Long‐term follow‐up, quality of life, and survival of patients with Lambert‐Eaton myasthenic syndrome

Abstract

ObjectiveTo study survival and to characterize long-term functional impairments and health-related quality of life (HRQOL) of patients with Lambert-Eaton myasthenic syndrome (LEMS). MethodsIn this observational study, survival of patients with LEMS, separately for nontumor (NT) and small cell lung cancer (SCLC), was compared to that of the Dutch general population and patients with SCLC. Disease course in patients with LEMS was recorded retrospectively. Several scales for functional impairments and health-related quality of life were assessed. ResultsWe included 150 patients with LEMS. Survival was similar to that of the general population in 65 patients with NT-LEMS. Tumor survival was significantly longer in 81 patients with SCLC-LEMS compared to patients with non-LEMS SCLC (overall median survival 17 vs 7.0 months, p < 0.0001). At diagnosis, 39 (62%) of 63 patients with complete follow-up data were independent for activities of daily living, improving to 85% at the 1-year follow-up. The physical HRQOL composite score (55.9) was significantly lower than in the general population (76.3, p < 0.0001) and comparable to that of patients with myasthenia gravis (60.5). The mental HRQOL composite score was 71.8 in patients with LEMS, comparable to that of the general population (77.9, p = 0.19) and patients with myasthenia gravis (70.3). ConclusionsThis study shows that patients with NT-LEMS have normal survival. Patients with SCLC-LEMS have an improved tumor survival, even after correction for tumor stage. A majority of patients with LEMS report a stable disease course and remain or become independent for selfcare after treatment. MORE ONLINE

Presynaptic Paraneoplastic Disorders of the Neuromuscular Junction: An Update

“…In patients with NT-LEMS, life expectancy appears comparable to that of the general population [112]. Overall, functional impairment tends to be more severe in patients with paraneoplastic LEMS, although a recent study showed that the health-related quality of life does not differ significantly between patients with SCLC-and NT-LEMS [112]. A follow-up study on 47 patients with LEMS showed that 88% had an improvement of muscle strength whereas a sustained clinical remission was achieved in 43%.…”

“…Given the rarity of LEMS in association with tumors other than SCLC, there are no data on the prognosis of this group of paraneoplastic patients. In patients with NT-LEMS, life expectancy appears comparable to that of the general population [112]. Overall, functional impairment tends to be more severe in patients with paraneoplastic LEMS, although a recent study showed that the health-related quality of life does not differ significantly between patients with SCLC-and NT-LEMS [112].…”

“…Independently from the tumor presence, patients with mod interfering with function, should be started on symptomatic tre choice is amifampridine (3,, which acts by blocking the p prolonging the presynaptic nerve terminal membrane depolari ing the calcium entry, and improving the ACh release (Figure 2 Survival is shorter in patients with SCLC-LEMS and depends on the disease stage at presentation. On the other hand, LEMS is associated with longer survival in patients with SCLC [109][110][111][112] with a median of 17.3 months in these patients versus 10 in those with SCLC without neurologic symptoms [111]. Given the rarity of LEMS in association with tumors other than SCLC, there are no data on the prognosis of this group of paraneoplastic patients.…”

Combination therapy: Future directions of immunotherapy in small cell lung cancer

“…Therefore, it is a promising method to restore antitumor immune responses for the treatment of SCLC through the inhibition of CTLA-4 and PD-1/PD-L1 pathways. Interestingly, some studies have reported that the presence of paraneoplastic syndromes, such as Lambert-Eaton syndrome, most commonly accompanied by SCLC, is associated with a better prognosis for SCLC [ 22 , 23 ]. Moreover, tumor tissue from SCLC patients with neurologic paraneoplastic syndromes presented with increased tumor infiltrating lymphocytes [ 24 ].…”

Accuracy of patient-reported data for an online patient registry of autoimmune myasthenia gravis and Lambert-Eaton myasthenic syndrome

“…Approximately 87% of all Dutch LEMS patients are registered in the database, when assuming a prevalence of 2.5 per million [7] . However, there are indications that the actual prevalence in the Netherlands is higher [19] . Two participants declined retrieval of medical information.…”