Our CenterThe USC Neuromuscular Center at the Leslie P. Weiner Neurological Care and Research Center at Keck Medicine of USC in Los Angeles provides an integrated approach to clinical diagnosis, evaluation, neurophysiologic testing and treatment options for patients with various neuromuscular disorders.
Physicians provide second opinion consultation, diagnostic evaluation and individualized treatment for patients with various neuromuscular disorders, including:
- Immune-mediated neuropathies
- Hereditary neuropathies such as hereditary transthyretin amyloidosis (hATTR)
- Myasthenia gravis
- Motor neuron diseases such as amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA)
- Diabetic neuropathies
- Neuropathic pain
Given the diversity of neuromuscular disorders, correct diagnosis, identification of the cause of the disorder and prompt appropriate therapy for treatable conditions, including counseling, are crucial to correcting the courses of these ailments. Advances in neuromuscular disorders have seen significant growth, with new technology in genetic and molecular diagnosis.
Specialized services include:
- A state-of-the-art neurophysiology electromyography laboratory for diagnostic testing
- An infusion center, used for infusion therapy such as intravenous immunoglobulin therapy (IVIG) or other immunomodulatory therapy for immune-mediated disorders
- A multidisciplinary ALS clinic, recognized as a Certified Treatment Center of Excellence by the ALS Association
- An amyloidosis program offering advanced treatments and leading-edge care
- Collaboration with neuropathology specialists for specialized evaluation of muscle and nerve biopsies using histochemical and advanced immunocytochemical techniques
- Clinical research trials that allow patients access to the most advanced, experimental medical technology
In many cases, patients are referred to the neuromuscular center for second opinions and advanced evaluations. Evaluations, diagnoses and treatments are conducted in a multidisciplinary fashion and in close collaboration with:
- Pain specialists
- Pulmonary medicine specialists
- Foregut physicians
- Spine specialists
- Palliative care specialists
- Physical therapists
- Speech therapists
- Occupational therapists
- Specialty infusion nurses
- Social workers
All evaluations, diagnostics and treatments are conducted on an outpatient basis.
Because the incidence of neuromuscular disorders is rising and the causes of these disorders are diverse, it is crucial that a patient receive a thorough evaluation and correct diagnosis of the underlying disease. Only once a diagnosis is made and the cause determined can the appropriate course of treatment be determined. The USC Neuromuscular Center combines its experience in clinical trials that study the latest advances in these diseases and its multidisciplinary approach to successfully treat the range of these diseases.
These approaches include:
- Comprehensive neurophysiologic electrodiagnostic testing and neuromuscular diagnostic studies, including genetic testing and muscle, nerve and skin biopsy
- Treatment of immune-mediated neuromuscular disorders, such as chronic inflammatory demyelinating neuropathy (CIDP), multifocal motor neuropathy (MMN), myasthenia gravis
- Appropriate selection of treatment, early institution of therapy and periodic close follow-up of patients, assessing treatment response and changes needed
- An ALS clinic that provides multidisciplinary care with a team of specialists dedicated to delivering the highest quality of care during a single visit
- An amyloidosis program that brings together an interdisciplinary team of experts to deliver exceptional care
- Evaluation and treatment of neuropathic pain and its associated conditions, such as diabetic neuropathies, postherpetic neuralgia and other conditions and causes of painful neuropathy
The physicians and staff in the USC Neuromuscular Center at Keck Medicine of USC in Los Angeles diagnose and treat a wide variety of diseases that affect the peripheral nervous system, which includes the motor neuron, peripheral nerves, neuromuscular junction, and the muscle.
Examples of disorders of the peripheral nerves or peripheral neuropathy:
Peripheral neuropathy affects 20 million individuals in the United States. There are various causes. It is very important to do a comprehensive work-up for diagnosis and treatment. When the cause is identified and treated early, the condition can improve and be reversed. Neuropathy can cause significant pain, which should be and can be treated effectively to improve quality of life.
