What are the common types of demyelinating disorders. How do these conditions affect the nervous system. What are the primary symptoms and available treatments for demyelinating diseases. Can early intervention improve outcomes for patients with myelin sheath damage.

What Are Demyelinating Disorders?

Demyelinating disorders are a group of neurological conditions characterized by damage to the myelin sheath, the protective covering that surrounds nerve fibers in the central and peripheral nervous systems. This damage disrupts the normal transmission of nerve signals, leading to various neurological symptoms.

The myelin sheath acts as an insulator, similar to the coating on electrical wires, allowing for rapid and efficient transmission of nerve impulses. When this protective layer is compromised, it can result in the formation of scar tissue, which impedes the smooth flow of electrical signals along the nerves.

How does myelin damage affect nerve function?

When myelin is damaged, several consequences can occur:

• Slowed nerve conduction
• Impaired signal transmission
• Increased energy expenditure for signal propagation
• Vulnerability of axons to further damage

These effects can lead to a wide range of neurological symptoms, depending on the location and extent of the demyelination.

Common Types of Demyelinating Disorders

Several demyelinating disorders affect the nervous system, each with its unique characteristics and progression. Understanding these conditions is crucial for proper diagnosis and treatment.

Multiple Sclerosis (MS)

Multiple Sclerosis is the most prevalent demyelinating disorder, affecting approximately 1 in 500 people. It is an autoimmune condition that primarily targets the brain, spinal cord, and optic nerves.

Key features of MS include:

• Higher prevalence in women
• Genetic predisposition
• Potential environmental triggers
• Four main disease courses with varying progression rates

Acute Disseminated Encephalomyelitis (ADEM)

ADEM is a rare but serious demyelinating condition that predominantly affects children. It is characterized by widespread inflammation and myelin damage in the brain and spinal cord, sometimes extending to the optic nerves.

Notable aspects of ADEM include:

• Rapid onset of symptoms
• Often triggered by viral or bacterial infections
• Rare cases linked to vaccine reactions
• Generally good prognosis with proper treatment

Balo’s Disease (Concentric Sclerosis)

Balo’s disease is a rare form of demyelinating disorder that shares similarities with MS. It is characterized by concentric rings of demyelination in the brain, visible on MRI scans.

Key points about Balo’s disease:

• Higher prevalence in Asian populations and individuals from the Philippines
• Can be rapidly progressive and potentially fatal
• Some cases may result in full recovery
• More common in adults than children

Charcot-Marie-Tooth Disease (CMT)

CMT is an inherited neurological disorder that affects the peripheral nerves, which are responsible for transmitting signals between the brain and spinal cord to the muscles in the limbs.

Distinguishing features of CMT include:

• Genetic inheritance pattern
• Onset typically in late teens or early adulthood
• Progressive muscle weakness and atrophy in the legs and feet
• Sensory loss in affected areas

Guillain-Barré Syndrome (GBS)

GBS is an acute demyelinating disorder that affects the peripheral nervous system. It is characterized by rapidly progressive weakness that can lead to paralysis in severe cases.

Important aspects of GBS include:

• Often triggered by infections or immune system dysregulation
• Ascending paralysis pattern (starting in the legs and moving upward)
• Potential for respiratory failure in severe cases
• Generally good prognosis with proper treatment and support

Symptoms of Demyelinating Disorders

The symptoms of demyelinating disorders can vary widely depending on the specific condition and the areas of the nervous system affected. However, there are some common symptoms shared across many of these disorders.

What are the most prevalent symptoms of demyelinating disorders?

While symptoms can differ between individuals and conditions, some of the most common manifestations include:

• Fatigue: Often described as overwhelming and disproportionate to activity levels
• Visual disturbances: Including blurred vision, double vision, or vision loss
• Motor difficulties: Such as weakness, paralysis, or coordination problems
• Sensory abnormalities: Including numbness, tingling, or burning sensations
• Cognitive changes: Affecting memory, concentration, and information processing
• Balance and coordination issues: Leading to difficulties with walking or fine motor tasks

It’s important to note that the presentation and severity of these symptoms can vary greatly between individuals and may fluctuate over time.

Causes and Risk Factors of Demyelinating Disorders

The exact causes of many demyelinating disorders remain unclear, but researchers have identified several factors that may contribute to their development.

What triggers demyelination in the nervous system?

While the specific triggers can vary between conditions, some common factors include:

• Autoimmune reactions: The body’s immune system mistakenly attacks myelin
• Viral infections: Certain viruses may directly damage myelin or trigger an autoimmune response
• Genetic predisposition: Some demyelinating disorders have a hereditary component
• Environmental factors: Exposure to toxins or certain pathogens may play a role
• Vascular issues: Damage to blood vessels in the brain can lead to demyelination
• Oxygen deprivation: Lack of oxygen to brain tissue can result in myelin damage

It’s important to note that in many cases, the exact cause may be multifactorial or remain unknown.

Diagnosis of Demyelinating Disorders

Diagnosing demyelinating disorders often requires a comprehensive approach, combining clinical evaluation, imaging studies, and laboratory tests.

How are demyelinating disorders diagnosed?

The diagnostic process typically involves several steps:

1. Medical history and physical examination: To assess symptoms and neurological function
2. Magnetic Resonance Imaging (MRI): To visualize areas of demyelination in the brain and spinal cord
3. Cerebrospinal fluid analysis: To check for signs of inflammation or abnormal antibodies
4. Evoked potential tests: To measure the electrical activity of the nervous system in response to stimuli
5. Nerve conduction studies and electromyography: To assess peripheral nerve function (for conditions like CMT and GBS)
6. Genetic testing: For inherited disorders like Charcot-Marie-Tooth disease

Early and accurate diagnosis is crucial for initiating appropriate treatment and management strategies.

Treatment Approaches for Demyelinating Disorders

While there is no cure for most demyelinating disorders, various treatment options are available to manage symptoms, slow disease progression, and improve quality of life.

What are the primary treatment strategies for demyelinating disorders?

Treatment approaches often include:

• Disease-modifying therapies: To reduce inflammation and slow disease progression (especially in MS)
• Corticosteroids: To manage acute flare-ups and reduce inflammation
• Immunosuppressants: To modulate the immune system and prevent further damage
• Plasma exchange: To remove harmful antibodies from the blood (used in some cases of GBS and severe MS relapses)
• Symptomatic treatments: To address specific symptoms like pain, fatigue, or spasticity
• Physical and occupational therapy: To improve strength, mobility, and daily functioning
• Cognitive rehabilitation: To address cognitive challenges and develop coping strategies
• Assistive devices: To aid mobility and independence

Treatment plans are typically tailored to the individual’s specific condition, symptoms, and disease course.

Living with Demyelinating Disorders: Coping Strategies and Support

Living with a demyelinating disorder can present numerous challenges, but there are many strategies and resources available to help individuals manage their condition and maintain a good quality of life.

How can individuals cope with the challenges of demyelinating disorders?

Some effective coping strategies include:

• Education: Learning about the condition and staying informed about new developments
• Stress management: Practicing relaxation techniques and mindfulness to reduce stress-induced flare-ups
• Regular exercise: Engaging in appropriate physical activity to maintain strength and flexibility
• Healthy lifestyle: Following a balanced diet and getting adequate rest
• Support groups: Connecting with others who have similar experiences
• Mental health support: Seeking counseling or therapy to address emotional challenges
• Adaptations: Making necessary modifications to home and work environments
• Planning: Developing strategies for managing energy levels and prioritizing activities

It’s important for individuals with demyelinating disorders to work closely with their healthcare team to develop a comprehensive management plan that addresses their unique needs and goals.

Research and Future Directions in Demyelinating Disorders

The field of demyelinating disorders is an active area of research, with ongoing efforts to better understand these conditions and develop more effective treatments.

What are the promising areas of research in demyelinating disorders?

Current research focuses on several key areas:

• Remyelination therapies: Developing treatments to promote the repair of damaged myelin
• Neuroprotective strategies: Exploring ways to prevent further nerve damage
• Stem cell therapies: Investigating the potential of stem cells to repair or replace damaged nervous tissue
• Biomarkers: Identifying biological indicators for earlier diagnosis and treatment monitoring
• Personalized medicine: Tailoring treatments based on individual genetic and environmental factors
• Advanced imaging techniques: Developing more sensitive methods to detect and monitor myelin damage
• Novel drug delivery systems: Improving the efficacy and targeting of medications

These research efforts hold promise for improving outcomes and quality of life for individuals affected by demyelinating disorders in the future.

As our understanding of demyelinating disorders continues to grow, so does the hope for more effective treatments and potential cures. The complex nature of these conditions requires a multidisciplinary approach, combining insights from neurology, immunology, genetics, and other fields. By advancing our knowledge and developing innovative therapies, we can work towards better management and outcomes for individuals living with demyelinating disorders.

Demyelinating Disorders: Types, Causers, Symptoms, Treatments

Most of the nerves in your body are covered with a protective layer called myelin. It’s a lot like the insulation on electric wires. It helps messages from your brain move quickly and smoothly through your body, the way electricity flows from a power source.

