What Is Demyelinating Disease?

Demyelinating diseases or disorders are any conditions that attacks and damages the myelin. The nervous system of the body is a network of complex pathways that the brain to the spinal cord branches to every part of the body.

This complex system contains cells called neurons which otherwise known as nerve cells; the nerve cells are transport vehicles that transmit successful signals to and from the brain to other parts of the body and vise versa.

The signals and processions allow an individual to speak, see, feel and think, that is why almost all the nerves are coated with protective myelin sheath.

Myelin is an insulating material or sheath just like an electric wire protecting these cells; just like an electric wire but unlike wires, the myelin helps messages or signals move quickly and smoothly throughout the body like electricity flowing from a power generator.

When myelin sheaths are damaged or injured, scar tissues begin to form in its place and signals from the brain or from the body and this cannot move freely across these scar tissues as swiftly as before, causing the nervous system not to function as well as before.

In turn, the reduction in this conductivity causes impairment in sensation, movement, cognition or other functions depending on the area of nerve cells affected.

Types of Demyelinating disease

Demyelinating diseases are basically classified into two groups, although other types have been drawn; but these two traditional classifications are demyelinating myelinoclastic disease are a group that entails a healthy myelin been destroyed by a toxic, chemical or autoimmune substance and demyelinating leukodystrophic disease is a disease condition where the myelin sheath is abnormal and degenerates.

Other types of demyelinating disorders and some their symptoms include:

Multiple Sclerosis (MS)

1 in every 500 people has this disease, making it the most common demyelinating disease. This is an autoimmune condition that affects the optic nerves (eye), spinal cord and the brain.

The condition is mostly genetically linked, but some cases may be triggered by environmental agents and it is most likely to affect women. Symptoms most common to MS are vision problems, extreme fatigue, locomotion hindrances and tingling, burning or other odd feelings.

There are several treatment techniques to help keep the symptoms in check and medications to change the coruse of the disease and help avoid relapses but there is no specific cure.

Concentric Sclerosis (Balo’s Disease)

According to medical experts, this condition is very similar to MS due to the similar symptoms but is not they are quite different.

Its cause is not totally known but the disease results in serious complications which can be fatal, but recovery is not impossible. Asians and Philippines are most at risk to get it, affecting children and more often adults.

Some symptoms for Concentric Sclerosis (CS) comes quickly and increases in severity within a short space of time while other symptoms go away quickly; headache, fever, memory loss, muscle spasms, seizures, paralysis and troubling processing information are some its symptoms.

There is no cure for CS but medications to help with symptoms can be prescribed, including steroids to help reduce the swelling in the brain and spinal cord.

Charcot-Marie-Tooth Disease (CMT)

This disease affects the peripheral nerves- nerves outside the brains and spinal cord, these peripheral nerves send signals to the muscles in the limbs. CMT is an inherited genetic disorder.

Symptoms do not show at early years but in teenage age and early adulthood, these symptoms include:

• weakness of the legs, ankles and feet,
• Loss of muscle mass in legs and feet,
• Troubles moving the legs and ankles,
• Less sensations in the legs and feet,
• Changes in the feet such as higher arches and curled toes,
• Trouble walking and running,
• Tripping and falling over.

There is no cure for CMT, but painkillers can be administered, physical therapy lessons and exercises to help build stamina and keep the muscles strong can be taken and occupational therapy to assist the individual learn to use any affected limbs.

Overtime, braces and splints may be required for weak joints.

Acute Disseminated Encephalomyelitis (ADEM)

This condition is a rare condition most likely to affect children. The condition is a brief but widespread assault of inflammation that destroys myelin in the brain and spinal cord.

It rare affects the optic nerve (which connects the eye and the brain). ADEM cause is mostly unknown but at times it is an autoimmune condition (a condition where the body attacks its own tissues in response to an infection of a virus or bacteria).

It can also be a result of a reaction to administered vaccine.

Symptoms include:

• High fever
• Low energy
• Irritation
• Confusion
• Nausea and vomiting
• Headaches
• Vision impairment
• Cognitive issues 

Medications used against ADEM are drugs that combat inflammation and can stop the damages to the nerves of the brain and spinal cord. Clinicians also can suggest medication to ease some ADEM symptoms. A recovery time varies with individuals as some persons can fully recover within 6 months, though in very rare cases, ADEM can be deadly.

HTLV-1 Associated Myelopathy (HAM)

This condition is caused by a virus called HTLV-1. The virus causes swelling of the brain and the spinal cord which certainly leads to some not so fatal symptoms. Not all individual infected with the virus might get HAM.

