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IVIG Therapy for Neuromuscular Disorders

Distinct neuromuscular conditions like chronic inflammatory demyelinating polyneuropathy (CIDP) and Guillain-Barré Syndrome (GBS) can be managed using a combination of steroids, plasmapheresis (PP), and immunosuppressive medications. While a significant number of patients exhibit an initial positive response to these therapies, some may eventually become resistant to treatment or encounter adverse side effects that necessitate discontinuation of the interventions.

Scientists are of the opinion that intravenous immunoglobulin (IVIG) offers a more enduring and safer alternative to conventional treatments for patients diagnosed with CIDP and GBS. IVIG has demonstrated its efficacy in treating various immune-related nervous system disorders and holds promise for providing sustained benefits to individuals with these conditions.

IVIG and plasmapheresis appear to exhibit comparable effectiveness in treatment. IVIG is typically favored over plasmapheresis due to its enhanced safety, greater accessibility, and reduced invasiveness. Prolonged steroid treatment can lead to significant side effects. IVIG, while effective in 70% to 90% of cases, often requires patients who initially improve to receive periodic, long-term IVIG doses to sustain clinical stability.

If you have received a diagnosis of CIDP or GBS and your doctor prescribes IVIG therapy, you will undergo regular IVIG treatments. Typically, these infusions are administered intravenously, requiring a needle to be inserted directly into a vein at a medical facility, hospital, or infusion center. In some cases, you may have the option to arrange for these infusions to be conducted in the comfort of your own home.

IVIG is typically well-tolerated, with adverse reactions being mostly minor. The most frequently encountered side effects include headaches, nausea, chills, flushing, muscle pain, fluctuations in blood pressure, chest discomfort, and fatigue. Less common adverse reactions encompass thromboembolic events, skin responses, aseptic meningitis, renal tubular necrosis, and severe anaphylactic reactions.

For further information, please refer to the list of frequently asked questions (FAQs) regarding Neuromuscular Diseases below:

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

CIDP is a neurological condition marked by a gradual onset of weakness in the limbs, which typically progresses over several months or even years. This weakness results from damage to the protective nerve covering known as myelin. Symptoms can vary in severity, ranging from mild to severely debilitating. Fortunately, CIDP can be effectively treated with IVIG home infusion therapy.

In individuals with CIDP, the typical presentation involves a progressive deterioration in walking ability, spanning from months to years. Additionally, sensations like weakness and tingling, often originating in both hands and feet, may be encountered. During a physical examination, a reduction in reflexes, such as those seen in knee and ankle jerks, is frequently observed. A neurologist’s assessment commonly incorporates an electrical test known as a nerve conduction velocity-electromyography study. To identify potential causes of CIDP, your physician might also order blood and urine tests, including protein analysis. It’s important to note that CIDP is typically a chronic condition, often necessitating long-term treatment.

While the precise cause remains elusive, there is a prevailing belief that the immune system, which typically serves a protective role, erroneously identifies myelin as foreign and mounts an attack against it. The trigger for this process remains unclear. Notably, some patients exhibit abnormal proteins in their bloodstream, which may play a role in promoting damage. Over time, this ongoing destruction of myelin results in weakness, along with sensations of numbness and tingling in the arms and legs.

Numerous treatment options are at your disposal, including steroids, plasmapheresis, and intravenous immune globulin (IVIG). The primary objectives of treatment encompass halting further myelin damage, safeguarding nerve fiber (axon) integrity, alleviating symptoms, preventing relapses, and, if feasible, fostering an environment conducive to myelin regeneration. In the case of CIDP, IVIG has demonstrated the ability to diminish disability, forestall relapses, and even enhance overall quality of life [1].

Reference:

1. Donofrio PD, Bril V, Dalakas MC, et al. Safety and tolerability of immune globulin intravenous in chronic inflammatory demyelinating polyradiculoneuropathy. Arch Neurol. 2010 Sep;67(9):1082-8.

CIDP can be managed through various methods, including the use of steroids, plasmapheresis (PP), and immunosuppressive medications. While many patients initially respond positively to these treatments, some eventually develop resistance or encounter adverse side effects, necessitating the discontinuation of these therapies.