Demyelinating disorders are any conditions that damage myelin. When this happens, scar tissue forms in its place. Brain signals can’t move across scar tissue as quickly, so your nerves don’t work as well as they should.

Symptoms: The most common symptoms of demyelinating disorders are:

Causes: In many cases, doctors aren’t sure what causes these conditions. They know that some result from:

• A virus
• Inflammation from an immune response that goes awry and causes your body to attack its own tissues; you may hear this called an autoimmune condition.
• Your genes
• Damage to blood vessels in your brain
• Lack of oxygen to the brain

Treatment: There’s no cure for these conditions, so early treatment is important. Your doctor will work with you to:

• Lessen the effects of the attack
• Control the disease course
• Manage your symptoms

Medications can ease your pain, fatigue, and stiff muscles. Physical therapy can help with muscles that don’t work the way they used to.

What Are the Types?

There are several demyelinating diseases:

Multiple Sclerosis (MS)

This is the most common demyelinating disorder. One in 500 people have it. It’s an autoimmune condition that attacks your brain, spinal cord, and optic nerve. There are four main courses of the disease that predict how fast it will progress. MS is more likely to affect women. It has a genetic link and may also be triggered by something in a person’s environment.

The most common symptoms are:

• Extreme fatigue
• Vision problems
• Trouble moving
• Tingling, burning, or other odd feelings

There’s no cure, but there are medications to change the course and lower the number of relapses. Plus there are many treatments and techniques to keep your symptoms in check. Know more about the early signs and symptoms of MS.

Acute Disseminated Encephalomyelitis (ADEM)

Children are more likely to get this brief but widespread bout of inflammation that damages myelin in the brain and spinal cord. Sometimes it affects the optic nerve, which connects your eye to your brain. You get ADEM when your body attacks its own tissues in response to an infection with a virus or bacteria. It’s rare, but it can also be a reaction to a vaccine. Sometimes the cause is unknown.

Symptoms usually come on quickly. They include:

Drugs that fight inflammation can stop the damage to the nerves in your brain and spinal cord. A doctor also can prescribe other medicine to ease some ADEM symptoms. Most people recover fully within 6 months, though in very rare cases, ADEM can be deadly. Learn more about the causes and symptoms of ADEM.

Balo’s Disease (Concentric Sclerosis)

Some doctors think of Balo’s disease as a rare form of MS because the symptoms are the same in many ways.

Experts don’t know why people get it, but it can cause serious problems. It can be fatal, but it’s possible to recover fully, too. Asians and people from the Philippines are the most likely to get it. It affects adults more often than children.

Symptoms might come on quickly and get worse in a short amount of time. Or they might go away quickly. They include:

There isn’t a cure for Balo’s disease, and no drugs treat it. Your doctor can suggest medications to help with your symptoms, including steroids to bring down the swelling in your brain and spinal cord. Get more information about Balo’s disease.

Charcot-Marie-Tooth Disease (CMT)

It affects peripheral nerves that lie outside your brain and spinal cord and send signals to the muscles in your limbs. It’s a condition you inherit from your parents when you’re born.

Symptoms usually show up in your late teens or early adult years. But they can come on in midlife, too. You may notice:

• Weakness in your legs, ankles, and feet
• Loss of muscle mass in your legs and feet
• Trouble raising your legs and moving your ankles
• Less feeling in your legs and feet
• Changes to your feet, like higher arches or curled toes
• Trouble walking or running
• Tripping or falling

There’s no cure, but your doctor may give you medicines for pain. They’ll also suggest physical and occupational therapy to help you learn to use any affected limbs. Exercise can help you build stamina and keep muscles strong. Over time, you may need braces and splints for weak joints. Find out more on treatments for Charcot-Marie-Tooth syndrome.

Guillain-Barre Syndrome (GBS)

Like CMT, this condition also attacks peripheral nerves. It often starts with weakness in your legs that moves to your arms and upper body. It can lead to paralysis. And it could be life-threatening if it causes trouble breathing. Doctors don’t know the cause, but it often follows a respiratory or digestive tract infection. Some people get it after surgery or a bout of the flu or a bacterial infection. Most people reach maximum weakness within 2-3 weeks.

The most common symptoms include:

• Tingling in your fingers, toes, ankles, or wrists
• Weakness in your legs that spreads to your upper body
• Trouble walking or climbing stairs
• Bowel or bladder problems
• Trouble moving your face, speaking, or chewing

There’s no cure for GBS. Doctors try to lessen its effects with medication and speed up recovery. Plasma exchange (PLEX) is a common treatment. It removes some of the liquid part of your blood, called plasma, and replaces it with a manmade version. Another option is intravenous immunoglobulin (IVIG). The doctor puts proteins called immunoglobulins into your veins. They’re the same proteins your body uses to attack invaders, but they come from healthy donors. If the disease affects body functions like breathing, you’ll need treatment in a hospital. Caregivers may help move your limbs when you can’t to prevent clots. Later, you’ll get physical therapy to help you use your limbs again. Know more about the causes and symptoms of Guillain-Barre syndrome.

HTLV-I Associated Myelopathy (HAM)

This condition results from a virus called HTLV-1. It can make your brain and spinal cord swell, which causes the symptoms of the disease. Not everyone who has the virus will get HAM. Some people also carry HTLV-1 but have no symptoms.

People with HAM usually live near the equator. You get it by coming into contact with blood or other body fluids of someone who has the disease. It isn’t usually fatal, but it can be. You might live with the disease for decades.

Symptoms include:

• Weakness in your legs that gets worse over time
• Numbness or tingling
• Stiff muscles
• Muscle spasms
• Bladder problems
• Constipation
• Double vision
• Deafness
• Coordination problems
• Tremors

There isn’t a cure, but steroids can help ease your symptoms.

Neuromyelitis Optica (Devic’s Disease)

This rare autoimmune disease can affect your eyes, arms, and legs. You may have blurred vision or lose your eyesight. If it’s in your spinal cord, your legs and arms might not work well.

If you have one attack of neuromyelitis optica, you’ll probably get another. But if your doctor catches the disease early, they’ll have a better chance of treating your symptoms. They may try drugs that turn down your immune system so you don’t have relapses.

Symptoms include:

• Blurred vision
• Loss of eyesight
• Eye pain
• Weak or numb arms and legs
• Bladder and bowel problems
• Vomiting
• Uncontrollable hiccups

Neuromyelitis optica doesn’t have a cure but the drugs eculizumab (Soliris), inebilizumab-Cdon (Uplizna), and satralizumab-mwge (Enspryng) have been approved for treatment. They work by targeting the defective antibodies that attack the healthy cells in your body, triggering NMO. Your doctor may give you a steroid shot to help with swelling. They may also try a treatment called plasma exchange.

Drugs that suppress your immune system, like azathioprine (Azasan, Imuran), methotrexate (Otrexup), mycophenolate (CellCept), and rituximab (Rituxan, Ruxience, Truxima), can help prevent further attacks. Read more on treatment for neuromyelitis optica.

Schilder’s Disease

This rare condition most often affects boys between ages 7 and 12. It wears away myelin in the brain and spinal cord. Severe cases can affect breathing, heart rate, and blood pressure.

Doctors aren’t sure what causes Schilder’s disease, but it usually starts with an infection. Often, a headache and fever are the first symptoms.

This disease is hard to predict. Some people will have flares of symptoms followed by times of recovery. For others, the disease slowly gets worse over time. Signs include:

• Weakness on one side of the body
• Slow movements
• Seizures
• Trouble speaking
• Vision and hearing problems
• Memory problems
• Change in personality
• Weight loss

There’s no cure, but some people can manage their symptoms well with steroids and drugs that calm the immune system. Learn more about Schilder’s disease and how to treat it.

Transverse Myelitis

This spinal cord disorder can cause symptoms throughout your body. It depends on where on your spinal cord you lose myelin. It also makes you more likely to be diagnosed with MS later on. There are about 1,400 new cases of transverse myelitis each year.

It affects kids and adults, but women are more likely to get it than men. Doctors aren’t sure about the cause, but it often follows an infection. Some people have long-lasting effects. Others recover with no problems.

Symptoms include:

• Problems moving your legs
• Bladder and bowel problems
• Lower back pain
• Muscle weakness
• Sensitivity to touch
• Tingling or numbness in your toes
• Fatigue

There’s no cure for transverse myelitis and no FDA-approved medication to treat it. Steroid shots or plasma exchange (PLEX) may bring down the swelling in your spinal cord and ease other symptoms. Get more information about transverse myelitis causes, symptoms, and treatment.

Demyelinating Diseases

Multiple Sclerosis (MS)

Multiple sclerosis (MS) is the most common demyelinating disease of the central
nervous system (CNS). It’s an autoimmune condition that attacks your brain,
spinal cord, and optic nerve. In this disorder, your immune system attacks the
myelin sheath or the cells that produce and maintain it.

This causes inflammation and injury to the sheath and ultimately to the nerve
fibers that it surrounds. The process can result in multiple areas of scarring
(sclerosis).

The most common symptoms are:

There’s no cure, but there are medications to change the course and lower
the number of relapses. Plus there are many treatments and techniques to
keep your symptoms in check.

