Treatment options include various immunosuppressants (including cyclophosphamide), but there has not been ample evidence in favor of any of these agents in randomized controlled trials. Intravenous immunoglobulins remain the mainstay for treating MMN. No full-text available...
Are anti-GM1 antibodies useful in the treatment of motor neuropathy?
This case emphasizes that determination of the specificity of anti-GM1 antibodies helps to determine their clinical relevance. The polyreactive antibodies in a patient with features atypical of a motor neuropathy were not, of themselves, an indication for immunosuppressive therapy.
Is multifocal motor neuropathy immune mediated?
A clinical response to immunotherapy remains a "gold standard", without which it is difficult to argue that a syndrome is immune mediated. In 1988 two patients with a multifocal motor neuropathy, motor conduction block, and serum IgM anti-GM1 antibodies were reported to improve after treatment with cyclophosphamide .
Are polyreactive antibodies an indication for immunosuppressive therapy of motor neuropathy?
The polyreactive antibodies in a patient with features atypical of a motor neuropathy were not, of themselves, an indication for immunosuppressive therapy. Bibliography Kornberg AJ, Pestronk A. Chronic motor neuropathies: Diagnosis, therapy, and pathogenesis.
Does cyclophosphamide improve multifocal motor neuropathy?
In 1988 two patients with a multifocal motor neuropathy, motor conduction block, and serum IgM anti-GM1 antibodies were reported to improve after treatment with cyclophosphamide .
How long is IVIg effective in multifocal motor neuropathy?
This decline started after 3 to 7 years (mean 4.8 years) of therapy and correlated with a reduction of distal compound muscle action potential amplitudes (p < 0.019). The effectiveness of IVIg in MMN often declines after several years possibly associated with the development of axonal degeneration.
Can multifocal motor neuropathy be reversed?
Researchers believe that conduction block in multifocal motor neuropathy is reversible. Affected individuals have autoantibodies. Antibodies are part of the immune system; they are specialized proteins that target foreign or invading organisms.
Is there a cure for MMN?
Unlike ALS, though, MMN is treatable. Your doctor may tell you to see a neurologist, a specialist who treats problems with the nervous system.
Is multifocal motor neuropathy an autoimmune neuropathy?
Multifocal motor neuropathy (MMN) is an immune-mediated chronic asymmetrical motor neuropathy [11, 12].
What is the latest treatment for neuropathy?
The most effective treatment was nortriptyline. Of the study subjects taking this medication, 25% reported their discomfort improved by at least 50%. The least effective treatment was pregabalin: only 15% of study subjects reported that much improvement. Side effects were common with all of the treatments.
Can MMN go into remission?
It is rare that MMN will go into remission. In some cases, MMN will initially respond to treatment (see “Therapy” section) but then stop responding to treatment and remain stable.
How quickly does MMN progress?
Typically, MMN is slowly progressive, resulting in asymmetrical weakness of a patient's limbs. Patients frequently develop weakness in their hand(s), resulting in dropping of objects or sometimes inability to turn a key in a lock.
How fast does MMN progress?
As a result, misdiagnosis does occur and contributes to delays in diagnosis. Unfortunately, for many patients it can take a considerable amount of time from symptom onset to receiving a diagnosis. The mean time to diagnosis is 6 years (range: <1-23 years). * If left undiagnosed and unmanaged, MMN continues to progress.
Is MMN considered a disability?
MMN can however, lead to significant disability, with loss of function in hands affecting ability to work and perform everyday tasks, and "foot drop" leading to inability to stand and walk; some patients end up using aids like canes, splints and walkers.
Does MMN make you tired?
Participants also reported that MMN impacts their life and restricts their activities. The most commonly reported symptoms included fatigue (21%), muscle cramping (20%), and weakness (53%). Many participants reported numbness, pain, and difficulty walking.
Is Motor Neuropathy painful?
Neuropathies affecting only motor nerves may cause painful cramps. This symptom is rare to absent in slowly evolving hereditary motor neuropathies, but is common in the rapidly evolving primary motor neuron diseases.
How many people have multifocal motor neuropathy?
It has been estimated that less than 1 per 100,000 people in the world have multifocal motor neuropathy (MMN). The number of people with MMN is different in different countries. Men are more likely to have MMN than women. [4]
Why is multifocal motor neuropathy considered an immune-mediated disorder?
It is considered an immune-mediated disorder (due to an abnormal immune system response), both because IVIG therapy improves symptoms, and many patients have anti-GM1 antibodies. Research to further understand the cause of MMN is underway.
What is a neuropathy NAF?
The Neuropathy Action Foundation (NAF) provides an online information about Multifocal motor neuropathy.
What is the diagnosis of MMN?
MMN is thought to be due to an abnormal immune response, but the underlying cause is not clear. Diagnosis is based on the symptoms, clinical exam, and specific laboratory testing. Guidelines for diagnosis have been published. Treatment includes intravenous immune globulin (IVIG).
What is the National Institute of Neurological Disorders and Stroke?
The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic. The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition.
Does IVIG help with MMN?
Most people with MMN have rapid improvement in weakness with IVIG, but this treatment needs to be repeated regularly. Cyclophosphamide has also been effective in treating MMN. Physical and occupational therapy may be helpful for some people with MMN. [1] [2] [3] [4]
Is multifocal motor neuropathy inherited?
We are not aware of any evidence that multifocal motor neuropathy (MMN) is inherited or of any reports of familial cases (occurring in more than one person in a family). Furthermore, to our knowledge, no specific genes known to be associated with MMN have been identified.
What is MMN?
MMN (Multifocal Motor Neuropathy) is a rare disorder in which focal areas of multiple motor nerves are attacked by one’s own immune system. Typically, MMN is slowly progressive, resulting in asymmetrical weakness of a patient’s limbs.
What causes MMN?
MMN is associated with increased levels of specific antibodies to GM1, a ganglioside or sugar-containing lipid found in peripheral nerve. Antibodies normally protect individuals from viruses and bacteria, but may under certain circumstances bind to and facilitate an immune attack on the peripheral nerve.
How is MMN diagnosed?
The diagnosis of MMN is a clinical one that depends on demonstrating that the patient:
How is MMN treated?
It is now established that intravenous immunoglobin (IVIg), a preparation of antibodies obtained from healthy volunteers, can be readily given through an arm vein and provides benefit to patients with MMN.
What is Anti-MAG Peripheral Neuropathy?
Anti-MAG peripheral neuropathy is a very rare disease, constituting perhaps 5% of CIDP-like disorders. Anti-MAG occurs when the body’s own immune system develops antibodies against a key glycoprotein (myelin-associated glycoprotein, or MAG). MAG is essential to maintaining a healthy peripheral nervous system.
What Causes Anti-MAG?
Myelin is an important part of the peripheral nervous system. It wraps around the nerve axon (the long, wire-like part of a nerve cell) much like insulation around an electrical wire.
How is Anti-MAG Diagnosed?
Detecting anti-MAG neuropathy starts with identifying the symptoms of the patient:
How is Anti-MAG Treated?
There are many therapeutic treatments that have been used for anti-MAG neuropathy. Most of these treatments are thought to reduce the levels of anti-MAG antibodies.
Living with Anti-MAG
The progression of anti-MAG is slower and less severe than CIDP, and many patients continue living relatively normal lives while managing their symptoms with simple exercises or drug therapies. Usually, only 10 percent of patients become severely disabled and wheelchair-bound.