Untitled Quiz

Neuromuscular Disorders

• Most neuromuscular disorders stem from issues with motor neurons, neurotransmitters, or the muscles' response to neurotransmitters.
• The neurological pathway transmits impulses from the brain to muscles, triggering contraction.
• The impulse travels down the axon, utilizing the myelin sheath for rapid transmission. Damage or lack of myelin impairs or slows impulse transmission.

Myasthenia Gravis - Pathophysiology

• Myasthenia gravis is a neuromuscular junction disorder.
• It impairs communication between the motor neurons and muscle cells.
• Antibodies target acetylcholine receptors, reducing their availability.
• This reduces the transmission of impulses, leading to muscle weakness.

Myasthenia Gravis - Risk Factors

• Predominantly affects young women (20-30 years old) and men over 50.
• Can occur at any age.
• An autoimmune disorder.

Myasthenia Gravis - Clinical Manifestations

• Diplopia (double vision).
• Ptosis (drooping of the upper eyelid).
• Facial weakness and expression.
• Dysphonia (voice impairment).
• Increased risk of choking and aspiration.
• Generalized muscle weakness.
• Weakness in extremities and intercostal muscles, leading to potential respiratory failure.

Myasthenia Gravis - Diagnosis

• Serum AChR antibody levels.
• Measures acetylcholine receptor autoantibodies.
• Electromyography (EMG).
• Records electrical activity in muscle tissue.

Myasthenia Gravis - Exacerbating Factors

• Infection
• Surgery
• Medications (e.g., aminoglycosides, quinine, magnesium sulfate).
• Pregnancy
• High environmental temperature.
• Emotional stress
• Vigorous physical activity
• Thymoma (abnormal thymus)

Myasthenia Gravis - Medical Management

• Pharmacological management:
  • Anticholinesterase drugs (e.g., pyridostigmine bromide) to block the breakdown of acetylcholine.
  • Corticosteroids (e.g., prednisone).
  • Immunosuppressant drugs (e.g., azathioprine) to curb the immune response.
• Surgical management:
  • Thymectomy (removal of the thymus).

Myasthenia Gravis - Nursing Considerations

• Medication adherence is critical.
• Schedule drug administration, especially around strenuous activities.
• Patient and family education on medication mechanisms.
• Support with extra medication supply.
• Precautions for choking and aspiration.
• Energy conservation measures.
• Monitor vision problems; consider artificial tears, glasses modifications, eye patching.
• Health teaching and provision of medical alert information.

Guillain-Barré Syndrome - Pathophysiology

• Damage or destruction of myelin sheaths in peripheral nerves.
• Reduced nerve conduction speed leads to progressive muscle weakness.
• Schwann cells, responsible for myelin production, are not affected; remyelination is possible.

Guillain-Barré Syndrome - Risk Factors

• Most common in young to middle-aged adults.
• Immune-mediated response, triggered by prior infections (e.g., Campylobacter jejuni) or vaccinations.
• Medical emergency due to rapid respiratory paralysis.

Guillain-Barré Syndrome - Clinical Manifestations

• Begins in the lower extremities and ascends.
• Flaccid weakness, difficulty walking, and progressing to paralysis.
• Symmetrical paresthesias, dysphagia, dyskinesia.
• Respiratory muscles are often affected.

Guillain-Barré Syndrome - Medical Management

• Medical emergency, requiring ICU monitoring, IV fluids, physical therapy, and potentially mechanical ventilation.
• Immunomodulating treatments like IV immunoglobulin (IVIg).
• Plasmapheresis to filter blood and remove antibodies.

Guillain-Barré Syndrome - Nursing Considerations

• Monitoring and maintaining effective respiratory function.
• Ensuring adequate nutrition, hydration, and mobility.
• Supportive care for communication difficulties.
• Addressing anxiety and potential for fatigue.

Parkinson's Disease - Pathophysiology

• Degeneration of dopamine-producing neurons in the basal ganglia.
• Depletion of dopamine leads to an imbalance between excitatory and inhibitory neurotransmitters.
• This affects voluntary movement, leading to motor symptoms.

Parkinson's Disease - Risk Factors

• Primarily affects individuals in their 50s and 60s, but younger onset is possible.
• Risk factors include genetic predisposition and environmental factors.
• Men are more commonly affected.
• Up to 80% may eventually develop dementia.

Parkinson's Disease - Clinical Manifestations

• Resting tremor (often first symptom).
• Bradykinesia (slowed movement).
• Rigidity (stiffness) of muscles.
• Postural instability — difficulty balancing.
• Shuffling gait.

Parkinson's Disease - Medical Management

• Pharmacological management:
  • Carbidopa-levodopa (conversion of levodopa to dopamine in the brain); carbidopa blocks peripheral conversion, making more levodopa available in the brain.
  • Dopamine agonists (e.g., pramipexole, ropinirole).

Parkinson's Disease - Nursing Considerations

• Enhancing mobility and promoting functional independence.
• Supporting self-care activities.
• Improving bowel elimination and nutrition.
• Encouraging healthy coping mechanisms and managing emotional distress.

Amyotrophic Lateral Sclerosis (ALS) - Pathophysiology

• Degeneration of both upper and lower motor neurons in the corticospinal tracts, anterior horn cells, and bulbar motor nuclei.
• Progressive, irreversible neurodegenerative disease.
• No known cure.

