Neuromuscular Disorders

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Lambert-Eaton Myasthenic Syndrome (LEMS): Signs, Symptoms, and Treatment Options

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With an estimated 400 known cases in the U.S.1, Lambert-Eaton Myasthenic Syndrome (LEMS) is a rare, autoimmune, neurological disorder in which the body's immune system attacks its own tissues. With LEMS, the body specifically attacks the neuromuscular junction—i.e. the connections between nerves and muscles. This causes weakness in the upper legs, hips, upper arms, and shoulders. Both walking and self-care can be difficult.

More than 50% of LEMS cases happen in middle aged or older people, with symptoms often occurring prior to the diagnosis of lung cancer.2 Because cancer cells share some of the same proteins as nerve endings, it’s thought that the body attacks nerve endings in an attempt to kill cancer cells. In the other half of LEMS cases, the cause is unknown and typically occurs around age 35.

Signs and Symptoms

LEMS is characterized by weakness starting in the legs and hips, progressing to the arms and shoulders. Onset of signs and symptoms is typically gradual. Additional signs and symptoms may include:

  • Muscle aches

  • Muscle weakness that gets worse with time

  • Fatigue

  • Difficulty walking and climbing stairs

  • Difficulty lifting objects and raising arms

  • Drooping eyelids

  • Dry eyes

  • Dry mouth

  • Blurred vision

  • Difficulty swallowing

  • Dizziness upon standing

  • Constipation

  • Erectile dysfunction

It’s important to note that because of similar symptoms of muscle weakness, LEMS is often misdiagnosed as Myasthenia gravis (MG). However key differences are in the severity and type of muscle weakness. For example, eye weakness tends to be milder in LEMS patients and unlike MG, is typically not the only symptom. Severe respiratory muscle weakness found in MG is also rare for people with LEMS.

Treatment Options

Although there is no cure for LEMS, treatment can help relieve and lessen symptoms. If LEMS is connected to a cancer diagnosis, treatment is first targeted at the cancer which may then greatly improve LEMS symptoms.

Medicines to help nerve signals reach the muscles as well as immunosuppressants (like steroids) can help relieve symptoms. If symptoms are advancing and other treatments have not helped, both plasmapheresis (plasma exchange) and intravenous immunoglobulin (IVIG) are used to treat LEMS. 

Plasmapheresis redirects blood through a machine which filters out the antibodies attacking the nerves. IVIG, which is immunoglobulin given intravenously or through a vein, suppresses the inflammatory response. Derived from thousands of healthy blood plasma donations, immunoglobulin therapy can help suppress an overactive immune system by preventing it from attacking healthy cells.

Helpful Resources

The Muscular Dystrophy Association provides support, education, care, and advocacy for patients with neuromuscular diseases, like LEMS, and their families and/or caretakers. Some of these resources include: 

Community resources like access to transportation, clinical trials finder tool, and equipment assistance

How BioMatrix Can Help

Though an LEMS diagnosis can feel overwhelming for you or a loved one, it’s important to know that you are not alone. Organizations like the Muscular Dystrophy Association mentioned above can provide a wealth of information and support as well as connect you to others in the community who have experienced the same diagnosis as well as others with neuromuscular conditions. In addition, your specialty pharmacy can offer individualized support to help manage treatment.

BioMatrix helps manage the individual needs of patients requiring IVIG therapy. Knowledgeable pharmacists and care coordination staff guide each patient through the potential medication side effects and, working with the prescribing physician, help manage treatment to reduce the prevalence and severity of symptoms.

The BioMatrix clinical team includes compassionate nurses who have extensive training and experience with rare diseases, infusion therapies, and complex medical conditions. Our nurses work together with patients, caregivers, pharmacists, and prescribers to coordinate the optimal site of care, conduct nursing interventions, and provide patient education.

Learn more about how our individualized specialty pharmacy services for patients with LEMS and other neurological and neuromuscular conditions.

