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Acquired neuromyotonia is an inflammatory disorder characterized by abnormal nerve impulses from the peripheral nerves that result in continuous muscle fiber activity spontaneous and continuous muscle fiber activity resulting from hyperexcitability of motor nerve terminals and autoimmune disorder, frequently associated with antibodies against voltage-gated potassium channel complex proteins. Affected individuals often experience progressive muscle stiffness and cramping especially in the hands and feet, increased sweating (hyperhidrosis), and delayed muscle relaxation. Symptoms may persist even during sleep or under general anesthesia.
Neuromyotonia (NMT) is a form of peripheral nerve hyperexcitability that causes spontaneous muscular activity resulting from repetitive motor unit action potentials of peripheral origin. NMT along with Morvan’s syndrome is the most severe type in the Peripheral Nerve Hyperexcitability spectrum. Examples of two more common and less severe syndromes in the spectrum are Cramp Fasciculation Syndrome and Benign Fasciculation Syndrome. [rx] NMT can have both hereditary and acquired (non-inherited) forms. The prevalence of NMT is unknown.[rx]
Symptoms
Acquired neuromyotonia is characterized by involuntary continuous muscle fiber activity (fasciculations, doublet, and triplet discharges on electromyography) that cause stiffness and delayed relaxation in the affected muscles. Muscle twitching with a rippling appearance (myokymia) may occur along with these symptoms. Affected individuals may, at times, be unable to coordinate voluntary muscle movement and find difficulty in walking (ataxia). Other symptoms may include staggering and reeling (titubation), stiffness, and lack of balance in response to being startled. There may be diminished spontaneous gross motor activity.
The disorder is characterized by progressive stiffness, cramping, and weakness. Muscle activity is constant, and patients describe the feeling of continuous writhing or rippling of muscles under the skin. These movements continue during sleep. Diminished reflexes are also frequently a sign of this disorder. In some instances, muscle relaxation following voluntary muscle movement is delayed (grip myotonia) in the affected muscles. For example, affected individuals may not be able to open their fists or eyes immediately after closing them tightly for a few seconds.
Affected individuals frequently have excessive sweating (hyperhidrosis), rapid heartbeats (tachycardia), and weight loss. Symptoms of pain are common.
In slightly fewer than 20% of patients, a set of symptoms, including arrhythmias, excessive salivation, memory loss, confusion, hallucinations, constipation, personality change, and sleep disorders, are found. In such cases, the disorder may be referred to as Morvan syndrome.
Approximately 20% of affected individuals have a tumor of the thymus gland (thymoma). The thymus glands are the source of several specialized cells associated with autoimmune functions. The disorder is also associated with peripheral neuropathies and autoimmune diseases including myasthenia gravis in some individuals. It has also been reported following infections and radiation therapy.
Acquired Neuromyotonia is a muscular disorder that involves hyperexcitation of motor peripheral nerves. It shows various symptoms which can range from being moderate to severe. It may also differ in frequency as well as severity. Some of those symptoms include the following:
- Cramps in muscles
- Stiffness in muscles
- Slow relaxation of muscles
- Difficulties in walking
- Over Sweating
- Fatigue
- Occurrence of jerks which are myoclonic in nature
- Intolerance to exercises
- Fasciculations
Causes
Acquired neuromyotonia is an autoimmune disease in which the immune system malfunctions so that it damages parts of one’s own body. Approximately 40% of affected individuals have antibodies to voltage-gated potassium channels (VGKCs) that affect the points at which the signals from the nerve fiber meet the muscle cell (neuromuscular junction).
Diagnosis
The diagnosis of acquired neuromyotonia is based on the presence of continuous muscle contractions (myokymia), especially in the face and hands, rhythmic tics or twitches (fasciculations), and muscle cramps. The diagnosis is confirmed by studies of the electrical signs of muscle activity (electromyography). Serum investigations include auto-immune serology and specific testing for the presence of anti-VGKC.
