Transcript Degenerative Neurologic Diseases

eg enera tive
D isea ses
Degenerative Disorders
Disorders of the central and peripheral nervous
system that are neurodegenerative characterized
by slow onset of signs and symptoms.
Signs and symptoms that leads to the deterioration
of normal cells or function of the nervous system.
Patients are mainly managed at home for as long as
possible and are admitted to the acute care setting
for exacerbations, treatments, and surgical
interventions as needed.
The Disorders Include:
 Parkinson’s Disease (PD)
 Huntington’s Disease (HD)
 Alzheimer’s Disease (AD)
 Amyotrophic Lateral Sclerosis (ALS)
 Muscular Dystrophies
 Degenerative Disk Disease (DDD)
 Herniation of a Cervical Intervertebral Disk
 Herniation of a Lumbar Disk
Parkinson’s Disease (PD)
Parkinson’s Disease
Parkinson's disease is a disorder that affects nerve cells in the
part of the brain controlling muscle movement.
People with Parkinson's disease often experience trembling,
muscle rigidity, difficulty walking, problems with balance
and slowed movements.
Symptoms usually develop after age 60, although some
people affected by Parkinson's disease are younger than
age 50.
Although Parkinson's disease may eventually be disabling, the
disease often progresses gradually, and most people have
many years of productive living after a diagnosis.
Causes
Most people with Parkinson's disease are
described as having idiopathic PD (having no
specific cause).
There are far less common causes of PD
including genetic, toxins, head trauma,
cerebral anoxia, and drug-induced PD.
Head Trauma
Past episodes of head trauma are reported more
frequently by sufferers than by others in the
population.
A methodologically strong recent study found
that those who have experienced a head injury
are four times more likely to develop
Parkinson’s disease than those who have
never suffered a head injury.
Drug Induced PD
Antipsychotics, which are used to treat
schizophrenia and psychosis, can induce the
symptoms of Parkinson's disease (or
parkinsonism) by lowering dopaminergic
activity.
Pathophysiology of PD
The symptoms of Parkinson's disease result from the
loss of pigmented dopamine-secreting
(dopaminergic) cells, secreted by the same cells, in
the pars compacta region of the substantia nigra
These neurons project to the striatum and their loss
leads to alterations in the activity of the neural
circuits within the basal ganglia that regulate
movement, in essence an inhibition of the direct
pathway and excitation of the indirect pathway
Pathophysiology of PD
The direct pathway facilitates movement and
the indirect pathway inhibits movement, thus
the loss of these cells leads to a hypokinetic
movement disorder.
The lack of dopamine results in increased
inhibition of the ventral lateral nucleus of the
thalamus, which sends excitatory projections
to the motor cortex, thus leading to
hypokinesia.
Bradykinesia
Rigid Muscles
Tremors
Dementia
Clinical Manifestations
Loss of
Automatic
Movements
Difficulty
Swallowing
Impaired
Speech
Impaired
Balance
Levodopa
Selegiline
Eldepryl
Medications
Anticholinergics
Cathechol – o –
Methyltransferase
Dopamine
Agonist
Thalatomy
Pallidotomy
Surgical Management
Deep Brain
Stimulation
Physical Exercise and
Speech Therapies
Complications
Difficulty chewing and swallowing.
Urinary problems.
Constipation
Sleep problems
Sexual dysfunction
Nursing Management
Provide information and support regarding Parkinson’s
Disease.
Health teaching, diet, high in fiber, increase oral fluid
intake to prevent constipation.
Promote relaxation exercises to promote proper sleep.
Improving mobility.
Enhancing self-care activities.
Improving bowel elimination
Improving nutrition.
Enhancing swallowing.
Huntington’s Disease (HD)
Huntington’s Disease
Huntington’s disease is a chronic, progressive, hereditary disease
of the nervous system that results in progressive involuntary
choreinform movement and dementia.
It is transmitted as an autosomal dominant genetic disorder.
Also called Huntington's chorea, “chorea” comes from the greek
word meaning “to dance” and refers to the incessant quick,
jerky, involuntary movements that are characteristic of this
condition.
Each child of a parent with Huntington’s disease has a 50% risk of
inheriting the illness.
It affects men and women.
