Management of Degenerative Neurological Disorders

Management of Patients With Oncologic or Degenerative Neurological Disorders

• Chapter 67 (4th ed.)
• NRSG 3013
• Adapted by J. Black RN, BScN, MN

Degenerative Neurological Disorders

• Huntington's Disease
• Amyotrophic Lateral Sclerosis
• Muscular Dystrophy

Huntington's Disease

• A chronic, progressive, hereditary disease.
• Causes progressive involuntary choreiform movements and dementia.
• Transmitted as an autosomal dominant trait.
• The pathologic process involves the loss of cells in the striatum of the basal ganglia and the cortex.
• Affects any age.
• Also known as Huntington's chorea (Greek: dance)
• Damages motor cortex
• Area of the brain most damaged in early Huntington's disease is the striatum.
• Microscopic image of medium spiny neurons (yellow) with nuclear inclusions (orange) is part of disease process.
• Huntington's disease is inherited in an autosomal dominant fashion.
• Probability of each offspring inheriting an affected gene is 50%.
• Inheritance is independent of gender, and the phenotype does not skip generations.

Huntington's - Clinical Signs and Symptoms

• Early: Mood swings (anger, depression, irritability), emotional disturbances, trouble driving, learning new things, remembering facts, making decisions.
• Late: Difficulty concentrating, difficulty feeding self, swallowing difficulties, chorea (uncontrolled movements), speech/visual impairment, impairment of voluntary movements.

Diagnosing Huntington's Disease

• History and physical: neurological assessment, mini-mental assessment, depression assessment
• Genetic testing

Huntington's - Symptom Management

• No treatment halts or reverses the disease.
• Pharmacology:
  • Chorea: Navane or Haldol (blocks dopamine receptors)
  • Rigidity: Levodopa
  • Involuntary writhing: Tetrabenazine
  • Depression: antidepressants
  • Anxiety: phenothiazine
• Speech impairment: difficulty understanding and expressing oneself.
• Counseling for depression.
• Loss of bowel/bladder function: continence products.
• Maintain physical fitness.
• Monitor for aspiration pneumonia.
• Fall risk.
• Risk for malnutrition.
• Risk for injury.

Huntington's - Client Teaching

• No way to stop or reverse the disease.
• Genetic counseling.
• The disease worsens over time.
• Individual and family may need grief counseling.
• Discussion of end-of-life issues.

Huntington's - Home Teaching

• Swallowing and feeding complications: high-calorie/protein, soft/pureed diets, thickened fluids.
• Importance of exercise (even passive ROM).
• Education regarding medications and side effects.
• Socialization and support networks.
• Home safety.
• Health promotion and sleep routines.
• Medical check-ups and follow-ups with care.

Huntington's - Strategies for Caregivers

• Respite care, homecare.
• Caregiver self-care and stress management strategies.
• Social worker.
• Speech pathologist (swallowing assessment).
• Physiotherapist (mobility assessment).
• Palliative care team.
• Genetic counseling.

Amyotrophic Lateral Sclerosis (ALS)

• Also called Lou Gehrig's disease.
• Rapidly progressive, invariably fatal neurological disease.
• Onset typically in 50s-60s; more common in men.
• Affects approximately 3000 Canadians per year.
• Loss of motor neurons in anterior horns of spinal cord and motor nuclei of lower brain stem.
• Attacks nerve cells responsible for voluntary muscles, causing muscle atrophy.
• Autoimmune disease and free radical damage.

ALS - Clinical Manifestations

• Depends on the location of affected motor neurons.
• Fatigue, muscle weakness, cramps, fasciculations (twitching), incoordination.
• Anterior horn weakness in arms, trunk, legs, and spasticity.
• Spinal cord involvement (25%): difficulty walking, swallowing, speaking, breathing; risk for aspiration.
• Onset to death is approximately 3 years.

ALS - Symptom Management

• No specific treatment.
• Interventions to maintain function, well-being, and quality of life.
• Medications (e.g., riluzole (Rilutek)) to slow deterioration of motor neurons.
• Symptomatic therapy, rehabilitation.

ALS - Patient Education

• Recognizing early symptoms of problems.
• Completing advanced directives (code status).
• Risk for falls (spasticity).
• Risk for aspiration.
• Risk for nutritional imbalances.

ALS - Strategies for Caregivers

• ALS Association for information.
• Respite care, VON, home care relief.
• Hospitalization, if necessary.
• Grief counseling (ALS support groups).

Information for PN (Huntington's Disease and ALS)-

• Huntington's disease and ALS can often be managed at home.
• Common reasons for hospitalization include dehydration, malnutrition, pneumonia, and respiratory failure.
• Early recognition is critical.
• Possible need for a G-tube for nutrition.

Muscular Dystrophy

• A group of chronic disorders characterized by progressive weakness and wasting of voluntary (skeletal) muscles.
• Duchenne muscular dystrophy is the most common type (1 in 3,000 male births).
• Inherited (genetic counseling recommended).
• Degeneration and permanent loss of muscle fibers.
• Often diagnosed in teenagers or young adults.

Muscular Dystrophy - Clinical Manifestations

• Varying degrees of muscle wasting and weakness.
• Abnormal increases in blood muscle enzymes.
• Myopathic findings on EMG and muscle biopsy.
• Lack of coordination.
• Progressive crippling, resulting in contractures of the muscles around the joints.
• Loss of mobility.
• Spinal deformity.

Muscular Dystrophy - Symptom Management

• No specific treatment.
• Physical, respiratory, and speech therapy.
• Orthopaedic appliances (braces).
• Individualized exercise program to prevent muscle tightness and contractures.
• Medications(e.g., corticosteroids, anticonvulsants, immunosuppressants, antibiotics)
• Importance of early recognition.

Muscular Dystrophy - Client Education

• Assessing client progression and status.
• Assessing home for safety.
• Working with physical therapy, occupational therapy, and other therapies.
• Assessing client needs for supplies and equipment.
• Assessing family needs and assistance needed (social work, counselling, financial status).
• Home care.
• Hospital care (when needed).
• Community resources.
• Online and reading resources.

Muscular Dystrophy - Strategies for Caregivers

• Muscular Dystrophy Association for information.
• Feelings of helplessness, grief, and mourning.
• End-of-life options.
• Psychiatric nurse/referral.
• Respite care.
• Occupational therapy/physical therapy on an outpatient basis.
• Hospitalization (if needed).

Information for PN (Muscular Dystrophy)

• Onset differs in age and gender depending on the type of MD.
• Spinal cord deformity is a significant problem (weak trunk muscles—use orthotic jacket for support).
• Potential for compromised pulmonary function.
• Dental and speech problems may impact communication.
• Prognosis varies by type and progression, potentially leading to early death.
• Hereditary—genetic testing is required.
• Risk of falls.

This quiz focuses on Chapter 67 of NRSG 3013, which covers the management of patients with oncologic and degenerative neurological disorders, particularly Huntington's Disease and other related conditions. You will explore the characteristics, inheritance patterns, and pathological aspects of these disorders that affect motor function and cognition.