ALS and GBS

NURSING CARE OF THE PATIENT WITH NEUROLOGICAL DISORDERS: ALS & GUILLAIN-BARRE

AMYOTROPHIC LATERAL SCLEROSIS (ALS)

Definition

• Amyotrophic Lateral Sclerosis (ALS):
    - A relentlessly progressive, presently incurable neurodegenerative disorder.
    - It causes muscle weakness, disability, and eventually death.

Survival Statistics

• Median survival: 3-5 years.

• Prolonged survival: 10% of patients can live 10 years or more.

Clinical Manifestations

• Asymmetric Limb Weakness:
    - Most common presentation of ALS.
    - Upper Extremity: Weakness can manifest as hand weakness or weakness in shoulder girdle muscles.
    - Lower Extremity: Often begins with weakness of foot dorsiflexion leading to a condition known as foot drop.
    - Symptom Spread: Symptoms initially spread within the segment of onset and then to other regions in a relatively predictable pattern.

• Other Symptoms:
    - Fatigue
    - Dysarthria/Dysphagia: Problems with speech and swallowing.
    - Muscle Atrophy & Spasticity
    - Fasciculations: Twitching of face or tongue muscles.
    - Respiratory Muscle Weakness: This condition can become life-threatening as respiratory muscles weaken.

Illness Trajectory

• The trajectory of ALS is devastating as patients typically remain cognitively intact while experiencing physical decline and muscle wasting.

Plan of Care

• Medication:
    - Riluzole (Rilutek): Only FDA-approved medication for ALS. While it is not a cure, it can extend survival time.

• Exercise Regimen: Guide the patient through moderate-intensity endurance-type exercises for trunk and limbs to:
    - Reduce spasticity.

• Consultations:
    - Physical Therapy: Obtain a consult to assist with mobility and exercises.
    - Speech Therapy: Facilitate communication and address swallowing difficulties.

• Nutritional Considerations:
    - Maintain NPO status due to the major risk for aspiration.

• Pain Assessment: Recognizing that pain may be secondary to muscle weakness.

• Injury Prevention: Minimize risk for injury due to muscle weakness and atrophy.

• Diversion Activities: Provide activities to occupy the patient’s mind and reduce feelings of despair.

• Emotional Support: Facilitate anticipatory grieving for both the patient and family members as the disease progresses.

GUILLAIN-BARRE SYNDROME (GBS)

Definition

• Guillain-Barre Syndrome:
    - An acute, immune-mediated polyneuritis characterized by progressive, usually ascending symmetric muscle weakness.
    - Accompanied by absent or depressed deep tendon reflexes.

Symptom Onset

• Patients typically present a few days to a week after the onset of symptoms.

• Weakness Variability:
    - Symptoms can range from mild difficulty walking to nearly complete paralysis.

Additional Symptoms

• Paresthesia: Numbness or tingling in hands and feet (80% of cases).

• Pain: Present in back and extremities (66%).

• Dysautonomia Symptoms:
    - Present in 70% of cases, may include:
      - Tachycardia: Most common.
      - Labile Blood Pressure
      - Orthostatic Hypotension
      - Ileus
      - Urinary Retention

Precipitating Factors

• Often preceded by a viral infection, immunization, trauma, or surgery.

• Progression Timeline:
    - Progresses over a period of about 2 weeks, with 90% of GBS patients reaching their lowest point (nadir) by 4 weeks.

Diagnosis

• Evaluation Components:
    - History and physical examination.
    - Cerebrospinal Fluid (CSF) Analysis: Conducted only after one week of onset. Findings typically include:
      - Increased Protein Levels.
      - Normal White Blood Cell (WBC) Count.
    - Nerve Conduction Studies & Needle Electromyography (EMG):
      - Valuable for confirming diagnosis and assessing prognosis.

Plan of Care

• Routine Physical Assessment:
    - Required to monitor for disease progression since 15-30% of patients may require mechanical ventilation.
    - Deterioration can occur rapidly, necessitating ongoing vigilance.
    - Assess gag and corneal reflexes as part of neurological assessment.
    - Conduct frequent vital signs checks, focusing on blood pressure changes.
    - Be alert for autonomic changes, which may necessitate ICU admission.

• Nutritional Interventions: Need for monitoring as swallowing difficulty may require dietary adjustments.

• Pain Management: Interventions for neuropathic pain.

• Urinary Monitoring: Regular assessment for urinary retention.

• Emotional Support: Provide encouragement and support to patients and families as they deal with uncertainties related to recovery.

Main Modalities of Therapy

• Plasmapheresis:
    - Procedure that removes circulating antibodies, complements, and soluble biological response modifiers.
    - Typically administered in 4-6 treatments over a span of 8-10 days.

• IV Immune Globulin (IVIG):
    - Administered for 5 days to help manage GBS, ideally given within 4 weeks of symptom onset.

Prognosis

• Approximately 95% of patients survive GBS.

• Around 75% of patients achieve complete recovery.

• Notably, 25-30% of patients may experience residual weakness after 3 years.

• About 3% of patients could suffer a relapse of muscle weakness and tingling symptoms many years after the initial episode.