Ailments of the Nervous Ststem

Polio (also called poliomyelitis) is a contagious, historically

devastating disease that was virtually eliminated from the Western
hemisphere in the second half of the 20th century. Although polio has
plagued humans since ancient times, its most extensive outbreak
occurred in the first half of the 1900s before the vaccination, created
by Jonas Salk, became widely available in 1955.

At the height of the polio epidemic in 1952, nearly 60,000 cases with
more than 3,000 deaths were reported in the United States alone.
However, with widespread vaccination, wild-type polio, or polio
occurring through natural infection, was eliminated from the United
States by 1979 and the Western hemisphere by 1991.

A Brain Tumor is an abnormal growth of cells within the brain or

inside the skull, which can be cancerous or non-cancerous (benign).

It is defined as any intracranial tumor created by abnormal and

uncontrolled cell division, normally either in the brain itself (neurons,
glial cells (astrocytes, oligodendrocytes, ependymal cells), lymphatic
tissue, blood vessels), in the cranial nerves (myelin-producing
Schwann cells), in the brain envelopes (meninges), skull, pituitary and
pineal gland, or spread from cancers primarily located in other organs
(metastatic tumors).

Primary (true) brain tumors are commonly located in the posterior

cranial fossa in children and in the anterior two-thirds of the cerebral
hemispheres in adults, although they can affect any part of the brain.

In the United States in the year 2005, it was estimated there were
43,800 new cases of brain tumors (Central Brain Tumor Registry of the
United States, Primary Brain Tumors in the United States, Statistical
Report, 2005-2006), which accounted for 1.4 percent of all cancers,
2.4 percent of all cancer deaths, and 20-25 percent of pediatric
cancers. Ultimately, it is estimated there are 13,000 deaths per year in
the United States alone as a result of brain tumors.
A Stroke is the rapidly developing loss of brain function(s) due to
disturbance in the blood supply to the brain. This can be due to
ischemia (lack of blood supply) caused by thrombosis or embolism or
due to a hemorrhage. As a result, the affected area of the brain is
unable to function, leading to inability to move one or more limbs on
one side of the body, inability to understand or formulate speech, or
inability to see one side of the visual field. In the past, stroke was
referred to as cerebrovascular accident or CVA, but the term
"stroke" is now preferred.[citation needed]

A stroke is a medical emergency and can cause permanent

neurological damage, complications, and death. It is the leading cause
of adult disability in the United States and Europe. In the UK, it is the
second most common cause of death, the first being heart attacks and
third being cancer. It is the number two cause of death worldwide and
may soon become the leading cause of death worldwide. Risk factors
for stroke include advanced age, hypertension (high blood pressure),
previous stroke or transient ischemic attack (TIA), diabetes, high
cholesterol, cigarette smoking and atrial fibrillation. High blood
pressure is the most important modifiable risk factor of stroke.

The traditional definition of stroke, devised by the World Health

Organization in the 1970s, is a "neurological deficit of cerebrovascular
cause that persists beyond 24 hours or is interrupted by death within
24 hours". This definition was supposed to reflect the reversibility of
tissue damage and was devised for the purpose, with the time frame
of 24 hours being chosen arbitrarily. The 24-hour limit divides stroke
from transient ischemic attack, which is a related syndrome of stroke
symptoms that resolve completely within 24 hours. With the
availability of treatments that, when given early, can reduce stroke
severity, many now prefer alternative concepts, such asbrain
attack and acute ischemic cerebrovascular syndrome (modeled
after heart attack and acute coronary syndrome respectively), that
reflect the urgency of stroke symptoms and the need to act swiftly.
Meningitis is inflammation of the protective membranes covering the
brain and spinal cord, known collectively as the meninges. The
inflammation may be caused by infection with viruses, bacteria, or
other microorganisms, and less commonly by certain drugs. Meningitis
can be life-threatening because of the inflammation's proximity to the
brain and spinal cord; therefore the condition is classified as a medical
emergency.

