Nursing Care of the Patient with Neurological Disorders: ALS & Guillain-Barre

Definition: Amyotrophic Lateral Sclerosis is a relentlessly progressive and presently incurable neurodegenerative disorder. It is characterized by muscle weakness and disability that eventually leads to death.
Prognosis and Survival Statistics: * The median survival for patients is approximately $3-5$ years. * Approximately $10\%$ of patients can live for $10$ years or more.
Cognitive Status: The illness trajectory is described as devastating because the patient remains cognitively intact while their physical body wastes away.

Limb Weakness: Asymmetric limb weakness is the most common initial presentation of the disease. * Upper Extremity: Often presents as hand weakness or weakness in the shoulder girdle muscles. * Lower Extremity: Frequently begins with weakness of foot dorsiflexion, commonly referred to as foot drop.
Pattern of Progression: Symptoms initially spread within the segment of onset and then move to other regions in a relatively predictable pattern.
Symptom Profile: * Fatigue. * Dysarthria (difficulty speaking) and Dysphagia (difficulty swallowing). * Muscle atrophy and spasticity. * Fasciculations: Visible twitching of the face or tongue. * Respiratory Muscle Weakness: This is a life-threatening manifestation of the disease.

Pharmacological Management: * riluzole (Rilutek): This is the only FDA-approved medication for ALS. It is not a cure, but it serves to extend survival time.
Physical Activity and Therapy: * Guide the patient through moderate intensity endurance-type exercises focused on the trunk and limbs. * These exercises are used to reduce spasticity. * Physical therapy consult is recommended.
Communication and Cognition: * Support cognitive and emotional functions. * Facilitate communication; a Speech therapy consult is often helpful for managing dysarthria.
Nursing Care and Safety: * Nutrition: Maintain NPO (nothing by mouth) status as there is a major risk for aspiration. * Pain Management: Conduct pain assessments, as pain is often secondary to muscle weakness. * Injury Prevention: Minimize the risk for injury. * Psychosocial Support: Provide diversional activities and facilitate anticipatory grieving for both the patient and the family.

Definition: An acute, immune-mediated polyneuritis characterized by progressive, usually ascending, symmetric muscle weakness. It is accompanied by absent or depressed deep tendon reflexes.
Onset and Progression: * Patients usually present to medical facilities within a few days to a week after symptoms begin. * The disease progresses over a period of about $2$ weeks. * By $4$ weeks, $90\%$ of GBS patients have reached the "nadir" (the lowest point or peak of the symptoms).
Potential Triggers: Often preceded by a viral infection, immunization, trauma, or surgery.

Motor Weakness: Varies from mild difficulty walking to nearly complete paralysis.
Sensory and Pain Symptoms: * Paresthesia: Occurs in the hands and feet in $80\%$ of cases. * Pain: Located in the back and extremities in $66\%$ of cases.
Dysautonomia: Occurs in $70\%$ of patients and includes: * Tachycardia (the most common autonomic symptom). * Labile blood pressure (fluctuating BP). * Orthostatic hypotension. * Ileus (intestinal obstruction/lack of movement). * Urinary retention.

History and Physical Examination: Primary diagnostic tools.
Cerebrospinal Fluid (CSF) Analysis: Performed only after one week of symptom onset. * Findings include increased protein levels but a normal White Blood Cell (WBC) count.
Diagnostic Testing: * Nerve conduction studies and needle electromyography (EMG) are valuable not only for confirming the diagnosis but also for determining the prognosis.

Monitoring and Assessment: * Perform astute, routine physical assessments to monitor for disease progression. * Respiratory Support: Since $15-30\%$ of patients require mechanical ventilation and deterioration can occur rapidly, vigilance is essential. * Assess gag and corneal reflexes. * Monitor frequent Vital Signs (V.S.), specifically checking for blood pressure changes. Patients with significant autonomic changes may require the Intensive Care Unit (ICU).
General Nursing Care: * Implement nutritional interventions. * Pain management specifically for neuropathic pain. * Monitor for urinary retention. * Provide support and encouragement to the patient and family, especially when dealing with concerns related to the variable nature of recovery.

Plasmapheresis: * This process removes circulating antibodies, complement, and soluble biological response modifiers. * Typically involves $4-6$ treatments over a period of $8-10$ days.
IV Immune Globulin (IVIG): * Administered over $5$ days. * Must be administered within $4$ weeks of symptom onset.

Survival Rate: $95\%$ of patients survive.
Recovery Rate: $75\%$ of patients achieve a complete recovery.
Long-term Outcomes: * $25-30\%$ of patients experience residual weakness after $3$ years. * Approximately $3\%$ of patients may suffer a relapse of muscle weakness and tingling many years after the initial attack.