Degenerative Diseases

Degenerative Diseases

Multiple SclerosisParkinson’sAlzheimer’s

Myasthenia GravisAmiotrophic Lateral Sclerosis

Huntington’s DiseaseTourette’s Syndrome

Degenerative Diseases

• “Degenerative Diseases” refers to neurological disorders in which there is a premature aging of nerve cells– Caused by suspected metabolic disturbance or

unknown cause

Multiple Sclerosis

• Multiple Sclerosis– Chronic, progressive– Cause is unknown; genetic factor suspected due to

the higher rate among relatives

– Something goes wrong and induces the T cells (normally the “field commanders” of the immune system) to attack the body myelin damage occurs

Multiple Sclerosis

– A viral infection may be the beginning mechanism, but a defective immune response seems to have an important role in the pathology

– People living in temperate climates have an increased risk of the disease

Multiple Sclerosis

– Pathology: multi-foci of demyelination are distributed randomly in the white matter of the brainstem, spinal cord, optic nerves, and cerebrum causes an interruption or distortion of the nerve impulse

– There is some evidence of partial healing in areas of degeneration

Multiple Sclerosis

• Clinical Manifestations:• Onset is insidious and gradual• Vague symptoms that occur intermittently over

months or years• Visual problems; urinary incontinence; fatigue,

weakness, incoordination of an extremity; swallowing difficulty; sexual problems

Multiple Sclerosis

• Assessment: – Subjective data: pt. c/o eye problems, weakness or

numbness of a part of the body; fatigue, emotional instability, bowel/bladder problems; vertigo; loss of joint sensation; involvement of the cerebellum ataxia and tremor

– Objective data: nystagmus, muscle weakness and spasms; changes in coordination; dysarthria, dysphagia; urinary incontinence; emotional instability

Multiple Sclerosis

• Medical Management:• No specific treatment• Favorable results with: corticosteroids;

prednisone, ACTH; dexamethasone• Valium to treat muscle spasms• Interferon reduces frequency of

exacerbations; slows progress of physical disability

– Interferon is a low molecular wt. protein that regulates the extent and duration of the immune and inflammatory response

Multiple Sclerosis

• Nursing Interventions:• Nutrition: well balanced diet; high fiber• Skin Care: frequent turning; pressure-relief

devices• Activity: exercise regularly; avoid fatigue;

daily rest periods• Control of Environment: air-conditioned

surroundings during hot weather; peaceful, relaxed setting

Multiple Sclerosis

• Patient Teaching:– Environmental control– 24 hr care in late stages of disease; need caregiver– Support

• Prognosis:– Average life expectancy after the onset of

symptoms is > 25 years

Parkinson’s Disease

• A syndrome that includes:– A slowing down in the initiation and execution of

movement (bradykinesia)

– Increased muscle tone (rigidity)

– Impaired postural reflexes


• Involves:– Damage or loss of the dopamine-producing cells

in the midbrain depletion of the dopamine that influences the initiation, modulation, and completion of movement


• Disease shows no gender, socioeconomic, or cultural preference

• Symptoms commonly occur after 50 years old• Many causes: encephalitis, chemical toxins,

drug-induced (Aldomet, Haldol, Thorazine)• Signs/symptoms: tremors, muscle rigidity,

slowed movements, impaired balance


• Clinical Manifestations:– Beginning stages: mild tremor, slight limp,

decreased arm swing– Later: shuffling gait, arms flexed, loss of

postural reflexes; slight changes in speech pattern

• Diagnosis based solely on the hx., neurological exam, and clinical features

• Dementia occurs in approx. 40% of pts.


