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Neuromyelitis optica

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Introduction


Neuromyelitis optica (NMO), also known as Devic's disease, is a rare type of demyelinating disease. It is called a demyelinating disease because it occurs when the protective covering (the myelin sheath) that surrounds the nerves in the brain and spinal cord is damaged.

The damage to the myelin sheath causes inflammation (redness and swelling) of the:

  • spinal cord - this is called transverse myelitis (TM)
  • optic nerve - this is called optic neuritis (ON)

NMO can also affect parts of the brain.

How common is NMO?

NMO is a very rare condition. In Europe, it is estimated that there are less than two cases of NMO for every 100,000 people. The condition is more common in people of Asian and African descent.

NMO can affect anyone, but it is more common in people who are around 40 years of age. The condition varies from person to person, depending on the individual and the type of NMO that they have.

There are two main types of NMO:

  • relapsing
  • monophasic

Relapsing NMO

Relapsing NMO is the most common type of NMO. After the initial attack of optic neuritis and transverse myelitis, there will be further recurrences, often over a number of years. This can sometimes lead to permanent disability.

Disability occurs because the body cannot always fully recover from the damage that is caused by the attacks on the spinal cord and the optic nerve. Many more women are affected by this type of NMO than men.

Monophasic NMO

Monophasic NMO is where people experience a few attacks in a short space of time - for example, over a few days or weeks, but do not have any further attacks. Equal numbers of men and women experience this form of NMO.

Outlook

NMO can sometimes be confused with multiple sclerosis (another condition that affects the brain and spinal cord). It is important that the condition is diagnosed correctly because this will affect the treatment.

Several medications are used to treat the different symptoms of NMO. Rehabilitation techniques, such as physiotherapy (when physical methods are used to promote healing), can also help with any reduced mobility that the relapses cause.

Spinal cord
The spinal cord is a column of nervous tissue located in the spinal column. It sends messages between the brain and the rest of the body.

Optic nerve
The nerve that connects your eye to your brain.

Brain
The brain controls thought, memory and emotion. It also sends messages to the body controlling movement, speech and senses.

Inflammation
Inflammation is the body's response to infection, irritation or injury. It causes redness, swelling, pain and sometimes a feeling of heat in the affected area.


Symptoms of neuromyelitis optica


The symptoms of neuromyelitis optica (NMO) vary from person to person. However, NMO is most commonly characterised by one or more episodes of:

  • optic neuritis - inflammation (swelling and redness) of the optic nerve (the nerve that connects your eye to your brain)
  • transverse myelitis - inflammation of the spinal cord

Optic neuritis

Symptoms of optic neuritis include:

  • inflammation of the optic nerve
  • reduced or lost vision in at least one eye - this is usually temporary, but it can be permanent, and is made worse by exercise or heat
  • swelling of the optic disc (the area at the back of your eye where the optic nerve is connected)
  • eye pain - which is usually made worse by movement, and is at its worst after a week before disappearing after several days
  • reduced colour vision - colours may appear 'washed out' or less vivid than usual

If you develop optic neuritis, it may have implications for driving (see box, to the right).

Transverse myelitits

Symptoms of transverse myelitis include:

  • inflammation of the spinal cord
  • pain in the neck or back
  • shooting sensations in the arms, legs, or abdomen (stomach)
  • altered sensations - you may be more sensitive to touch, cold, and heat, and you may feel numbness, tingling, coldness, or burning below the affected area of your spinal cord
  • weakness in your arms or legs - this can range from a mild 'heavy' feeling in one limb, to complete paralysis in all four limbs
  • bladder and bowel problems - increased frequency or need to urinate, difficulty urinating, urinary, or bowel, incontinence (unintentional passing of urine or stools) or constipation (being unable to completely empty your bowels)
  • muscle spasms - when your muscles contract tightly and painfully
  • headaches
  • a high temperature (fever) of 38C (100.4F) or over
  • loss of appetite

Cases of NMO can range from one mild attack of optic neuritis and one episode of transverse myelitis with near complete recovery and no further relapses to a number of attacks throughout your life, possibly resulting in permanent disability.

In cases where a person experiences only relapsing optic neuritis, or relapsing myelitis, it is known as NMO spectrum disorder.

Generally, NMO affects only the optic nerve and spinal cord. However, in rare cases it can also affect parts of the brain. When the brain stem (the inner part of the brain that is connected to the spinal cord) is affected, it can cause symptoms such as uncontrollable vomiting and hiccups.

Spinal cord
The spinal cord is a column of nervous tissue located in the spinal column. It sends messages between the brain and the rest of the body.

Optic nerve
The nerve that connects your eye to your brain.

Brain
The brain controls thought, memory and emotion. It also sends messages to the body controlling movement, speech and senses.

Inflammation
Inflammation is the body's response to infection, irritation or injury. It causes redness, swelling, pain and sometimes a feeling of heat in the affected area.