DISCLAIMER: THIS IS NOT MEDICAL ADVICE. All information, content, and material is for informational purposes only and is not intended to serve as a substitute for the consultation, diagnosis, and/or medical treatment of a qualified physician or healthcare provider. Please consult a physician or other health care professional for your specific health care and/or medical needs or concerns and never disregard professional medical advice or delay in seeking it because of something you have read here or on our website.

Stay informed on the latest trends in healthcare and specialty pharmacy.

Sign up for our monthly e-newsletter, BioMatrix Abstract.

By giving us your contact information and signing up to receive this content, you'll also be receiving marketing materials by email. You can unsubscribe at any time. We value your privacy. Our mailing list is private and will never be sold or shared with a third party. Review our Privacy Policy here.

References

  1. (2019). Lambert-Eaton Myasthenic Syndrome. National Organization for Rare Disorders (NORD). https://rarediseases.org/rare-diseases/lambert-eaton-myasthenic-syndrome/

  2. (2023). Jayarangaiah A, Theetha Kariyanna P. Lambert-Eaton Myasthenic Syndrome. National Library of Medicine (NIH). https://www.ncbi.nlm.nih.gov/books/NBK507891/

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Guillain-Barré syndrome (GBS): Signs, Symptoms, and Treatment Options

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With an estimated 3,000 to 6,000 people in the U.S. developing Guillain-Barré syndrome (GBS) every year1, GBS is a rare neurological disorder in which the body's immune system attacks its own nerves causing muscle weakness and sometimes complete paralysis. It’s most common between ages 30-50, though onset can happen at any age.2

Though the cause of GBS is not fully understood, two-thirds of patients report a viral or bacterial infection in the weeks prior to GBS symptoms—typically gastrointestinal or respiratory. Evidence suggests that this infection triggers the immune response which damages the myelin sheath that protects the nerves, leading to numbness and weakness. Most people recover fully from GBS and are able to walk within 6 months to two years of when symptoms began. However, some have lasting effects, and for some, the disease can be fatal.3

Signs and Symptoms

Early signs of GBS often include a rapid onset of tingling, weakness, and numbness that start in the feet and legs and spread to the upper body. Some people notice these symptoms first in their arms or face. While initial weakness may be mild, symptoms can progress rapidly over just a few days. Additional signs and symptoms may include:

  • Difficulty raising a foot or walking without assistance

  • Paralysis or the loss of ability to move one’s legs, arms, breathing muscles, and face

  • Paralysis that travels up the limbs from fingers and toes towards the torso

  • Loss of reflexes such as the knee jerk

  • Pain in the muscles

  • Blurred vision

  • Difficulty swallowing or chewing

  • Difficulty speaking

  • Shortness of breath or difficulty breathing

  • Low or high blood pressure

  • Difficulty with bladder control or bowel function

  • Rapid heart rate

It’s important to note that not all patients with GBS experience paralysis. Some with mild cases may just experience tingling and weakness throughout their body.

Treatment Options

For some with mild symptoms, no treatment is necessary. For most new cases of GBS, however, patients need to be hospitalized. To monitor breathing and other body functions, patients are typically admitted to the ICU (Intensive Care) until the condition is stabilized. Although there is no cure for GBS, immune-suppressing treatments can prevent breathing problems and relieve symptoms by reducing the inflammation caused by the immune system’s response to the disease. Both plasmapheresis (plasma exchange) and intravenous immunoglobulin (IVIG) are used to treat GBS, and IVIG suppresses the inflammatory response.

Derived from thousands of healthy blood plasma donations, immunoglobulin therapy can help suppress an overactive immune system by preventing it from attacking healthy cells. Immunoglobulin given intravenously, or through a vein, is called intravenous immunoglobulin (IVIG).

Helpful Resources

The GBS/CIDP Foundation International provides support and resources for GBS patients and their families. Some of these resources include:

  • Local community groups and chapter meetings

  • Community forums

  • GPS educational webinars and conferences

  • Rehabilitation guide for caregivers

How BioMatrix Can Help

Though a GBS diagnosis can feel overwhelming for you or a loved one, it’s important to know that you are not alone. Organizations like GBS/CIDP Foundation International mentioned above can provide a wealth of information and support as well as connect you to others in the community who have experienced the same diagnosis. In addition, your specialty pharmacy can offer individualized support to help manage treatment.

