ALS + GBS

AMYOTROPHIC LATERAL SCLEROSIS (ALS)

  • Progressive, incurable neurodegenerative disorder.

  • Causes muscle weakness, disability, and mortality.

  • Median survival: 3-5 years; 10% can live for 10+ years.

ALS: CLINICAL MANIFESTATIONS

  • Asymmetric limb weakness (initial presentation).

    • Upper: Hand weakness, shoulder girdle involvement.

    • Lower: Weakness in foot dorsiflexion (foot drop).

  • Symptoms spread predictably after initial onset.

  • Additional signs:

    • Fatigue, dysarthria/dysphagia, muscle atrophy, spasticity, fasciculations, respiratory muscle weakness.

  • Cognition usually remains intact while physical condition deteriorates.

ALS: PLAN OF CARE

  • Riluzole (Rilutek): FDA approved, extends survival but not a cure.

  • Exercise: Moderate intensity endurance training for trunk/limbs to reduce spasticity.

  • Physical therapy and speech therapy consultations suggested.

  • Risk management: Maintain NPO to prevent aspiration; pain assessment; minimize injury risks; facilitate communication.

  • Offer support for anticipatory grieving.

GUILLAIN-BARRE SYNDROME (GBS)

  • Acute, immune-mediated polyneuritis leading to progressive muscle weakness with absent/depressed reflexes.

  • Symptoms may appear days to weeks post-trigger (infection, vaccination, trauma).

  • Weakness ranges from mild to complete paralysis.

GBS: CLINICAL MANIFESTATIONS

  • Common symptoms:

    • Paresthesia (80%), back/extremity pain (66%), dysautonomia (70%):

    • Tachycardia, labile BP, orthostatic hypotension, ileus, urinary retention.

  • Progression typically within two weeks; by four weeks, most patients reach nadir.

GBS: DIAGNOSIS

  • Assessment through history and physical examination.

  • CSF analysis after one week: Increased protein, normal WBC count.

  • Nerve conduction studies and EMG for diagnosis and prognosis.

GBS: PLAN OF CARE

  • Regular assessment to monitor progression (15-30% may require ventilation).

  • Monitoring gag/corneal reflexes, vital signs for BP changes.

  • Nutritional and pain management interventions; support for patient/family.

GBS: MAIN MODALITIES OF THERAPY

  • Plasmapheresis: 4-6 treatments over 8-10 days to remove antibodies.

  • IV immune globulin (IVIG): Administer within 4 weeks of onset for 5 days.

GBS: PROGNOSIS

  • 95% survival rate; 75% complete recovery.

  • 25-30% may have residual weakness after 3 years.

  • About 3% may experience muscle weakness relapse years after initial attack.