The most common symptoms of meningitis are headache and neck

stiffness associated with fever, confusion or altered consciousness,
vomiting, and an inability to tolerate light (photophobia) or loud noises
(phonophobia). Sometimes, especially in small children, only
nonspecific symptoms may be present, such as irritability and
drowsiness. If a rash is present, it may indicate a particular cause of
meningitis; for instance, meningitis caused by meningococcal bacteria
may be accompanied by a characteristic rash.

A lumbar puncture may be used to diagnose or exclude meningitis.

This involves inserting a needle into the spinal canal to extract a
sample of cerebrospinal fluid (CSF), the fluid that envelops the brain
and spinal cord. The CSF is then examined in a medical
laboratory. The usual treatment for meningitis is the prompt
application of antibiotics and sometimes antiviral drugs. In some
situations, corticosteroid drugs can also be used to prevent
complications from overactive inflammation. Meningitis can lead to
serious long-term consequences such as deafness, epilepsy,
hydrocephalus and cognitive deficits, especially if not treated
quickly. Some forms of meningitis (such as those associated with
meningococci, Haemophilus influenzae type B, pneumococci or mumps
virus infections) may be prevented by immunization.

Broca Aphasia
Aphasia is defined by Websters Dictionary as a "loss or impairment of
the power to use or comprehend words usually resulting from brain
damage."

In most people the Broca's area is in the lower part of the left frontal
lobe. It is one of the main language areas in the cerebral cortex
because it controls the motor aspects of speech. Persons with a Broca
aphasia can usually understand what words mean, but have trouble
performing the motor or output aspects of speech. Thus, other names
for this disorder are 'expressive' and 'motor' aphasia. Depending on
the severity of the lesion to Broca's area, the symptoms can range
from the mildest type (cortical dysarthria) with intact comprehension
and the ability to communicate through writing to a complete loss of
speaking out loud.

Cerebello-Olivary Degeneration of Holmes

This is a rare, autosomally-inherited disease that leads to the

progressive degeneration of the cerebellar cortex and the inferior
olivary nucleus within the medulla. Onset is generally in the fourth
decade. The disease results in ataxic gait, dysarthria, and tremor of
the limbs.

Autopsy reveals pronounced atrophy of the cerebellum. There is

almost complete loss of Pukinje cells and an associated
astrocytosis. Shrinkage is not as prominent in the olives. It is thought
that the disease begins in the cerebellum and that in the inferior
olivary nucleus the neuronal loss is secondary since the Purkinje cell is
the synaptic target of the inferior olivary nucleus neurons. There is
some similarity pathologically between this disease and chronic
alcoholic degeneration in that both affect the cerebellum.

Choroid Plexus Papilloma

These tumors form most commonly in the fourth ventricle and mainly
affect children during the first year of life. If untreated they result in
hydrocephalus and symptoms of headache, lethargy, stupor and
weakness of the legs. The tumor takes the form of a giant choroid
plexus and has a cellular epithelium that is closely related to the
ependyma. This form of tumor is often accompanied by hemorrhage
which increases the complications of treatment. A shunt is the most
immediate form of treatment if the patient is in a condition where
surgical removal of the tumor cannot be performed right away. The
shunt removes the excess CSF thereby reducing the damaging affects
of pressure.

Another problem that can occur with the choroid plexus is that a
cyst(s) can form within it.

Huntington Disease

This is an autosomally-inherited, dominant disorder in which the

patient begins to exhibit symptoms in the third to fourth decades.
Patients with Huntington Disease (HD) initially have a tendency to
fidget which over months or years develops into jerky, choreiform
movements. HD usually progresses over a 10 to 25 year period. As
the disease progresses it leads to dementia and usually death from
incurrent infection. There is a high incidence of suicide among
patients with HD.

Pathologically, there is atrophy of certain forebrain structures including

the entire cerebral cortex and even more notably of the caudate
nucleus and putamen The head of the caudate is reduced to a narrow
brownish band of tissue that is flattened or concave. In normal brain
the ratio of small neurons to large neurons in the corpus striatum is
approximately 160:1 in Huntingtons patients the ratio is reduced to
40:1 with a marked decrease in the number of astrocytes. The gene
for this disease has been isolated to the short arm of chromosome 4.

