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Other Neurological Conditions
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This section doesn't talk about multiple sclerosis (MS), but rather other
neurological conditions or disorders. It's important to understand the basics of
other disorders if one wants to better understand about MS. There are
differences and similarities with each disorder and those can vary significantly
between each person affected.
Neurological disorders are health conditions that involve the nervous system and
causes paralysis to any part of the body. Sometimes physical injury to the
brain, spinal cord, or nerves can be the cause of neurological disorders and
sometimes they can result from biochemical causes. Other times, the cause may be
unknown and only the effects are seen. Neurological disorders can also be a sign
that there is an imbalance of some type in your system, but when you have an
imbalance, you are also susceptible to various diseases which can settle in weak
areas of your body.
Neurological disorders are a group of disorders that involve the central nervous
system (CNS), the peripheral nervous system (PNS) including the cranial nerves,
and the autonomic nervous system which is located in both CNS and PNS.
Neurological disorders are quite diverse, chronic, challenging to treat, and
often disabling. They can be caused by many different factors, including
inherited genetic abnormalities, problems in the immune system, injury to the
brain or nervous system, or diabetes. Many mental illnesses are believed to be
neurological disorders of the CNS, but they are classified separately. They
aren't traditionally listed as neurological diseases because their causes are
not definitely determined as biological, although there are good reasons to
suspect that some have neuro-chemical causes.
Neurological disabilities are associated with damage to the nervous system
(including the brain and spinal cord) that results in the loss of some bodily or
mental functions. Acquired Brain Injury (ABI), and Epilepsy are two of the most
prevalent neurological disabilities. Heart attacks, infections, genetic
disorders, and lack of oxygen to the brain may also result in a neurological
disability.
The CNS along with the PNS comprise a primary division of controls that command
all physical activities of a vertebrate. Neurons of the CNS affect consciousness
and mental activity while spinal extensions of CNS neuron pathways affect
skeletal muscles and organs in the body.
The immune system defends the body against foreign invaders such as bacteria and
viruses. It does so by recognizing that foreign invaders have special markers
distinguishing them as "non-self," compared to the body's own tissue or "self."
Normally, the immune system reacts only to non-self invaders, not to its own
tissues. This, however, is not always the case. Autoimmunity is an immune
response mounted against antigens that are naturally produced within the body,
or self-antigens, to cause lasting tissue damage.
In the strictest sense, an autoimmune disease must meet several criteria. First,
the disease must be reproduced by transfer of autoantibodies or autoreactive T
lymphocytes (T cells) from affected to unaffected individuals. Second, the
self-antigen that elicits the immune attack must be identified. Third, this
antigen (or a closely related one) should cause a similar disease in an animal
model.
There are several human and animal diseases of known etiology or pathogenesis
that resemble either the clinical or the pathological features of MS. CNS
demyelinating diseases include those mediated by immune responses, infection,
and toxins, as well as inherited disorders. Infectious agents can induce direct
injury of oligodendrocytes and their myelin membranes, as well as indirect
injury via the immune system.
A variety of toxins, such as diphtheria, lysolecithin, cuprizone, and ethidium
bromide, have been associated with demyelinating lesions. Many of these toxins
induce lysis of the oligodendrocyte, with demyelination as a secondary effect.
In addition, nutritional deprivation can be associated with demyelination in the
CNS and PNS.
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Neurological Conditions and Diseases
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Alzheimer's disease
Alzheimer's disease (AD) is an irreversible, progressive disorder in which brain
cells or neurons deteriorate, resulting in the loss of cognitive functions,
primarily memory, judgment and reasoning, movement coordination, and pattern
recognition. In advanced stages of the disease, all memory and mental
functioning may be lost. A person with Alzheimer's disease usually has a gradual
decline in mental functions, often beginning with slight memory loss, followed
by losses in the ability to maintain employment, to plan and execute familiar
tasks, and to reason and exercise judgment. The ultimate cause or causes of
Alzheimer's disease are still unknown; there are several risk factors that
increase a person's likelihood of developing the disease.
Amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS), also called Lou Gehrig’s disease, is a
disease in which certain nerve cells in the brain and spinal cord slowly die.
