ALS: Common Issues and Nursing Interventions

• These notes cover neuromuscular impairments, focusing on Multiple Sclerosis (MS), Amyotrophic Lateral Sclerosis (ALS), Guillain-Barré Syndrome (GBS), and Myasthenia Gravis (MG), including their characteristics, management, and nursing care.
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Objectives of the Lecture

• Identify risk factors for neuromuscular function problems.
• Recognize significant cues related to these problems.
• Interpret test results and their implications.
• Formulate nursing diagnoses and collaborative problems.
• Identify appropriate NOCs and NICs for nursing diagnoses.
• Discuss pharmacological indications and nursing responsibilities.
• Discuss standards of care, including biobehavioral interventions.
• Discuss bio-ethical-legal principles in caring for clients with neuromuscular impairments.

General Overview

• The diseases discussed are progressive disorders.

Multiple Sclerosis (MS)

• MS is a chronic, degenerative, progressive disease of the central nervous system.
• It involves small patches of demyelination in the brain and spinal cord.
• Characterized by remissions and exacerbations (flare-ups/attacks).
• Demyelination, inflammation, and plaques are key features.
• Immune-mediated destruction of oligodendrocytes leads to loss of the myelin sheath (demyelination).
• Damaged myelin slows down electrical impulses transmission.
• Inflammation involves autoreactive T cells crossing the blood-brain barrier.
• This is followed by demyelination, degeneration, and axonal damage, resulting in plaque formation.
• Injured blood brain barrier allows surface antigens to enter.
• Multiple sclerosis is characterized by multiple plaques/scars in the brain.
• Inflammation makes the blood-brain barrier permeable, leading to oligodendrocyte destruction.

MS: Signs and Symptoms

• Primary symptoms include fatigue, depression, weakness, numbness, difficulty in coordination, loss of balance, and pain.
• MS typically affects optic nerves, pyramidal tracts, posterior columns, brainstem nuclei, and the ventricular region of the brain.
• Charcot's Neurologic Triad is a pathognomonic sign:
  • Nystagmus (involuntary rapid eye movements).
  • Dysarthria/dysphagia (difficult or unclear speech or swallowing).
  • Intention tremor (involuntary, rhythmic muscle contractions during purposeful movement).

MS: Assessment Findings

• Visual disturbances are often the first symptom.
• Interference with eating, talking, and swallowing.
• Spastic weakness of the extremities, loss of abdominal reflexes, ataxia, and tremor.
• Potential bladder, bowel, and sexual problems.
• Cognitive and psychosocial problems such as depression, emotional lability, and euphoria.

MS: Movement-Related changes

• Fatigue and stiffness of the legs are common.
• Flexor spasms at night, hyperactive DTRs, and a positive Babinski's reflex can occur.
• Absent abdominal reflexes may also be noted.
• Gait may be unsteady due to leg weakness and spasticity from cerebral motor strip damage.
• Cerebellar issues include intention tremor, dysmetria (lack of coordination), and dysdiadochokinesia (inability to perform rapid alternating movements).

Other areas affected by MS

• CNS and Brainstem: Tinnitus, vertigo, hearing loss, facial weakness, dysphagia, blurred vision, diplopia, decreased visual acuity, scotomas, and nystagmus.
• Sensory: Hypalgesia (diminished sensitivity to pain), paresthesia (tingling, burning, pricking), facial pain, and decreased temperature perception. Numbness, tingling, burning, or crawling sensations.
• Spinal Cord Involvement: Bowel and bladder dysfunction (constipation or incontinence), sexual dysfunction (impotence, decreased vaginal secretions).
• Psychosocial: Cognitive changes, mood changes (anxiety, apathy, emotional lability, and depression).

MS: Diagnostics

• There is no single definitive test for MS.
• MRI is used to detect lesions (damaged areas) in the central nervous system.
• Spinal/Lumbar Tap helps determine the presence of oligoclonal bands in the CSF, indicating ongoing inflammation.
  • It is 80% positive for MS patients.

MS: Medical Management

• There is no cure, treatment focuses on reducing disability and managing symptoms, and acute exacerbations.
• Goals include delaying disease progression, managing symptoms, and treating acute exacerbations.
• Pharmacologic interventions:
  • Interferon beta-1a (Rebif) and interferon beta-1b (Betaseron).
  • Glatiramer acetate (Copaxone).
  • IV methylprednisolone for acute relapses.
  • Baclofen and benzodiazepines for spasticity.
  • Amantadine and Fluoxetine for fatigue.
  • Anti-seizure medications and beta-blockers for ataxia.

MS: Nursing Diagnoses and Collaborative Management

• Nursing Diagnoses: Impaired physical mobility, risk for injury, aspiration, impaired urinary and bowel elimination, disturbed thought process, ineffective individual coping.
• Collaborative Management:
  • Promoting physical mobility through relaxation and coordination exercises, progressive resistance exercises, warm packs, and encouraging specific activities.
  • Preventing injury by patient teaching, providing assistive devices, assessing skin, and proper positioning.
  • Enhancing bowel and bladder control with regular elimination, bladder training, self-catheterization, high-fiber nutrition, and bowel-training programs.
  • Managing speech and swallowing difficulties with speech therapy and a plan of care to reduce aspiration risk.
  • Improving sensory and cognitive function through diplopia management, psychosocial support, and a structured environment.

