Management of Neurological Disorders

Management of Patients with Oncologic or Degenerative Neurological Disorders

• This chapter covers the management of patients with oncologic or degenerative neurological disorders.
• Specific focus on Huntington's disease, Amyotrophic Lateral Sclerosis (ALS), and Muscular Dystrophy.

Huntington's Disease

• A chronic, progressive, hereditary disease.
• Results in involuntary choreiform movements and dementia.
• Transmitted as an autosomal dominant trait.
• Pathologic loss of cells in the striatum of the basal ganglia and cortex.
• Affects any age.
• Often called Huntington's chorea.
• Inherited chronic, progressive neurological disease.
• Involves involuntary choreiform movements and dementia.
• Autosomal dominant.
• Damages motor cortex.
• Affects most damaged area in early stages of the disease is the striatum.
• Inherited in an autosomal dominant pattern.
• Chance of inheriting affected gene is 50%.

Huntington's Disease - Clinical S&S

• Early: Mood swings (anger, depression, irritability), emotional disturbances, trouble driving, learning new things, remembering facts, and making decisions.

• Late: Difficulty concentrating, difficulty feeding self/swallowing, uncontrollable movements (chorea), speech/visual impairment, impairment of voluntary movements.

Diagnosing Huntington's Disease

• History and physical exam (neurological & mini-mental assessment, depression assessment)
• Genetic testing

Huntington's Disease - Symptom Management

• No treatment reverses the disease.
• Pharmacology:
  • Chorea: Navane or Haldol (blocks dopamine receptors).
  • Rigidity: Levodopa
  • Involuntary movement: Tetrabenazine
  • Depression: Antidepressants
  • Anxiety: Phenothiazines
• Speech impairment: Difficulty understanding or expressing oneself.
• Counseling for depression and other issues.
• Continence products for bowel/bladder function issues.
• Maintaining physical fitness.
• Monitor for aspiration pneumonia
• Fall risk assessment.
• Risk for malnutrition.
• Risk for injury.

Huntington's Disease - Client Teaching

• No cure or reversing the disease, Worsens over time
• Genetic counseling
• Individual and family grief counseling
• End-of-life discussions
• Swallowing and feeding issues - soft/pureed diets, and thickened fluids.
• Importance of exercise, including passive range of motion (ROM).
• Medication education and side effect recognition.
• Socialization and support systems.
• Safety measures in the home.
• Health promotion and sleep routines.
• Medical checkups and follow-up care.

Huntington's Disease - Strategies for Caregivers

• Respite care or home care.
• Caregiver self-care strategies.
• Social workers.
• Speech pathologists (swallowing assessment).
• Physiotherapists (mobility assessment).
• Palliative care team.
• Genetic counseling.

Amyotrophic Lateral Sclerosis (ALS)

• Also known as Lou Gehrig's disease.
• Rapidly progressive, invariably fatal neurological disease.
• Onset typically in the 50s or 60s; more common in men.
• Approximately 3000 Canadians affected per year.
• Loss of motor neurons in the anterior horns of the spinal cord and lower brain stem.
• Attacks nerve cells controlling voluntary muscles, resulting in muscle atrophy.
• Possible autoimmune disease and free radical damage.

Amyotrophic Lateral Sclerosis (ALS) - Clinical Manifestations

• Depends on location of affected motor neurons
• Fatigue, muscle weakness, cramps, fasciculations (twitching), and incoordination
• Weakness in arms, trunk, and legs, spasticity
• Difficulty walking, swallowing, speaking, or breathing – risk for aspiration
• Onset to death ~3 years

Amyotrophic Lateral Sclerosis (ALS) - Symptom Management

• No specific treatment halts or reverses the disease.
• Interventions aim to maintain function.
• Medication (Rilutek): glutamate antagonist that can slow deterioration of motor neurons.
• Symptomatic treatment, such as physical therapy, respiratory care, and speech therapy

Amyotrophic Lateral Sclerosis (ALS) - Patient Education

• Recognizing early symptoms.
• Completing advanced directives and code status.
• Fall risks (spasticity).
• Aspiration risks.
• Nutritional imbalances.

Amyotrophic Lateral Sclerosis (ALS) - Strategies for Caregivers

• ALS Association for information.
• Respite care, VON, home care relief.
• Hospitalization when necessary.
• Grief counseling and ALS support groups.

Information for PN (Nursing Professionals) - ALS

• Most cases managed at home.

• Common reasons for hospitalization: dehydration, malnutrition, pneumonia, and respiratory failure.

• Early recognition is essential.

• G-tube feeding might be considered for nutrition.

Muscular Dystrophy

• A group of chronic disorders marked by progressive weakness and wasting of voluntary (skeletal) muscles.
• Duchenne muscular dystrophy (DMD) is the most common type. - 1 in 3,000 male births.
• Inherited; genetic counseling recommended.
• Progressive 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 muscles around the joints, loss of mobility, and spinal deformity.
• Characteristic physical appearance in children (e.g., swayback, poor posture, difficulty walking).

Muscular Dystrophy - Symptom Management

• No specific treatment to halt or reverse disease.
• Physical therapy and speech therapy.
• Orthopaedic appliances (braces).
• Individualized exercise programs to limit muscle tightness and contractures.
• Corticosteroids (short-term use), anticonvulsants to control seizures, immunosuppressants, and antibiotics (for respiratory infections)

Muscular Dystrophy - Client Education

• Assessing client progression and status
• Assessing home safety
• Collaboration with physical therapists, occupational therapists, and other specialists.
• Assessing client needs for supplies and equipment.
• Assessing family needs and assistance (social work/counseling, financial status).
• Providing home care, as needed
• Providing hospital care if needed.
• Providing community resources.
• Providing information and online resources

Muscular Dystrophy - Strategies for Caregivers

• Muscular Dystrophy Association for information
• Feelings of helplessness and grief counseling
• End of life care options
• Psychiatric nurse or referral
• Respite care
• Occupational therapy / physical therapy on an outpatient basis.
• Hospitalization when needed
• Providing information for PN

General Information for PN

• Onset in age and gender depends on the specific type of muscular dystrophy.
• Spinal cord deformities and loss of trunk muscle strength present significant challenges, potentially requiring orthotic interventions and awareness of pulmonary function.
• Dental and speech concerns impact communication.
• Prognosis varies significantly.
• Risk factor for falls.
• Hereditary disorder warrants genetic testing.

This quiz focuses on the management of patients with oncologic and degenerative neurological disorders, highlighting conditions such as Huntington's disease, Amyotrophic Lateral Sclerosis (ALS), and Muscular Dystrophy. It covers key clinical features, genetic implications, and the progression of these diseases.