According to a story from Lambert-Eaton News, a recent large scale study using data from the Veterans Affairs has determined that the prevalence of Lambert-Eaton myasthenic syndrome (LEMS) is similar across the US and Europe. The study also confirmed that most patients are able to improve with the use of 3,4-DAP, also known as Firdapse.
About Lambert-Eaton Myasthenic Syndrome (LEMS)
Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune disease which is characterized by weakness of the limb muscles. In this autoimmune disease, the immune system develops antibodies against an ion channel that plays a vital role in neuromuscular function. Lambert-Eaton myasthenic syndrome commonly appears as the result of cancer, often small cell lung cancer. Genetic variants are often the cause when cancer is not involved. The disease presents with muscle weakness of the upper arms and legs, with the legs usually being most affected. Other symptoms include difficulty swallowing, double vision, drooping eyelids, orthostatic hypotension, ataxia, and reduced reflexes. Weakness may be temporarily relieved after exercise; hot temperatures seem to worsen symptoms. Treatment of comorbid cancer can resolve the disease; other treatments include immune system suppressors (IVIg and prednisone) and drugs that improve neuromuscular function. To learn more about Lambert-Eaton myasthenic syndrome, click here.
About The Study
The study also found that patients with lung cancer were often diagnosed with the disease more quickly than those that did not have cancer. This unfortunately results in treatment delays. The research was one of very few studies that have been conducted in an effort to determine the prevalence of Lambert-Eaton myasthenic syndrome. The researchers accessed a massive trove of data from the VA. They looked at records from the years 1999 to 2013 which included data from over 12 million patients.
Mean time to diagnosis was 10 months and mean age at diagnosis was 60 years. Throughout this massive resource of data, only 48 confirmed cases of Lambert-Eaton myasthenic syndrome were identified, which is a testament to just how rare this syndrome really is. This gives a frequency rate of about 2.6 cases per every million people. 58 percent of patients presented with small cell lung cancer at some point in their lives.
This study was originally published in the journal Muscle & Nerve.