Acute Disseminated Encephalomyelitis (ADEM)

Children are more likely to get this brief but widespread bout of inflammation
that damages myelin in the brain and spinal cord. Sometimes it affects the
optic nerve, which connects your eye to your brain. You get ADEM when your
body attacks its own tissues in response to an infection with a virus or bacteria.
It’s rare, but it can also be a reaction to a vaccine. Sometimes the cause is unknown.

Symptoms usually come on quickly. They include:

Drugs that fight inflammation a can stop the damage to the nerves in your
brain and spinal cord. A doctor also can prescribe other medicine to ease
some ADEM symptoms. Most people recover fully within 6 months, though in
very rare cases, ADEM can be deadly.

Balo’s Disease (Concentric Sclerosis)

Some doctors think of Balo’s disease as a rare form of MS because
the symptoms are the same in many ways. Experts don’t know why people
get it, but it can cause serious problems. It can be fatal, but it’s
possible to recover fully, too. Asians and people from the Philippines are
the most likely to get it. It affects adults more often than children.

Symptoms might come on quickly and get worse in a short amount of time and
then they might go away quickly. They include:

There isn’t a cure for Balo’s disease, and no drugs treat it. Your doctor
can suggest medications to help with your symptoms, including steroids to bring
down the swelling in your brain and spinal cord.

Charcot-Marie-Tooth Disease (CMT)

It affects peripheral nerves that lie outside your brain and spinal cord and
send signals to the muscles in your limbs. It’s a condition you inherit
from your parents when you’re born.

Symptoms usually show up in your late teens or early adult years. But they can
come on in midlife, too. You may notice:

There’s no cure, but your doctor will may give you medicines for pain. They
should also suggest physical and occupational therapy to help you learn to use
any affected limbs. Exercise can help you build stamina and keep muscles strong.
Over time, you may need braces and splints for weak joints.

Guillain-Barre Syndrome (GBS)

Like CMT, this condition also attacks peripheral nerves. It often starts with
weakness in your legs that moves to your arms and upper body. It can lead to
paralysis. And it could be life-threatening if it causes trouble breathing.
Doctors don’t know the cause, but it often follows a respiratory or digestive
tract infection. Some people get it after surgery or a bout of the Zika virus.
Most people reach maximum weakness within 2-3 weeks.

The most common symptoms include:

There’s no cure for GBS. Doctors try to lessen its effects with medication
and speed up recovery. Plasma exchange (PLEX) is a common treatment. It removes
some of the liquid part of your blood, called plasma, and replaces it with a
manmade version. Another option is intravenous immunoglobulin (IVIG). The doctor
puts proteins called immunoglobulins into your veins. They’re the same proteins
your body uses to attack invaders, but they come from healthy donors. If the
disease affects body functions like breathing, you’ll need treatment in a hospital.
Caregivers may help move your limbs when you can’t to prevent clots. Later, you’ll
get physical therapy to help you use your limbs again.

HTLV-I Associated Myelopathy (HAM)

This condition results from a virus called HTLV-1. It can make your brain and
spinal cord swell, which causes the symptoms of the disease. Not everyone who
has the virus will get HAM. Some people also carry HTLV-1 but have no symptoms.

People with HAM usually live near the equator. You get it by coming into contact
with blood or other body fluids of someone who has the disease. It isn’t
usually fatal, but it can be. You might can live with the disease for decades.

Symptoms include:

There isn’t a cure, but steroids can help ease your symptoms.

Neuromyelitis Optica (Devic’s Disease)

This rare disease can affect your eyes, arms, and legs. Doctors don’t know
what causes it, but they do know it makes your body attack your optic nerve
and spinal cord. You may have blurred vision or lose your eyesight. If it’s
in your spinal cord, your legs and arms might not work well.

If you have one attack of neuromyelitis optica, you’ll probably get another.
But if your doctor catches the disease early, they will have a better chance
of treating your symptoms. He may try drugs that turn down your immune system
so you don’t have relapses.

Symptoms include:

Neuromyelitis optica doesn’t have a cure or FDA-approved medications to
treat it. Your doctor may give you a steroid shot to help with swelling. He
may also try a treatment called plasma exchange.

Drugs that suppress your immune system, like azathioprine, methotrexate,
mycophenolate, and rituximab, can help prevent further attacks.

Schilder’s Disease

This rare condition most often affects boys between ages 7 and 12. It wears
away myelin in the brain and spinal cord. Severe cases can affect breathing,
heart rate, and blood pressure.

Doctors aren’t sure what causes Schilder’s disease, but it usually starts
with an infection. Often, a headache and fever are the first symptoms.

This disease is hard to predict. Some people will have flares of symptoms
followed by times of recovery. For others, the disease slowly gets worse
over time. Signs include:

There’s no cure, but some people can manage their symptoms well
with steroids and drugs that calm the immune system.

Transverse Myelitis

Transverse myelitis is an inflammation of your spinal cord. It results
from damage to nerve cells in a certain area. This spinal cord disorder
can cause symptoms throughout your body. It depends on the specific
location of the spinal cord that myelin is lost.

The condition may show up as a symptom of neuromyelitis optica. It also
makes you more likely to be diagnosed with MS later on. There are about
1,400 new cases of transverse myelitis each year.

It affects kids and adults, but women are more likely to get it than
men. Doctors aren’t sure about the cause, but it often follows
an infection. Some people have long-lasting effects. Others recover
with no problems.

Symptoms include:

There’s no cure for transverse myelitis and no FDA-approved medication
to treat it. Steroid shots or plasma exchange (PLEX) may bring down the
swelling in your spinal cord and ease other symptoms.

Demyelinating disease: What can you do about it?

Answer Section

A demyelinating disease is any condition that results in damage to the protective covering (myelin sheath) that surrounds nerve fibers in your brain, optic nerves and spinal cord. When the myelin sheath is damaged, nerve impulses slow or even stop, causing neurological problems.

Multiple sclerosis

Multiple sclerosis (MS) is the most common demyelinating disease of the central nervous system. In this disorder, your immune system attacks the myelin sheath or the cells that produce and maintain it.

This causes inflammation and injury to the sheath and ultimately to the nerve fibers that it surrounds. The process can result in multiple areas of scarring (sclerosis).

Other causes

Other types of demyelinating disease and their causes include:

• Optic neuritis — inflammation of the optic nerve in one or both eyes
• Neuromyelitis optica (Devic’s disease) — inflammation and demyelination of the central nervous system, especially of the optic nerve and spinal cord
• Transverse myelitis — inflammation of the spinal cord
• Acute disseminated encephalomyelitis — inflammation of the brain and spinal cord
• Adrenoleukodystrophy and adrenomyeloneuropathy — rare, inherited metabolic disorders

MS and other demyelinating diseases most commonly result in vision loss, muscle weakness, muscle stiffness and spasms, loss of coordination, change in sensation, pain, and changes in bladder and bowel function.

Treatment

No cures exist for demyelinating diseases and their progression, and symptoms are different for everyone. Getting treatment early is important. Treatment focuses on:

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

A variety of drug therapies are recommended depending on your specific disorder. Strategies to treat symptoms include physical therapy, muscle relaxing drugs, and medications to reduce pain and fatigue. Talk with your doctor about the best course of treatment for your specific disorder.

Last Updated: May 5th, 2020

Adrenoleukodystrophy (ALD) | Johns Hopkins Medicine

What is ALD?

Adrenoleukodystrophy (ALD) is a genetic condition that damages the membrane (myelin sheath) that covers nerve cells in the brain and spinal cord. Myelin acts as insulation around the nerve fibers. When this insolating layer is damaged, nerve signals from the brain cannot communicate across the body properly, causing impaired bodily functions or paralysis.

ALD prevents the body from breaking down very long chain fatty acids (VLCFAs), causing these fatty acid chains to build up in the brain, nervous system and adrenal gland. The accumulation is thought to cause inflammation in the body, damaging the myelin sheath.

How is ALD Inherited?

ALD is a genetic condition that may be inherited from one or both parents. ALD most severely affects males when it can either present during childhood or during adulthood. Women who are carriers for ALD develop a milder form of the disease during adulthood.

ALD Symptoms

Symptoms of ALD often begin between the ages of 4 and 10 but can also present much later in life. ALD symptoms include:

• loss of vision
• learning disabilities
• dysphagia (difficulty swallowing)
• seizures
• deafness
• lack of coordination and balance
• fatigue
• intermittent vomiting
• weight loss
• lack of appetite
• nausea
• darkening of the skin
• progressive dementia
• muscle weakness
• low blood glucose (blood sugar)
• headaches in the morning

Adrenomyeloneuropathy

Adrenomyeloneuropathy is an adult-onset form of ALD that progresses slowly over decades. Symptoms may include a stiff gait when walking and bladder and bowel dysfunction. Many male patients often end up in need of a wheelchair. The adrenal glands often fail to produce enough steroid (cortisol) in people who have ALD, causing Addison’s disease.

