What is Demyelinating Disease and Why Does it Occur?

What is Demyelinating Disease?

A demyelinating disease is a condition in which the protective covering of the nerve, i.e. the myelin sheath gets damaged. The nerves in the body are covered by a layer known as the myelin sheath. The myelin sheath protects the nerves and helps the messages from the brain to move quickly and smoothly through the body.

What are the Symptoms of Demyelinating Disease?

The symptoms associated with a demyelinating disease are: loss of vision, muscle weakness, muscular spasms, stiffness of muscles and changes in working of bladder and bowel.

Why Does Demyelinating Disease Occur?

The different types of demyelinating diseases and its causes are:

Multiple Sclerosis (MS)

Multiple Sclerosis is a very common demyelinating disease. The cause of Multiple Sclerosis is not known. Multiple sclerosis is an autoimmune condition, which attacks the brain, spinal cord, and the optic nerve. The severity of Multiple Sclerosis ranges from mild to severe and this condition is most likely to affect women. Multiple sclerosis brings along fatigue, vision problems, and difficulty in movement, tingling, burning, and other weird feelings. A cure for multiple sclerosis is not yet known, but the symptoms can be controlled with various treatments.

Acute Disseminated Encephalomyelitis (ADEM)

The cause of this demyelinating disease, which is Acute Disseminated Encephalomyelitis, is that the body destroys its own tissues in response to viral or bacterial infections. Children are most likely to get affected by Acute Disseminated Encephalomyelitis, which leads to damage to the myelin sheath of the brain and spinal cord. Acute Disseminated Encephalomyelitis is a rare condition and sometimes occurs due to a reaction to a vaccine. Sometimes the cause of Acute Disseminated Encephalomyelitis remains unknown.

The symptoms of ADEM are fever, low energy, irritation, nausea and vomiting, eyesight problem, confusion, headache, and trouble in coordination. Drugs to fight inflammation are given to the patient suffering from ADEM and stop the damage to the nerve and spinal cord. Medicines are given to ease the symptoms of Acute Disseminated Encephalomyelitis. Recovery time from Acute Disseminated Encephalomyelitis is around 6 months, though in rare cases ADEM proves to be deadly.

Balo’s Disease (Concentric Sclerosis)

This demyelinating disease is a rare type of multiple sclerosis, as the symptoms are very similar. Though the cause of this demyelinating disease, i.e. Balo’s Disease is unknown; it can prove to be fatal at times. Balo’s Disease is a demyelinating disease that occurs more in adults than children.

The symptoms of Balo’s Disease include: high fever, headache, memory loss, seizures, paralysis, difficulty in talking and understanding information. The cure for Balo’s Disease is not known and symptomatic relief can be given to the patient with certain medications.

Charcot-Marie-Tooth disease (CMT)

In this demyelinating disease, the peripheral nerves, which lie outside the brain and the spinal cord are affected. These nerves send signals to the limbs. The Charcot-Marie-Tooth disease is mostly inherited. The cause of Charcot-Marie-Tooth disease is genetic mutations or a defective gene inherited from either of the parent. The symptoms of Charcot-Marie-Tooth disease are:

  • Weakness in the legs, ankle and feet.
  • Loss of sensation in legs and feet.
  • Tripping or falling.
  • Trouble in raising the leg and moving the ankle.
  • Difficulty in walking and running.
  • Guillian-Barr Syndrome (GBS)

Similar to CMT, Guillian-Barr syndrome is a demyelinating disease that affects the peripheral nerves. Guillian-Barr syndrome starts from the lower limb and extends to the upper body. Guillian-Barr syndrome can lead to paralysis and can prove to be fatal if it affects respiration.

The cause of Guillian-Barr syndrome is not known, but it often occurs after respiratory or digestive infection.

Tingling in toes, ankle, and wrist, trouble in walking and ascending stairs, bowel and bladder problem, and trouble in moving face, speaking and chewing, are the symptoms faced by the patient suffering from Guillian-Barr syndrome.

The cure of GBS is not known, but symptomatic relief can be attained with medications. Plasma exchange is also a treatment for Guillian-Barr syndrome in which plasma from the blood is replaced with the manmade version.

HTLV-1 Associated Myelopathy (HAM)

HTLV-1 Associated Myelopathy is a demyelinating disease which occurs after an infection from the HTLV-1 virus. It leads to swelling in the brain and the spinal cord. Most of the time people affected with the virus stay symptom-less.

The symptoms of HTLV-1 Associated Myelopathy are: weakness in the legs, numbness, tingling, stiff muscles, constipation, tremors, double vision, deafness and coordination problem.

Steroids help in easing the symptoms of HTLV-1 Associated Myelopathy and there is no other cure yet known.

Neuromyelitis Optica (Devic’s Disease)

Neuromyelitis Optica is a rare demyelinating disease and affects the eyes, arm, and legs. Neuromyelitis Optica attacks the optic nerve and spine. The cause of Neuromyelitis Optica is not known; however, this demyelinating disease seems to occur along with another autoimmune condition or after an infection. Neuromyelitis Optica attacks have relapse; therefore if the doctor detects the disease early he can give a medicine which can turn down the immune system to stop the recurrence. The symptoms of Neuromyelitis Optica are eye pain, blurred vision, and loss of eyesight, vomiting, weakness, numbness in limbs and uncontrolled hiccups. Neuromyelitis Optica doesn’t have any cure, but medicines help relieve symptoms.

Schilder’s Disease

Schilder’s disease is a demyelinating disease, which wears away the myelin sheath of the brain and spinal cord and affects boys between 7-12 years of age. The exact cause of Schilder’s disease is not clear; however, there seem to be a genetic connection to it. The progress of Schilder’s disease varies in different people. In some, the symptoms of Schilder’s disease flare up followed by recovery; and in some patients, Schilder’s disease gets worse slowly.

Weakness on one side of the body, seizures, and slow movements, difficulty in speaking, weight loss, memory problems, and change in personality are some symptoms of Schilder’s disease.

Similar to other demyelinating diseases, Schilder’s disease has no cure, but the symptoms can be managed with steroids and other drugs.

Transverse Myelitis

Transverse Myelitis is a demyelinating disease characterized by inflammation of the spinal cord. The exact cause of Transverse Myelitis is not clear. Transverse Myelitis is thought to occur due to certain infections, with multiple sclerosis, Devic’s disease, after vaccinations and along with other autoimmune disorders.

The presentation of the Transverse Myelitis symptoms depends on which part of the spinal cord is affected. In later stages of Transverse Myelitis, it is more likely to be diagnosed with multiple sclerosis.

The symptoms of Transverse Myelitis include: bowel and bladder problems, fatigue, sensitivity to touch, lower back pain, problems in moving legs and tingling and numbness in toes. Treatment of Transverse Myelitis lays stress on symptom reduction by steroid shots or plasma exchange (PLEX), which helps bring down swelling in the spinal cord.