• Assessment:– Subjective data: c/o fatigue, incoordination of

movement, judgment defects, emotional instability, heat intolerance

– Objective data: presence of tremor (pill-rolling style), bradykinesia, muscle rigidity; mask-like facial appearance, drooling; swallowing may be abnormal


• Diagnostic Tests:– No specific dx. test for Parkinson’s

Firm dx. can be made when 2 of the 3 classic triad of symptoms present: tremor, rigidity, bradykinesia

– Dx. Confirmed with clinical exam and history

– CT scan may show cerebral atrophy

Ultimate confirmation of dx. is a positive response to antiparkinsonian drugs


• Medical management:– Goal: to ease the s/sx of the disease– Medications: esp. Carbid-levo (Sinemet)– Surgery:

• Ablation surgery: destroying portions of the brain that control the rigidity or tremor

• Deep brain stimulation: electrode placement connected to a generator implanted in the chest. A specific current is delivered to the targeted brain location improved motor performance and gait


• Nursing Interventions:– Activity needs: pay special attention to posture– Ambulation: when “freezes up”, usually need prn

medication; can also teach techniques such as stepping over pretend or real lines on the floor; avoid hurrying the patient

– Nutrition: easy to chew and swallow foods; cut into bite-size pieces; 6 small meals vs 3 large; aspiration precautions; don’t hurry.


• Nursing Interventions:– Elimination: toileting schedule and prn sense of

urgency; may also experience urinary hesitancy– Chronic constipation: high fiber diet; encourage

oral fluid intake; stool softeners, prune juice; mild cathartics/laxatives

– Patient Teaching: Medication schedule, skin care, keeping active; safe ambulation and positioning; proper feeding techniques

Alzheimer’s Disease

• Chronic, progressive, degenerative disorder that affects the cells of the brain and causes impaired intellectual functioning

• Possible genetic link

• Changes in the brain include “plaques” in the cortex and “neurofibrillary tangles” ( a tangled mass of non-functioning neurons) decrease in brain size


• Clinical manifestations:– 4 stages:

• Early: mild memory lapses; difficulty using the correct word; decreased attention span; disinterest in surroundings

• 2nd stage: increased memory lapses – esp. short-term memory; disorientation; “loses” personal belongings; confabulation; loss of ability to recognize familiar faces; loss of impulse control agitation


• 3rd stage: total disorientation, motor problems; unable to recognize objects; difficulty carrying out ADLs; wandering

• Terminal Stage: severe mental and physical deterioration; total incontinence


• Assessment: usually characterized by:– Memory loss – Inability to carry out normal activities

• Diagnostic Tests: CT or PET scan, EEG, MRI may be used to rule out other pathologic conditions. Family hx. of Alzheimer’s is significant


• Medical Management – Options are limited– To treat agitation: small doses of Lorazepam

(Ativan) or haloperidol (Haldol)– Mild cognitive impairment may respond to

Donepezil (Aricept) reducing the progression of AD; Memantine (Namenda)slows symptoms of moderate to severe AD


• Nursing Interventions/Patient Teaching– Maintaining adequate nutrition/hydration– Safety – Support with ADL– Education usually directed to the family

Amyotrophic Lateral Sclerosis


• Rare, progressive neurological disease death in 2-6 years

• Also known as Lou Gehrig’s disease

• Motor neurons in the brainstem and spinal cord gradually degenerate Chemical and electrical messages originating in the brain do not reach the muscles to activate them

• Primary symptoms: weakness of the UE, dysarthria, and dyphagia


• Death usually results from respiratory infection secondary to compromised respiratory function

• No cure• Medication: Rilutek slows the progression• Support and teaching provided by OT, ST, PT,

RD, RN, and psychological support• Nursing care is to support patient’s cognitive

function, provide diversional activities, help pt. and family do future planning and anticipatory grieving

Huntington’s Disease


• A genetically transmitted autosomal dominant disorder

• Affects men and women of all races

• Offspring have 50% chance of inheriting it

(diagnosis often occurs after the affected individual has had children)


• Pathology: affects the basal ganglia and extrapyramidal motor system– Overactivity of the dopamine pathway

• Clinical Manifestations: abnormal and excessive involuntary movements (chorea)– Writhing, twisting movements of the face, limbs, and

body. – Speech, chewing, and swallowing are affected

malnutrition and aspiration


• Clinical Manifestations cont.– Gait deteriorates ambulation difficulties

wheelchair bound– Mental function declines– Emotional lability– Psychotic behavior– Death usually occurs 10-20 yrs after the onset


• No cure• Therapeutic Management is palliative• Medications: antipsychotic, antidepressant,

and antichorea


• Nursing Interventions:– Goal: provide the most comfortable environment

possible for the patient and the family• Maintain physical safety• Treat physical symptoms• Provide emotional and psychological support• Nutritional / caloric needs

– Genetic counseling for the family

Tourettes Syndrome

Tourettes Syndrome

• A neurological disorder characterized by repetitive, stereotyped, involuntary movements and vocalizations called tics.