BioMatrix helps manage the individual needs of patients requiring IVIG therapy. Knowledgeable pharmacists and care coordination staff guide each patient through the potential medication side effects and, working with the prescribing physician, help manage treatment to reduce the prevalence and severity of relapses.

The BioMatrix clinical team includes compassionate nurses who have extensive training and experience with rare diseases, infusion therapies, and complex medical conditions. Our nurses work together with patients, caregivers, pharmacists, and prescribers to coordinate the optimal site of care, conduct nursing interventions, and provide patient education.

Learn more about our individualized specialty pharmacy services for patients with GPS and other neurological conditions.

Insurance Appeal Letter Sample & Template

Have you been denied insurance coverage for much needed treatment? Use this appeal letter template as a guide to help you or a loved one appeal insurance claim denials.

DISCLAIMER: THIS IS NOT MEDICAL OR LEGAL ADVICE. All information, content, and material is for informational purposes only and is not intended to serve as a substitute for the consultation, diagnosis, and/or medical treatment of a qualified physician or healthcare provider or as legal advice. Please consult a physician or other health care professional for your specific health care and/or medical needs or concerns and never disregard professional medical advice or delay in seeking it because of something you have read here or on our website.

Stay informed on the latest trends in healthcare and specialty pharmacy.

Sign up for our monthly e-newsletter, BioMatrix Abstract.

By giving us your contact information and signing up to receive this content, you'll also be receiving marketing materials by email. You can unsubscribe at any time. We value your privacy. Our mailing list is private and will never be sold or shared with a third party. Review our Privacy Policy here.

References

  1. (2023). Guillain-Barré Syndrome and Vaccines. CDC. https://www.cdc.gov/vaccinesafety/concerns/guillain-barre-syndrome.html

  2. Guillain-Barré Syndrome. Mount Sinai Today Blog. https://www.mountsinai.org/health-library/diseases-conditions/guillain-barr-syndrome

  3. (2022). Guillain-Barré Syndrome. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/guillain-barre-syndrome/symptoms-causes/syc-20362793

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Myasthenia Gravis (MG): Signs, Symptoms, Positive Self-Management, and Treatment Options

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Myasthenia gravis (MG) is a rare, autoimmune neuromuscular disease that’s characterized by weakness in the voluntary muscles (muscles you can control) such as the eyes, face, jaw, neck, arms, and legs. With MG, the immune system attacks the communication between the nerve signals and the muscles, causing weakness and loss of control of these muscles.

Though MG can occur at any age, the onset of symptoms most commonly happens in women under 40 and men over 60.1

Signs and Symptoms

Ocular weakness is typically one of the first signs of MG. This is when muscles that control eye and eyelid movement cause a partial paralysis of eye movements, double vision, and droopy eyelids. Roughly half of those who first experience these symptoms will go on to develop muscle weakness and/or fatigue in the rest of their body within two years—typically in their neck and arms first, then their legs.2 Lifting one’s arms over their head, standing up from a seated position, walking long distances, and climbing stairs may become more and more difficult. MG symptoms can appear suddenly with rapid fatigue and loss of muscle control.

Positive Self Management

The Myasthenia Gravis Foundation provides a wealth of resources for MG patients and their families including disease education, research, community events, and wellness strategies. In addition to a patient’s medical/treatment protocol, wellness strategies and positive self management can improve day-to-day quality of life for those living with MG. According to the Myasthenia Gravis Foundation, some of those strategies include:

  • Sharing facts about MG with family, friends, and coworkers as you feel comfortable

  • Learning ways to cope

  • Conserving energy

  • Staying cool

  • Accepting help

  • Potentially recovering some strength through low impact movement and exercise

  • Proper nutrition and diet modification to help with chewing and swallowing

  • Learning your rights in the workplace

You can learn more and dive deeper into these wellness strategies here.

