Home Health Topics What are Demyelinating Diseases, and What Causes Them

What are Demyelinating Diseases, and What Causes Them

by Gauri Kolhe

Any ailment that harms the myelin sheath, which protects the nerve fibres in the brain, the nerves leading to the eyes, the optic nerves, and the spinal cord, is referred to as a demyelinating disease.

Nerve impulses slow or even cease when the myelin sheath is compromised, leading to neurological issues. Myelin, a fatty white material, coats or sheaths many of the nerves in the nervous system.

This myelin sheath shields nerve fibres and allows electrical impulses to move swiftly and effectively between nerve cells. The myelin sheath can be harmed by some disorders, which can lead to issues with the spinal cord, brain, eyes, and other body nerves. These conditions are known by doctors as demyelinating disease.

Genesis for the demyelinating disease:

Demyelination can be caused by a variety of different situations. Those are

Multiple sclerosis

The most prevalent demyelinating condition affecting the central nervous system is multiple sclerosis (MS). The immune system targets the myelin sheath or the cells that create and maintain it in this condition. The onslaught damages and inflames the nerve sheath, ultimately harming the surrounding nerve fibres. Multiple areas of scarring sclerosis may develop as a result of the process.

In MS, the immune system targets the myelin that covers the brain, spinal cord, and optic nerves, which are all parts of the central nervous system. During MS exacerbations, demyelinating disease can cause a variety of neurological symptoms. When myelin is severely damaged, it might result in scars that are permanently calcified and provide enduring symptoms.

Some common symptoms of MS include:

  • numbness and tingling
  • weakness and fatigue
  • pain
  • vision changes, such as blurry vision or double vision.
  • decreased bladder control
  • difficulty with balance and coordination
  • problems with concentration and memory

Depression

Only a small percentage of people continue to have mild symptomsTrusted Source. But for many others, they might even get really bad. Most people have relapses occasionally. When symptoms temporarily get worse and when they dramatically lessen during remission.

Certain MS varieties can get worse over time. For instance, Schilder’s illness is a progressive MS variation that typically first manifests in children. The brain and spinal cord experience extensive demyelinating disease as a result of the illness.

The underlying reasons of the illness are unclear, and it is extremely uncommon. MS often strikes people between the ages of 20 and 50, and two to three times as many women as males are affected. Most persons with a diagnosis of MS live as long as those without a diagnosis.

Optic neuritis

When the optic nerve is affected by inflammation and demyelination, optic neuritis develops. Working to transmit visual data from the eye to the brain is the optic nerve. One or both eyes may be impacted by optic neuritis. Eye pain and visual abnormalities are some of its symptoms.

It occurs in about 50% of instances of MS and is a prevalent component of the disease. Optic neuritis, however, can also develop without MS. Lupus is one example of an inflammatory disease that can cause the syndrome. Additionally, it frequently affects those who have viral illnesses including the flu, mumps, and measles.

Neuromyelitis optica

When myelin in the spinal cord and eye is attacked and destroyed by the immune system, it results in neuromyelitis optica (NMO), also known as Devic’s disease. The spinal cord and eyes may be affected on either one or both sides by NMO. The effects of this could include signs and complications such:

  • vision loss from optic neuritis
  • Weakness, pain, or both in the limbs
  • bladder and bowel problems

Neuromyelitis optica patients frequently experience symptomatic episodes that can happen months or years apart. The cause of neuromyelitis optica is still unknown, like that of many other demyelinating disease. But those who get it frequently have a personal or family history of autoimmune diseases.

Transverse myelitis

Inflammation that occurs at the same level on both sides of the spinal cord is referred to as transverse myelitis. Myelin may be harmed in this situation. Regardless of age, transverse myelitis can afflict anyone, but it most frequently People in the Trusted Source age ranges of 10 to 19 and 30 to 39 are affected. There is no evidence that the illness is genetic in nature.

 leading to symptoms such as:

  • pain and weakness in the limbs
  • unusual sensations, such as burning, numbness, or tickling
  • bladder and bowel problems.
  • neuromyelitis optica
  • certain infections, such as varicella-zoster, syphilis, or tuberculosis.
  • autoimmune conditions, such as lupus or Sjögren’s

Acute disseminated encephalomyelitis

The brain and spinal cord are both affected by the extensive inflammation that leads to acute disseminated encephalomyelitis (ADEM). Myelin injury and early symptoms may result from this.