These nerve cells are motor neurons, and they control the muscles that allow you
to move the parts of your body. Those with ALS gradually become more disabled,
but how quickly the disease gets worse is different for everyone. Some people
live with ALS for several years. Over time, ALS makes it difficult to walk,
speak, eat, swallow, and breathe. These problems can lead to injury, illness,
and eventually death.
Acquired Brain Injury
Acquired Brain Injury (ABI) occurs when a sudden, external, physical assault
damages the brain. It's one of the most common causes of disability and death in
adults. ABI is a broad term that describes a vast array of injuries that occur
to the brain. The damage can be focal (confined to one area of the brain) or
diffuse (occurs in more than one area of the brain). The severity of a brain
injury can range from a mild concussion to a severe injury that results in coma
or even death. Acquired brain injuries are also commonly referred to as
traumatic brain injuries (TBI).
Bell's palsy
Bell's palsy is a paralysis or weakness of the muscles on one side of your face.
Damage to the facial nerve that controls muscles on one side of the face causes
that side of your face to droop. The nerve damage may also affect your sense of
taste and how you make tears and saliva. In most cases, the nerve that controls
muscles on one side of the face is damaged by inflammation. A person's speech
can be affected and sound "slurred" due to the effects of paralysis.
Carpal tunnel syndrome
Carpal tunnel syndrome occurs when tendons in the wrist become inflamed after
being aggravated. Tendons can become aggravated when the carpals and the
ligaments in the wrist narrow, pinching nerves that reach the fingers and the
muscle at the base of the thumb. Repetitive flexing and extension of the wrist
may cause a thickening of the protective sheaths that surround each of the
tendons, which narrows the tunnel. Women are three times more likely to develop
CTS than men, and the risk increases with age. People between the ages of 40 and
60 are more commonly affected.
Cerebral palsy
Cerebral palsy (CP) is a group of disorders associated with developmental brain
injuries that occur during fetal development, birth, or shortly after birth.
It's characterized by a disruption of motor skills, with symptoms such as
spasticity, paralysis, or seizures. Cerebral palsy is also known as static
encephalopathy and Little's disease (which is strictly speaking only the
"spastic diplegia" form of CP). It's no longer considered a disease, but rather
it is a chronic nonprogressive neurological disorder. The incidence is about 1.5
to 4 per 1000 live births. There is no cure, but therapy may be helpful. It has
one of the highest lifetime costs of any birth defect.
Chronic fatigue syndrome
Chronic fatigue syndrome (CFS) is an illness characterized by prolonged,
debilitating fatigue and multiple nonspecific symptoms such as headaches,
recurrent sore throats, muscle and joint pain, memory and concentration
difficulties. Profound fatigue, the hallmark of the disorder, can come on
suddenly or gradually and persists or recurs throughout the period of illness.
Unlike the short-term disability, chronic fatigue syndrome symptoms linger for
at least six months and often for years. The cause of chronic fatigue syndrome
remains unknown.
Epilepsy
Epilepsy is a chronic neurological disorder in which clusters of nerve cells, or
neurons, in the brain sometimes signal abnormally. In epilepsy, the normal
pattern of neuronal activity becomes disturbed, causing strange sensations,
emotions, and behavior or sometimes convulsions, muscle spasms, and loss of
consciousness. Epilepsy is a disorder with many possible causes. Anything that
disturbs the normal pattern of neuron activity - from illness to brain damage to
abnormal brain development - can lead to seizures. Epilepsy may develop because
of an abnormality in brain wiring, an imbalance of nerve signaling chemicals
called neurotransmitters, or some combination of these factors. The seizures are
transient signs and/or symptoms of abnormal, excessive or synchronous neuronal
activity in the brain.
Guillain-Barré syndrome
Guillain-Barré syndrome (GBS) is a rare nervous system disorder that results
from nerve damage caused by the body's immune system, usually in response to an
infection or other illness. As the immune system produces antibodies to fight
the infection or illness, it may also produce antibodies that attack the myelin
sheath of the peripheral nerves and sometimes the nerve fibers or axons. The
resulting nerve damage leads to tingling and numbing sensations, muscle
weakness, and paralysis. GBS causes muscle weakness, loss of reflexes, and
numbness or tingling in the arms, legs, face, and other parts of the body. It
may also progress to complete paralysis.