Klüver-Bucy Syndrome
This syndrome has only really been seen in experimental monkeys. If
part of the temporal lobes are removed the monkey cannot recognize
objects (visual agnosia). These monkeys exhibit bizarre oral
behaviors such as sticking things in their mouths that normal monkeys
would have nothing to do with. They also become hyperactive and
hypersexual making physical contact with almost anything in their
environment. Wild, fearful and aggressive monkeys become tame and
showed no fear when handled by humans or presented with snakes
which rhesus monkeys normally avoid.

Locked-In Syndrome

This syndrome is due to stroke, tumor or trauma to the ventral part of

the rostral pons. Lesions there render the individual quadriplegic,
unable to speak and incapable of facial movement. One would think
these individuals were in a coma except that they are able to move
their eyes and if given an eye communicating device they can
communicate.
Alzheimer disease (AD) is the most common cause of dementia in
western civilization. It affects more women than men, and the clinical
course generally lasts approximately five years. The younger the
individual is at the onset of the disease, the more severe the deficits
for the patient. One famous contemporary who suffers from the
disease is former U.S. President Ronald Reagan.

The cerebral cortex and some other forebrain regions atrophy so

severely that the brain may weigh less than 1000g at death. Shrinkage
is most pronounced in the frontal and temporal lobes. The insula and
the medial part of the temporal lobe tend to demonstrate the highest
number of neuritic plaques. The greater the number of plaques the
higher the degree of dementia. The disease often causes vacuolization
of the subpial layers of the temporal and parietal lobes. The spongy
state is associated with neuronal loss and is similar to the effects of
Creutzfeldt-Jakob disease.

Researchers continue to search for causes and cures for AD. The gene
that codes for the B-amyloid protein located on chromosome 21 is
implicated in the 20% of patients for whom there is a family history of
AD. Head injury has been implicated in 3 to 5 % of AD cases. There
is a 70 to 90% decrease in the production of the enzyme that makes
acetylcholine. Other neurotransmitter abnormalities have also been
implicated.
Multiple Sclerosis

Multiple sclerosis (MS) is the most common demyelinating disease

afflicting humans. It is a chronic disease affecting more women than
men often leading to progressive neurological deterioration and ataxic
paraplegia. MS has cycles of remission and relapses with remission
occurring less frequently as the disease progresses. Lesions occur in
all parts of the CNS white matter appearing gray and translucent.
Some of the more prominent structures affected are the medial
longitudinal fasciculus and the optic tract and chiasm. Damage to the
neurons in these structures can lead to optic neuritis (with complete
loss of vision in one eye), diplopia (double vision) and problems
coordinating eye and head movements. Onset of the disease is usually
between the ages of 20 and 40 years of age.

Histologically there is a large decrease in the number of

oligodendrocytes with 90% of the patients having antibodies to these
cells. Many times there is a cuffing of blood vessels due to the high
number of lymphocytes, macrophages and plasma cells found in
plaques. Macrophages take a principal role in stripping and
phagocytosis of myelin from intact axons. Efforts to link this
autoimmune disease with a virus have been unsuccessful perhaps do
to the difficulty of isolating and culturing virus from MS patients.

Parkinson Disease
Parkinson disease (PD) is characterized by a slowing of voluntary
movements, bradykinesia, muscular rigidity and tremor at rest. These
abnormalities result from a reduction of neurons that make dopamine
in the pars compacta of the substantia nigra. The axons of these
neurons normally release this neurotransmitter where they synapse, in
parts of the basal ganglia called the caudate nucleus and putamen or,
collectively, the corpus striatum. Dopamine usually works as an
inhibitory neurotransmitter in the corpus striatum where it acts on
cholinergic neurons.

Lewy bodies, a hyaline inclusion, are seen microscopically in the

cytoplasm of residual neurons in the substantia nigra of nearly all
patients with Parkinsonism. The loss of neurons results in a grossly
evident depigmentation of the substantia nigra because those
dopaminergic neurons also contain the pigment neuromelanin in their
cytoplasm.