ALD Diagnosis

After a thorough evaluation of the patient’s medical history, if doctors think the patient may have ALD, they will request additional testing: First, a blood test is performed to measure the levels of VLCFA. High levels of VLCFA suggest a possible ALD diagnosis. To confirm this diagnosis, a genetic test is ordered. If ALD is diagnosed, the doctor may recommend that the patient’s family members receive genetic testing. Many states have also started newborn screening for ALD.

ALD Treatment

Medications and physical therapy may be used to treat ALD symptoms. If the patient is diagnosed as a child or at an early stage of ALD, a stem cell transplant may be a promising treatment to stop the progression of ALD.

Myelin Sheath Definition, Function & Demyelinating Diseases

The myelin sheath helps insulate the nervous system and is vital for optimal cognitive function and brain health. Read on to learn more about its purpose and diseases associated with its loss or dysmyelination.

What Is the Myelin Sheath?

Definition & Facts

The myelin sheath is a cover made out of fats and proteins that wraps around the axons (projection) of nerve cells. It insulates neurons so they can send electrical signals faster and more efficiently. This supports brain health and nervous system function [1, 2].

Here are some quick facts about myelin:

• About 80% fats/cholesterol and 20% proteins.
• Considered an outgrowth or extension of a type of glial cell (oligodendrocyte – CNS, Schwann cell – PNS).
• Continues to grow throughout adolescence and even into our early 20s.
• Myelinated axons are white in appearance, hence the term “white matter” of the brain.

Function

Myelin improves the conduction of action potentials, which are needed to send information down the axon to other neurons [3].

The myelin sheath increases the speed of impulses in neurons. It facilitates conduction in nerves while saving space and energy [1].

Myelin helps prevent the electrical current from leaving the axon. It allows for larger body sizes by maintaining efficient communication at long distances.

When babies are born, many of their nerves lack mature myelin sheaths. As a result, their movements are jerky, uncoordinated, and awkward. Scientists think that, as myelin sheaths develop, movements become smoother, more purposeful, and more coordinated [4, 5].

Research suggests that myelination might improve children’s cognitive performance improves as they grow and develop [6].

Additionally, when a peripheral fiber is severed, the myelin sheath provides a track along which regrowth can occur [7].

The myelin sheath enables neurons to conduct action potentials, increasing the speed of their transmission.

When Does Myelination Stop?

Researchers think that myelination occurs most significantly during childhood, but some brain imaging studies suggest it may continue until 55 years of age and possibly even throughout life [8].

Oligodendrocytes vs. Schwann Cells

Oligodendrocytes and Schwann cells are types of cells that produce, maintain, and repair myelin [9].

Schwann cells normally produce myelin in peripheral nerves (outside the brain), but can enter the brain when needed [9].

On the other hand, oligodendrocytes are found solely in the brain. They are responsible for the formation of new myelin in both the injured and healthy adult brains [9].

Symptoms and Conditions Linked With Myelin Loss or Damage

Demyelination

Demyelination refers to myelin damage or loss. It disrupts signals between neurons and may result in a diverse range of neurological symptoms. These depend on whether peripheral (outside the brain) or central (in the brain and spinal cord) neurons are affected, and to what extent [10].

Symptoms

Symptoms differ from patient to patient and have different presentations, depending on the specific demyelinating disorder. The most common demyelinating disorder affecting the central nervous system is Multiple Sclerosis [10].

Thus, symptoms shown here are commonly associated with demyelinating disorders. This list is not exhaustive. The most important step is to see your doctor or other health care professional to get an accurate diagnosis and treatment [11].

• Blurred vision that may affect only one eye
• Double vision
• Loss of vision/hearing
• Odd sensation in legs, arms, chest, or face, such as tingling or numbness (neuropathy)
• Muscle weakness
• Cognitive dysfunction, including speech impairment and memory loss
• Heat sensitivity
• Loss of dexterity
• Difficulty coordinating movement and/or balance
• Difficulty controlling bowel movements and/or urination
• Fatigue
• Tinnitus

Symptoms of demyelinating disorders include complex visual and sensory changes that vary from person to person depending on the underlying cause.

Demyelinating Disorders

Multiple sclerosis is the most common demyelinating disorder. The cause of multiple sclerosis is unknown, though many contributing factors have been proposed [10].

The following are more rare types of demyelinating disorders [10, 12]:

• Acute disseminated encephalomyelitis
• Acute hemorrhagic leukoencephalitis
• Neuromyelitis optica
• Chronic inflammatory demyelinating polyneuropathy
• Central pontine myelinosis
• Inherited demyelinating diseases such as leukodystrophy
• Charcot-Marie-Tooth disease
• Adrenoleukodystrophy and adrenomyeloneuropathy
• Leber hereditary optic neuropathy
• Progressive multifocal leukoencephalopathy

The exact cause of many demyelinating disorders is often an enigma. Science suggests that certain primary demyelinating disorders develop after a viral infection or vaccination against viral infection [10].

Some researchers hypothesize that this might be because a virus or another substance somehow triggers the immune system to attack the body’s own tissues (autoimmune reaction). The autoimmune reaction results in inflammation, which damages the myelin sheath and the nerve fiber under it [10, 12].

However, this hypothesis holds only for specific, rare demyelinating disorders [10, 12].

HIV infection can also cause white matter abnormalities, including myelin damage [10].

Multiple sclerosis is the most common demyelinating disorder. It causes progressive loss of the myelin sheath.

Genetic Myelin Sheath Disorders

The following are some genetic disorders of myelin [13, 14]:

• Adrenoleukodystrophy
• Tay-Sachs disease
• Niemann-Pick disease
• Gaucher disease
• Hurler syndrome
• Canavan disease
• Charcot-Marie-Tooth disease
• Krabbe’s leukodystrophy
• Phenylketonuria

Other Poorly-Researched Associations

Aside from demyelinating disorders, limited studies have linked the following disorders to white matter or myelin loss or damage:

• PTSD [8]
• ADHD [15]
• Depression [16]
• Chronic Fatigue Syndrome [17]
• Schizophrenia [8]
• Mild cognitive impairment in Alzheimer’s disease [18]
• Traumatic brain injury [19]
• Tourette’s syndrome [8]
• Tone deafness [8]
• Pathological lying [8]
• Guillain-Barré syndrome [20]
• Diabetes [21]
• Nutritional deficiencies (such as B12 deficiency) [22]
• Poisoning with lead, carbon monoxide, or deadly plants like rosary pea [23, 24, 25]
• Drugs (such as the antibiotic ethambutol used to treat tuberculosis) [26]

According to some theories, reduced white matter in the brain is a contributing factor to some brain-related conditions. Also, scientists think that certain conditions are caused by white matter reductions. At other times, science suggests that specific conditions themselves may cause white matter reduction [27].

However, many of these links are purely investigational and lack large-scale human data as support.

Additionally, the majority of studies that focused on these conditions dealt with associations only, which means that a cause-and-effect relationship hasn’t been established.

For example, just because depression has been linked with altered white matter (made up of myelin) in certain brain areas doesn’t mean that depression is caused by myelin damage. Data are lacking to make such claims.

Also, even if a study did find that poor myelination contributes to depression, myelin is highly unlikely to be the only causative factor. Complex disorders like depression always involve multiple possible factors – including brain chemistry, environment, health status, and genetics – that may vary from one person to another.

Therefore, more research is needed to determine the association between these disorders and myelin or white matter abnormalities.

Myelin loss has been linked with many disorders (including ADHD and TBI) in small-scale experimental studies; large-scale research is needed.

What Happens to Myelin in Multiple Sclerosis?

Multiple sclerosis is an autoimmune condition in which the person’s immune system attacks the myelin sheath or cells that maintain it [28].

As myelin continues to degrade, symptoms such as impaired balance, and cognition may arise [10].

Cognitive impairment occurs in 40 to 65% of multiple sclerosis patients. The deficits are typically in complex attention, information processing speed, (episodic) memory, and executive functions [29].

Myelin loss can impair or stop the conduction of signals along nerves, eventually causing groups of nerves to wither. Some scientists believe that inflammatory cytokines reduce myelination [30, 10].

Myelin & Intelligence

Do We Have a Clear Link?

Since science discovered that myelin works as a sort of a fine cable for the brain – helping carry electrical signals between neurons at high speeds – some have suggested that it may play a critical role in determining intelligence [31].

Since myelin allows signals to travel faster, researchers think it might make the brain as a whole work better. We know that white matter – nerve fibers coated with myelin – support normal cognitive function, learning, and IQ. But the specifics of the “myelin intelligence” theory have yet to be determined in humans [8, 32].

It’s important to know that studies attempting to find links between brain factors like myelin and intelligence are bound to have inconsistencies and flaws. Intelligence is a complex trait and most studies can only point to possible links. But many factors interact to shape its various aspects: general, verbal, mathematical, emotional, and other types of intelligence [32].

Myelin controls the speed of impulse conduction through axons, and the synchrony of impulse traffic between distant brain regions seems to be critical for optimal mental performance and learning [8].

Limited studies suggest that myelination of appropriate brain regions coincides with the development of specific cognitive functions, such as reading, development of vocabulary, and proficiency in executive decision making [8].

