ALS and GBS

Definition: ALS is a relentlessly progressive, presently incurable neurodegenerative disorder.
Pathophysiology: The disease causes muscle weakness and disability, eventually leading to death.
Prognosis and Survival Statistics: - The median survival rate is between $3-5$ years. - Approximately $10\%$ of patients can live for $10$ years or more.
Cognitive Impact: A devastating aspect of the illness trajectory is that the patient remains cognitively intact while their body physically wastes away.

Presentation Pattern: Symptoms initially spread within the segment of onset and then progress to other regions in a relatively predictable pattern.
Asymmetric Limb Weakness: This is the most common presentation of ALS. - Upper Extremity: Manifests as hand weakness or weakness in the shoulder girdle muscles. - Lower Extremity: Often begins with weakness of foot dorsiflexion, commonly referred to as foot drop.
Neuromuscular Symptoms: - Fatigue. - Dysarthria (difficulty speaking). - Dysphagia (difficulty swallowing). - Muscle atrophy and spasticity. - Fasciculations: Twitching of the face or tongue.
Life-Threatening Complications: Respiratory muscle weakness is a critical and life-threatening manifestation.

Pharmacological Treatment: - riluzole (Rilutek): This is the only FDA-approved medication for ALS. It is not a cure but is used to extend survival time.
Physical Activity and Therapy: - Guide the patient through moderate intensity endurance-type exercises for the trunk and limbs to help reduce spasticity. - Initiate a Physical Therapy (PT) consult.
Communication and Cognitive Support: - Support both cognitive and emotional functions. - Facilitate communication; a Speech Therapy consult can be helpful for this purpose.
Nutritional and Safety Interventions: - Maintain NPO status: This is critical as there is a major risk for aspiration. - Pain Assessment: Pain is typically secondary to muscle weakness. - Minimize the risk for injury.
Psychosocial and Diversional Care: - Provide diversional activities for the patient. - Facilitate anticipatory grieving for both the patient and their family.

Definition: An acute, immune-mediated polyneuritis characterized by progressive, symmetric muscle weakness.
Progression Style: The weakness is usually ascending.
Reflexes: Accompanied by absent or depressed deep tendon reflexes.
Symptom Onset: Patients usually present for care a few days to a week after the onset of symptoms.
Severity Spectrum: Weakness varies from mild difficulty walking to nearly complete paralysis.
Etiology: Often preceded by a viral infection, immunization, trauma, or surgery.
Timeline of Progression: - The disease typically progresses over a period of about $2$ weeks. - By $4$ weeks, $90\%$ of GBS patients have reached their nadir (the lowest point of the disease or maximum weakness).

Sensory and Pain Symptoms: - Paresthesia: Numbness or tingling in the hands and feet occurs in $80\%$ of cases. - Pain: Pain in the back and extremities occurs in $66\%$ of cases.
Dysautonomia: Occurs in $70\%$ of patients and includes: - Tachycardia: The most common autonomic symptom. - Labile blood pressure. - Orthostatic hypotension. - Ileus. - Urinary retention.

Diagnostic Methods: - History and physical examination. - Cerebrospinal Fluid (CSF) Analysis: Conducted only after one week of onset. Indicators include increased protein levels and a normal White Blood Cell (WBC) count. - Nerve Conduction Studies and Needle Electromyography (EMG): These are valuable for confirming the diagnosis and determining the prognosis.
Monitoring and Physical Assessment: - Conduct astute, routine physical assessments to monitor disease progression. - Respiratory Monitoring: $15-30\%$ of patients require mechanical ventilation. Vigilance is essential because deterioration can occur rapidly. - Reflex Checks: Regularly assess gag and corneal reflexes. - Vital Signs: Perform frequent checks, specifically looking for blood pressure changes; if autonomic changes occur, the patient may need ICU admission.

Nursing Interventions: - Nutritional interventions. - Pain-management interventions for neuropathic pain. - Monitor for urinary retention. - Provide support and encouragement to the patient and family. - Address concerns related to the variable nature of recovery.
Main Modalities of Therapy: - Plasmapheresis: Used to remove circulating antibodies, complement, and soluble biological response modifiers. This typically involves $4-6$ treatments over $8-10$ days. - IV Immune Globulin (IVIG): Administered for $5$ days. It should be given within $4$ weeks of symptom onset.

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