Incontinence
Incontinence is when you pass urine (urinal incontinence), or stools or gas (faecal incontinence), because you cannot control your bladder or bowels.

Constipation
Constipation is when you pass stools less often than usual, or when you are having difficulty going to the toilet because your stools are hard and small.


Causes of neuromyelitis optica


The exact cause of neuromyelitis optica (NMO) is not fully understood because very little is known about it. However, a number of factors that are thought to play a part are outlined below.

Genetics

NMO is not hereditary (it is very rarely found in more than one family member), but it is more common in people of Asian and African descent. This suggests that people with these genes (units of genetic material), may be predisposed (have a tendency) to develop the condition.

Infectious agents

An infectious agent is something that causes an infection. It is possible that NMO could be caused by an infectious agent. In rare cases, tuberculosis (a bacterial infection) and other environmental organisms have been associated with NMO, although no specific organism has yet been identified for being responsible.

Autoimmune disorder

NMO is an autoimmune disorder, which means that the immune system (your body's own defence system) starts to attack your tissues and organs. In NMO, your immune system attacks your myelin sheath (the protective covering that surrounds the optic nerve and spinal cord).

Antibodies

Research has found that about 70% people with NMO have an antibody called Neuromyelitis Optica Immunoglobin G (NMO lgG) present in their blood. An antibody is a protein that is produced by the body to destroy disease-carrying organisms and toxins.

It is thought that the NMO lgG antibody may influence the development of NMO by damaging the water channel (aquaporin 4) that surrounds the cells of the spinal cord and optic nerve.

Bacteria
Bacteria are tiny, single-celled organisms that live in the body. Some can cause illness and disease and some are good for you.

Genetic
Genetic is a term that refers to genes. Genes are the characteristics inherited from a family member.

Gene
A gene is a unit of genetic material that determines your body's characteristics.

Immune system
The immune system is the body's defence system, which helps protect it from disease, bacteria and viruses.

Antibodies
Antibodies are your body's natural defence against any foreign antigens that enter your blood. An antibody is a protein that is produced by the body to neutralise or destroy disease-carrying organisms and toxins.


Diagnosing neuromyelitis optica


Neuromyelitis optica (NMO) can easily be mistaken for a number of other conditions that have similar symptoms. These include:

  • multiple sclerosis (MS) - a condition of the central nervous system (the brain and spinal cord), which affects movement and balance
  • acute demyelinating encephalomyelitis (ADEM) - an acute (short-term) condition that affects the brain and spinal cord
  • Sjogren's syndrome - an autoimmune condition (when the body's defence system starts attacking the body) that affects the saliva and tear glands
  • systemic lupus erythematosis (SLE) - an autoimmune condition that causes joint pain and fatigue (extreme tiredness)
  • mixed connective tissue disease (MCTD) - inflammation of the connective tissue (tissues that provides support and structure to other tissues)
  • virus induced inflammation - inflammation that is caused by a viral infection
  • cancer associated inflammation (paraneoplastic optic neuropathy)

As NMO can be similar to each of these conditions, it can be difficult to diagnose. However, there are a number of diagnostic tests that your GP may carry out in order to rule out any other conditions. You may also be referred to a specialist at a hospital to help with your diagnosis, such as:

  • an ophthalmologist - a medical doctor who specialises in eye conditions
  • a neurologist - a specialist in conditions of the central nervous system

Blood test

A sample of your blood may be tested for the presence of the NMO lgG antibody, which is commonly found in people with NMO. An antibody is a protein that is produced by your body to destroy disease-carrying organisms.

If the test is positive, it means that you are at increased risk of experiencing multiple attacks of transverse myelitis (inflammation of the spinal cord).

Lumbar puncture

A lumbar puncture, also known as a spinal tap, is used to collect a small amount of cerebrospinal fluid (CSF). CSF is the clear fluid that surrounds your brain and spinal cord.

The CSF can be tested for white blood cells (cells in the blood that help fight infections) and certain proteins. This will help determine whether you have NMO or another condition.

See the Health A-Z topic about Lumbar puncture for more information.

Magnetic resonance imaging (MRI)

A magnetic resonance imaging (MRI) scan uses strong magnetic fields and radio waves to produce a detailed image of the inside of the body. The procedure scans the brain and the body for any lesions (wounds) that may be present.

Generally, test results for people with NMO show no abnormalities in the brain, but often show lesions over three or more segments of the spinal cord. This type of damage is very unusual in MS, so it is often used to rule this condition out.

See the Health A-Z topic about MRI scan for more information.

Spinal cord
The spinal cord is a column of nervous tissue located in the spinal column. It sends messages between the brain and the rest of the body.

Optic nerve
The nerve that connects your eye to your brain.

Brain
The brain controls thought, memory and emotion. It also sends messages to the body controlling movement, speech and senses.

Inflammation
Inflammation is the body's response to infection, irritation or injury. It causes redness, swelling, pain and sometimes a feeling of heat in the affected area.