Incomplete myelination of the forebrain until the early twenties has been suggested as a neurological basis for weaker decision-making skills in adolescence, though this association remains controversial [33].

Small studies have suggested a correlation between individual differences in normal cognitive development, IQ, reading skills, working memory, and musical proficiency with differences in white matter in specific brain regions mediating these tasks. Future large-scale studies are needed to clarify this link [33].

In another small study, white matter levels in certain regions were also associated with arithmetic ability, reaction time, and cognitive control [27].

Learning complex skills, such as playing the piano, appear to be accompanied by increased white matter in brain areas involved in musical performance. White matter increased proportionately to the number of hours each subject had practiced the instrument, indicating white matter increases when acquiring certain skills [33].

However, such studies have limitations that make it difficult to determine whether learning a new skill can directly impact myelination.

A couple of studies have noted a positive correlation between white matter volume and intelligence at the level of whole brain white matter volume as well as in specific white matter regions [34].

Additionally, researchers suggest that prefrontal white matter volume is disproportionately larger in humans compared to non-human primates [35].

One unverified theory says that the more myelin a person has, the higher their cognitive capacities. Although plausible, this theory is highly uncertain.

Summary

To sum it up, white matter and myelin have been associated with the following aspects of intelligence in limited, small studies [27]:

• Working memory
• IQ
• Verbal ability
• Reaction time
• Cognitive control
• Musical ability
• Attention
• Arithmetic capacities

However, larger and better-designed studies are needed before we can draw any clear conclusions.

Takeaway

The myelin sheath is a protective coating that wraps around brain cells, enabling them to send out signals at high speed.

Demyelination refers to a loss or damage of the myelin sheath. The most common demyelinating disorder is multiple sclerosis, though many other disorders have been associated with demyelination.

Symptoms of demyelinating disorders include complex visual, sensory, and other neurological changes. These symptoms vary from person to person depending on the underlying cause. It’s essential to see a doctor to get an accurate diagnosis and treatment.

Learn More

Chronic Inflammatory Demyelinating Polyradiculoneuropathy | Cedars-Sinai

Not what you’re looking for?

What is chronic inflammatory demyelinating polyradiculoneuropathy?

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare type of
autoimmune disorder. In an autoimmune disease, the body attacks its own tissues. In
CIDP, the body attacks the myelin sheaths. These are the fatty coverings on the fibers
that insulate and protect the nerves.

Experts think that CIDP is related
to the more commonly known disease Guillain-Barré syndrome (GBS). GBS is generally
considered a short-term (acute) disease. CIDP is considered a long-term (chronic)
disease. CIDP is less common than GBS.

CIDP is usually classified as follows:

• Progressive. The disease continues to worsen over
  time.
• Recurrent. Episodes of symptoms stop
  and start.
• Monophasic. This means one bout of the disease lasts 1 to
  3 years and doesn’t recur.

What causes CIDP?

CIDP occurs when the body’s immune
system attacks the myelin sheaths around nerve cells. But exactly what triggers this
isn’t clear. Unlike GBS, there usually isn’t an infection preceding CIDP.
There doesn’t seem to be a genetic link to CIDP.

Who is at risk for CIDP?

CIDP can occur in anyone. But
people in their 50s and 60s seem more likely to develop it than people in other age
groups. Men are twice as likely as women to get the disease.

What are the symptoms of CIDP?

Symptoms are usually the same for
all types of CIDP. They can include:

• Tingling in the arms and legs
• Gradual weakening of the arms and
  legs
• Loss of reflexes
• Loss of balance and your ability to
  walk
• Loss of feeling in the arms and legs,
  which often starts with not being able to feel a pin prick

How is CIDP diagnosed?

Because CIDP is rare, it’s often
hard to correctly diagnose the disease, at least at first. Healthcare providers can
confuse its symptoms with those of GBS because of the similarity between the diseases.
If symptoms last longer than 8 weeks, a provider may suspect CIDP.

After taking a medical history and
doing a physical exam, a healthcare provider may do two or more tests to confirm a
diagnosis. These may include:

• Blood and urine tests
• A nerve conduction study
  (electromyogram) to look for myelin damage in peripheral nerves. This involves using
  low electrical currents to test nerve function and response.
• A lumbar puncture to see if levels of
  certain proteins related to the disease are higher than normal. To do this,a small
  needle is inserted into the back and a small amount of the fluid that surrounds the
  spinal cord (cerebrospinal fluid) is withdrawn. .
• A nerve biopsy to look at microscopic changes in the nerves.
  This test is rarely done.

How is CIDP treated?

Treatment for CIDP is often
effective. Some studies show that up to 4 in 5 people respond well to therapy. Because
it’s an autoimmune disorder, healthcare providers use medicines that suppress the
immune
response to treat CIDP. Medical teams tailor your treatment to each person and closely
monitor their progress. Treatments for CIDP include:

• Immunosuppresive medicines
• Steroids
• Intravenous immunoglobulin
• Plasma exchange (plasmapheresis)
• Monoclonal antibody therapy

Living with CIDP

The course of CIDP can vary greatly
among people, as can the response to treatment.

Getting treatment as early as
possible is very important because it gives them the best chance of limiting symptoms
and keeping this condition under control. If they don’t seek treatment for CIDP,
symptoms will likely get worse over the course of several years. These can range from
sensory symptoms, such as tingling and numbness, to weakness and loss of balance.
Without treatment, 1 in 3 people with CIDP will need a wheelchair.

In people with permanent physical
impairments, physical therapy can be very important. In this treatment, specialists
work
with them to maintain or increase strength and improve coordination. Another type
of
therapy is occupational therapy. It helps people learn new ways of doing everyday
tasks
in spite of new physical limitations.

Some people with physical
disabilities often feel sad or depressed. If this happens, a healthcare provider may
recommend seeing a mental health provider. Antidepressants and psychotherapy can help
treat depression. So can support groups for people managing chronic health
conditions.

When should I call my healthcare provider?

If you have been diagnosed with
CIDP, talk with your healthcare provider about when you might need to call them. They’ll
likely advise you to call if you notice worsening of any symptoms or if you develop
any
new symptoms.

Key points about CIDP

• Chronic inflammatory demyelinating
  polyradiculoneuropathy (CIDP) is a slowly developing autoimmune disorder in which
  the
  body’s immune system attacks the myelin that insulates and protects the body’s
  nerves. The exact cause isn’t known.
• Common symptoms are gradual weakness or sensation changes in the arms or legs. This
  might get worse over time, or it might come and go.
• Early treatment is important to limit
  disease progression. It includes medicines or other treatments to suppress the immune
  system.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

• Know the reason for your visit and what you want to happen.
• Before your visit, write down questions you want answered.
• Bring someone with you to help you ask questions and remember what your provider tells
  you.
• At the visit, write down the name of a
  new diagnosis and any new medicines, treatments, or tests. Also write down any new
  instructions your provider gives you.
• Know why a new medicine or treatment
  is prescribed, and how it will help you. Also know what the side effects are.
• Ask if your condition can be treated in other ways.
• Know why a test or procedure is recommended and what the results could mean.
• Know what to expect if you do not take
  the medicine or have the test or procedure.
• If you have a follow-up appointment, write down the date, time, and purpose for that
  visit.
• Know how you can contact your provider if you have questions.

Medical Reviewer: Joseph Campellone MD

Medical Reviewer: Rita Sather RN

Medical Reviewer: Raymond Kent Turley BSN MSN RN

© 2000-2021 The StayWell Company, LLC. All rights reserved. This information is not intended as a substitute for professional medical care. Always follow your healthcare professional’s instructions.

Not what you’re looking for?

Multiple sclerosis

2

Not All Multiple Sclerosis-Like Diseases Are Alike

May 19, 2020 — Scientists say some myelin-damaging disorders have a distinctive pathology that groups them into a unique disease …

Slowing the Progression of Multiple Sclerosis

Nov. 13, 2019 — Over 77,000 Canadians are living with multiple sclerosis, a disease whose causes still remain unknown. Presently, they have no hope for a cure. Researchers have now identified a molecule named ALCAM …

Simple Sugar Possible Therapy for Repairing Myelin in Multiple Sclerosis

Oct. 7, 2020 — N-acetylglucosamine, a simple sugar found in human breast milk and sold as an over-the-counter dietary supplement in the United States, promotes myelin repair in mouse models and correlates with …

Gut Microbes Protect Against Neurologic Damage from Viral Infections

July 16, 2019 — Gut microbes produce compounds that prime immune cells to destroy harmful viruses in the brain and nervous system, according to a mouse …

A Tool to Interrogate a New Class of Drugs

Sep. 30, 2021 — Reactive electrophilic drugs like Tecfidera, approved for the treatment of relapsing multiple sclerosis, show a lot of potential but are also mystery. Scientists have now used an innovative chemical …

In Children With Multiple Sclerosis, Teriflunomide Tempers Lesion Growth

Nov. 30, 2021 — A phase 3 clinical trial tested the safety and efficacy of teriflunomide, an oral immunomodulatory drug, in children with multiple sclerosis. Although the medication did not prevent disease relapses …