Headaches
A headache is a condition of mild to severe pain in the head or sometimes upper
back or neck pain may also be interpreted as a headache. Most headaches are due
to tension, migraine, or a combination of the two. Serious underlying causes of
headaches, like a tumor or a stroke, are extremely rare, despite the fact that
many people worry about these possibilities. Migraine headache is a primary
headache disorder with, almost certainly, a genetic basis. Activation of a
mechanism deep in the brain causes release of pain-producing inflammatory
substances around the nerves and blood vessels of the head.
Meningitis
Meningitis is an infection of the meninges, which is the coverings around the
brain and spinal cord. The infection occurs most often in children, teens, and
young adults. Also at risk are older adults and people who have long-term health
problems, such as a weakened immune system. There are two main kinds of
meningitis: Viral meningitis is fairly common and it usually doesn't cause
serious illness. It can, however, cause prolonged fever and seizures. The other
is bacterial meningitis isn't as common but is very serious. It needs to be
treated right away to prevent brain damage and death.
Neuropathy
Neuropathy is the disease of the nervous system. Neuropathy is a disturbance in
the function of a nerve or particular group of nerves. Many people who have had
diabetes for a while have nerve damage. The three major forms of nerve damage
are: peripheral neuropathy, autonomic neuropathy, and mononeuropathy. The most
common form is peripheral neuropathy, which mainly affects the feet and legs.
Neuropathy can lead to disability, amputation, decreased ambulation as well as
foot and leg ulceration because of loss or damage to nerves which feel sensation
in the lower limbs.
Parkinson's disease
Parkinson's disease is a neurodegenerative disease of the substantia nigra which
is an area in the basal ganglia. Parkinson's disease involves a breakdown of the
nerve cells in the motor area of the brain. As the cells break down, there is a
shortage of dopamine. Dopamine is a neurotransmitter, or chemical that carries
messages to the body. When there is a shortage of dopamine, the messages that
regulate movement aren't sent properly. Parkinson's disease happens when nerve
cells or neurons in a part of the brain called the substantia nigra gradually
die. These cells normally produce dopamine, a chemical that helps to relay
messages between areas of the brain that control body movement.
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Other Demyelinating Diseases
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ADEM
Acute disseminated encephalomyelitis (ADEM), also known as post vaccination
encephalomyelitis, occurs as a consequence of vaccination with neural antigens.
EAE, the most widely used animal model of MS, is the animal counterpart of this
human disease. ADEM is characterized pathologically by widespread perivenular
inflammation and demyelination. The uniformity of lesions differs from the
multi-age lesions found in even the most acute case of MS. Post vaccination
immune-mediated damage can also affect the PNS.
This disorder usually develops after a viral infection. Acute disseminated
encephalomyelitis is thought to be a misguided immune reaction triggered by the
virus. In the United States, this disorder usually results from some types of
influenza, hepatitis A or B, or infection with enteroviruses, Epstein-Barr
virus, or human immunodeficiency virus (HIV). Measles, chickenpox, and rubella
used to be common causes before childhood vaccination became widespread.
Typically, the inflammation develops 1 to 3 weeks after the viral illness
begins. It can be treated with corticosteroids given intravenously.
Guillain-Barré syndrome seems to be a similar disorder of the peripheral nerves.
Adrenoleukodystrophy and Adrenomyeloneuropathy
Both are rare hereditary metabolic disorders. Adrenoleukodystrophy affects young
boys, usually between the ages of 4 and 8. A milder, more slowly developing form
of the disorder can begin in adults in their 20s or 30s. Adrenomyeloneuropathy
affects adolescent boys.
In these disorders, widespread demyelination is accompanied by adrenal gland
dysfunction. Boys have behavioral problems and problems with hearing and vision.
Eventually, mental deterioration, involuntary and uncoordinated muscle
contractions (spasticity), and blindness occur.
No cure for either disorder is known. Dietary supplements with glycerol
trioleate and glycerol trierucate (known as Lorenzo's oil) have not been shown
to slow the progression of the disease. Bone marrow transplantation is an
experimental treatment.
