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DEGENERATIVE DISORDER

DEGENERATIVE NEUROLOGIC DISORDERS

– Disorders of the central and peripheral nervous system that are neurodegenerative
– Slow onset of signs and symptoms.
– Deterioration of normal cells or function of the nervous system.
The Disorders Include:
– Parkinson’s Disease (PD) Muscular Dystrophies (MD)
– Huntington’s Disease (HD) Degenerative Disk Disease (DDD)
– Alzheimer’s Disease (AD) Herniation of a Cervical & Lumbar Disk
– Amyotrophic Lateral Sclerosis (ALS)

PARKINSON’S DISEASE
– Affects nerve cells in the part of the brain controlling muscle movement.
– Trembling, muscle rigidity, difficulty walking, problems with balance and slowed movements.
– after age 60, some younger than age 50. eventually become disabling,
– progresses gradually
– Most people have many years of productive living after a diagnosis.
Causes
• Most people with Parkinson's disease are described as having idiopathic
• There are far less common causes of PD:
• Genetic, toxins, 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.
• those who have experienced a head injury are four times more likely to develop Parkinson’s
Drug Induced PD
• Antipsychotics, by lowering dopaminergic activity.
Pathophysiology of PD
• result from the loss of dopamine-secreting (dopaminergic) cells, in the substantia nigra
• their loss leads to alterations in the activity of the neural circuits within the basal ganglia
• loss of these cells leads to a hypokinetic movement disorder.
• The lack of dopamine results in increased inhibition of the thalamus, which sends excitatory projections to the motor
cortex,
• thus leading to hypokinesia.
Clinical Manifestations
• Tremors • Impaired
• Bradykinesia • Speech
• Rigid Muscles • Impaired
• Dementia • Balance
• Loss of • Difficulty
• Automatic • Swallowing
• Movements
Medications
• Levodopa
• Selegiline Eldepryl
• Anticholinergics
• Cathechol – o –Methyltransferase
• Dopamine
• Agonist
Surgical Management
• Thalatomy
• Pallidotomy
• Deep Brain Stimulation
Physical Exercise and Speech Therapies
Nursing Management • Improving mobility.
• Provide information
• Health teaching, diet, high in fiber, increase oral
• Self-care activities.
fluid • Improving nutrition.
• Enhancing swallowing.
• Relaxation exercises to promote proper sleep.

HUNTINGTON’S DISEASE (HD)


– Chronic, progressive, hereditary disease of the nervous system progressive involuntary choreinform movement and dementia.
– autosomal dominant genetic disorder.
– Also called Huntington's chorea, “chorea” comes from the greek word meaning “to dance” incessant quick, jerky, involuntary
movements
Cause of HD
• 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
• Premature death of cells in the striatum (caudate and putamen of the basal ganglia) which involve the control movement.
• Loss of cells in the cortex, region of the brain associated with thinking, memory, perception, and judgement and also in
the cerebellum that coordinates voluntary muscle activity.
• Gluthamine abnormally collects in the nucleus, causing cell death.
• 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
• A genetic marker has been identified through 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 as the 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, eventually confined to a wheelchair.
Management
Medications
• Tranquilizers such as clonazepam (Klonopin) andantipsychotic haloperidol (Haldol) and clozapine (Clozaril) ---can help
control movements, violent outbursts and hallucinations.
Side effects: sedation, stiffness and rigidity.
• fluoxetine (Prozac, Sarafem), sertraline (Zoloft) and nortriptyline (Aventyl, Pamelor)---can help control depression and
the obsessive-compulsive rituals
• Medications such as lithium (Eskalith, Lithobid)--- control extreme emotions and mood swings.
Physical and Occupational Therapy
• Physical therapy
• Occupational therapy
---Later can assist you with eating, dressing and hygiene challenges.
Complications
• Swallowing, eating, speaking and walking, usually degenerate over time.
• Depression, and some are at risk of suicide.
• Death generally occurs as a result of complications of the disease, such as an infection or a fall.
Nursing Management
• takes emotional, physical, social, and financial tolls
• genetic counseling
• marriage counseling

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.
Cause of AD
• Alzheimer's damages and kills brain cells.
• plaques and tangles from the brains
Neural Plaques and AD
• Plaques are made up of a normally harmless protein called beta-amyloid, which later on 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
Clinical Manifestations
• Disorientation • Difficulty finding the right word
• Loss of • Increasing and persistent forgetfulness
• Judgement • Difficulty Performing Familiar Tasks
• Personality • Difficulty with Abstract Thinking
• Changes
Management of AD
• Currently, there is no cure for AD.
• Medications are prescribed to offer relatively small symptomatic relief.
Cholinesterase Inhibitors
• 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, ---protects brain cells from damage caused by
the chemical messenger glutamate.
• Most common side effect is dizziness, and may increase agitation and delusional behavior.

AMYOTROPHIC LATERAL SCLEROSIS (ALS)


– serious neurological disease
– attacks the nerve cells that control voluntary muscles.
– begin with muscle twitching or weakness in an arm or leg, or with slurring of speech.
– Eventually, affects your ability to control the muscles needed to move, speak, eat and breathe.

Cause of ALS
• 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.
• inherited disease.
Clinical Manifestations
• Difficulty lifting the front part of your foot • Muscle cramps and twitching in your arms,
(footdrop) shoulders and tongue
• Weakness in your leg, feet or ankles • Eventually, paralyzes the muscles needed to
• Hand weakness or clumsiness breathe.
• Slurring of speech or trouble swallowing • Die of respiratory failure, usually within three to
five years after symptoms begin.
Treatment and Management
• Currently, there is no treatment for ALS. • Activity as tolerated
• Treatment focuses on efforts to relieve symptoms • Dantrolene (Dantrium) or baclofen (Lioresal) for
and maintain quality of life muscle spasticity
• May need tube feedings • Riluzole (Rilutek) to delay progression of disease

MUSCULAR DYSTROPHIES
– Muscular dystrophy (MD) is a group of inherited muscle diseases
– Muscles, primarily voluntary muscles, become progressively weaker.
– In the late stages of muscular dystrophy, fat and connective tissue often replace muscle fibers.
Causes of Muscular Dystrophies
• Inherited diseases involving a defective gene.
• 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)
• 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 • Surgery, to release contractures that may develop
• Treatment and management are aimed to • Anti-inflammatory corticosteroids
decrease the severity of symptoms and reduce • Medications to manage muscle spasms
deformities brought about by the disease.

HERNIATION OF A INTERVEBRAL DISK


– Low back pain
– 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.
– 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. .
– 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.
Clinical Manifestations
• 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.
Treatment and Management
• Majority of herniated disks will heal themselves in • Bed rest and lumbo-sacral support belt
about six weeks and do not require surgery. • Physical therapy
• Often first attempted with NSAIDs. • Osteopathic / chiropractic manipulations
• Epidural steroid injection for short term relief of • Massage therapy
pain • Oral steroids (for pain)

DEGENERATIVE DISK DISEASE (DDD)


– Degeneration of the intervertebral disk, often called "degenerative disk disease" (DDD) of the spine,
– 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, anti-inflammatory medications,
• Anterior cervical discectomy and fusion
• Spinal laminectomy