Immune system
The immune system is the body's defence system, which helps protect it from disease, bacteria and viruses.

Antibodies
Antibodies are your body's natural defence against any foreign antigens that enter your blood. An antibody is a protein that is produced by the body to neutralise or destroy disease-carrying organisms and toxins.


Treating neuromyelitis optica


There is no cure for neuromyelitis optica (NMO), but some treatments are available.

Corticosteroids

Corticosteroids (steroids) are any type of medication containing hormones (powerful chemicals that have a wide range of effects on the body).

Corticosteroids may be used to treat relapses of NMO by reducing the inflammation (redness and swelling) that they cause. You may be treated with corticosteroids:

  • intravenously (through a vein)
  • orally (taken by mouth)

See the Health A-Z topic about Corticosteroids for more information about corticosteroids and their side effects.

Other medications

NMO is an autoimmune disorder, which means that it is caused by the immune system (the body's defence system) attacking its own tissues.

Therefore, medication to suppress the immune system (prevent it working) is thought to be an effective treatment. One possible medication is azathioprine, but it can cause a number of side effects including:

  • dizziness
  • vomiting
  • fever
  • diarrhoea
  • hypotension (low blood pressure)
  • hair loss
  • pancreatitis - inflammation of the pancreas (a
  • small organ behind the stomach)
  • pneumonitis - inflammation of the lungs caused by a virus
  • colitis - inflammation of the colon (large intestine)

If you experience any of these side effects, visit your GP or call NHS Direct on 0845 46 47.

Pain relief

Episodes of NMO can cause pain, and you may need to take painkillers to relieve this. Your GP can advise you about what medication will work best. Some pain relief medication is available over-the-counter (OTC) from pharmacies, or your GP may be able to prescribe something stronger, if necessary.

Symptom control

NMO can cause a number of other symptoms, such as difficulty controlling your bladder, or muscle spasms (when the muscles contract tightly and painfully). These can be treated separately with a number of other medications. For example, you may be prescribed an anticonvulsant (antiseizure) medication to help relax your muscles

Future research

There is currently a lack of research into possible future treatments for NMO. This may be because the condition is so rare that it is difficult to organise clinical trials (medical research that tests one type of treatment against another).

Multiple sclerosis (MS), a condition that affects the central nervous system (the brain and spinal cord) is similar to NMO. However, some medication that works on MS, such as beta interferons, has so far not proven effective for treating NMO.

Further research is under way to find possible treatments for NMO. For example, one small study has found that a medication that is usually used to treat people having organ transplants can help improve relapse rates in people with NMO.

Rehabilitation

NMO can result in some degree of disability. You may require mobility or visual aids, such as:

  • a wheelchair
  • a walking frame
  • magnifying lenses
  • large print books

You may also need to have physiotherapy (see below).

Physiotherapy

If you are experiencing physical problems, such as weakness, stiffness, or poor co-ordination, you may be referred to a physiotherapist.

A physiotherapist uses a variety of treatments, such as massage, exercise, and hydrotherapy (special exercises in warm, shallow, water) to help you recover physically.

See the Health A-Z topic about Physiotherapy for more information.

Hormones
Hormones are groups of powerful chemicals that are produced by the body and have a wide range of effects.

Inflammation
Inflammation is the body's response to infection, irritation or injury. It causes redness, swelling, pain and sometimes a feeling of heat in the affected area.

Immune system
The immune system is the body's defence system, which helps protect it from disease, bacteria and viruses.


Complications of neuromyelitis optica


Particularly severe cases of neuromyelitis optica (NMO) can result in:

  • permanent loss of vision as a result of damage to the optic nerve
  • paralysis in all four limbs due to damage to the spinal cord in the neck, which may mean you will require a wheelchair
  • breathing difficulties due to muscle weakness which, in extreme cases will require artificial ventilation (a machine that helps you breathe)
  • osteoporosis (brittle bones) - this may be the result of long-term use of steroid medication, or a lack of weight-bearing activities
  • sexual dysfunction - men may experience difficulty getting an erection, or reaching orgasm, and women may also have difficulty reaching orgasm
  • depression - changes in lifestyle associated with the complications of NMO can increase the risk of developing depression

Spinal cord
The spinal cord is a column of nervous tissue located in the spinal column. It sends messages between the brain and the rest of the body.

Optic nerve
The nerve that connects your eye to your brain.


The materials in this website are provided by Medicine Chest and NHS Choices.  Neither Co-operative Group Limited or Co-operative Healthcare Limited (trading as The Co-operative Pharmacy or otherwise) shall be in any way responsible or liable for its content.

The materials in this website are in no way intended to replace the professional medical care, advice, diagnosis or treatment of a doctor.  The website does not have answers to all problems and answers to specific problems may not apply to everyone.  If you notice medical symptoms or feel unwell, you should consult your doctor.  For further information, consult the terms and conditions.


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