Management of Neurological Disorders - NRSG 3013

Management of Patients With Oncologic or Degenerative Neurological Disorders

• Covers management of patients with oncologic or degenerative neurological disorders.
• This focuses on Huntington's disease, Amyotrophic Lateral Sclerosis (ALS), and Muscular Dystrophy (MD).
• The content is adapted from Chapter 67 (4th ed.) of a nursing textbook, NRSG 3013.

Huntington's Disease

• A chronic, progressive, hereditary disease.
• Results in involuntary choreiform movements and dementia.
• Transmitted as an autosomal dominant trait.
• Pathologic process involves loss of cells in the striatum of the basal ganglia and cortex.
• Early brain damage occurs in the striatum.
• Microscopically, medium spiny neurons show yellow nuclear inclusions.

Huntington's Disease - Clinical S&S

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

Diagnosing Huntington's Disease (HD)

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

Huntington's: Symptom Management (cont.)

• Counseling for depression
• Continence products for bowel/bladder function
• Maintaining physical fitness
• Monitoring for aspiration pneumonia
• Fall risk assessment
• Risk for malnutrition
• Risk for injury

Huntington's: Client Teaching

• No cure or reversal of HD.
• Genetic counseling
• Worsens over time
• Grief counseling for individuals and families
• Discussion of end-of-life care

Huntington's: Home Teaching

• Swallowing and feeding complications: need for high calorie/protein, soft or pureed diet, thickened fluids.
• Importance of exercise (even passive ROM).
• Education on medications and side effects (S/E).

Huntington's: Caregiver Strategies

• Respite care and home care
• Caregiver self-care and stress management strategies
• Social worker involvement
• Speech pathologist for swallowing assessment
• Physiotherapist for mobility assessment
• Palliative care team
• Genetic counseling

Amyotrophic Lateral Sclerosis (ALS)

• Information for PN: Most cases manageable at home.
• Common hospitalizations from dehydration, malnutrition, pneumonia, and respiratory failure.
• Early recognition is needed.
• G-tube placement may be a possibility for nutrition

Muscular Dystrophy

• A group of chronic disorders with progressive muscle weakness and wasting.
• Duchenne muscular dystrophy is the most common type, affecting 1/3,000 male births
• Inherited genetic counseling recommended.
• Results in degeneration and permanent loss of muscle fibers, affecting many as teenagers or young adults
• Clinical manifestations: Progressive crippling, loss of mobility, spinal deformities.

Muscular Dystrophy: Symptom Management

• No specific treatment
• Physical, respiratory, and speech therapy
• Orthopaedic appliances (braces)
• Individualized exercise programs to prevent muscle tightness and contractures.

Muscular Dystrophy: Pharmacology

• Corticosteroids (not long-term): To slow muscle degeneration
• Anticonvulsants: To control seizures and some muscle activity
• Immunosuppressants: To slow damage to dying muscle cells.
• Antibiotics: To fight respiratory infections.

Muscular Dystrophy: Caregiver Strategies

• Muscular Dystrophy Association information
• Feelings of helplessness, grief, mourning
• End-of-life options
• Psychiatric nurse consultation/referral
• Respite care
• Outpatient therapy (OT, PT)
• Hospitalization when necessary.

Muscular Dystrophy: Information for PN

• Onset differs by age and gender, depending on the type of MD.
• Spinal cord deformity and weak trunk muscles; orthotic jackets may be needed; assess for compromised pulmonary function.
• Dental and speech problems related to communication.
• Prognosis varies based on type and progression of MD; can lead to early death.
• Hereditary component necessitates genetic testing
• Risk of falls.

General Patient Education for Neurological Disorders

• Recognizing early symptoms of problems
• Completing advanced directives (code status)
• Risk for falls (spasticity)
• Prevention of aspiration
• Risk of nutritional imbalances
• Home care
• Hospital care (when needed)
• Community resources
• Online and reading resources.

Review Questions and Case Studies

• Questions cover diagnosis, assessment, interventions (e.g., splints, orthotic jackets, tube feeding), and appropriate nursing actions. Specific information for patients with Huntington's, ALS, and Muscular Dystrophy provide detailed clinical reasoning and decision-making.

This quiz focuses on the management of patients with oncologic or degenerative neurological disorders, specifically Huntington's disease, ALS, and muscular dystrophy. Drawing from Chapter 67 of the NRSG 3013 nursing textbook, it examines the clinical signs and symptoms associated with these conditions, as well as their pathophysiology.