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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.

Degenerative Disorders

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

The Disorders Include:

Parkinson’s Disease (PD)

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.

Parkinson’s Disease

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.

Causes

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.

Head Trauma

Antipsychotics, which are used to treat schizophrenia and psychosis, can induce the symptoms of Parkinson's disease (or parkinsonism) by lowering dopaminergic activity.

Drug Induced 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.

Pathophysiology of PD

Clinical Manifestations

Tremors

Bradykinesia Rigid Muscles

ImpairedBalance

Dementia

DifficultySwallowing

ImpairedSpeech

Loss of Automatic

Movements

Medications

Anticholinergics

Cathechol – o –Methyltransferase

SelegilineEldepryl

DopamineAgonist

Levodopa

Surgical Management

Pallidotomy

Deep BrainStimulation

Thalatomy

Physical Exercise andSpeech Therapies

Difficulty chewing and swallowing.

Urinary problems.

Constipation

Sleep problems

Sexual dysfunction

Complications

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 eliminationImproving nutrition.Enhancing swallowing.

Nursing Management

Huntington’s Disease (HD)

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.

Huntington’s Disease

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.

Cause 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.

Pathophysiology of HD

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.

Assessment and Diagnosis

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.

Clinical Manifestations

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.

Management

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.

Physical and Occupational Therapy

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.

Complications

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.

Nursing Management

Alzheimer’s Disease (AD)

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.

Alzheimer’s Disease

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.

Cause of 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.

Neural Plaques and AD

Medical History

Basic Medical Test (e.g. blood tests)

Mental Status Evaluation

Neuropsycological Testing

Brain Scans

Assessment and Diagnosis

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

AD’s Different Stages

Clinical Manifestations

Disorientation

Loss ofJudgement

Difficulty FindingThe Right Word

Difficulty PerformingFamiliar Tasks

Difficulty withAbstract Thinking

Personality Changes

Increasing andPersistent

Forgetfulness

Currently, there is no cure for AD. Medications are prescribed to offer relatively small symptomatic relief.

Management of AD

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.

Cholinesterase Inhibitors

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.

Memantine (Namenda)

Amyotrophic Lateral Sclerosis (ALS)

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.

Amyotrophic Lateral Sclerosis

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.

Cause of ALS

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.

Clinical Manifestations

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 feedingsActivity as toleratedDantrolene (Dantrium) or baclofen (Lioresal)

for muscle spasticityI.V. or intrathecal administration of

thyrotropin-releasing hormoneRiluzole (Rilutek) to delay progression of

disease

Treatment and Management

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.

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.

Causes of Muscular Dystrophies

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.

Clinical Manifestation

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 TherapyAssistive DevicesSurgery, to release contractures that may

develop and that can position joints in painful ways.

Anti-inflammatory corticosteroidsMedications to manage muscle spasms

Treatment and Management

Herniation of A Intervebral 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.

Herniation of an Intervertebral Disk

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.

Pathophysiology

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

Clinical Manifestations

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)

Treatment and Management

Anterior cervical discectomy and fusion

LaminectomyHemilaminectomyLumbar fusionsDisk arthroplastyArtificial disk replacement

Surgical Options

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.

Degenerative Disk Disease (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.

Causes of DDD

Most DDD can be successfully treated without surgery.

One or combinations of physical therapy, osteopathy, anti-inflammatory medications, chiropractic treatments, or spinal injections.

Anterior cervical discectomy and fusion Cervical corpectomy Facetectomy Foraminotomy Laminoplasty Laminotomy Micro-discectomy Spinal laminectomy

Treatment and Management