Motor Nerve Disease

Motor nerve disease is a group of nerve disorders that cause loss of muscle strength step by step. Often, the designation of motor neuron disease refers to amyotrophic lateral sclerosis (ALS) or Lou Gehrig's disease. This disease is the most common and most well-known disease in the motor nerve disease group, especially after the ice bucket challenge activity aimed at increasing people's awareness about ALS becoming viral in 2014. But ALS is just one of the diseases that are included in the neurological disease group motor, among other conditions which include:

•     Spinal muscular atrophy (Spinal Muscular Atrophy / SMA).
•     Primary lateral sclerosis (PLS).
•     Hereditary spastic paraparesis (HSP).
•     Kennedy disease or X-linked spinobulbar muscular atrophy / SBMA.
•     Postpolio Syndrome.
•     Progressive bulbar palsy (PBP).
•     Brachial plexus injury.
•     Benign focal amyotrophy.
•     Progressive muscle atrophy (PMA).

 Motor nerves are divided into two types, namely motor nerves (superior motor neurons / UMN) and lower motor neurons (LMN). UMN originates from the brain and is responsible for sending signals from the brain to the brain stem or spinal cord. While LMN originates from the brain stem and spinal cord, LMN functions to forward signals to all muscles. The signals sent are used to regulate the movement of body muscles, such as walking, grasping, breathing, talking or swallowing. When motor nerve function is disrupted, patients will have difficulty doing this movement.
Symptoms of motor nerve disease

The symptoms caused depend on the type of motor nerve. When abnormalities occur in the upper motor nerve (UMN), muscles become stiff and tense, movements become slow and are carried out with more force than usual, the body's reflexes become hyperactive. When abnormalities occur in the lower motor nerve (LMN), it causes symptoms such as muscle weakness and contraction (atrophy), and there are uncontrolled muscle contractions.


The causes and symptoms of motor nerve disease, seen from the disease itself, include:

• Amyotrophic lateral sclerosis (ALS). ALS is the most common type of motor nerve disease and usually affects men aged 40 to 60 years. This disease causes damage to the motor nervous system, both UMN and LMN. Symptoms that first appear in the muscles of the arms and hands, legs and muscles to swallow. People with ALS will have difficulty swallowing, chewing, and talking. In addition, sufferers will have difficulty moving their arms, hands, legs and keep their bodies straight. Other problems that occur are muscle cramps, muscle contractions and muscle stiffness. For diaphragm muscles, people with ALS will have difficulty breathing, which is a major cause of death in people with ALS. The time needed to develop ALS while waiting for respiratory failure is between 3 and 10 years, but it can also be longer. Until now, the cause is unknown, but is thought to be caused by various internal factors, such as genetic disorders, abnormalities in immunological processes, and external factors such as the environment.

• Spinal muscular atrophy (SMA). This is a hereditary disease that attacks LMN. SMA is caused by lesions of the SMN1 gene that produce the SMN protein, essential for the survival of motor nerve cells. Lack of SMN protein will cause atrophy and weak muscle strength in the body, legs, arms, hands and feet.

• Primary lateral sclerosis (PLS). This is a motor nerve disease that attacks UMN through the muscles of the arms, legs, and face. This disease is characterized by speech disorders, difficulty walking and labor obstruction that requires coordination of hand muscles. Unlike ALS, PLS is not fatal, but can disrupt quality of life. PLS generally attacks men aged between 40 and 60 years. PLS is caused by nerve cell disorders in the brain, but the cause of this disorder is unknown.

• Hereditary spastic paraparesis (HSP). HSP or Strumpell-Lorrain disease is a congenital condition caused by genetic disorders that result in weakness and lower body stiffness. In addition, reflexes in the ankle become excessive. This happens slowly and can be serious.

• Parallel bulbar paralysis (PBP). PBP or also called progressive bulbar atrophy is a disease that attacks LMN and UMN in the brain stem. This disease weakens certain parts of the muscles and interferes with activities such as chewing, swallowing, and conversing. There are also weaknesses in membership, but they are not important, and people with PBP are emotionally unstable. Because of swallowing disorders, people with PBP can choke and experience aspiration pneumonia. No less than 20% of people with motor nerve disease usually suffer from this type of disease. The cause of PBP is still difficult to understand. 

• Spinal muscular atrophy (SMA). This is a hereditary disease that attacks LMN. SMA is caused by damage to the SMN1 gene that functions to produce the SMN protein that is important for the survival of motor nerve cells. Lack of SMN protein will result in atrophy and weakened muscle strength in the body, legs, arms, hands and feet.

• Primary lateral sclerosis (PLS). This is a motor nerve disease that attacks the UMN from the muscles of the arms, legs, and face. This disease is characterized by speech disorders, difficulty walking, and obstruction of work that requires hand muscle coordination. Unlike ALS, PLS is not fatal, but can disrupt quality of life. PLS usually attacks men aged 40-60 years. PLS occurs due to nerve cell disorders in the brain, but the cause of the disorder is unknown.

• Hereditary spastic paraparesis (HSP). HSP or Strumpell-Lorrain disease is a hereditary disease caused by genetic disorders that cause weakness and lower body rigidity. In addition, reflexes in the ankle become excessive. This happens slowly and can be severe.