Amyotrophic Lateral Sclerosis (ALS)

• ALS, also known as Lou Gehrig's disease, has a poor prognosis.
• The affected typically survive 3-5 years, most commonly to respiratory insufficiency.
• It is characterized by progressive weakness, muscle wasting, and spasticity, leading to paralysis.
• The definite cause is unknown, although several factors associated with oxidative stress.

ALS: Manifestation

• Symptoms of ALS include fatigue, progressive muscle weakness, cramps, fasciculations (twitching), incoordination, and dysarthria/dysphagia.
• The clinical features depend on the location of affected motor neurons; clinical features includes, progressive weakness of the arms, trunk or leg muscles, spasticity, and anal sphincters usually remain intact.
• Weakness in muscles supplied by cranial nerves leads to talking, swallowing, and breathing difficulties.
• Soft palate and esophageal weakness causes regurgitation through the nose
• Bulbar muscle impairment, including difficulty speaking and swallowing, nasal voice, and compromised respiratory function.

ALS: Assessment & Diagnostics, and Treatment

• Assessment & Diagnostics: There is no definitive test, diagnosis is based on ruling out other causes.
• Treatment: There is no cure currently available.
• Riluzole is a medication that slows the degeneration of motor neurons.
• The focus is on interventions to maintain/improve function, well-being, and quality of life.
• Symptomatic treatment and rehabilitative measures are for patient support, the most common reasons for hospitalization are malnutrition and dehydration.

ALS: Goals of Care

• Maintain a patent airway since the disease will cause respiratory failure.
• Initiate palliative consultation, discuss acceptable death.

Comparison of MS and ALS

• MS (Chronic neurologic disease affecting the brain and spinal cord due to immune-mediated demyelination and nerve injury) and ALS (Chronic neurologic disease of unknown cause).
• MS affects women twice as often as men, typically between 20 and 50 years and ALS affects more men than women, between age 40.
• Visual field loss in MS and progressive muscle atrophy in ALS.
• Fatigue, muscle spasticity and paresthesias are symptoms in MS, while fatigue, muscle atrophy (tongue), twitching of the face and tongue are symptoms of ALS.
• Inter-professional collaborative care in MS includes immunomodulating drugs. ALS includes only one approved drug to slow disease progression.
• Both include psychosocial support.

Guillain-Barré Syndrome (GBS)

• GBS, or Landry-Guillain-Barré-Strohl Syndrome (characterized by ascending paralysis); symmetric, affects peripheral nervous system, or autoimmune attack.
• It is associated with Campylobacter jejuni bacteria.

GBS: Stages of Acute Course

• Acute/Initial Period (1-4 weeks): Onset of symptoms until no further deterioration.
• Plateau Period (days-2 weeks).
• Recovery Period (4-6 months to 2 years): Coincides with re-myelination and axonal regeneration.

GBS: Assessment

• Motor Manifestations: Ascending, symmetric weakness -> flaccid paralysis-without muscle atrophy, respiratory compromise (dyspnea, diminished breath sounds, decreased tidal volume and vital capacity/VF and respiratory failure, decreased or absent DTRs, ataxia.
• Sensory Manifestation: Tingling in hands and feet.
• Autonomic Dysfunction Manifestations: Unstable blood pressure/BP, cardiac dysrhythmias, and tachycardia.
• Facial weakness, Dysphagia,Diplopia, Speech difficulties.

GBS: Diagnostics & Medical Management

• Changes in VF and elevated CSF protein levels after 1-2 weeks.
• Electrophysiologic studies: EMG (4th week or longer), Nerve Conduction Velocity (NCV).
• Medical Management: Removal of circulating antibodies by way of Plasma Exchange, IV Immunoglobulin Therapy or Cerebrospinal Fluid Filtration.

GBS: Nursing Responsibilities & Diagnoses

• Ensure weight checks, and Provide information and reassurance.
• GBS:Ineffective breathing pattern, impaired gas exchange, physical mobility.

Myasthenia Gravis (MG)

• A chronic autoimmune neuromuscular disorder
• Antibodies blocking acetylcholine receptors at the neuromuscular junction
• More prone for women > men (20-40Yrs)

MG: Assessment

• Ptosis- Dropping of the eyelid
• Diplopia- seeing double

MG: Diagnosis and Nursing Diagnoses

• Repetitive nerve stimulation (RNS) of proximal nerves & Electromyography (EMG).
• Nursing diagnoses: Pt with MG- Ineffective breathing patterns, impaired verbal communication,, Activity and tolerance, Self deficiency

Comparison of MG and GBS and ALS and MS

• GBS: Ascending Distribution, Weakness, Symmetrical distribution.
• MG: Distribution Eyes and CN's , and increased CSF Protein.
• ALD: Increased weakness and asymmetry distribution.
• MS: Fatigue and the most commonly observed is Yes

Ethical and Legal Aspects of Care

• Respect for Persons: Autonomy- Pt does not feel able to express his/her own needs and thoughts.
• Veracity: Be truthful about diagnosis, treatment and benefits.
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This quiz covers common reasons for hospitalization, nursing interventions, and palliative care goals for patients with Amyotrophic Lateral Sclerosis (ALS). It addresses issues such as difficulty swallowing, muscle weakness, cramps, and bulbar involvement to provide comprehensive care strategies.