Researchers believe that intravenous immunoglobulin (IVIG) offers a safer and more enduring alternative to standard CIDP therapies. IVIG has demonstrated success in treating other immune-related nervous system disorders and is considered to have a longer-lasting effect.

Comparatively, IVIG and PP appear to be equally effective, with IVIG often preferred due to its superior safety profile, increased accessibility, and reduced invasiveness. In contrast, long-term steroid use can lead to significant side effects. Although IVIG proves effective in 70% to 90% of cases, it’s important to note that most patients who initially experience improvement require ongoing periodic IVIG doses to maintain their clinical stability [1].

At least five small randomized controlled studies have provided evidence of the benefits of IVIG for the majority of CIDP patients [2-7]. Among these, the ICE study stands out as the largest and most recent trial investigating IVIG’s efficacy in CIDP treatment [7]. This comprehensive study not only reaffirmed IVIG’s short-term effectiveness but also demonstrated that maintenance therapy can sustain improvements, enhance quality of life over a 12-month period, and prevent further axonal degeneration [7-10]. The ICE study strongly advocates IVIG as a first-line treatment, leading to FDA approval for a specific brand of IVIG. Furthermore, the study revealed that most responsive patients often require repeated therapy to initially improve symptoms and then maintain that improvement. The recommended dosages typically involve repeat administrations every 1 to 6 weeks. Interestingly, a significant portion of IVIG-responsive patients were able to gradually reduce their reliance on therapy over a 24-week period without experiencing relapses before the study concluded. Hence, the dosage and frequency of repeated doses can vary widely among patients and are usually determined by individual drug response and the physician’s experience with similar cases in the past.

References:

1. Van Doorn PA. Treatment of chronic inflammatory demyelinating polyneuropathy in patients. Rev Neurol (Paris). 1996;152:383-386.

2. Mendell JR, et al. Randomized controlled trial of IVIg in untreated chronic inflammatory demyelinating polyradiculoneuropathy. Neurology. 2001;56:445-449.
3. Hahn AF. Treatment of chronic inflammatory demyelinating polyneuropathy with intravenous immunoglobulin. Neurology. 1998;51(6 Suppl. 5):S16-S21.
4. Vermeulen M, et al. Intravenous immunoglobulin treatment in patients with chronic inflammatory demyelinating polyneuropathy: a double-blind, placebo-controlled study. J Neurol Neurosurg Psychiatry. 1993;56:36-39.
5. Dyck PJ, et al. A plasma exchange versus immune globulin infusion trial in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol. 1994;36:838-845.
6. van Doorn PA, et al. High-dose intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy: a double-blind, placebo-controlled, crossover study. Neurology. 1990;40:209-212.
7. Hughes RAC, et al. Intravenous immune globulin (10% caprylate-chromatography purified) for the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (ICE study): a randomized placebo-controlled trial. Lancet Neurol. 2008;7:136-144.
8. Merkies IS, et al. Health-related quality-of-life improvements in CIDP with immune globulin IV 10%: the ICE Study. Neurology. 2009;72:1337-1344.
9. Bril V, et al. Electrophysiologic correlations with clinical outcomes in CIDP. Muscle Nerve. 2010;42:492-497.
10. Latov N, et al. Timing and course of clinical response to intravenous immunoglobulin in chronic inflammatory demyelinating polyradiculoneuropathy. Arch Neurol. 2010;67:802-807.

The typical IVIG treatment regimen begins with an initial dose of 0.4 g/kg per day for 5 consecutive days, followed by a single infusion of 1.0 g/kg or less administered on a monthly or bimonthly basis. The patient’s response to treatment is evaluated after 1 to 2 months [11]. In specific cases, a weekly dosing schedule may also prove beneficial for maintenance therapy [12].

References:

11. Hahn AF, Bolton CF, Zochodne D, Feasby TE. Intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy: a double-blind, placebo-controlled, cross-over study. Brain. 1996;119(pt 4):1067-1077. 12. Dyck PJ, Litchy WJ, Kratz KM, et al. A plasma exchange versus immune globulin infusion trial in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol. 1994;36:838-845.