On examination, he had generalized myokymia and fasciculations involving all parts of his face, tongue, trunk, and all four limbs. Power, coordination, and reflexes were normal. He had impaired pain sensation in his ankles and wrists; other modalities of sensation were normal.
Electromyography (EMG) revealed widespread continuous semi-rhythmic doublets and triplets of low frequency with interspersed silent periods. There were fasciculations in the right tibialis anterior and right opponents pollicis with some polyphasic in the tibialis anterior muscle. Motor and sensory nerve conduction velocities were normal. He tested negative for anti-voltage-gated potassium channel (VGKC) antibodies, antinuclear antibodies, and anti-acetylcholine receptor antibodies. He was also negative for the anti-neuronal antibodies anti-Hu, anti-Yo, and anti-Ri. His creatinine kinase was mildly elevated at 474 U/L as was serum creatinine at 111-micromol/L. eGFR was mildly reduced at 67. A computerized tomography scan of his chest was normal.
Treatment
Acquired neuromyotonia may be treated with anticonvulsant drugs such as phenytoin or carbamazepine, which may stop the abnormal impulses and prevent the symptoms from reoccurring. Plasma exchange (plasmapheresis) and intravenous immune globulin have been effective in a few cases but no long-term, controlled, clinical studies have been carried out.
Testing for acetylcholine receptor antibodies should be done if a thymoma is suspected. The thymus gland should be surgically removed if a thymoma is present.
Acquired Neuromyotonia can be treated under the supervision of a well-specialized team of neuromuscular specialists. A proper diagnosis is followed by an adequate treatment which includes the following:
- Application of anticonvulsants: These drugs include phenytoin and carbamazepine which are important to treat conditions like spasms of muscles, stiffness, and pain associated with the muscles.
- Plasma exchange therapy: This is a treatment procedure based on a short-term relief plan from the symptoms of Acquired Neuromyotonia. The therapy disturbs the functioning of potassium channels on the motor nerves which is linked to hyper-excitability of the neuromuscular junction.
- IVIg therapy: This is also a part of the short-term relief plan in case of Acquired Neuromyotonia.
- Administration of Botox injection: It provides relief on a short-term basis.
- Application of immunosuppressants: These include the use of prednisolone for providing relief on a long-term basis.
A proper diagnosis of Acquired Neuromyotonia is followed by well-planned treatment procedures under the supervision of a specialized team of neuromuscular specialists. Plasma exchange therapy, administration of botox injection, IVIg therapy, and immunosuppressants are some of the common methods to treat the condition.
How to prevent Acquired Neuromyotonia?
Acquired Neuromyotonia is a chronic or lifelong muscular abnormality for which no cure has been discovered so far. It is caused due to the self-attacking tendency of our immune system. The immune system forms antibodies against the neuromuscular junction which bind to the potassium channels of the motor nerves, causing its hyper-excitability. There are no prevention methods for this disease, only treatment is possible to control and manage the symptoms.
Acquired Neuromyotonia, which is a chronic or lifelong muscular abnormality, has no cure been discovered so far. Hence, there are no prevention methods for this disease, only treatment is possible to control and manage the symptoms.
What to do when Acquired Neuromyotonia?
Acquired Neuromyotonia is a nongenetic autoimmune disease that can be present in an individual since birth or may be acquired later in some stages of life. When symptoms related to spontaneous muscular activities are noticed in any individual, the first important step is to get to a neuromuscular specialist.
A physical examination followed by a proper diagnosis should be done for the confirmation of Acquired Neuromyotonia. After the final diagnosis, the treatment plan is decided, followed by its execution by an experienced team of doctors.
The first important step in the case of Acquired neuromyotonia is to get to a neuromuscular specialist when symptoms related to spontaneous muscular activities are noticed in any individual. A proper treatment plan is decided based on the diagnosis, followed by its execution by an experienced team of doctors.
