11. is a type of inflammatory demyelinating disease.
A demyelinating disease occurs when the myelin sheath, a
protective covering that surrounds brain and spinal cord nerves
is damaged. In the case of Devic's disease, the patient develops
myelitis (inflammation of the spinal cord) and optic neuritis
(inflammation of the optic nerve).
The disease more commonly affects people when they are
about 40 years of age
12. There are two main types of neuromyelitis optica:
Relapsing neuromyelitis optica
There is an initial attack of optic neuritis and transverse myelitis, and then
subsequent attacks over a period of several years. Sometimes the patient
cannot recover fully from the neurological damage the attacks cause to the
optic nerve and/or spinal cause and the damage is permanent, causing
disability. This type of neuromyelitis optica affects females more frequently
than males.
Monophasic neuromyelitis optica
Few attacks are experienced over a period of days or weeks. There are no
subsequent attacks. This form of neuromyelitis optica affects both sexes
equally.
13. What are the signs and symptoms of neuromyelitis optica?
A symptom is something the patient feels and reports, while a sign is something other
people, including a doctor or nurse may detect. For example, pain may be a symptom
while a rash may be a sign.
Signs and symptoms of neuromyelitis optica (NMO) vary according to many factors,
including the type of NMO the patient has. Typically, a person with NMO will have at
least one episode of optic neuritis and transverse myelitis.
Signs and symptoms of optic neuritis include (ON):
The optic nerve becomes inflamed
Loss of eyesight, which affects at least one eye. In most cases this is temporary;
however, there is a risk of permanent vision loss, which is exacerbated by heat or
exercise.
Swelling of the optic disc.
Pain in the eye. The pain generally worsens with movement. Pain becomes more intense
after about one week, and then goes away after a period of days.
The eyes become less sensitive to colors.
Patients who develop optic neuritis may have problems driving a vehicle.
14. Signs and symptoms of transverse myelitis (TM):
The spinal cord becomes inflamed
Back pain
Neck pain
Shooting sensations in the limbs and abdomen
Below the affected area of the spinal cord the patient may have altered sensations, with
sensitivity to cold/heat, numbness, tingling, and a sensation of coldness or burning.
Limbs may become weak. Some patients may describe their limbs as feeling heavy,
while others may develop total paralysis.
Urinary incontinence
More frequent urination
Difficulty urinating
Fecal incontinence (bowel incontinence) constipation
15. A patient with NMO may have just one mild attack of ON and
one episode of TM, recover completely, or almost completely,
and have no more relapses ever. Others may have several
attacks throughout their life and experience lifelong disability.
NMO spectrum disorder is a term used for patients who
experience only relapsing optic neuritis or relapsing myelitis
(but not both).
In most cases, just the optic nerve and spinal cord of patients
with NMO are affected. In very rare cases parts of the brain may
also be affected.
Patient's whose brain stem has been affected may experience
uncontrollable hiccups or vomiting.
16. What is the cause of neuromyelitis optica?
NMO is an autoimmune disorder.
Experts are not sure what the exact causes of NMO are. We do know that the following
factors are or aren't implicated in the disease:
It is not apparently, a strongly hereditary condition; it does not usually run in families.
A higher percentage of people from Asia or Africa, as well as individuals of Asian or African
ancestry develop NMO, compared to other people. This fact indicates that hereditary must
play some part.
In some rare cases, TB (tuberculosis) and some environmental organisms have been linked
to the development of NMO. However, scientists have not been able to isolate specific
organisms so far.
Healthy people's immune systems attack pathogens; organisms and substances that are bad
for us, such as some bacteria, viruses, parasites, cancer cells and fungi. If the person's
immune system starts attacking good tissue, they have an autoimmune disease (disorder) -
the immune system is attacking parts of the person's body that are needed for good health.
NMO is an example of an autoimmune disease. Experts are not sure why autoimmune
diseases, such as NMO occur. The immune system of a patient with NMO attacks the myelin
sheath, the protective blanket for the spinal cord and optic nerve.
17. How is neuromyelitis optica (NMO) diagnosed?
NMO is not an easy condition to diagnose because it presents signs and symptoms which
are often similar to those found in other diseases, such as:
MS (multiple sclerosis)
ADEM (acute demyelinating encephalomyelitis)
SLE (systemic lupus erythematosis), also known as Sjogren's syndrome
MCTD (mixed connective tissue disorder)
Some inflammations caused by viruses
Paraneoplastic optic neuropathy (inflammation linked to cancer)
The doctor will have to carry out some tests to rule out the above-mentioned conditions.
Blood test - the aim here is to find out whether the antibody NMO IgG is present. A positive
blood test result usually means that the patient risks having multiple attacks of transverse
myelitis.
Lumbar puncture test (spinal tap) - a small amount of fluid surrounding the brain and
spinal cord is collected. Results will indicate whether levels of white blood cells are high,
and also whether specific proteins linked to NMO or other conditions are present.
MRI (magnetic resonance imaging (MRI) scan - this scan can show up any damage to
nerves and their surroundings (lesions). MRI scans of NMO patients hardly ever reveal any
brain abnormalities, but will show up lesion consisting of three or more segments of the
spinal cord. This type of spinal cord lesion makes it easier for the doctor to rule out multiple
sclerosis.
18. What are the treatment options for neuromyelitis optica (NMO)?
NMO is not a curable disease. However, there are some treatments which may help some of the symptoms and
relapse frequency and intensity.
Treatment of relapses
Steroids - such steroids as injected methylprednisone may be administered, after which the patient would take
a course of oral steroids.
Plasma exchange therapy (Plasmapheresis) - this treatment is usually only given to patients who have not
responded to the attacks with steroid treatment. Plasma exchange removes the inflammation-causing
antibodies from the blood. The patient's blood is removed and blood cells are separated from plasma. The
blood cells, diluted with fresh plasma or substitute are returned to the patient's bloodstream.
Preventing relapses
If the patient's immune system can be slightly suppressed the chances of relapses are significantly reduced. AZT
(azathioprine), an immunosuppressant medication is sometimes prescribed. The doctor may prescribe a combination
of AZT and steroids. AZT may cause vomiting, diarrhea, fever, hypotension (low blood pressure), dizziness,
pneumonitis, colitis, hair loss and pancreatitis.
Controlling symptoms
The anticonvulsant drug, carbamazepine, may be prescribed for symptoms of pain, urinary problems, muscle spasms,
and stiffness. Carbamazepine is often used with patients who have other demyelinating diseases.
Rehabilitation
If damage is permanent and the patient experiences disabilities, physical therapy (physiotherapy), rehabilitation,
mobility and visual aids may also be given.
19. What are the possible complications of neuromyelitis optica (NMO)?
Breathing problems - in severe cases muscle weakness may be such that the patient
needs artificial ventilation.
Depression - the mental strain of living with NMO, especially if symptoms are severe, can
lead to clinical depression.
Erectile dysfunction and sexual dysfunction - some men may experience problems getting
or maintaining an erection. Both men and women may experience problems reaching
orgasm.
Fragile bones - long-term steroid therapy can lead to osteoporosis.
Paralysis - in severe cases when there is damage to the spinal cord, there can be limb
paralysis.
Vision loss - in severe cases there can be damage to the optic nerve, leading to
permanent loss of vision.