Additionally, if ADEM worsens, it can produce symptoms like weakness, poor coordination, and eye impairments. When ADEM is severe, it can be fatal and cause coma or seizures. ADEM often starts after a bacterial or viral infection.

Adrenoleukodystrophy and adrenomyeloneuropathy

A hereditary disorder called adrenoleukodystrophy (ALD) causes myelin degradation. It is thought that 1 in 20,000 people have it. dependable source With the same gene that causes ALD in adulthood, adrenomyeloneuropathy (AMN) can also develop. It usually starts between the ages of 21 and 35 and is milder. ALD often manifests in childhood and may cause symptoms like:

  • adrenal gland dysfunction
  • vision or hearing loss
  • impaired coordination and movement
  • changes in behavior, such as withdrawal or aggression
  • problems with memory and learning
  • difficulty with speech
  • swallowing difficulties
  • seizures
  • progressive weakness or stiffness in the legs
  • impaired coordination and movement
  • bladder and bowel problems
  • sexual dysfunction
  • adrenal gland dysfunction (Addison’s disease)
  • peripheral neuropathy

Guillain-Barré syndrome

The immune system assaults myelin in the uncommon autoimmune illness known as Guillain-Barré syndrome (GBS). In extreme circumstances, it can also target the peripheral nervous system’s nerves. Only if inflammation persists after destroying the myelin does this happen.

GBS is primarily characterised by weakness, numbness, and tingling in the legs that frequently extends to the arms and chest. In certain instances, this might get so bad that paralysis could happen. Respiratory support will be required throughout the course of treatment if this paralysis affects the muscles that govern breathing.

It is understood that a variety of infections can result in GBS. One of the most frequent causes of food poisoning is an infection with Campylobacter jejuni. Experts believe that up to 40%Trusted Source of GBS cases in the US may be caused by infections with these bacteria.

GBS can also be brought on by the cytomegalovirus, the flu, and the Epstein-Barr virus. The flu vaccine is just one of several vaccinations that may cause GBS. According to the Centers for Disease Control and Prevention (CDC)Trusted Source, a person is more likely to get GBS from having the flu than from getting a flu vaccination.

Charcot-Marie-Tooth disease

Peripheral nerves are harmed by Charcot-Marie-Tooth disease (CMT). It is a hereditary disorder caused by a genetic mutation that may affect the myelin’s structure or function. The symptoms of CMT increase over time, meaning they progressively get worse. Those who have the illness frequently have growing difficulty moving about and performing daily duties.

Some symptoms of CMT include:

  • weak muscles in the limbs.
  • changes to walking, often with frequent tripping or falling
  • foot irregularities, such as high arches or curled toes
  • a reduced ability to feel sensations such as touch and temperature
  • muscle cramping
  • peripheral neuropathy

HTLV-I-associated myelopathy (HAM)

The myelopathy brought on by the HTLV-I virus might develop slowly. Myelopathy refers to spinal cord injury. HAM results in symptoms by demyelinating the spine. The signs and symptoms of HAM are comparable to those of main progressive MS (PPMS). However, the majority of those with HTLV-1 infection do not experience myelopathy.

Some of the Symptoms that impacts:

The following are some of the parts of the nervous system that can be impacted by demyelinating illnesses.

Vision

These disorders can cause temporary or permanent vision loss, hazy or double vision, or both.

Reflexes and movement

Muscle stiffness, spasms, weakness, and balance issues can all be caused by changes to the motor system. Due to this, it may be difficult for the person to speak and swallow. Blood pressure and breathing are examples of automatic actions that are rarely impacted by demyelinating disease.