There are various causes for the loss of dopaminergic neurons in the
substantia nigra and the resultant signs of PD. Some evidence exists
for involvement of genetic factors in the pathogenesis of PD. For
example, several large families with an autosomal, dominant
inheritance pattern of PD have now been described, and the first
genetic locus for PD has been identified in one of those families.

Parinaud Syndrome

Also called dorsal midbrain or collicular syndrome, this disorder is

characterized by an upward fixed gaze and pupils. Structures in the
vicinity of the dorsal midbrain area, including cranial nerve IV, the
superior and inferior colliculi, pretectal area, and pineal gland, are
affected. Pineal tumors and hydocephalus are main causes of damage
to these structures.

Pituitary Adenoma
These tumors are benign and are usually composed of secretory cells
from the anterior lobe of the pituitary. Initially the tumor results in
either an increase or decrease of hormone production depending on
whether or not the adenoma is composed of cells capable of producing
hormone. Increased levels of hormones can lead to either Cushing
disease or acromegaly.

Secondary effects of tumor growth can lead to compression of the

optic chiasm which has a position slightly above and in front of the
pituitary gland. Such damage leads to visual difficulties.

Tourette Syndrome

This syndrome begins in childhood and manifests itself through various

forms of tics. These tics include frequent, irregular movements of the
head, neck, or shoulders. They also may be more complex motor
behaviors such as snorting, sniffing, and involuntary vocalization. As
the syndrome progresses repetitive behaviors such as touching others,
obsessive compulsive symptoms, and explosive involuntary cursing
can be more common. Aggressive behavior and improper sexual
impulses are the rarest and most severe expressions of the syndrome.
Most often the child is hyperactive and has been given some form of
CNS stimulant.

The cause of Tourette syndrome is not known, but it is believed to

have a genetic component. A total absence of the protein dynorphin in
the striatal fibers of the globus pallidus is found in some patients.
Other studies have shown a deficit in the uptake or release of
dopamine and serotonin at synapses.

Wallenberg Syndrome
This syndrome is characterized by difficulty in swallowing and
hoarseness due to paralysis of the ipsilateral vocal cord. In some
cases taste may also be affected in the ipsilateral half of the tongue.
The glossopharyngeal (IX) & vagus (X) are the primary cranial nerves
involved in this syndrome.

Occlusion of the posterior inferior cerebellar artery (PICA) or branches

from it leads to damage to the posterior lateral region of the medulla.
Thus another name for this disorder is the 'lateral medullary plate
syndrome'.

There is also likely to be loss of pain and temperature sensation on the

ipsilateral face and contralateral body because of damage to the spinal
trigeminal tract and spinothalamic tract, respectively. If there is
damage to the medullary reticular formation, an ipsilateral Horner
syndrome may also be apparent in the patient.

Wernicke Aphasia

There are two main components to this type of aphasia. The first is
impairment in the comprehension of written and spoken language.
The second is an inability to speak substantive language; often times
these patients are unable to form some words correctly and seem to
inject words in their sentences that do not fit (paraphasia). These
persons cannot function independently in society because they are
unable to understand the meaning of written and spoken words, and
they are unable to tell others what they want or think because their
own speech is devoid of content. Since there may not be any difficulty
making the sounds of language, another name for this disorder is
'fluent or sensory' aphasia.

Lesions producing this disorder can be found in the boundary region of

the temporal and parietal lobes on the dorsolateral surface of the left
(in most persons) cerebral hemisphere. When this disorder is due to a
stroke there may be improvement to the point that the aphasia can no
longer be detected except through specific tests.
Table of Contents

Ailments of the Nervous System

1 Polio
2 Brain Tumor
3 Stroke
4 Meningitis
5 Broca Aphasia
6 Cerebello-Olivary Degeneration of Holmes
7 Choroid Plexus Papilloma
8 Huntington Disease
9 Klüver-Bucy Syndrome
10 Locked-In Syndrome
11 Alzheimer
12 Multiple Sclerosis
13 Parkinson Disease
14 Parinaud Syndrome
15 Pituitary Adenoma
16 Tourette Syndrome
17 Wallenberg Syndrome
18 Wernicke Aphasia