If you’ve received a CIDP diagnosis and your doctor prescribes IVIG therapy, you’ll undergo regular IVIG treatments. Typically, these infusions are administered intravenously, requiring a needle to be inserted directly into a vein either at a medical facility, hospital, or infusion center. In some cases, you may also have the option to arrange for these infusions to be conducted in the comfort of your own home.
IVIG is generally well-tolerated with most adverse reactions being minor. The most frequently reported side effects include headaches, nausea, chills, flushing, myalgia, changes in blood pressure (both low and high), chest discomfort, and fatigue. Less commonly, IVIG treatment may lead to infrequent adverse reactions such as thromboembolic events, skin reactions, aseptic meningitis, renal tubular necrosis, and severe anaphylactic reactions.

Dermatomyositis (DM)

Dermatomyositis is a rare muscle disorder characterized by the presence of a skin rash, and it can affect both adults and children. In adults, dermatomyositis typically manifests between the late 40s and early 60s, while in children, it commonly appears between the ages of 5 and 15. This condition is more prevalent in females than in males. The good news is that dermatomyositis can be effectively managed with IVIG home infusion therapy.
Dermatomyositis is a rare muscle disorder marked by the presence of a skin rash, and it can affect individuals of all ages, both adults and children. In adults, dermatomyositis typically develops between the late 40s and early 60s, while in children, it commonly emerges between 5 and 15 years of age. This condition is more prevalent among females than males. Fortunately, DM can be effectively managed through IVIG home infusion therapy.
While the precise origin of dermatomyositis remains elusive, it shares similarities with other autoimmune conditions where the body’s immune system targets its own tissues. Ongoing research aims to uncover additional contributing factors in its development, including the potential role of infections, the higher likelihood of underlying skin cancer (especially in the elderly), and genetic predisposition.
While there is no known cure for dermatomyositis, the central objective of treatment revolves around managing both the skin condition and muscle strength and function. Treatment alternatives encompass antimalarial medications, corticosteroids to mitigate inflammation, immunosuppressive medications, intravenous immunoglobulins (IVIG) to temper the immune response, pain relief medications, and steroid-sparing agents. Complementary to these treatments, essential measures in dermatomyositis management encompass physical therapy, speech therapy, and dietary guidance.

The primary approach to treating dermatomyositis typically involves the use of oral corticosteroids to slow the progression of the disease. Immunosuppressive medications may also be prescribed alongside corticosteroids. However, it’s important to note that these drugs often come with significant side effects, and the response to this conventional therapy is frequently less than optimal.

For individuals with dermatomyositis who do not respond well to or cannot tolerate conventional therapy, IVIG serves as an effective supplementary treatment. Clinical trials have demonstrated an overall response rate of approximately 80% after about 2 months, with the maximum response typically achieved at around 4 months. In many cases, patients continue to require ongoing IVIG therapy alongside conventional treatments, albeit at lower and more tolerable dosages.

 
It is typically advised to administer IVIG on a monthly basis, with a dosage ranging from 1 to 2 g/kg distributed over 5 days. The effects of IVIG typically endure for up to a month following each administration. IVIG is administered through an intravenous infusion, which typically takes place over a period of time, typically spanning from 4 to 6 hours.

Skin reactions to IVIG are infrequent. Among the reported cases of rashes, a blistering form of eczema is the most commonly observed [1]. This type of rash typically emerges around 8 to 10 days after IVIG exposure. It often starts as small, itchy blisters on the palms and/or soles before spreading to other areas of the body. The affected person may experience generalized redness and itching. Switching to a different type of IVIG may resolve the reaction.

In most cases, the skin lesions tend to resolve within 1 to 4 weeks. Symptoms can be managed and recovery expedited with the use of steroids.

Reference:

1. Vecchietti G, et al. Severe eczematous skin reaction after high-dose intravenous immunoglobulin infusion: report of 4 cases and review of the literature. Arch Dermatol. 2006;142(2):213-7.