Some people also carry HTLV-1 but have no symptoms. The virus causing HAM disease is contaminated through contacts with body fluids or blood from an infected person.

The virus and disease can live in the body for decades.

Symptoms include:

• Stiff muscles
• Muscle spasms
• Numbness or tingling
• Weakness in legs that worsens overtime.
• Constipation
• Double vision
• Deafness
• Tremors
• Coordination problems
• Bladder issues

  There is no cure for HAM, but steroids can be prescribed to help ease symptoms.

Guillain-Barre Syndrome (GBS)

Just like CMT, this disease condition also attacks peripheral nerves. It always begins with weakness in your legs that moves to the arms and upper body. GBS can lead to paralysis and can be life threatening if it causes trouble breathing.

The primary cause is still unknown, but it often follows a respiratory or digestive tract infection. Some persons are infected after surgery or after a bacterial and flu infection.

There is no cure for GBS just like some other demyelinating disease, but effects of the symptoms can be lessened by medications and speed up recovery; Plasma exchange (PLEX) is a common treatment, it involves the removal of some liquid part of the blood (plasma) and replacing it with manmade substance.

Another type of treatment is intravenous immunoglobulin (IVIG); in this treatment, the doctor puts proteins called immunoglobulin into the veins.

When the disease affects the respiratory functions of the body, like breathing, carefully close hospital treatment will be needed. Caregivers might need to assist patients move their limbs.

The most common symptoms include: 

• Weakness in legs that spread to the upper body.
• Bowel or bladder problems
• Trouble using staircases.
• Tingling in your fingers, toes, ankles or wrists
• Trouble moving your face, speaking or chewing.

Neuromyelitis Optica (Devic’s Disease)

This is a rare autoimmune condition that affects the legs, arms and eyes. Loss of sight or blurred vision. When then disease affects the spinal cord, the legs and arms might be drastically affected.

This condition usually has relapses but if the disease is attended to early enough, there may be better chances of treating the symptoms. Drugs may be used to suppress the immune system in other to avoid relapses.

Some of the symptoms include:

• Loss of sight
• Blurred vision
• Pains in the eyes
• Vomiting
• Uncontrollable hiccups
• Bladder and bowel problems
• Weak or numb arms and legs

Neuromyelitis optica does not have a cure but some medications like inebilizumab-Cdon (Uplizna), satralizumab-mwge (Enspryng) and eculizumab (soliris) have been approved for treatment.

These drugs function by attacking defective antibodies that attack the healthy cells in your body (triggering Neuromyelitis Optica). Steroids are also administered to reduce swelling. PLEX is also an option of treatment.

Drugs that suppress the immune system include azathioprine (Imuran, Azasan), Mycophenolate (CellCept), Methotrexate (Otrexup), and Rituximab (Rituxan, Ruxience, Truxima), these can help prevent further attacks.

Transverse Myelitis

This is a demyelinating spinal cord disease that result in whole body symptoms depending on the part of the spinal cord myelin is affected.

There are about 1400 reported cases of demyelinating transverse myelitis each year as it is most likely diagnosed alongside Multiple Sclerosis. Statistics show more women been affected than men, the cause is uncertain, but it usually follows an infection.

Symptoms include:

• Fatigue
• Lower back pain
• Muscles weakness
• Bladder and bowel problems
• Tingling and numbness in the toes
• Increased sensitivity to touch.

Plasma exchange and steroid administration are some of the treatment approach used to reduce swellings of the spinal cord and to ease symptoms as there is no cure for transverse myelities and no FDA-approved drug for it yet.

Schilder’s Disease

This is a rare type of demyelinating disease that affects males between ages 7 and 12. The condition is characterized by wearing out of myelin sheath in the brain and spinal cord. Breathing, blood pressure and heart rate are affected in most cases.

Research points that Schilder’s demyelinating disease begins with an infection; headaches and fever been its early symptoms. The condition is hard to predict as its symptoms come like a flare in some cases but in others, it is slowly progressing and worsens over time.

Symptoms of Schilder’s demyelinating disease are as follows:

• Seizures
• Lagging movements
• Weakness on a particular side of the body
• Vision and hearing issues
• Weight loss
• Memory issues
• Personality disturbance

Causes of Demyelinating disease

The most common cause of demyelinating diseases and damage to myelin is inflammation (the body’s process of fighting against foreign bodies or things that harms it such as toxins, infection and injuries in an attempt to heal itself).