Diabetic neuropathy is a disorder of the nervous system that results as a complication of either type 1 or type 2 diabetes. While years of high blood sugar can damage any nerve in the body, most diabetic neuropathies occur in the legs and feet, causing pain and limiting movement.
Immune-mediated neuropathies are disorders that destroy the protective sheath of myelin that surrounds peripheral nerve axons. These disorders include Guillain-Barre syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP), multifocal motor neuropathy (MMN), and neuropathies associated with other systemic or autoimmune conditions. These disorders are treatable and can be reversible, if treated early.
Chronic inflammatory demyelinating polyneuropathy (CIDP) usually presents with slowly progressive weakness and sensory symptoms in the legs and arms, but one also can experience variations in progression of the disease and area affected. Immune therapy is highly effective.
Multifocal motor neuropathy (MMN) is marked by painless, slowly progressive asymmetric weakness in the distal part of the extremities (hands and feet). It usually affects the arms but can also involve the legs. If untreated, MMM can result in muscle atrophy and progressive weakness. It responds well to IVIG therapy which, when started early, can result in significant improvement.
Hereditary transthyretin amyloidosis (hATTR) is a rare, progressive, debilitating systemic disorder that manifests in several organs. hTTR is frequently diagnosed late because it is difficult to recognize and often resembles other more common conditions. Early recognition of hATTR is crucial so that patients can begin receiving disease-modifying therapy, such as gene silencers, which is essential to improving outcomes.
Examples of disorders of the motor neuron:
Motor neuron diseases are disorders that affect the motor neuron. These disorders can have varied onset ages, and different progressions and prognoses. In addition to weakness, some of the complaints include cramps, twitching, stiffness and muscle atrophy. These diseases include:
Amyotrophic lateral sclerosis (ALS) (also known as “Lou Gehrig’s disease”) is a degenerative disease of the motor neurons. It is characterized by progressive muscle weakness, stiffness and atrophy that can affect muscles of the arms and legs as well as muscles of speech, swallowing, emotional control and breathing. ALS can present and progress differently in different people.
Spinal muscular atrophy (SMA) is a rare neuromuscular disorder that causes loss of motor neurons and progressive muscle wasting, resulting in progressive weakness, breathing and swallowing difficulties. It is usually diagnosed in infancy or early childhood but may also appear later in life. Treatments are available to improve functional outcomes and slow the progression of disease.
Examples of disorders of the neuromuscular junction:
Neuromuscular junction diseases are disorders of the neuromuscular junction. There are many types of these disorders and most of those are autoimmune disorders, which are treatable.
Examples of neuromuscular junction disorders include: Myasthenia Gravis (MG), Lambert Eaton Myasthenic Syndrome (LEMS).
Autoimmune myasthenia gravis is the most common type of neuromuscular junction disorder, producing fluctuating weakness affecting the eye muscles, muscles of chewing, swallowing, breathing and the arm and leg muscles. Weakness is usually most pronounced during activity and decreases in severity during rest. It is a treatable disease, but when severe and generalized, it can be quite disabling.
Examples of disorders of the muscle or myopathy:
Myopathies can be genetic (inherited) or acquired. The latter include autoimmune causes, such as polymyositis or dermatomyositis. Other inflammatory myopathies include inclusion body myositis, known also as IBM. Diagnostic and genetic studies are very useful to identify the type of myopathy. Certain myopathies can affect other organs such as the heart.
The neuromuscular program at Keck Medical Center of USC in Los Angeles diagnoses and treats patients with a wide range of neuromuscular disorders. Since these disorders are very complex and often mimic one another, it is essential to have an accurate diagnosis. Our team of physicians and other specialists are experienced in detecting, diagnosing and treating all neuromuscular disorders. Nearly all diagnoses and treatments are performed on an outpatient basis. Patients will first receive a thorough medical and neurophysiological evaluation. Specific tests will depend on what type of disorder is suspected.