• Progressive bulbar palsy (PBP). PBP or also commonly called progressive bulbar atrophy is a disease that attacks LMN and UMN in the brain stem. This disease causes some parts of the muscles to become weak and interfere with activities, such as chewing, swallowing, and talking. There are also limb weaknesses but they are not prominent, and PBP sufferers are emotionally unstable. Because of swallowing disorders, PBP sufferers will be at risk of choking and suffering from aspiration pneumonia. As many as 20 percent of people with motor nerve disease generally suffer from this type of disease. The cause of PBP is still difficult to understand.

• Progressive muscular atrophy (progressive muscular atrophy / PMA). PMA attacks LMN with early symptoms appearing on the hands and spreading to the lower body. Other symptoms can include muscle atrophy, slow hand movements, muscle twitching, and muscle cramps. PMA attacks men more often and can turn into ALS.

• Kennedy disease. Also referred to as X-linked spinobulbar muscular atrophy (SBMA), because this disease is caused by gene mutations on the X chromosome that are inherited. Women have 2 X chromosomes, so women who have this chromosomal abnormality are only carriers of nature, and can be passed down to their sons which then causes symptoms. Early symptoms can begin at the age of 15-60 years, namely weakness and atrophy of the facial muscles, jaw and tongue. As a result, sufferers will have difficulty chewing, talking, and swallowing. Weakness in arm and leg muscles can develop later. Other symptoms that can arise are loss of sensation or taste in the hands and feet, gynecomastia, high cholesterol, and having type 2 diabetes.

• Post polio syndrome. Is a complication of polio which was suffered decades ago. Post-polio syndrome is a new complaint that arises in people who have had polio before. The suspected cause is nerve cells that are already weak due to previous polio, then suffer damage due to the aging process or other diseases. The symptoms caused by this disease are muscle weakness and atrophy, muscle twitching, muscle aches, joint pain, and not being able to withstand cold temperatures. Unlike ALS, post-polio syndrome is usually not life-threatening.

Diagnosis of motor nerve disease
The doctor can diagnose motor nerve disease by using symptoms experienced by the patient, physical examination and neurological examination.

Neurological examination is useful for measuring motor and sensory abilities, nerve function, visual strength, ability to hear and speak, balance and coordination of movements, mental states, and changes in behavior and mood. the mood.

Several investigations can be carried out to rule out other diagnoses and see how much muscle is involved, including:

• Electromyography (EMG). Made to see lower motor nerve disorders (LMN). This test is done to measure the electrical activity of muscles, which will show how nerves work and relate to muscles.
• Blood test. Creatine kinase examination is a marker of muscle weakness.
• Analysis of cerebral fluid and spinal cord. To see inflammation or infection can make muscles stiff. Samples were taken using a lumbar puncture technique, which involves collecting brain fluid between the needle and the spine.
• MRI. This scanned image will help the doctor see the state of the brain and spinal cord.
• Muscle or nerve biopsy. The doctor will take a small sample of muscle or nerve tissue under a microscope. This examination is useful to see the level of damage, but rarely done because of the risk of injury to the muscles or nerves themselves.

Specific genetic tests for spinal muscular atrophy (SMA) can be done to detect abnormalities in these genes.

Treatment of motor nerves

There is no treatment that can cure motor nerve disease. However, therapeutic steps can still be taken to relieve symptoms and reduce the severity of the disease.

A number of processing steps are adapted to the conditions, usually in the form of:

• Riluzole medicine. This drug is used to treat amyotrophic lateral sclerosis (ALS) by inhibiting the development of nerve damage. Riluzole can extend the patient's life from 2 to 3 months. Some of the side effects that can occur when taking riluzole are nausea, palpitations and fatigue.

• Nusinersen's medicine. Nusinersen is used to treat spinal muscular atrophy by giving intra-administered injections into brain fluid and spinal cord. This procedure is almost identical to a lumbar puncture.

• muscle relaxant. This drug is given to reduce the symptoms of muscle stiffness. Baclofen, tizanidine and benzodiazepines are examples of drugs given.

• Quinine medicine. In some cases, quinine can be used to relieve symptoms of muscle cramps. Side effects that can be caused by quinine medications include vision and hearing problems, tinnitus and vertigo.

• Physiotherapy. Made to improve posture, prevent joint stiffness and slow down symptoms of muscle weakness and atrophy. In some people, occupational therapy and speech therapy are also needed. If the condition worsens, you may need a tool such as a wheelchair or splint.

• Medication for pain Although motor nerve disorders often do not cause pain, doctors sometimes prescribe painkillers to relieve the pain felt by the patient.

• Installation of supply pipes. Symptoms of motor nerve disease is dysphagia. However, a balanced diet is important for maintaining ideal body weight and muscle strength. For people who cannot chew or swallow, it is possible to install percutaneous gastronomic tube (PEG). A PEG is a filling tube that is directly implanted in the stomach with a small incision in the abdominal wall.

• Noninvasive ventilation (NIV). This is a respirator that absorbs air and filters, and then air is pumped into the lungs. Unlike respirators in hospital intensive care wards, air flows through a nasal tube or respirator rather than a tube that is inserted from the mouth to the throat. NIV helps prevent sleep apnea and helps people with respiratory problems because they have respiratory muscle weakness.

Complications of motor nerve disease

Motor nerve disease can cause a number of complications, such as urinary tract infections, aspiration pneumonia, constipation, depression and paralysis. The most dangerous complication that can cause death is respiratory failure.

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