Guillian-Barre Syndrome (GBS)

Guillain-Barré Syndrome (GBS) is a neurological condition marked by the progressive weakening of both the legs and arms, typically occurring over a span of days to weeks. This condition arises due to the damage of the protective sheath surrounding the nerves, known as myelin. The range and severity of symptoms can vary, ranging from mild to severely incapacitating. Fortunately, GBS can be effectively treated through intravenous immunoglobulin (IVIG) home infusion therapy.

A patient diagnosed with Guillain-Barré Syndrome (GBS) typically exhibits initial muscle weakness and unusual sensations primarily in the feet and legs, which can rapidly extend to affect the arms and upper body. A physical examination often reveals diminished reflexes, including the knee and ankle jerks.

In contrast, individuals with Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) typically experience symptoms that develop gradually over several months to years. Conversely, GBS tends to progress more swiftly, often reaching a critical stage of illness within a period of just 2 to 3 weeks or even less. In severe instances, patients may encounter difficulties with breathing and/or experience paralysis. In such critical cases, hospitalization and the use of a ventilator may become necessary.
While the precise cause remains elusive, there is a prevailing theory that suggests the immune system, typically a defender of the body, misidentifies myelin as a foreign entity and launches an attack against it. What initiates this process, however, remains unclear. It is worth noting that certain patients have been found to possess abnormal proteins in their bloodstream, which may contribute to the damage. The abrupt onset of symptoms serves as a distinguishing feature, setting it apart from other medical conditions.
Plasmapheresis or IVIG therapy is typically administered promptly upon the confirmation of a GBS diagnosis, with the aim of halting the progression of ascending paralysis and preventing any compromise to the patient’s breathing. For some individuals, a limited number of IVIG treatment courses suffice to manage their symptoms effectively and prevent substantial disability. Clinical evidence supporting the use of IVIG for treating GBS patients can be found in the following reference:

Reference:

1. Donofrio PD, Berger A, Brannagan TH 3rd, et al. Consensus statement: the use of intravenous immunoglobulin in the treatment of neuromuscular conditions report of the AANEM ad hoc committee. Muscle Nerve. 2009 Nov;40(5):890-900.

The management of GBS involves a combination of supportive and immunomodulatory therapies, with intravenous immunoglobulin (IVIG) and plasmapheresis (PP) emerging as the most widely regarded options. Several randomized, controlled studies have demonstrated that IVIG is at least as effective as plasmapheresis in GBS treatment, and in certain instances, it has shown superiority. The selection of the most appropriate treatment for a given patient is typically made by their attending physician, taking into account the patient’s specific condition and, on occasion, the hospital where the patient is receiving care.
Not all medical facilities possess the necessary equipment and staff to provide plasmapheresis (PP) therapy. Additionally, this method poses challenges when administered to elderly patients or those with limited venous access. In contrast, intravenous immunoglobulin (IVIG) offers greater convenience in terms of administration and reception and has, therefore, frequently supplanted plasmapheresis as the preferred treatment option. IVIG can be readily administered in most hospitals through a 4 to 6-hour infusion. Furthermore, unlike PP, IVIG preserves other valuable blood components, can be more cost-effective, and is associated with fewer complications.
Furthermore, IVIG has demonstrated a higher safety profile compared to PP, with a reduced incidence of complications. Extensive research has also established the effectiveness and safety of IVIG in managing GBS in pediatric patients[1,2]. Consequently, its demonstrated efficacy, safety record, and widespread availability position it as the preferred treatment for numerous GBS patients.
In certain cases where initial IVIG treatment fails to yield the desired response in select patients, there is some evidence to suggest that a second dose may offer potential benefits[3]. However, it’s important to note that this approach is not yet considered standard therapy and requires further investigation.

References:
1. Hughes, R. A., Wijdicks, E. F., Barohn, R., et al. (2003). Immunotherapy for Guillain-Barré Syndrome: Practice Parameter. Report of the Quality Standards Subcommittee, American Academy of Neurology. Neurology, 61(6), 736-740.