• Cause is unknown• Early Symptoms:

– Noticed first in childhood– Males affected 3-4x more than females

Tourettes Syndrome

• Symptoms: simple or complex– Simple motor tics: sudden, brief, repetitive

movements that involve a limited number of muscle groups

• E.g. eye blinking, facial grimacing, shoulder shrugging

– Complex tics: involve several muscle groups• E.g. facial grimacing combined with a head twist and a

shoulder shrug

Tourettes Syndrome

• Characteristics: – Tics are often worse with excitement; better during

calm, focused activities

– Tics come and go over time and vary in type, frequency, location and severity

– Tics peak in severity before mid-teen years; improvement for the majority of patients in late teen and early adulthood

Tourettes Syndrome

• Diagnosis: made after verifying that the patient has had both motor and vocal tics for at least 1 year.

• Neuroimaging tests and lab work may be used to rule out other conditions that might be confused with TS

Tourettes Syndrome

• Treatment: – Medication for those whose tics interfere with

functioning – e.g. neuroleptics (e.g.Thorazine)– Psychotherapy

• Prognosis: no cure; tics tend to decrease with age; some become symptom-free for a period of time. Neurobehavioral disorders may hamper normal functioning in adulthood.

Miscellaneous Slides

Myasthenia Gravis

• An autoimmune disease of the neuromuscular junction

• Characterized by fluctuating weakness of certain skeletal muscle groups

Myasthenia Gravis

Nerve impulses fail to pass at the myoneural junction muscle weakness

• Caused by an autoimmune process a decreased number of ACh receptor sites interference with impulse transmission to the muscle

Myasthenia Gravis

– Note: About 25% of pts. with MG have been found to have a thymoma and about 80% have changes in the cellular structure of the thymus gland

• Clinical Manifestations:– Ocular MG: drooping of eyelid, double vision– Generalized MG: Skeletal weakness of the

extremities, neck, shoulder, hands, diaphragm, vocal cords

Myasthenia Gravis

Ocular MG• Drooping of eyelid• Double vision

General MG• Skeletal weakness of the:

– Extremities– Neck– Shoulders– Hands– Diaphragm– Vocal cords

Myasthenia Gravis

• Assessment:– Subjective: pt. understanding of the disease; c/o

weakness, double vision; difficulty chewing or swallowing ; presence of bowel or bladder incontinence

– Objective: documented muscle weakness on neurological assessment; nasal-sounding speech; drooping eyelids; weight loss with swallowing problems

Myasthenia Gravis

• Diagnostic Tests: – Have the pt. look upward for 2-3 min. MG

confirmed if increased droop of eyelids so that the person can barely keep the eyes open

– EMG– Lab tests: serum testing for antibodies to

acetylcholine receptors– IV infusion of a short-acting cholinesterase

Myasthenia Gravis

• Medical Management– Use of medications:

• Anticholinesterase drugs: Prostigmine, Mestonin

• Corticosteroids• Immunosuppressive medications: e.g. Imuran,

Cytoxan– Plasmapheresis - Short term

Myasthenia Gravis

• Medical Management• Thymectomy – indicated for almost all patients.

improvement in all patients

• Administration of IV immune globulin to reduce the production of acetylcholine antibodies

Myasthenia Gravis

• Nursing Interventions/Patient Teaching• Priority: Facilitate effective airway exchange.

Respiratory problems frequently occur in MG patients

• Teach patient airway protective techniques

• Pace daily activities• ROM exercises

• Medication management based on sx.

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Degenerative Diseases