Cause of HD
Huntington's disease is an inherited condition caused by
a single abnormal gene.
Because only one copy of the defective gene, inherited
from either parent, is necessary to produce the
disease.
Because signs and symptoms typically first appear in
middle age, some parents may not know they carry
the gene until they've already had children and
possibly passed on the trait.
Pathophysiology of HD
Pathophysiology involves premature death of cells in the
stiatum (caudate and putamen of the basal ganglia, which
involve the control movement.
There is also loss of cells in the cortex, it is the region of the
brain associated with thinking, memory, perception, and
judgement and also the cerebellum that coordinates
voluntary muscle activity.
Building block for protein called gluthamine abnormally
collects in the nucleus, causing cell death.
The cell’s destruction results in a lack of the
neurotransmitters gamma-aminobutyric acid. This is
usually occurs in ages between 35 to 45 years.
Assessment and Diagnosis
CT scan and MRI may show atrophy of the Caudate
Nuclei once the disease is well established.
A genetic marker for Huntington’s disease has been
identified through the use of recombinant DNA
technology. As a result, researchers can now identify
presymptomatic individual who will develop this
disease.
Clinical Manifestations
Abnormal involuntary movements (chorea), intellectual
decline, and emotional disturbances.
Constant writhing and uncontrollable movements of the
entire body occur as this disease progresses.
Facial movements produce ticks and grimaces; speech
becomes slurred, hesitant, open explosive, and then
eventually unintelligible.
Chewing and swallowing are difficult, and aspiration and
choking are dangers.
Gait becomes disorganized, and ambulation is eventually
impossible; patient is eventually confined to a
wheelchair.
Management
Medications
Tranquilizers such as clonazepam (Klonopin) and
antipsychotic drugs such as haloperidol (Haldol) and
clozapine (Clozaril) can help control movements, violent
outbursts and hallucinations.
While these medications can be helpful, a common side effect
is sedation, and in some cases, these medications may
cause additional stiffness and rigidity.
Various medications, including fluoxetine (Prozac, Sarafem),
sertraline (Zoloft) and nortriptyline (Aventyl, Pamelor), can
help control depression and the obsessive-compulsive
rituals that some people with Huntington's disease develop.
Medications such as lithium (Eskalith, Lithobid) can help
control extreme emotions and mood swings.
Physical and Occupational Therapy
Physical therapy can help keep muscles stronger and
more flexible, which helps maintain balance and may
lessen the risk of falling.
Occupational therapy can help make your home safer
and give you strategies for coping with memory and
concentration problems.
Later in the disease, occupational therapy can assist you
with eating, dressing and hygiene challenges.
Complications
Though the signs and symptoms vary from person to
person, vital functions, such as swallowing, eating,
speaking and walking, usually degenerate over time.
Many people with Huntington's disease develop
depression, and some are at risk of suicide. However,
death generally occurs as a result of complications of
the disease, such as an infection or a fall.
Nursing Management
Reinforcement the understanding that Huntington’s
disease takes emotional, physical, social, and
financial tolls on every member of the patient’s
family.
Encourage genetic counseling, long term psychological
counseling, marriage counseling, and financial and
legal support.
Teach patient and family about the medication,
including signs indicating the need for change in
dosage or medication.
Nursing Management
Address strategies to manage symptoms (chorea,
swallowing problems, ambulation problems, or
altered bowel or bladder function).
Emphasize the need for regular follow-up.
Refer for home care nursing assistance, respite care,
day care centers, and eventually skilled long-term
care to assist patient and family cope.
Provide information about the Huntington’s Disease
Foundation of America, which gives information,
referrals, education, and support for reaserch.
Alzheimer’s Disease (AD)
Alzheimer’s Disease
Alzheimer's disease is the most common cause
of dementia, which is the loss of intellectual
and social abilities severe enough to interfere
with daily functioning.
Dementia occurs in people with Alzheimer's
disease because healthy brain tissue
degenerates, causing a steady decline in
memory and mental abilities.
Cause of AD
The causes of Alzheimer's are poorly
understood, but its effect on brain tissue has
been demonstrated clearly. Alzheimer's
damages and kills brain cells.
Studies of plaques and tangles from the brains of
people who have died of Alzheimer's suggest
several possible roles these structures might
play in the disease.