Treatment Options

Although there is no cure for MG, treatment can help relieve and lessen symptoms. The type of treatment depends on age, disease severity, and how fast it’s progressing. Medications like cholinesterase inhibitors can improve muscle contraction and muscle strength in some people. Corticosteroids, like prednisone, can lessen the immune system attacks. Immunosuppressants can help alter the immune system. All of these medications, however, can cause serious side effects with prolonged use.3

Infusion treatments such as IVIG and other infused medications are typically used to treat worsening MG symptoms. Because medication is infused directly into the bloodstream, a healthcare professional should be present to administer the infusion and monitor the patient for side effects.

How BioMatrix Can Help

BioMatrix helps manage the individual needs of patients requiring infused medications by providing options for administration site of care, education, and support to help improve quality of life. Knowledgeable pharmacists and care coordination staff guide each patient through the potential medication side effects and, working with the prescribing physician, help manage treatment to reduce the prevalence and severity of relapses.

The BioMatrix clinical team includes compassionate nurses who have extensive training and experience with rare diseases, infusion therapies, and complex medical conditions. Our nurses work together with patients, caregivers, pharmacists, and prescribers to coordinate the optimal site of care (including home infusion), conduct nursing interventions, and provide patient education.

Learn more about our individualized specialty pharmacy services for patients with MG and other neurological disorders.

DISCLAIMER: THIS IS NOT MEDICAL ADVICE. All information, content, and material is for informational purposes only and is not intended to serve as a substitute for the consultation, diagnosis, and/or medical treatment of a qualified physician or healthcare provider. Please consult a physician or other health care professional for your specific health care and/or medical needs or concerns and never disregard professional medical advice or delay in seeking it because of something you have read here or on our website.

Stay informed on the latest trends in healthcare and specialty pharmacy.

Sign up for our monthly e-newsletter, BioMatrix Abstract.

By giving us your contact information and signing up to receive this content, you'll also be receiving marketing materials by email. You can unsubscribe at any time. We value your privacy. Our mailing list is private and will never be sold or shared with a third party. Review our Privacy Policy here.

References

  1. (2023). Myasthenia Gravis. National Institute of Neurological Disorders and Stroke. https://www.ninds.nih.gov/health-information/disorders/myasthenia-gravis

  2. Myasthenia Gravis (MG) Signs and Symptoms. Muscular Dystrophy Association. https://www.mda.org/disease/myasthenia-gravis/signs-and-symptoms

  3. (2021). Myasthenia Gravis Diagnosis & Treatment. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/myasthenia-gravis/diagnosis-treatment/drc-20352040

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Multiple Sclerosis (MS): Signs, Symptoms, Positive Self-Management, and Treatment Options

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Multiple sclerosis (MS) is a disease of the central nervous system (CNS)—i.e. the brain, spinal cord, and optic nerves. The CNS, which controls the entire body, is attacked by the body’s own immune system, damaging the protective layer, or myelin, that insulates the wire-like nerve fibers.

This nerve damage disrupts signals to and from the brain. MS is an immune-mediated disease, which is when the body’s immune system overreacts and attacks itself. Though similar, an immune-mediated disease is different from an autoimmune disease. Both involve the immune system attacking and damaging the body’s own healthy cells via proteins (or autoantigens—antigens produced by one’s own body). However with an autoimmune disease, the proteins/autoantigens which attack the cells have been identified. With an immune-mediated disease, these proteins/autoantigens have not been identified.1

Signs and Symptoms

Though everyone’s experience with MS is different, there are some common signs and symptoms to look out for. These include:3

  • Numbness or weakness in one or more limbs, typically occurring on one side of the body at a time

  • Tingling

  • Electric-shock sensations that occur with certain neck movements, especially when bending the neck forward (Lhermitte sign)

  • Lack of coordination

  • Unsteady gait (having trouble with balance) or inability to walk

  • Partial or complete loss of vision, usually in one eye at a time with pain during eye movement

  • Prolonged double vision

  • Blurry vision

  • Vertigo

  • Problems with sexual, bowel, and bladder function

  • Fatigue

  • Slurred speech

  • Cognitive problems

  • Mood disturbances

Positive Self Management

In addition to a patient’s medical/treatment protocol, positive self management can improve day-to-day quality of life for those living with MS. Patients who self-manage their condition have the skillset to accept and communicate with others their need to move at their own pace. Research has shown that those who have developed self-management skills have more confidence to better communicate their needs to others, therefore receiving improved support.4 Found to be an empowering strategy to improve health for many people living with chronic conditions, self management is a philosophy that acknowledges living with a condition like MS is an ongoing experience.