Food Allergies and Multiple Sclerosis: New Link

Feb. 19, 2019 — Investigating the correlation between allergy and inflammatory disease activity, a team of investigators has found new evidence connecting food allergies and relapses of multiple …

Multiple Sclerosis: Helping Cells to Help Themselves

Jan. 22, 2019 — Diseases such as multiple sclerosis are characterized by damage to the ‘myelin sheath’, a protective covering wrapped around nerve cells akin to insulation around an electrical wire. …

Medicare Patients With Multiple Sclerosis Bear the Burden of Rising Drug Prices

Aug. 26, 2019 — In a decade, Medicare recipients saw a sevenfold increase in out of pocket costs for multiple sclerosis drugs. Spending on these drugs by Medicare itself increased by …

Vegetable-Rich Diet Lowers Fatigue in Multiple Sclerosis Patients by Raising Good Cholesterol

Aug. 15, 2019 — Higher levels of blood high-density lipoprotein (HDL) — or good cholesterol — may improve fatigue in multiple sclerosis patients, according to a new …

90,000 Demyelinating diseases “Lakhta Clinic

Demyelinating diseases

Communication between individual functional nerve cells (neurons) is carried out through a complex system of thin conductive outgrowths, their branches and plexuses (respectively, axons and dendrites, as well as synapses as switching points). These microscopic “connecting cables” of the nervous system are coated on the outside with a special type of membrane – the myelin sheath.Myelin is about 25-30% protein; the remainder is accounted for by lipid (fatty) compounds. As far as is known today, the myelin sheath performs several functions: ensuring the mechanical strength of the conductor, protecting against destructive external influences at the cellular and molecular levels, nourishing the axon, its electrochemical isolation, and accelerating the transmission of nerve impulses. In the future, it is likely that ideas about the purpose of the myelin sheath will expand; today it is generally accepted that the last two functions, the insulating and accelerating ones, are the most important.It is also known that in terms of the degree of myelination of axons of the central and peripheral nervous system (respectively, the central nervous system and the PNS), the human body surpasses the body of any other type of living creature, including the great apes. Perhaps it was this biochemical mechanism, which accidentally appeared due to endless spontaneous mutations, and then fixed and developed in the course of evolution, which provided the Homo Sapiens species not only with reliable survival, but also a dominant position in the biosphere of the planet.

Normally, the processes of demyelination (natural disintegration of the myelin layer) and remyelination (its restoration) are balanced and continue, apparently, throughout life.However, due to a number of unfavorable conditions, external and / or internal, the process of destruction of the myelin sheaths may be forced. In general, breakdown or destruction of the myelin layer is the most common type of damage to nerve cells. If demyelination becomes progressive, i.e. progresses steadily, then when 40-60% of all myelin sheaths are damaged, polymorphic or, less often, monosymptomatic neurological syndromes develop, the totality of which is summarized by the term “demyelinating diseases”.

Thus, demyelinating diseases are an extensive group of pathological processes, the basis of which is the destruction of the myelin sheaths of axons. In this case, various parts of the central nervous system or PNS can be predominantly affected, which inevitably affects the functional viability of these zones and is manifested by the appropriate clinic, or the nervous system as a whole can be involved, which leads to the generalization of symptoms.

More than two dozen distinctly demyelinating diseases are currently known, and the list is likely to grow.Some of them are congenital, some are acquired. The prevalence, annual incidence and other epidemiological parameters of this group of diseases fluctuate in a wide range – from very rare hereditary syndromes to ubiquitous forms. In particular, multiple sclerosis, which today is the leading frequency of occurrence among all known demyelinating processes, in some regions is recorded with an annual frequency of 1: 1000 of the population and even more often.

Reasons

According to the pathogenetic criterion, myelinopathies and myelinoclasts are conventionally distinguished in the group of demyelinating diseases. The first term implies an abnormal, unstable biochemical structure of myelin itself (usually due to genetic disorders), the second – the destruction of the membranes by some factors. The conventionality of such a classification is that genetically determined myelinopathy can be triggered by external causes, and vice versa, in patients with acquired myelinoclasts in history, a hereditary predisposition is often found.

Demyelinating diseases are hereditary diseases of Tay-Sachs, Leber, Niemann-Pick, Canavan, Alexander, some leukodystrophies and fermentopathies.

Acquired myelinoclasts with an inflammatory component include some processes caused by chronic hypoxia or local ischemia (lack of blood supply), alimentary factors (vitamin-poor diet, lack of nutrients), viral infection, immunopathological response to vaccines, as well as intoxication, incl.including alcoholic.

It has been established that the direct factor in myelin destruction is, as a rule, self-immunity, i.e. most demyelinating diseases are simultaneously autoimmune.

In some cases, demyelinating disease is primary, independent, moreover, idiopathic and / or polyetiological in nature (see “Multiple sclerosis”). The central nervous system (spinal cord and brain) is affected more often and more intensively, an example of which is, in particular, Binswanger’s disease; examples of demyelination of the peripheral nervous system are Guillain-Barré syndrome, Charcot-Marie-Tooth disease, etc.In some cases, demyelination is selective, selective, clearly localized (see, for example, “Devik’s opticomyelitis”).

Symptoms

Considering the above, it is easy to imagine the diversity of the clinical picture of demyelinating diseases: it depends on the preferential localization (or generalization) of foci of myelin destruction, that is, it can include virtually any neuropsychiatric symptoms and syndromes: motor, perceptual, cognitive (dementia), atactic ( disorders of balance and coordination), speech, pelvic, affective, extrapyramidal, etc.At the same time, demyelination itself does not have its own symptomatology – at least its pathognomonic signs are currently unknown.

Such diseases are very different in terms of prognosis: from a slow smooth progression over decades to a rapid unstoppable increase with a lethal outcome after a few months.

Diagnostics

In many cases, the intravital diagnosis of demyelinating diseases is very difficult and problematic.A deep study of all available anamnestic information is necessary in comparison with the clinic and the data of objectifying studies. From instrumental methods, the most informative are MRI and electroneuromyography, from laboratory methods – various immunological tests.

Treatment

The main directions of therapy for demyelinating diseases are, as a rule, the appointment of anti-inflammatory hormones, immunosuppressants, immunocorrectors and immunomodulators, as well as nootropics, neuroprotective agents and other drugs according to indications.As a rule, it is necessary to reconsider the way of life, a categorical rejection of bad habits, adherence to a special gentle regimen of stress and rest. There is still no single etiopathogenetic treatment regimen; therapy is most often palliative, symptomatic. Active research is underway to find ways of intensive remyelination; developments in the field of gene and hormonal therapy are considered to be the most promising.

90,000 Glial cell injections relieve multiple sclerosis in mice – Science

TASS, May 19.Neurophysiologists have successfully suppressed all the symptoms of multiple sclerosis in mice by introducing into their brains a culture of “blanks” of glial cells – auxiliary bodies of the nervous tissue. The press service of the University of Rochester writes about this with reference to an article in the journal Cell Reports.

On this topic

“Our experiments show that transplantation of glial cells can completely restore the structure of the myelin sheath of nerve cells in the human brain. the authors of the study, professor at the University of Rochester (USA) Stephen Goldman.

Multiple sclerosis is an autoimmune disease of the nervous system in which cells of the immune system begin to attack the sheath of nerve fibers, the so-called myelin. Because of this, the nerves begin to conduct signals worse and “short circuit”. At first, as a result of this, the limbs become slightly numb, and if the disease progresses, paralysis, blindness may appear and, in the end, the patient may die.

According to current statistics from the World Health Organization (WHO), about 2.3 million people now suffer from multiple sclerosis.At the same time, there are no full-fledged drugs that could completely suppress it. Thousands of biologists are working on solving the problems associated with this disease.

Goldman and colleagues conducted the first trials of an experimental treatment for multiple sclerosis. It is based on the culture of so-called glial cells – auxiliary bodies of the nervous system, some of which protect neurons from damage and pathogens, while others produce myelin.

Brain Cell Therapy

Scientists have long assumed that multiple sclerosis can be stopped or significantly slowed down with the help of “blanks” of glial cells, which can turn into so-called oligodendrocytes – cells that produce myelin.Injections of these cells can repair damage to the glial tissue and make it work more efficiently.

On this topic

Danish and American neurophysiologists have tested this hypothesis using human glial cells and transgenic mice. Changes were made in the genome of these animals, due to which mutations appeared in the MBP gene, which is responsible for the synthesis of myelin in oligodendrocytes. These rodents are born with symptoms similar to human multiple sclerosis and die 2-3 months after birth.

Scientists tried to protect them from a similar fate by introducing “blanks” of glial cells into the brain of animals. Experiments have shown that after injections, this culture was distributed throughout the nervous tissue of mice. As a result, in the first few weeks after the start of the experiment, the myelin sheath of the nerves had time to form.

This prolonged the life of the animals: they were able to live for more than four months, until the very end of the experiment, when scientists euthanized all the mice and began to study the structure of their brains.These studies showed that human cells successfully engrafted and formed a tough myelin sheath that was thicker than normal in mice.

