LEMS Treatment
Goals of LEMS Therapy
There is currently no cure for Lambert-Eaton myasthenic syndrome (LEMS); however, there are treatments available that can significantly reduce the impact of LEMS on patients’ lives.11,27
In general, the goals for LEMS treatment include3,11,27,31:
- Relief of autonomic symptoms
- Restoration/maintenance of muscle function and strength
- Improved patient mobility and independence
- Improved quality of life
- Assessing for/treating associated malignancies
LEMS Treatment Categories
Neuromuscular Junction Modulation
- Increase availability of ACh (eg, amifampridine) or inhibit degradation of ACh (eg, pyridostigmine) in the neuromuscular junction31
- In a double-blind, randomized, controlled trial comparing the efficacy of amifampridine and pyridostigmine in patients with LEMS, only amifampridine consistently produced significant improvements in muscle strength and compound muscle action potential (CMAP) amplitudes32
- Clinical data suggest that cholinesterase inhibitors, such as pyridostigmine, are generally not effective as monotherapies for LEMS27,32
Immunotherapy
- Recommended for flares or when symptomatic treatment is not sufficient31
- In addition, limited clinical data suggest that IVIG therapies may provide a short-term benefit for adult patients with LEMS33
Anticancer Therapy
- Symptomatic treatment of LEMS is recommended in addition to anticancer therapy for patients with cancer-associated LEMS31
- In a case study that included 16 patients, treatment with amifampridine was continued in conjunction with antineoplastic therapy34