Other general causes include:

• Metabolic problems
• Inherited genetic factors.
• Autoimmune reactions to some vaccines
• Certain viral infections
• Loss of oxygen to the brain
• Physical blows or compression of blood vessels in the brain

Symptoms of Demyelinating disease

Most Signs and Symptoms of demyelinating diseases are unique for each type and condition depending on the nature of the demyelinating disease.

But generally, some signs and symptoms can be common to all demyelinating disease, such as:

• Ataxia- lack of coordination during voluntary activites
• Diplopia- blurred double vision.
• Clonus- muscular spasm with regular contrations
• Incoordination and Clumsiness
• Hand or leg paralysis
• Dysarthria- difficulty in articulating words due to disturbance in the function of the structures that modulate voice into speech.
• Genital anesthesia
• Hemiparesis- muscular weakness that affect only one side of the body.
•  Paresthesia- a sensation of burning, prickling, itching or tingling of the skin with no obvious cause.
• Impaired vision
• Ocular paralysis
• Loss of sensation
• Memory problems
• Neurological symptoms
• Unsteady gait
• Speech and hearing problems
• Incontinence
• Spastic paraparesis- progressive weakness and stiffness of the legs.
• Pain
• Loss of bladder or bowel control
• Uncontrolled blood pressure
• Irregular heart beats

Diagnosis of Demyelinating diseases

MRI

MRI scans can show demyelinating activities in the brain and nerves, especially those caused by Multiple Sclerosis. The clinicians may also be able to locate lesions affecting the nervous system, thereby giving a specific direction for treatments against the source of the demyelination.

Fluid Attenuated Inversion Recovery (FLAIR)

This diagnostic technique uses a pulse timer sequence to cerebrospinal fluid in the spinal cord in other to reveal lesions more clearly. It is very effective for diagnosing most demyelinating diseases.

Quantitative Proton Magnetic Resonance Spectroscopy (MRS)

This is a non-invasive technique used to study the metabolic changes in a brain tumor, stroke, seizure disorders and other diseases and abnormalities that affects the brain. The technique is also employed in studying the metabolism of other structures such as the muscles.

Cerebrospinal Fluid Analysis (CSF)

This is a culture examination of the cerebrospinal fluid of the spinal cord. This analytical technique can be used to reveal the microorganism that causes any infection. it is very effective method that benefits the diagnosis of the Central nervous System.

Diagnostic Criteria

This technique refers to a specific combination of symptoms and lab test results that a dotor uses in attempt to find out a correct diagnosis.

Treatment of Demyelinating disease

There is no specific cure for demyelinating diseases as clinical trials are still been conducted and symptoms varies vastly with individuals. Getting medications early is very necessary to combat issues of demyelinating disease.

Medication for treatments focuses on:

• Managing the symptoms
• Minimizing the effects of the attacks
• Modifying the course of the disease

Also, most treatment for these conditions retracts the immune responsiveness of the body using medications like interferon beta-1a and glatiramer acetate.

Persons with considerably low levels of Vitamin D are more subtle to Multiple Sclerosis or other demyelinating conditions; high levels of vitamin D reduce inflammatory response of the immune system (which is the most common cause of demyelinations). Strategies to treat symptoms may include physical therapy, drugs to reduce pain and fatigue and muscle relaxing drugs.

  Administration of some kind of vaccines can produce an antonymic response in persons with relatively higher sensitive immune systems, which can lead to an activation of an autoimmune reaction.

Vaccines for influenza or HPV have been discovered to cause “acute demyelinating syndromes” in some children and adults. But it is not certain that these vaccines can cause demyelination.    

Conclusion

Demyelinating diseases and its conditions can be very painful and seemly unmanageable at first, but it is not impossible as it is very possible to live well with these conditions.

There are promising new research that been carried out to ascertain causes and treatments of demyelinating conditions. Pain management treatments are also been improved.

Demyelinating conditions may not be curable but if attended to early, it is very well manageable.

Sources;

• Impact of Demyelinating Diseases; https://www.hopkinsmedicine.org/neurology_neurosurgery/centers_clinics/project_restore/conditions/
• Demyelinating disorder; https://www.msdmanuals.com/professional/neurologic-disorders/demyelinating-disorders/overview-of-demyelinating-disorders
• Demyelinating disease; https://en.m.wikipedia.org/wiki/Demyelinating_disease
• Third edition/Cambridge priced edition/Biological Science