Neural Plaques and AD
Plaques are made up of a normally harmless
protein called beta-amyloid.
Although the ultimate cause of neuron death in
Alzheimer's isn't known, mounting evidence
suggests that a form of beta-amyloid protein
may be the culprit.
Assessment and Diagnosis
Medical History
Basic Medical Test (e.g. blood
tests)
Mental Status Evaluation
Neuropsycological Testing
Brain Scans
AD’s Different Stages
Stage 1. Mild memory lapses
Stage 2. Obvious short- term memory lapses
Stage 3. Disintegrations of personality
Stage 4. Terminal Stage – physical and mental
deterioration
Loss of
Judgement
Disorientation
Personality
Changes
Difficulty Finding
The Right Word
Clinical Manifestations
Increasing and
Difficulty with
Persistent
Abstract Thinking
Forgetfulness
Difficulty Performing
Familiar Tasks
Management of AD
Currently, there is no cure for AD. Medications
are prescribed to offer relatively small
symptomatic relief.
Cholinesterase Inhibitors
This group of medications, which includes
donepezil (Aricept), rivastigmine (Exelon) and
galantamine (Reminyl) works by improving the
levels of neurotransmitters in the brain.
Side effects include diarrhea, nausea, and
vomiting.
Memantine (Namenda)
The first drug approved to treat moderate to severe
stages of Alzheimer's, memantine (Namenda),
protects brain cells from damage caused by the
chemical messenger glutamate.
It sometimes is used in combination with a
cholinesterase inhibitor.
Most common side effect is dizziness, and may increase
agitation and delusional behavior.
Amyotrophic Lateral Sclerosis (ALS)
Amyotrophic Lateral Sclerosis
Amyotrophic lateral sclerosis (ALS) is a serious
neurological disease that attacks the nerve cells that
control voluntary muscles.
It may begin with muscle twitching or weakness in an
arm or leg, or with slurring of speech.
Eventually, amyotrophic lateral sclerosis affects your
ability to control the muscles needed to move, speak,
eat and breathe.
Cause of ALS
Amyotrophic lateral sclerosis is a disease that involves
death of motor neurons, the nerve cells that control
voluntary muscles.
In ALS, both upper motor neurons, located in your
brain, and lower motor neurons, located in your spinal
cord, gradually die.
ALS is an inherited disease.
The other type of ALS appears to occur randomly with
no identifiable cause and no obvious risk factors.
Clinical Manifestations
Difficulty lifting the front part of your foot (footdrop)
Weakness in your leg, feet or ankles
Hand weakness or clumsiness
Slurring of speech or trouble swallowing
Muscle cramps and twitching in your arms, shoulders and
tongue
Fatigue in combination with the above signs and symptoms
Eventually, amyotrophic lateral sclerosis paralyzes the muscles
needed to breathe.
Most people with ALS die of respiratory failure, usually within
three to five years after symptoms begin.
Treatment and Management
Currently, there is no treatment for ALS. Treatment of
amyotrophic lateral sclerosis typically focuses on
efforts to relieve symptoms and maintain quality of
life in the years after diagnosis.
May need tube feedings
Activity as tolerated
Dantrolene (Dantrium) or baclofen (Lioresal) for muscle
spasticity
I.V. or intrathecal administration of thyrotropinreleasing hormone
Riluzole (Rilutek) to delay progression of disease
Muscular Dystrophies
Muscular Dystrophies
Muscular dystrophy (MD) is a group of inherited muscle
diseases in which muscle fibers are unusually
susceptible to damage.
Muscles, primarily voluntary muscles, become
progressively weaker. In the late stages of muscular
dystrophy, fat and connective tissue often replace
muscle fibers.
Some types of muscular dystrophy affect heart
muscles, other involuntary muscles and other organs.
Causes of Muscular Dystrophies
Muscular dystrophy is a general term for a group of
inherited diseases involving a defective gene.
Each form of muscular dystrophy is caused by a genetic
mutation that's particular to that type of the disease.
The most common types of muscular dystrophy appear
to be due to a genetic deficiency of the muscle
protein dystrophin.
