Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune disease that affects signaling at the junctions between nerve cells and muscle cells. Its symptoms include muscle weakness (particularly in the arms and legs), fatigue, and muscle pain and cramping. As LEMS progresses, difficulties with speaking and swallowing food can also arise.
There is currently no cure for LEMS, but available treatments can help to ease symptoms. A primary LEMS treatment is Firdapse, which stimulates the release of cell signaling molecules from nerve cells to stimulate muscle cells. Several other symptomatic drug treatments, such as immunosuppressive medications, are also available.
Several non-drug therapies may also help to ease symptoms associated with LEMS and improve patients’ quality of life. These include plasmapheresis, intravenous immunoglobulin (IVIG) replacement therapy, physiotherapy, occupational therapy, and speech therapy.
Plasmapheresis, or plasma exchange, is a treatment used to remove circulating autoantibodies from the liquid portion of the blood that’s known as the plasma. It is used to treat several autoimmune diseases.
During plasmapheresis, the patient’s blood is passed through a machine, which separates the blood cells from the plasma. The plasma generally contains soluble components of blood, including circulating antibodies and blood clotting factors. A patient’s blood cells are then mixed with replacement plasma that is derived from healthy donors, and transferred back to the patient.
Plasmapheresis is a relatively safe and routine technique. It is generally used when the concentration of autoantibodies is very high and causing more severe symptoms. However, its beneficial effects are transient, because the plasma cells responsible for generating the autoantibodies are not cleared through this process.
IVIG replacement therapy
IVIG replacement therapy is also used to treat several different autoimmune diseases. IVIG contains purified antibodies, or immunoglobulins, that are prepared from matched healthy donors. When these antibodies are injected directly into the bloodstream of LEMS patients, they compete with the autoantibodies present in the patient’s blood, decreasing their ability to bind to the nerve cells. In this way, this therapy eases disease symptoms.
Studies have shown that IVIG replacement therapy improves muscle strength in the limbs and respiratory system by lowering autoantibodies levels in blood plasma for a few weeks. IVIG replacement therapy can be used to supplement other treatments in LEMS patients.
Physiotherapy is recommended for LEMS patients to improve muscle strength and help slow disease progression. A physiotherapist will analyze the patient’s symptoms and abilities, and identify areas of muscle weakness. This therapist can also design an exercise plan that involves stretching, strengthening, movements and massages that best enable the patient to remain as active and independent as possible in everyday life.
Occupational therapy involves helping LEMS patients with physical difficulties to gain independence in performing daily activities at home, work, school, and social events. An occupational therapist will analyze an individual’s functional issues, and suggest strategies that includes aids and adaptations to help LEMS patients perform daily tasks more independently and safely. The occupational therapist can also educate family members and caregivers about medical equipment and other aids and strategies of help to the patient.
LEMS patients may require speech therapy when the muscles of their face, tongue, and larynx are affected. Problems in these muscles are likely to cause slurred speech, altered speech quality, and dysphagia, or difficulties in chewing and swallowing. A speech and language pathologist can identify problems in articulation, fluency, voice, receptive and expressive language disorders, and food and fluid ingestion, and can suggest strategies to overcome them. These may include augmentative and alternative communication (AAC) aids to help with speaking, facial and throat muscle exercises to improve swallowing food and fluids, and alternative diet and food textures that may be easier to swallow.
Last updated: Oct. 20, 2019
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