American Board of Internal Medicine (ABIM) Certification Practice Exam

What is the primary focus of therapy in Lambert-Eaton Myasthenic Syndrome?

1. Treating any underlying malignancy

2. Increasing acetylcholine receptor activity

3. Providing symptomatic relief

4. Administering high-dose steroids

The correct answer is: Treating any underlying malignancy

In Lambert-Eaton Myasthenic Syndrome (LEMS), the primary focus of therapy indeed involves addressing any underlying malignancy, particularly if there is a paraneoplastic syndrome associated with the condition. LEMS is commonly associated with small cell lung cancer, and treating the underlying malignancy can lead to a significant improvement in the symptoms of LEMS. Management may improve muscle strength and reduce neuromuscular transmission problems as the malignancy is controlled, leading to improved outcomes for the patient. While other treatment options such as symptomatic relief, enhancing acetylcholine receptor activity with medications like guanidine or 3,4-diaminopyridine, or corticosteroids may play a role in managing symptoms or improving neuromuscular transmission, they are supportive strategies rather than primary focuses. Establishing a diagnosis and managing any potential malignancy effectively addresses the root cause of the syndrome and can lead to a more comprehensive resolution of symptoms.