Senses and feeling

In the arms, legs, or feet, a person may feel numbness and tingling, scorching, or prickling sensations. When lightly touched, they could also experience pain. An MS patient may experience a symptom known as Lhermitte’s sign. A person may experience what seems like an electric charge travelling down the back of their neck, down their spine, and then out into their limbs and legs as they move their head toward their chest.

Cerebellum

The brain’s motor cortex is in charge of balance and coordination. Tremors or lack of coordination can result from issues in this area. For instance, some individuals can discover that swallowing, writing, eating, and walking become challenging.

Genitourinary system

Problems with urination and bowel movements might occur in some persons. The ability to get an erection or have orgasms may be hampered as a result.

Mood and thinking

The individual may struggle with thinking, memory, and focus issues, as well as sadness, anxiety, and impatience. Additionally, some people could process thoughts more slowly.

Fatigue

A person may also have ongoing weariness from demyelinating illnesses without engaging in excessive activity or sleeping poorly.

Treatment for demyelinating disease

The precise ailment a person has will determine how they are treated for demyelinating illnesses. A list of potential treatments is provided below.

MS

MS presently has no known cure. However, recent years have seen considerable advancements in science. Now that there is a treatment for demyelinating disease, some patients with MS may experience fewer flare-ups and a slower rate of disease development.

Given that early therapy can assist improve long-term outcomes, current guidelines advise doctors to start prescribing one of several disease-modifying treatments (DMT) as soon as a patient is diagnosed. DMT for MS aids in lowering too much immune system activity.

The effects of inflammation can be lessened during a relapse (when symptoms go worse). Specific symptoms can be treated with a variety of drugs, such as antidepressants and painkillers. Strengthening muscles and issues with coordination and balance can both benefit from physical therapy.

Optic neuritis

Some persons with optic neuritis discover that their illness gets better on its own. To alleviate their symptoms or stop them from getting worse, the majority of people, however, require some type of medical intervention. A popular short-term treatment for acute flare-ups is corticosteroids.

Neuromyelitis optica

The symptoms of acute episodes can be treated with corticosteroids and plasma exchanges. In plasma exchange therapy, blood is infused through a venous catheter into a device that separates the antibodies before reintroducing the blood into the body. On the other hand, medications that lower immune system activity, like methotrexate and azathioprine, are useful as maintenance treatments and may help avoid further attacks.

Transverse myelitis

Corticosteroids can lessen inflammation and assist in calming the immunological system. Muscle pain can also be treated with painkillers like acetaminophen and ibuprofen. In the event that these therapies are unsuccessful, plasma exchange therapy or intravenous immunoglobulin (IVIG) may be used to treat acute bouts. The healthy donor antibodies in IVIG bind to the autoantibodies that are causing inflammation and help to eliminate them from circulation.

ADEM

In general, corticosteroids aid in the recovery of ADEM patients. In dire circumstances, IVIG or plasma exchange therapy may be helpful.

ALD and AMN

The goal of treating ALD and AMN is to reduce individual symptoms and raise quality of life. Steroid replacement treatment is frequently used by doctors to treat adrenal gland dysfunction. To aid in movement and muscle strength, they might also suggest physical therapy.

GBS

Patients with GBS typically receive IVIG or plasma exchange therapy from medical professionals. Some people might start feeling better in a matter of weeksReliable Source. Some people, however, may suffer from irreversible nerve damage and need years to fully recover.

CMT

Physical and occupational therapy are a big part of CMT treatment. These treatments can support patients with CMT in keeping their mobility, muscle strength, and capacity for daily activities. Surgery could be required in some cases to correct foot abnormalities. A person can take painkillers if they have peripheral neuropathy.

Outlook

Demyelinating disorders currently have no effective treatments. The sort of demyelinating disease a person has and how severe their symptoms are determine their prognosis as a whole. Demyelination symptoms can be managed with treatment. The medical staff will collaborate with a patient’s family to help them manage their symptoms and enhance their quality of life.

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