1. Parkinson’s disease: progressive disease of the brain occurring generally in later life; characterized by stiffness of muscles and by tremors.
    1. Pathophysiology: depigmentation of the substantia nigra of basal ganglia —> decreased dopamine (neurotransmitter necessary for proper muscle movement) —> decreased and slowed voluntary movement, masklike facies, and difficulty initiating ambulation. Decreased inhibitions of alpha-motoneurons —> increased muscle tone —> rigidity of both flexor and extensor muscles and tremors at rest.
    2. Risk factors:
      1. Age >40; most often 50-60 yr.
      2. Affects men and women equally.
      3. Cause unknown; possibly connected to arteriosclerosis or viral infection.
      4. Drug-induced parkinsonian syndromes have been linked to:
        1. Phenothiazines.
        2. Reserpine (Serpasil).
        3. Butyrophenones (haloperidol).
    3. Assessment:
      1. Subjective data:
        1. Insomnia.
        2. Depression.
        3. Defects in judgment related to emotional instability; dementia (memory loss).
      2. Objective data:
        1. Limbs, shoulders: stiff, offer resistance to passive ROM.
        2. Loss of coordination, muscular weakness with rigidity.
        3. Shuffling gait: difficulty in initiating, then propulsive, trunk bent forward.
        4. Tremors: pill-rolling of fingers, to-andfro head movements.
        5. Loss of postural reflexes.
        6. Weight loss, constipation.
        7. Difficulty in maintaining social interactions because of impaired speech, lack of facial affect, drooling.
        8. Facies: wide-eyed, decreased eye blinking, decreased facial expression.
        9. Akinesia (abnormal absence of movement).
        10. Excessive salivation, drooling.
        11. Speech: slowed, slurred.
        12. Judgment defective (e.g., poor decision making); intelligence intact.
        13. Heat intolerance.
    4. Analysis/nursing diagnosis:
      1. Impaired physical mobility related to loss of coordination.
      2. Altered health maintenance related to defective judgment.
      3. Risk for injury related to altered gait.
      4. Dressing/grooming self-care deficit related to muscular rigidity.
      5. Sleep pattern disturbance related to insomnia.
      6. Body image disturbance related to tremors and drooling.
      7. Social isolation related to altered physical appearance.
      8. Altered nutrition, less than body requirements, related to lack of appetite.
      9. Impaired swallowing related to excessive drooling.
      10. Constipation related to dietary changes.
    5. Nursing care plan/implementation:
      1. Goal: promote maintenance of daily activities.
        1. ROM exercises, skin care, physical therapy.
        2. Encourage ambulation; discourage sitting for long periods.
        3. Assist with meals - high protein, high calorie; soft diet; small, frequent feedings; encourage increased fluids.
        4. Encourage compliance with medication regimen:
          1. Dopamine agonists:
            1. Levodopa: given in increasing doses until symptoms are relieved; given with food to decrease GI symptoms. Side effects: nausea, vomiting, anorexia, postural hypotension, mental changes, cardiac arrhythmias. Levodopa (L-Dopa) helps restore dopamine deficiency in striated muscles: 500-1000 mg/day in divided doses; increase by 100-750 mg/day every 3-7 days until response reached; usual maintenance dose should not exceed 18 g/day.
            2. Carbidopa-levodopa (Sinemet): 25/250 mg/day in 3-4 divided doses. Limits the metabolism of levodopa peripherally and provides more levodopa for the brain.
          2. Anticholinergics: effective in lessening muscle rigidity.
            1. Benztropine mesylate (Cogentin): 0.5-6 mg/day in 1-2 divided doses.
            2. Biperiden (Akineton): 2 mg 3-4 times daily, not to exceed 16 mg/day.
            3. Trihexyphenidyl (Artane): 1-2 mg/day; increased by 2 mg every 3-5 days; usual maintenance dose 5-15 mg/day in 3-4 divided doses.