Can Acquired Neuromyotonia go away on its own?
Acquired Neuromyotonia is a non-curable disease. Till now, the only treatment has been possible to be done to control and manage the symptoms throughout life. Hence, this condition cannot be resolved spontaneously. It remains with the affected individual for a lifetime and the treatment procedures can only assure a good quality of life minimizing the risks of complications.
What to eat in Acquired Neuromyotonia?
In the case of Acquired Neuromyotonia, the diet has an important link with the control and management of the condition. Some of the food items which need to be consumed while suffering from Acquired Neuromyotonia include:
- low fat-containing substances
- Almonds and walnuts
- Green and leafy vegetables such as cabbage, spinach, and broccoli
- Fresh fruits
- Whole grains and cereals
- Beans, lentils, and soy milk
Summary: Diet is indirectly related to the control and management of the condition. Low fat-containing food items such as milk or cheese, almonds, fresh fruits and vegetables, whole grains, and soy milk are some better options to be preferred in such conditions.
What not to eat in Acquired Neuromyotonia?
Since diet has a close link with the control and management of Acquired Neuromyotonia, we need to know about certain food items which need to be avoided in this condition. Consumption of those may worsen the symptoms. Some of those food items include:
- High sugar beverages like sweetened tea, soft drinks, carbonated drinks, and energy drinks.
- Sauces and baked foods.
- Sugary dishes like pastries, candies, sauces, cookies, crackers, pizza, etc.
- Dairy products are high in fat content such as butter, high-fat milk, cheese, lard, etc.
- Fried and deep-fried foods, packaged and ready-to-eat food items, etc.
Diet being closely linked with the management of Acquired Neuromyotonia, it is important to know about food items to be avoided in this condition. High sugar beverages, foods containing high sugar and fat, deep-fried eatables, candies, pastries, etc. are some of them.
What are the side effects of Acquired Neuromyotonia treatments?
Treatment methods related to Acquired Neuromyotonia show certain side effects. Those side effects include:
- Stiffness in the muscles.
- Cramps in muscles of hands and legs.
- Excessive sweating.
- Slow or delayed relaxation of muscles.
The treatment of Acquired Neuromyotonia is associated with some side effects which may include over-sweating, muscle cramps stiffness, and slow muscle relaxation.
Should I go to urgent care for Acquired Neuromyotonia?
Acquired Neuromyotonia is accompanied by several symptoms including stiffness in muscles, cramps in the muscles of the hands and legs, over sweating, slow or delayed relaxation of muscles, and difficulty in walking. These symptoms are serious but incurable. However, their proper control and management are possible. For this, the affected individual must look for urgent medical care and attention.
The symptoms shown in the case of Acquired Neuromyotonia are serious and non-curable. Hence, the condition needs medical care and attention on an urgent basis.
How long does it take to recover from Acquired Neuromyotonia?
Acquired Neuromyotonia is a lifelong condition and does not have any cure till now. It can only be controlled and managed by several treatment methods including plasma therapy, IVIg therapy, and certain medications. The treatment involved in this disease is lifelong and carried out throughout the life of the affected individual.
The treatment involved in Acquired Neuromyotonia is lifelong and carried out throughout the life of the affected individual. Hence, complete recovery is not possible in such cases but patients are enabled to lead a healthy normal life.
What is the price of Acquired Neuromyotonia treatments in India?
The price of treatment of Acquired neuromyotonia is the cumulative sum of expenses related to its treatment modalities including plasma exchange therapy, anticonvulsant drugs, immunosuppressants, IVIg therapy, and botox injections. This reaches a considerably high amount which seems to be unaffordable, especially for middle-class people. Hence, it can be said the treatment of this disease is quite expensive in countries like India.