Amyotrophic Lateral Sclerosis (ALS) - Risk Factors

• Typically develops between ages 40-60.
• Men are more frequently affected.

Amyotrophic Lateral Sclerosis (ALS) - Clinical Manifestations

• Muscle weakness and cramps.
• Difficulty speaking/swallowing/respiration.
• Fasciculations (muscle twitching).
• Spasticity and difficulty with coordination.

Amyotrophic Lateral Sclerosis (ALS) - Medical Management

• No cure; treatment focuses on symptom management and delaying disease progression.
• Medications to manage symptoms and slow disease progression.

Amyotrophic Lateral Sclerosis (ALS) - Nursing Considerations

• Support respiratory function.
• Address issues of pain, dyspnea.
• Provide support for emotional and psychosocial needs.
• Teach adaptive strategies and assistive devices. Implement strategies to manage spasticity.

Systemic Lupus Erythematosus (SLE) - Pathophysiology

• Autoimmune disease that occurs because the immune system mistakenly attacks healthy tissue.
• The immune system produces antibodies (autoantibodies).
• Antibody-antigen complexes form and deposit in tissues, causing inflammation and damage.

Systemic Lupus Erythematosus (SLE) - Risk Factors

• Predominantly affects women of childbearing age.
• More common in African Americans than in Caucasians.
• Environmental factors, such as UV light exposure, cigarette smoking, and infections, may play a role.

Systemic Lupus Erythematosus (SLE) - Clinical Manifestations

• Most show widespread symptoms, often involving multiple organ systems.
• Typical symptoms can include skin rashes, arthritis, kidney disease, cardiovascular issues, blood disorders, and neurological problems.

Systemic Lupus Erythematosus (SLE) - Diagnosis

• Diagnosis is primarily clinical, based on symptoms and laboratory tests. Specific tests include complete blood counts (CBC) and antinuclear antibody (ANA).

Systemic Lupus Erythematosus (SLE) - Medical Management

• Anti-inflammatory drugs (including corticosteroids).
• Antimalarials (e.g., hydroxychloroquine) to prevent exacerbations.
• Immunosuppressants (e.g., belimumab) that modulate the immune system.

Systemic Lupus Erythematosus (SLE) - Nursing Considerations

• Support healthy coping mechanisms.
• Implement skin care, especially if there are significant skin manifestations.
• Ensure appropriate nutrition and fluid balance.
• Monitor kidney function for those with lupus nephritis.

Multiple Sclerosis (MS) - Pathophysiology

• Autoimmune disease targeting the myelin sheath, causing inflammation and damage in the central nervous system, leading to demyelination.

Multiple Sclerosis (MS) - Risk Factors

• Predominantly affects women between ages 20 and 50.
• Causes unknown, yet genetics and environmental factors may play a role.

Multiple Sclerosis (MS) - Clinical Manifestations

• Symptoms are highly variable and can include numbness/tingling, pain, fatigue, difficulty walking, vision problems, bladder/bowel issues, cognitive impairments, and speech difficulties.

Multiple Sclerosis (MS) - Medical Management

• No cure; treatment focuses on reducing symptom severity, slowing disease progression, and managing relapses.
• Pharmacological treatments (e.g., disease-modifying therapies, symptomatic medication).

Multiple Sclerosis (MS) - Nursing Considerations

• Support mobility and activity.
• Address fatigue and bowel/bladder problems.
• Promote emotional well-being.
• Provide education on disease and treatment.

Fibromyalgia - Pathophysiology

• Chronic pain disorder characterized by widespread pain and fatigue.
• The exact cause is not fully understood, but it may be influenced by immune system components, genetic makeup, and environmental factors.

Fibromyalgia & Who is at risk

• Primarily affects women.
• Often associated with anxiety, depression, sleep disorders, and stress.

Fibromyalgia - Medical Management

• Diagnosis is based on clinical presentation and physical examination. There is no definitive test.
• Treatment focuses on symptom relief with medication, therapies, and changes in lifestyle.

Fibromyalgia - Nursing Considerations

• Support psychosocial needs, emphasizing coping mechanisms.
• Implement strategies to reduce pain and fatigue.
• Promote self-care, healthy diet, and exercise to aid in managing symptoms.

Degenerative Disc Disease (DDD) - Pathophysiology

• Degenerative changes in the intervertebral discs, leading to pain and reduced spinal function, often accompanied by fatigue.

Degenerative Disc Disease (DDD) - Risk Factors

• Age-related wear and tear is a key factor. Obesity, poor posture, strenuous activities, and genetic predispositions also contribute.

Degenerative Disc Disease (DDD) - Clinical Manifestations

• Pain in the neck or back, often shooting down the arms or legs.
• Stiffness and difficulty with movement.
• Muscle weakness or spasms.

Degenerative Disc Disease (DDD) - Medical Management

• Non-surgical: Heat therapy, spinal manipulation
• Surgical: Microdiscectomy, laminectomy with fusion.

Degenerative Disc Disease (DDD) - Nursing Considerations

• Support pain management and activity modifications.
• Promote patient education on posture, weight, exercises, and adaptive strategies.