2. Yata, J., Nihei, K., Ohya, T., et al. (2003). High-dose Immunoglobulin Therapy for Guillain-Barré Syndrome in Japanese Children. Pediatric International, 45(5), 543-549.
3. Van Doorn, P. A., Kuitwaard, K., Walgaard, C., et al. (2010). IVIG Treatment and Prognosis in Guillain-Barré Syndrome. Journal of Clinical Immunology, 30(Suppl 1), S74-S78.
4. Research on Demyelinating Polyradiculoneuropathy. Archives of Neurology, 67, 802-807 (2010).
Typically, the initial dosage is 2 grams per kilogram of the patient’s weight, administered in five separate doses over the course of five days. Additional doses are determined based on the patient’s response. The impact of IVIG on Guillain-Barré syndrome (GBS) can become evident within a few days and may persist for several weeks to months. In cases where symptoms reoccur, some patients may require additional doses in the future.
IVIG is administered via intravenous infusion.
In general, IVIG is considered safe. Patients may encounter mild side effects, which can include headaches, neck stiffness, nausea, dizziness, vomiting, chills, fever, low blood pressure, and arrhythmia within the first 48 hours of treatment or during the initial stages. These symptoms typically resolve within a few hours or days. If they arise during treatment, they can often be mitigated by reducing the rate of IVIG infusion.
The treatment may also potentially trigger allergic reactions, such as the development of a rash on the palms of the hands. Serious complications, although rare in otherwise healthy patients, can include kidney damage and the formation of blood clots.

Multifocal Motor Neuropathy (MMN)

Multifocal Motor Neuropathy (MMN) is a rare condition where one’s immune system targets multiple motor nerves, resulting in weakness but without any loss of sensation. What makes this condition particularly puzzling is that the immune attack selectively affects the motor nerves, even though motor and sensory fibers coexist within the nerve trunks of the limbs.[1] Fortunately, MMN can be effectively managed through IVIG home infusion therapy.

Reference:

1. Katz J, Lewis R. Multifocal Motor Neuropathy. Neuropathy Action Foundation, 2013.

By its definition, MMN induces weakness, typically devoid of numbness or tingling sensations, and noteworthy absence of significant pain. The onset of MMN often displays an asymmetric progression, with an initial manifestation in the hands. Frequently, the weakness corresponds to specific nerve territories, allowing for identification of the affected nerve. For instance, an individual experiencing wrist and finger drop likely has radial nerve involvement, and when new weakness arises, it suggests the implication of a different nerve. This phenomenon of targeting multiple individual nerves is termed a multiple mononeuropathy syndrome. While similar patterns may appear in other conditions, MMN stands alone in exclusively affecting motor nerve fibers. Consequently, this represents the most crucial clinical indicator for diagnosing MMN.
Individuals diagnosed with MMN may experience additional symptoms, such as muscle twitching or small, random dimples in the muscles, referred to as fasciculations by neurologists. Fasciculations result from the spontaneous activation of a motor unit, which consists of all the muscle fibers controlled by a single motor neuron. It’s worth noting that fasciculations are also a hallmark of ALS (amyotrophic lateral sclerosis). This resemblance in symptoms sometimes leads to initial misdiagnoses of ALS in many MMN patients. Both diseases exhibit lower motor neuron signs like muscle atrophy, reduced muscle tone, and absent reflexes. However, in MMN, these signs typically affect specific nerve territories, while in ALS, they impact all muscles in the limb. In contrast to MMN, ALS patients may manifest “upper motor neuron signs” (UMN) due to brain-related motor control damage. These signs encompass heightened muscle tone, increased reflexes, and abnormal reflexes such as the Babinski sign.

Reference:

1. Katz J, Lewis R. “Multifocal Motor Neuropathy.” Neuropathy Action Foundation, 2013.

This suggests the potential need for extended treatment.