The National Multiple Sclerosis Society and Multiple Sclerosis Foundation both give resources for developing positive self management skills. 

The National Multiple Sclerosis Society has a section on their website dedicated to “Living Well with MS”. They provide guidance and resources for diet, exercise, and healthy behaviors; emotional well-being (i.e. managing stress and coping skills); spiritual well-being (i.e. building on values and beliefs that provide purpose); and cognitive health (i.e. keeping your mind engaged and challenged). Learn more about these resources here. 

The Multiple Sclerosis Foundation is helping MS patients get connected to support groups and grants. Some of their grants and programs include help with rent or utilities, homecare assistance, and transportation. They also host multiple events for the MS community to join together and learn more about managing their condition. To learn more about these resources and events, click here.

Treatment Options

For some with mild symptoms, no treatment is necessary. For others, treatment to help ease MS symptoms may include corticosteroids or plasma exchange. Although there is no cure for MS, treatment can also help slow disease progression by reducing the amount of damage and scarring to the myelin—the layer surrounding the nerves. This reduction in damage can help MS patients have fewer and less severe relapses. Treatment to slow progression may include injectable, oral, and infusion medications. 

Infusion treatments may help slow the progression of MS and lessen flare-ups for those with aggressive or advanced MS. Because medication is infused directly into the bloodstream, a healthcare professional should be present to administer the infusion and monitor the patient for side effects.

How BioMatrix Can Help

BioMatrix helps manage the individual needs of patients requiring infused medications by providing options for administration site of care, education, and support to help improve quality of life. Knowledgeable pharmacists and care coordination staff guide each patient through the potential medication side effects and, working with the prescribing physician, help manage treatment to reduce the prevalence and severity of relapses.

The BioMatrix clinical team includes compassionate nurses who have extensive training and experience with rare diseases, infusion therapies, and complex medical conditions. Our nurses work together with patients, caregivers, pharmacists, and prescribers to coordinate the optimal site of care (including home infusion), conduct nursing interventions, and provide patient education.

Learn more about how our individualized specialty pharmacy services for MS patients.

Financial Resource Guide

Living with a chronic condition can create additional healthcare costs while also impeding one’s ability to work. Our financial resource guide can help. 

DISCLAIMER: THIS IS NOT MEDICAL OR LEGAL ADVICE. All information, content, and material is for informational purposes only and is not intended to serve as a substitute for the consultation, diagnosis, and/or medical treatment of a qualified physician or healthcare provider or as legal advice. Please consult a physician or other health care professional for your specific health care and/or medical needs or concerns and never disregard professional medical advice or delay in seeking it because of something you have read here or on our website.

Stay informed on the latest trends in healthcare and specialty pharmacy.

Sign up for our monthly e-newsletter, BioMatrix Abstract.

By giving us your contact information and signing up to receive this content, you'll also be receiving marketing materials by email. You can unsubscribe at any time. We value your privacy. Our mailing list is private and will never be sold or shared with a third party. Review our Privacy Policy here.

References

  1. What is an immune-mediated disease? The National Multiple Sclerosis Society. https://www.nationalmssociety.org/What-is-MS/Definition-of-MS/Immune-mediated-disease

  2. About Multiple Sclerosis (MS). Penn Medicine. https://www.pennmedicine.org/for-patients-and-visitors/patient-information/conditions-treated-a-to-z/multiple-sclerosis-ms

  3. (2022). Multiple Sclerosis. Mayo Foundation for Medical Education and Research (MFMER). https://www.mayoclinic.org/diseases-conditions/multiple-sclerosis/symptoms-causes/syc-20350269

  4. (2010). Wazenkewitz, J., McMullen, K., Ehde, D. Self-Management: Keys to Taking Charge of Your MS. Multiple Sclerosis Foundation.

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