Comprehensive Diagnostic Services
Nerve conduction studies and electromyography (NCS/EMG): NCS is a test to assess the function and integrity of peripheral nerves. EMG assesses the health and function of muscles. NCS is very useful in detecting neuropathies and differentiating among the different pathologies. EMG is an important test that can help further in localization and in identifying muscle conditions and their severity.
Other diagnostic studies include: specialized immunologic blood studies, genetic blood studies, biopsies of muscle, nerve and skin. Depending on the patient’s diagnosis, these may be used to a different extent. But any one of these tools help further identify the patient’s neuromuscular disease, and play important roles in determining and implementing appropriate treatment.
Treatment experiences will vary greatly, depending on the nature and degree of the diagnosed disease. For some disorders, treatments will be more symptomatic, and involve pain management, physical therapy or social/psychological counseling.
Immune therapy is recommended for patients with immune-mediated neuro muscular disorders. These include infusions such as intravenous immunoglobulin therapy (IVIG). Treatments will consist of visits to the infusion center. While there, patients are closely monitored and supervised throughout their infusion of immunoglobulin. A nurse dedicated to the infusion center provides direct and constant supervision.
Treatments for neuropathy will vary according to the cause of the neuropathy. In many cases, our physicians will work with the USC Pain Clinic to develop a treatment program that includes pain relief.
Treatments for motor neuron diseases will depend on the type of disorder and the stage at which the disease was diagnosed. Treatments are designed for each patient, in close collaboration with USC subspecialists in pulmonary medicine and rehabilitation services including physical therapy, occupational therapy, speech therapy and social services.
ResourcesThere is a wide range of resources for patients with neuromuscular disorders. Most are organized according to a specific disorder, e.g. amyotrophic lateral sclerosis (ALS or Lou Gehrig’s disease), diabetic-based neuropathy or myasthenia gravis. The Neuromuscular Program at Keck Medicine of USC also conducts clinical trials in which patients can participate.
As the nation’s primary organization for patients with amyotrophic lateral sclerosis (ALS or Lou Gehrig’s disease), the association provides information, connections to patient support groups, and patient advocacy.
This organization supports patients with Guillain-Barre Syndrome or chronic inflammatory demyelinating polyneuropathy.
Myasthenia Gravis Foundation of California
This organization is dedicated to education, patient support and services for people with myasthenia gravis.
Neuropathy Action Foundation
This organization provides education and support for patients with neuropathy.
This group serves physicians treating and patients who have peripheral neuropathy.
Patients have the option of taking part in clinical trials being conducted at Keck Medical Center of USC and elsewhere. While clinical trial participation does not ensure that an effective treatment will be received, participation does help provide invaluable information for future treatments and possibly a cure in the future.
Randomized, Multicenter, Double-Blind, Placebo-Controlled, Parallel-Group Phase III to Investigate the Efficacy, Safety, and Tolerability of 2 Different Doses of IGPRO20 (Subcutaneous Immunoglobulin) for the Treatment of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) – The PATH Study. CSL Behring
A Randomized, Placebo-Controlled, Double-Blind Study to Evaluate the Efficacy, Safety, Tolerability, and Pharmacodynamics of Belimumab in Subjects with Generalized Myasthenia Gravis (MG), GlaxoSmithKline
A Randomized, Double-Blind, Placebo-Controlled, Multi-Center Study to Evaluate the Safety and Efficacy of Eculizumab in Subjects with Refractory Generalized Myasthenia Gravis (gMG), Alexion Pharmaceuticals
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no complaints at all.Review for SOMA SAHAI SRIVASTAVA MD
Division Chief, Neuromuscular Medicine
Keck Hospital of USC
USC Healthcare Center 2
USC Norris Comprehensive Cancer Center
Practicing LocationsKeck Hospital of USC
USC Norris Comprehensive Cancer Center
Additional Faculty Physicians
Frank P. Lin, MD