Systemic inflammatory or autoimmune diseases
Multifocal CNS lesions can occur as a component of an array of systemic collagen
vascular disorders including systemic lupus eryhemtosus and polyarteritis
nodosa. The CNS manifestations may be the presenting feature. The PNS is also
frequently involved.
HTLV-1 myelopathy
Human T cell lymphotropic virus type I (HTLV-1) infection is sometimes
associated with a neurological syndrome called HTLV-1-associated
myelopathy/tropical spastic paraparesis (HAM/TSP). Patients with HAM/TSP have a
progressive myelopathy, usually with spastic paraparesis, sensory disturbance,
bladder dysfunction, and occasionally, optic neuritis.
Leber's Hereditary Optic Neuropathy
This disorder causes demyelination leading to partial blindness. The disorder is
more common among men. Usually, symptoms begin between the ages of 15 and 35.
This disorder is inherited through the mother, and the defective genes seem to
be located in mitochondria (structures in cells that provide energy for the
cell).
No treatments are available. But limiting consumption of alcohol, which may
affect the mitochondria, and not using tobacco products may help.
Schilder's Disease
Schilder's disease is a rare progressive demyelinating disorder which usually
begins in childhood. Symptoms may include dementia, aphasia, seizures,
personality changes, poor attention, tremors, balance instability, incontinence,
muscle weakness, headache, vomiting, and vision and speech impairment.
Schilder's disease is not the same as Addison-Schilder disease (adrenoleukodystrophy)
and is a variant of MS.
Treatment for the disorder follows the established standards in MS and includes
corticosteroids, beta-interferon or immunosuppressive therapy, and symptomatic
treatment.
As with MS, the course and prognosis of Schilder's disease are unpredictable.
For some individuals the disorder is progressive with a steady, unremitting
course. Others may experience significant improvement and even remission and in
some cases, Schilder's disease is fatal.
Tropical Spastic Paraparesis
Also called HTLV-associated myelopathy, this disorder causes demyelination in
the spinal cord and results from infection with the human T cell lymphotropic
virus (HTLV). The disorder worsens over several years. Gradually, the legs
become weak and muscle spasms occur—a condition called spastic weakness.
Frequent, strong urges to urinate, urinary incontinence, and bowel dysfunction
also develop.
No cure is available, but corticosteroids may help, as may interferon-beta or
immune globulin given intravenously (these drugs help prevent the immune system
from attacking myelin sheaths). Muscle relaxants such as baclofen or tizanidine
help relieve spasms.
Neuromyelitis Optica
Also called Devic disease, this disorder causes symptoms similar to those of MS
and for a time was considered a variant of MS. However, neuromyelitis optica
typically affects only the eyes and the spinal cord, and MS also affects the
brain.
Neuromyelitis optica causes inflammation of the optic nerve (optic neuritis).
One or both eyes may be affected. The disorder causes episodes of eye pain and
dim, blurred, or lost vision. Days to weeks (sometimes years) later, the limbs
are affected. People may temporarily lose sensation, and the arms and legs may
become weak and sometimes paralyzed. People may be unable to control of bladder
and bowel function.
In some people, the part of the spinal cord that controls breathing is inflamed,
leading to difficulty breathing, which is life threatening.
The disorder progresses differently in each person. As the disorder progresses,
people may have brief, frequent, painful muscle spasms. Eventually, blindness,
loss of sensation and muscle weakness in the limbs, and bladder and bowel
dysfunction may become permanent.
To diagnosis the disorder, doctors evaluate the nervous system (neurologic
examination) during a physical examination. The optic nerve is examined with an
ophthalmoscope. Tests include magnetic resonance imaging (MRI) and a blood test
to detect specific antibodies in people with neuromyelitis optica.
There is no cure, however treatments can stop episodes, control symptoms, and
prevent episodes from recurring. A corticosteroid (such as methylprednisolone)
and a drug that suppresses the immune system (an immunosuppressant, such as
azathioprine) are often used to stop and prevent episodes. Rituximab, a
relatively new drug, may be used to reduce the number of abnormal antibodies and
to control disorder. Plasma exchange (plasmapheresis) may help people who do not
respond to corticosteroids. For this treatment, blood is removed, then abnormal
antibodies are removed, and the blood is returned to the person.
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