Goldman and his colleagues have achieved similar results while experimenting with healthy mice. Before the experiment, scientists destroyed oligodendrocytes in their brains with cuprizone, a toxin that only kills auxiliary brain cells. Injections of “blanks” of glial cells saved this group of animals from the development of multiple sclerosis and other consequences of the death of oligodendrocytes.

In both cases, as scientists note, such therapy improved the condition and well-being of rodents, returning them to control of the limbs and suppressing a number of other unpleasant symptoms associated with the development of multiple sclerosis. All this, as the researchers conclude, speaks of the prospects of using such a technique for the treatment of humans.

Immunotherapy for peripheral neuropathy caused by paraprotein, an IgM antibody that can bind to MAG, a protein of the myelin sheath of nerve fibers

Review Question

What are the benefits and harms of immunotherapy for peripheral neuropathy caused by the presence of paraprotein, an IgM antibody that can bind to myelin-associated glycoprotein (MAG)?

Relevance

There are several types of antibodies in the human body.They are more or less adapted to target recognition, as a rule, these are foreign proteins, for example, particles of bacteria, viruses or neoplasms. Some people make too many antibodies of the type called paraproteins. Some of these paraproteins are classified as class M immunoglobulins (IgM antibodies are generally referred to as a “rapid response” type of antibody). Some of them can react with a myelin-associated glycoprotein known as MAG.MAG is a molecule on the insulating myelin sheath of nerve fibers. It is likely that this antibody damages the myelin of the nerve fibers to which it binds, leading to a specific type of nerve injury known as peripheral neuropathy. Anti-MAG paraprotein-associated peripheral neuropathy is a disorder that affects men more often than women, usually over 60 years of age. It causes progressive symptoms of sensory impairment, loss of balance and tremors, and sometimes weakness from the feet to the lower leg.

Treatments that affect the immune system, such as plasmapheresis (a procedure in which circulating antibodies are removed and the blood plasma is replaced with pure plasma substitute), intravenous immunoglobulin injections (IVIG; antibodies isolated from donated blood), rituximab (which kills some cells, producing antibodies), corticosteroids, or antineoplastic agents can reduce levels of IgM antibodies that cause neuropathy and slow or prevent disease progression.

Research characteristics

Many of the above treatments have been tried in non-randomized trials, but we found only eight small randomized controlled trials (RCTs), involving 236 people, that met our eligibility criteria.

Results and quality of evidence

Two clinical trials involving 22 and 11 participants (20 of whom had anti-MAG antibodies) suggest that, in some cases, intravenous immunoglobulin (IVIG) may provide short-term, measurable benefits, and it is relatively safe, but this benefit is of questionable clinical meaning.During these clinical trials, there were no reports of severe adverse effects caused by IVIG. A clinical trial of cyclophosphamide and corticosteroids has shown moderate benefit. Two clinical trials of rituximab demonstrated beneficial effects of rituximab, but the evidence was of low quality due to the small number of participants and doubts about the methods of one of the two studies. Several adverse effects of rituximab have been reported, most of them minor.The remaining clinical trials failed to draw conclusions about the effectiveness of other agents and reported a number of serious adverse events. To assess the effectiveness of existing and new treatments, we need well-designed, large-scale RCTs, as well as new approaches by researchers and clinicians to identify changes that people report as a result of treatment.

Evidence is current to February 2016.

Multiple sclerosis: symptoms, causes, diagnosis, treatment

Multiple sclerosis is a chronic neurological pathology characterized by damage to the myelin sheath of the nerve fibers of the spinal cord and brain.The disease leads to the formation of sclerotic plaques in the primary foci of myelin destruction. The symptomatology of the pathology is specific and includes movement disorders, sensitivity disorders, suppression of the functions of the pelvic organs, neuropsychiatric changes.

Drug therapy is aimed at stopping the main manifestations of pathology. In the absence of treatment, the disease leads to a change in the psyche, limitation of motor capabilities, loss of the ability to independently perform elementary actions.

General

Damage to the myelin sheath of nerve fibers is a chronic disease with increasing symptoms. Lesions are formed in the central and peripheral nervous system. In medical reference books, the pathology can be referred to as multiple sclerosing periaxial encephalomyelitis, plaque, multiple or spotted sclerosis.

The first symptoms of the disorder appear in patients aged 20–45 years. Most often, the disease is diagnosed in people engaged in intellectual work. Multiple sclerosis is common in temperate climates. In these geographic areas, there is a record incidence of up to 100 cases per 100 thousand inhabitants.

The reasons for the development of pathology

Periaxial encephalomyelitis is a multifactorial autoimmune pathology.The disease develops under the influence of external factors and hereditary predisposition. In most cases, it is impossible to identify the basic trigger provoking the development of destructive processes in the myelin sheaths of nerve fibers.

Adverse external factors include:

• viral, bacterial and fungal infections;
• human interaction with toxic and radioactive substances;
• unbalanced diet;
• living in an unfavorable ecological environment;
• frequent stress and high intellectual stress.

Genetic susceptibility to multiple sclerosis arises from mutations in genes responsible for immunoregulation. Due to this, the cells of the immune system perceive the nerve fibers as foreign structures and attack them.

Types of disease

Neurologists use a classification based on the clinical manifestations of periaxial encephalomyelitis.Allocate:

• remitting course of pathology;
• secondary progressive course of the disease;
• primary progressive course of the disorder.

The remitting form is diagnosed in 90% of patients suffering from multiple sclerosis. The course of the pathology is accompanied by a regular increase in primary symptoms (attacks last up to a day) and remission that replaces them.Gradually, the periods of stabilization of the state become shorter and shorter.

The secondary progressive course replaces the remitting form of the disease. At this stage, there is a steady progression of symptoms with periods of sharp exacerbations. Strengthening signs of myelin sheath degeneration occurs due to accelerating damage to axons and a decrease in compensatory functions of the brain.

Primary progressive course of multiple sclerosis is detected in 8-10% of clinically registered cases.Patients suffer from steadily increasing signs of nervous system damage without periods of exacerbation and remission.

Multiple Sclerosis Symptoms

The clinical manifestations of pathology are varied. At the onset of the disease, neurologists can record one persistent symptom.As new foci of lesion of nerve fibers form, others join the primary symptom. The most common violations are:

• weakness in the legs;
• partial loss of sensitivity;
• visual defects;
• wobbly gait;
• bouts of vomiting;
• dizziness.

The early stages of multiple sclerosis are characterized by fragmented symptoms.Individual manifestations of pathology rarely overlap.

As the disease progresses, pelvic dysfunction and paresis appear. Muscle weakness is due to damage to the motor pathways of the nervous system. The formation of foci of demyelination of nerve fibers in the cerebellum causes:

• dynamic and static ataxia;
• dysmetria;
• asynergy;
• intentional tremor;
• megalography;
• chanted speech.

Over time, the tremor spreads to the limbs, affects the body and head, pathological wrist reflexes of flexion and extensor types appear.

Diagnostics of pathology

Neurologists use several diagnostic criteria when making a diagnosis of periaxial encephalomyelitis:

• the patient has pronounced signs of a multifocal lesion of the central nervous system;
• growing symptomatology of pathology;
• the unstable nature of individual symptoms;
• remitting or progressive course of the disease.

Laboratory and instrumental diagnostic methods are used to identify foci of damage to the nerve fibers of the spinal cord and brain. The main way to confirm the primary diagnosis is MRI. CT scans indicate areas of demyelination.

In the early stages of pathology, ophthalmological examinations are prescribed. Doctors record disorders caused by optic neuritis. Audiometry and stabilography are used as additional diagnostic measures.

Multiple Sclerosis Treatment

Damage to the myelin sheaths of nerve fibers is a chronic disease. Drug therapy reduces symptoms but does not restore normal functioning of damaged axons.Surgical treatment for multiple sclerosis is not used.

Conservative therapy is based on the following drugs:

• methylprednisolone;
• gastroprotective agents;
• potassium solutions;
• immunosuppressants and immunomodulators;
• antidepressants;
• central nervous system stimulants;
• barbiturates and anticonvulsants.

Treatment is aimed at preventing exacerbations and stabilizing the condition. Doctors seek to stop the transition from relapsing-remitting multiple sclerosis to progressive multiple sclerosis. The achievement of this goal is facilitated by the use of a combination of immunosuppressants and immunomodulators. These agents change the immune balance in the patient’s body and slow down the destruction of the myelin sheaths of nerve fibers by antibodies.

Forecast

Early detection of signs of pathology allows you to form a favorable prognosis.Drug therapy remains the main means of preventing death, which is possible with depression of the functions of the central nervous system. The progressive nature of the pathology implies that within 8–10 years after the diagnosis is made, patients become disabled.

Prevention

Specific measures for the prevention of pathology have not been developed.Persons at risk are advised to undergo regular neurological examinations and promptly treat viral or bacterial infections.

Questions and Answers

– Which doctor treats multiple sclerosis?

– The diagnosis and the development of treatment tactics are carried out by a neurologist.Patients may require the advice of therapists, immunologists, ophthalmologists, and other doctors.

– Why are antidepressants prescribed for patients with multiple sclera?