Physical Exercises for the people suffering from Acquired Neuromyotonia
Physical exercise is a key to good health. When it comes to acquired Neuromyotonia, mild to moderate forms of exercise is preferable since it is related to an abnormality in muscles. High-intensity exercises causing too much exertion to the muscles should be avoided. Some of the exercises which can be done in this condition include:
- Mild exercises: These include stretching, warm-up, and basic exercises. It can be done slowly and easily without facing too much exertion.
- Walking: This includes mainly brisk walking. It is good for improved blood circulation and the overall health of the individual.
- Regular activities: These include doing our regular work in-home or lawn or gardens. These should be mild and not exhaustive.
- Yoga and meditation: These are the most preferred form of exercise as they bring an overall physical, physiological, and psychological development to the body.
Acquired Neuromyotonia is associated with abnormality and dysfunctioning of muscles, hence vigorous exercises should not be preferred in such conditions. Mild to moderate forms of exercises can be done by the individual while taking care of his/her health status.
Which is the best medicine for Acquired Neuromyotonia?
The best medicines for the treatment of Acquired Neuromyotonia include anticonvulsant drugs. These drugs work effectively against abnormal impulses through the neuromuscular junction of the peripheral motor nerves, thereby stopping them. They also prevent the recurrence of these impulses. Some of the anticonvulsant drugs which are preferred in this condition include Phenytoin and carbamazepine.
Anticonvulsant drugs are considered to be the best medicines for treating Acquired Neuromyotonia. They work effectively against hyperactivity of motor nerves and prevent their recurrence.
Are the results of the Acquired Neuromyotonia treatment permanent?
The treatment methods for Acquired Neuromyotonia usually involve plasma exchange therapy, immunosuppressive therapy, and the application of anticonvulsants. These methods cannot cure the disease permanently but can only minimize the severity of symptoms and prevent further complications. The treatment enables the affected individual to lead a normal healthy life but cannot make him get rid of this completely.
The results of the treatment of Acquired Neuromyotonia are not permanent since it is a non-curable disease. Its treatment methods can only enable the affected individual to lead a normal healthy life but cannot make him get rid of this completely.
What are the alternatives to the Acquired Neuromyotonia treatment?
The usual methods for treatment for acquired neuromyotonia involve plasma therapy, immunosuppressive therapy, IVIg administration, and the application of anticonvulsants like carbamazepine and phenytoin. But there may be some other alternatives to these methods which include investigating receptors like acetylcholine in case of thymoma and surgical removal of thymus glands.
Investigating receptors like acetylcholine in case of thymoma and surgical removal of thymus glands are considered as alternative methods for treating Acquired neuromyotonia, while the common treatment methods include plasma therapy, immunosuppressive therapy, and anticonvulsants.
Who is eligible for the Acquired Neuromyotonia treatment?
Every individual who is suffering from Acquired Neuromyotonia is eligible for the treatment. The symptoms of the disease which include spontaneous contraction of muscles, cannot be cured by any treatment methods. Hence, the affected person must undergo the required treatment under the supervision of a specialist.
Who is not eligible for the Acquired Neuromyotonia treatment?
Acquired Neuromyotonia is a lifelong condition. Any individual suffering from it has to live with symptoms that cannot be cured but can only be controlled and managed. Hence, treatment is the only option as the condition cannot resolve spontaneously. Every affected person is eligible for the treatment.
What are the Acquired Neuromyotonia post-treatment guidelines?
The post-recovery phase is important for an individual suffering from serious diseases like Acquired Neuromyotonia. Important post-treatment guidelines which need to be followed in such conditions include:
- Proper care and attention in terms of treatment should be given.
- Dietary changes should be done according to health needs.
- Regular medical checkups are important to avoid chances of any further complications.
- Lifestyle modifications are important which include doing regular physical activities or mild exercises regularly.
Acquired Neuromyotonia is a lifelong condition and does not have any cure till now. It can only be controlled and managed by several treatment methods including plasma therapy, IVIg therapy, and certain medications such as phenytoin and carbamazepine. The treatment enables the affected individual to lead a normal healthy life but cannot make him get rid of this completely.
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