? ›

3,4-Diaminopyridine and pyridostigmine are widely used to treat Lambert–Eaton myasthenic syndrome (LEMS), either alone or in combination. 3,4-Diaminopyridine enhances the release of acetylcholine at the neuromuscular synapse, and pyridostigmine inhibits the degradation of this neurotransmitter.

What is the difference between myasthenia gravis and Lambert-Eaton? ›

Unlike MG, which affects the eye muscle first, LEMS causes proximal muscle weakness in the lower limbs (upper legs and hips), which leads to difficulty walking. With time, the muscle weakness temporarily improves after repetitive muscle activity, which is a unique feature of this condition.

Is amifampridine the same as fampridine? ›

Fampridine is twice as toxic and more convulsive than amifampridine in laboratory animals after intravenous administration. In vitro studies demonstrated that amifampridine is 7–10 times more potent than fampridine in enhancing the quantal release of acetylcholine.

What is the brand name for 3,4-diaminopyridine? ›

FIRDAPSE is the approved brand name for amifampridine phosphate, which is the active ingredient in FIRDAPSE. Amifampridine phosphate quickly becomes amifampridine freebase in the body and may also be referred to as 3,4-diaminopyridine or 3,4-DAP.

What is LEMS autoimmune disease? ›

Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune disorder of the neuromuscular junction. It is a miscommunication between the nerve cell and the muscles that lead to the gradual onset of muscle weakness.

What is the new drug for Lambert-Eaton syndrome? ›

Amifampridine to treat Lambert-Eaton myasthenic syndrome. Drugs Today (Barc) 2020;56:623–641. Oh SJ, Shcherbakova N, Kostera-Pruszczyk A, Alsharabati M, Dimachkie M, Blanco JM, et al. Amifampridine phosphate (Firdapse®) is effective and safe in a phase 3 clinical trial in LEMS.

What medication is used for Lambert-Eaton syndrome? ›

Treatments for LEMS

medicine to help nerve signals reach the muscles – commonly used medicines include 3,4-diaminopyridine and pyridostigmine. medicine to reduce the activity of the immune system (immunosuppressants) – commonly used medicines include steroid tablets (such as prednisolone), azathioprine and methotrexate.

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