– Persons suffering from the pathology under consideration often face depressive disorders due to the irreversibility of degenerative processes in the central nervous system. Antidepressants are designed to normalize the condition of patients and reduce the psychoemotional load on them.

– Is multiple sclerosis inherited?

– The disease can develop against the background of genetically determined mutations, but European and American neurologists were unable to detect signs of its inheritance during a long-term study (2011-2016).

Sources and literature

In preparing the article, the following materials were used:

• Korkina M.V., Martynov Yu.S., Malkov G.F. Mental disorders in multiple sclerosis. M., 1986.
• Gusev E.I., Demina T.L. Multiple sclerosis // Consilium Medicum: 2000. – No. 2.
• Jeremy Taylor. Darwin’s health: why we get sick and how it relates to evolution. – M .: Alpina Publisher, 2016.

90,000 New Treatment for Multiple Sclerosis Found?

According to the latest results of the work of scientists, they have made a breakthrough in understanding how the brain can repair itself. This knowledge, in turn, can be used to develop a new revolutionary method for the treatment of multiple sclerosis.It has been shown that a specific cell type responsible for immune control is also involved in the release of a protein that causes myelin growth (a substance that forms the myelin sheath of nerve fibers). The study was carried out by a team of scientists from Queen’s University Belfast in Northern Ireland, UK, and the results are published in the journal Nature Neuroscience. According to one of the authors of the publication, their work will help to understand how the self-healing of the brain and spinal cord occurs.In the study, the researchers used a mouse model.

Multiple sclerosis is a serious chronic disease characterized by damage to the brain and spinal cord. It is believed that the cause of the disease is a malfunction of the immune system, which attacks the myelin sheaths of nerve fibers, while destroying them. As damage accumulates throughout the nervous system, foci of sclerosis are formed, namely, the places where the nerve tissue is replaced by connective tissue. As a result, the patient develops symptoms of the disease such as severe fatigue, pain, problems with vision and coordination of movements, muscle fatigue, numbness, loss of sensitivity, depression, and cognitive impairment.At the moment, around the world, about 2.3 million people have multiple sclerosis. Young people are most often affected by this ailment, in most cases women. As you know, a drug for the treatment of multiple sclerosis has not yet been developed, which is why the researchers are so encouraged by the results of the work.

In this study, scientists drew attention to T cells that are able to enter the central nervous system from the bloodstream. It should also be noted that these cells play an important role in the development of multiple sclerosis.A team of British researchers found that a subtype of lymphocytes – regulatory T cells – are involved not only in immune processes, but also in the initiation of the growth of myelin fibers. This is due to the effect of regulatory T cells on oligodendrocytes – the cells that form myelin.

Scientists have found that regulatory T cells secrete a protein that promotes the conversion of progenitor cells into mature oligodendrocytes. Moreover, it was noted that the regenerative capacity of the myelin sheath was impaired in mice with a deficiency of regulatory T cells.Consequently, scientists have identified the regenerative capacity of regulatory T cells, which is decoupled from their role in immune processes.

Thus, an understanding of the myelin regeneration process can help in the development of new therapies for multiple sclerosis.

Based on materials from www.medicalnewstoday.com

90,000 treatment in Moscow, diagnosis and symptoms

What is neuralgia

Neuralgia – damage to peripheral nerves, which is characterized by excruciating paroxysmal pain.Unlike neuritis, motor and sensory disorders do not occur with neuralgia.

Reasons for the development of neuralgia

The development of neuralgia in certain nerve fibers is due to their anatomical features. So, pathological damage often develops in the nerves that pass through narrow channels and holes.

Essential (primary) neuralgia develops for no apparent reason, clinical examination in this case does not reveal other diseases.It is believed that the cause of essential neuralgia may lie in the pressure exerted on the nerve fibers by the vascular arteriovenous connections.

Symptomatic (secondary) neuralgia is caused by systemic pathological processes such as multiple sclerosis. In multiple sclerosis, the myelin sheath surrounding the nerve fiber is destroyed. The myelin sheath ensures the conduction of nerve impulses and protects the nerve fiber. Further destruction of the nerve fiber occurs.Neuralgia can also be caused by a growing tumor that squeezes the nerve fiber, traumatic injury.

Symptoms of neuralgia

Neuralgia is characterized by sudden, paroxysmal, piercing, “shooting” pains. Pain syndrome may be accompanied by a change in the color and temperature of the skin in the affected area, as well as local muscle cramps.

Most often, patients suffer from trigeminal neuralgia, intercostal neuralgia.

Trigeminal neuralgia is characterized by intolerable pain in the face, gums, lacrimation.Pain attacks are provoked by talking, chewing, brushing teeth, shaving, and low ambient temperatures. At the same time, there is a reddening of the face, a spasm of the facial muscles. Trigeminal neuralgia is characterized by the presence of trigger zones – areas of the skin, touching which can provoke a pain attack.

It is important when examining patients to exclude all possible secondary variants of trigeminal neuralgia.

With the development of intercostal neuralgia, patients complain of severe pain in the rib area, aggravated by coughing or sneezing.It is noted that intercostal neuralgia often accompanies osteochondrosis of the thoracic spine.

Diagnosis of neuralgia

The diagnosis “Neuralgia” is made by a neurologist on the basis of typical complaints presented by the patient. The doctor asks questions about the presence of chronic diseases, conducts an examination in the affected area, assesses the soreness at the points of exit of the branches of the trigeminal nerve.

If a secondary nature of neuralgia is suspected, the doctor may prescribe an MRI scan to clarify the diagnosis (multiple sclerosis, tumor process).

Treatment of neuralgia

In primary neuralgia, the appointment of B vitamins, injections of novocaine, vascular preparations is indicated.

Treatment of secondary neuralgia should be aimed at eliminating the cause of the damage to the nerve fibers.

Anticonvulsants are used in the treatment of trigeminal neuralgia. The group of these drugs has a pronounced analgesic effect.

In some cases, if indicated, it is possible to carry out neurosurgical intervention – vascular decompression.

90,000 Multiple sclerosis, treatment of multiple sclerosis in Moscow

Benefits of treatment at NEARMEDIC

Highly qualified neurologists guarantee an accurate diagnosis and effective treatment of multiple sclerosis of the brain.

High-tech diagnostic equipment (Japanese tomograph HITACHI APERTO for MRI) allows you to detect foci of the disease and comprehensively assess the degree of brain damage.

Often, patients with this pathology suffer from concomitant diseases (diabetes mellitus, lung disease, coronary heart disease), which significantly worsen the course of the disease.Their qualified treatment is an additional measure that improves the quality of life of patients and reduces the number of deaths. Experienced doctors of various specialties practice in NEARMEDIC multidisciplinary clinics, who will provide timely effective medical assistance and psychological support in the progressive development of the disease.

In addition, NEARMEDIC provides its patients with the possibility of treatment abroad in partner clinics and consultation with the world’s leading specialists.

Features of the course of the disease, diagnosis and treatment of multiple sclerosis in NYARMDIK

Unlike most neurological disorders, the disease begins at a young age (20-40 years). Multiple sclerosis in women is observed more often than in men, but the disease is more active in the stronger sex. In addition, multiple sclerosis in men tends to be more severe.

The first signs of multiple sclerosis, contrary to the erroneous opinion, are not associated with forgetfulness and absent-mindedness of a person.

Initial symptoms:

• coordination disorder;
• Weakness in the limbs, tingling and numbness;
• paresis of the facial muscles
• impaired vision, double vision
• Pelvic disorders (impaired sexual function, defecation and urination).

Disease diagnosis

The initial stage of the disease is characterized by the transient nature of clinical manifestations, since the functions of the destroyed nerve pathways are taken over by healthy cells.A person may feel quite healthy, but MRI of the brain and spinal cord already shows the presence of lesions.

Studies are recommended to be repeated at least once a year, and with frequent exacerbations every 6 months, to monitor the formation of new plaques over time.

An important place in the diagnosis of the disease is occupied by the study of cerebrospinal fluid, the detection of specific immunoglobulins. An auxiliary role in the diagnosis of the disease is played by monitoring the state of the immune system, examination by an ophthalmologist, and a study of the bioelectric activity of the brain.

Treatment of the disease

Disease therapy is divided into the following stages:

1. Treatment of exacerbations with corticosteroid drugs. Pharmaceutical agents are prescribed individually, depending on the severity of exacerbations, the course of the disease, and the presence of contraindications.
2. Pathogenetic therapy. The choice of drugs is based on the duration of the disease, the volume of the lesion, the severity of the neurological deficit.
3. Symptomatic drug therapy and rehabilitation measures (physiotherapy, exercise therapy, apparatus rehabilitation) aimed at correcting disorders.

Progressive multiple sclerosis is a severe and progressive disease, but following the doctor’s instructions, a person can remain active and completely self-sufficient. It is important to remain optimistic and maintain a healthy lifestyle. The main thing is to consult a doctor on time and start treatment with a qualified specialist.

Contact the specialists on time!

Timely therapy after the first clinical attack slows down the progression of the disease. Therefore, it is extremely important to recognize the signs of the disease as early as possible.

If you suspect a neurological pathology, see your doctor immediately.

Call us and make an appointment.

.