Clinical Manifestation
Muscle weakness
Apparent lack of coordination
Progressive crippling, resulting in fixations
(contractures) of the muscles around your joints and
loss of mobility
Weakening of voluntary muscles that control your arms
and legs, usually beginning with the limb muscles
farthest from the torso — the muscles of the feet,
hands, lower legs and forearms.
Treatment and Management
There are currently no cures for any forms of muscular
dystrophies.
Treatment and management are aimed to decrease the
severity of symptoms and reduce deformities brought
about by the disease.
Physical Therapy
Assistive Devices
Surgery, to release contractures that may develop and
that can position joints in painful ways.
Anti-inflammatory corticosteroids
Medications to manage muscle spasms
Herniation of A Intervebral Disk
Herniation of an Intervertebral Disk
Low back pain is a significant public health disorder.
Acute low back pain lasts less than 3 months, whereas
chronic or degenerative disk disease lasts 3 months or
longer.
Cervical disc herniations occur in the neck, most often
between the sixth and seventh cervical vertebral
bodies.
Symptoms can affect the back of the skull, the neck,
shoulder girdle, scapula, shoulder, arm, and hand. The
nerves of the cervical plexus and brachial plexus can
be affected.
Herniation of an Intervertebral Disk
Lumbar disk herniations occur in the lower
back, most often between the fourth and fifth
lumbar vertebral bodies or between the fifth
and the sacrum.
Symptoms can affect the lower back, buttocks,
thigh, and may radiate into the foot and/or
toe.
The sciatic nerve is the most commonly affected
nerve, causing symptoms of sciatica.
Pathophysiology
The intervertebral disk is a cartilaginous plate that
forms a cushion between the vertebral bodies.
This tough, fibrous material is incorporated in a capsule.
A ball like cushion in the center of the disk is called
the nucleus pulposus.
In herniation, the nucleus of the disk protrudes into the
annulus, with subsequent nerve compression.
Pathophysiology
Protrusion or rupture of the nucleus pulposus usually is
preceeded by degenerative changes that occur with
changing.
Loss of protein polysaccharides in the disk decreases
the water content of the nucleus pulposus.
The development of radiating cracks in the annulus
weakens resistance to nucleus heriation.
Clinical Manifestations
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Chief complaint is leg pain greater than back pain.
Intense pain in the hip joint from a lumbar disk herniation.
Unrelenting neck or lower back pain.
Numbness
Tingling
Muscular weakness
Paralysis
Paresthesia
Affection of reflexes
Sciatica
Treatment and Management
Majority of herniated disks will heal themselves in about six
weeks and do not require surgery.
 If pain due to disc herniation, protrusion, bulge, or disc tear is
due to chemical radiculitis pain, then prior to surgery it may
make sense to try an anti-inflammatory approach.
 Often first attempted with NSAIDs.
 Epidural steroid injection for short term relief of pain
 Bad rest and lumbo-sacral support belt
 Physical therapy
 Osteopathic / chiropractic manipulations
 Massage therapy
 Oral steroids (for pain)
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Surgical Options
Anterior cervical discectomy and
fusion
Laminectomy
Hemilaminectomy
Lumbar fusions
Disk arthroplasty
Artificial disk replacement
Degenerative Disk Disease (DDD)
Degenerative Disk Disease (DDD)
Degeneration of the intervertebral disk, which is often
called "degenerative disk disease" (DDD) of the
spine, DDD is in fact a condition that can be painful
and can greatly affect the quality of one's life.
While disk degeneration is not a normal part of aging
and for most people is not a problem, for certain
individuals a degenerated disk can cause severe
constant chronic pain.
Causes of DDD
Trauma micro or macro as a simple
lifting accident is the most
common cause of disc
degeneration.
After trauma the discs in the spine
dehydrate, or dry out, and lose
their ability to act as shock
absorbers between the
vertebrae.
There is minimal blood supply to
the discs so they lack the ability
to heal or repair themselves.
Treatment and Management
Most DDD can be successfully treated without surgery.
One or combinations of physical therapy, osteopathy, antiinflammatory medications, chiropractic treatments, or spinal
injections.
 Anterior cervical discectomy and fusion
 Cervical corpectomy
 Facetectomy
 Foraminotomy
 Laminoplasty
 Laminotomy
 Micro-discectomy
 Spinal laminectomy
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