Class
- Cholinergic agonist, potassium channel blocker, neuromuscular drug
3,4-Diaminopyridine
Commonly Prescribed for
(FDA approved in bold)
- Lambert-Eaton myasthenic syndrome (LEMS)
- Congenital myasthenia gravis (CMG)
- Multiple sclerosis (MS)
3,4-Diaminopyridine
How the Drug Works
- Potassium channel blocker. Reduces flow of potassium across nerve terminal membranes and increases calcium influx with prolongation of action potential. This promotes presynaptic release of acetylcholine and may improve weakness and autonomic dysfunction
3,4-Diaminopyridine
How Long Until It Works
- About 20 minutes, but maximum effect might take a few days
3,4-Diaminopyridine
If It Works
- Continue to use to reduce symptoms of LEMS or CMG at lowest required dose. In LEMS, disease-modifying treatments, such as plasma exchange, intravenous immune globulin, corticosteroids, or immunosuppressives, are useful
- Identifying malignancy such as small-cell lung cancer is essential
3,4-Diaminopyridine
If It Doesn’t Work
- LEMS – Treat with immunologic therapy. Removal of neoplasm may improve symptoms
- CMG – Establish the type. Presynaptic forms may respond to 3, 4 diaminopyridine. Acetylcholinesterase inhibitors may improve or worsen symptoms, depending on the disorder
3,4-Diaminopyridine
Best Augmenting Combos for Partial Response or Treatment-Resistance
- May be combined with pyridostigmine, which increases the available amount of acetylcholine for receptor binding and may allow reduction of dose
3,4-Diaminopyridine
Tests
- Obtain baseline CBC, electrolytes, glucose, BUN, creatinine, liver function tests. Repeat monthly for 3 months, then every 6 months while on treatment
Adverse Effects (AEs)
3,4-Diaminopyridine
How Drug Causes AEs
- Some AEs are related to acetylcholine release, others are unknown
3,4-Diaminopyridine
Notable AEs
- Paresthesias, perioral numbness, insomnia, abdominal pain
3,4-Diaminopyridine
Life-Threatening or Dangerous AEs
- Seizures, delirium – most common at doses of 100 mg or greater
3,4-Diaminopyridine
Weight Gain
- Unusual
3,4-Diaminopyridine
Sedation
- Unusual
3,4-Diaminopyridine
What to Do About AEs
- Lower dose, supplement with pyridostigmine in LEMS. For first seizure, lower dose or discontinue and evaluate for metastatic brain tumor. For recurrent seizure, discontinue