          3. Antihistamines: have mild central anticholinergic properties.
      2. Goal: protect from injury.
        1. Monitor BP, side effects of medications, , orthostatic hypotension.
        2. Monitor for GI disturbances.
        3. Avoid pyridoxine (vitamin B6): cancels effect of levodopa.
        4. Levodopa contraindicated with:
          1. Glaucoma (causes increased intraocular pressure).
          2. Monoamine oxidase (MAO) inhibitors (causes possible hypertensive crisis).
      3. Goal: health teaching.
        1. Teach patient and family about medications: dosage range, side effects, not discontinuing medications abruptly.
        2. Exercise program to maintain ROM and normal body posture; also to get adequate rest to prevent fatigue.
        3. Dietary adjustment and precautions regarding cutting food in small pieces to prevent choking, taking fluid with food for easier swallowing.
        4. Importance of adding roughage to diet to prevent constipation.
        5. Assist patient and family to adjust to this chronic debilitating illness.
    6. Evaluation/outcome criteria:
      1. Activity level maintained.
      2. Symptoms relieved by medications; no drug interactions.
      3. Complications avoided.

  2. Amyotrophic lateral sclerosis (ALS; Lou Gehrig’s disease): progressive degeneration of motor neurons within the brain, spinal cord, or both, leading to death within 2-7 years, usually from infection (pneumonia) or consequences of respiratory or bul- bar paralysis. There are three types of ALS: classic sporadic, familial, and Mariana Islands (Guam).
    1. Pathophysiology: myelin sheaths destroyed, replaced by scar tissue; involves lateral tracts of spinal cord, eventually medulla and ventral tracts.
    2. Risk factors:
      1. Affects men more than women.
      2. Usually in middle age.
      3. Viral infection possible causal agent.
      4. Possible familial or genetic component.
    3. Assessment:
      1. Subjective data:
        1. Early symptoms: fatigue, awkwardness.
        2. Dysphagia, dysarthria.
        3. Alert, no sensory loss.
      2. Objective data:
        1. Symptoms depend on which motor neurons affected.
        2. Decreased fine finger movement.
        3. Progressive muscular weakness, atrophy, especially lower limbs at onset of disease; later, arms, hands, and shoulders.
        4. Spasticity of flexor muscles; one side of body becomes more involved than other.
        5. Progressive respiratory difficulties —> diaphragmatic paralysis.
        6. Progressive disability of upper and lower extremities.
        7. Fasciculations (muscle twitching visible under skin).
        8. Diagnostic tests:
          1. Electromyography (EMG).
          2. Nerve conduction velocities (NCV).
    4. Analysis/nursing diagnosis:
      1. Ineffective airway clearance related to difficulty in coughing.
      2. Ineffective breathing pattern related to progressive respiratory difficulties and eventually respiratory paralysis.
      3. Altered health maintenance related to inability to perform self-care activities.
      4. Impaired physical mobility related to progressive muscular weakness.
      5. Self-care deficits: bathing/hygiene and dressing/grooming.
      6. Powerlessness related to lifestyle of progressive physical helplessness.
      7. Impaired swallowing related to disease progression.
    5. Nursing care plan/implementation:
      1. Goal: maintain independence as long as possible.
        1. Assistance with ADL; splints, prosthetic devices to support weak limbs and maintain mobility.
        2. Skin care to prevent decubiti.
        3. Respiratory assistance as needed; ventilators as disease progresses and diaphragm becomes involve
        4. Soft/liquid diet to aid in swallowing, prevent choking; suction prn; head of bed elevated when eating.
        5. Arrange long-term care arrangements if home maintenance no longer feasible.
        6. Emotional support, when patient is alert; continue involving patient in decisions regarding care.
        7. Medications: riluzolet (Rilutek); has some effect in reducing disease progression; other drugs offer temporary relief of symptoms.
      2. Goal: health teaching.
        1. Skin care to prevent decubitus ulcer.
        2. Explain ramifications of disease so patient and family can make decisions regarding future care, whether patient will remain at home as disease progresses or enter a long-term care facility.
        3. How to use suction apparatus to clear airway.
        4. Care of nasogastric or gastrostomy feeding tube.
    6. Evaluation/outcome criteria:
      1. Obtains physical and emotional support.
      2. Complications avoided in early stage of disease.
      3. Remains in control of ADL as long as possible.
      4. Skin breakdown avoided.
      5. Peaceful death.