The diagnosis of MMN relies on establishing that a patient exhibits a purely motor disorder impacting individual nerves. Specific criteria have been developed to distinguish this disorder from ALS (amyotrophic lateral sclerosis), the Lewis-Sumner syndrome variation of chronic inflammatory demyelinating polyneuropathy (CIDP) (which shares similarities with MMN but often presents with significant sensory loss), and “vasculitis” (a type of multiple mononeuropathy syndrome characterized by inflammatory damage to nerve blood vessels, leading to both sensory and motor symptoms).[1]

Reference:

1. Katz J, Lewis R. “Multifocal Motor Neuropathy.” Neuropathy Action Foundation, 2013.

It is now established that intravenous immunoglobulin (IVIG) provides benefits to individuals with MMN. IVIG treatment can result in improvements for most MMN patients, although the extent of response varies, ranging from minimal to substantial. It’s important to note that IVIG typically does not fully reverse all symptoms, and patients who do respond may require recurrent treatments to maintain their progress. The timing and dosage of IVIG need to be personalized, as there is no universal formula for success. Adjustments may be necessary if the treatment’s effectiveness diminishes or if symptoms worsen despite ongoing therapy.

While IVIG is not a cure for MMN, currently, no other therapies have proven effective. Corticosteroids are generally ineffective and can potentially exacerbate the disease. Although some reports suggest that cyclophosphamide may control the disease in certain patients, it carries greater side effects and risks. On the other hand, results for Rituximab are not promising. Clearly, there is a pressing need for novel therapies, and researchers worldwide are diligently working to gain a better understanding of MMN and develop more definitive treatments.[1]

Reference:

1. Katz J, Lewis R. “Multifocal Motor Neuropathy.” Neuropathy Action Foundation, 2013.

2010 Sep;67(9):1082-8.

Myasthenia Gravis (MG)

The term “myasthenia gravis” originates from Greek and Latin, signifying “serious muscle weakness.” It is widely regarded as a chronic autoimmune neuromuscular condition characterized by the weakening of voluntary muscle groups, the ones we use for physical activities. While the precise reasons behind this phenomenon remain unclear, individuals with MG have immune systems that produce antibodies against the receptor sites at the neuromuscular junction. Typically, certain muscles, such as those governing eye and eyelid movements, facial expressions, chewing, speaking, and swallowing, are affected by the disorder, though not universally. Muscles responsible for breathing, neck control, and limb movements may also be impacted. Fortunately, MG can be managed with IVIG home infusion therapy.
A patient displaying muscle weakness is a common initial presentation of MG. Nonetheless, the diagnosis of MG might experience delays since muscle weakness is a prevalent symptom. To confirm the condition, a physician conducts a thorough evaluation that includes a detailed medical history and physical examination. During this assessment, the physician looks for specific muscle impairments, particularly related to eye movements, as well as instances of weakness that emerge during physical activity and improve after rest. The diagnosis is further solidified through various tests, including blood examinations to detect acetylcholine antibody receptors and anti-MuSK antibodies, electromyography, nerve conduction studies, and potentially a Tensilon test, which evaluates the drug’s effectiveness in temporarily alleviating fatigue.
MG arises from a disruption in the transmission of nerve signals to muscles, yet frequently the specific trigger for MG in an individual remains unidentified. This condition is prevalent among individuals of all ethnic backgrounds and both genders. It tends to manifest most frequently in young adult women (under 40) and older men (over 60), although it can develop at any age. In a majority of MG patients, the thymus gland, a component of the immune system, exhibits abnormalities. Some individuals with MG may also have non-cancerous thymus gland tumors known as thymomas. However, the precise relationship between the thymus gland and MG remains incompletely understood.
The primary objective of MG treatment is to diminish or remove antibodies and hinder their binding using various medications and therapies. Although there is no known cure for MG, there exist treatments that enable many individuals with MG to lead fulfilling lives. Commonly prescribed medications for MG management encompass pyridostigmine, prednisone, mycophenolate mofetil, azathioprine, and cyclosporine. For certain patients, thymectomy, the surgical extraction of the thymus gland, may prove to be an effective treatment option. Clinical evidence supports the utilization of intravenous immunoglobulin (IVIG) infusions in MG patients, particularly during exacerbations, to prevent complications.
Please be aware that the information provided on this website is not meant to replace professional medical advice, diagnosis, or treatment. Medrx Infusion does not advocate for or endorse any particular tests, products, procedures, or other information mentioned on this website.

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