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

Comprehensive practice questions covering clinical manifestations, diagnosis, and plan of care for Amyotrophic Lateral Sclerosis (ALS) and Guillain-Barre Syndrome (GBS).

How is Amyotrophic Lateral Sclerosis (ALS) defined in terms of its progression and prognosis?

A relentlessly progressive, presently incurable neurodegenerative disorder that causes muscle weakness, disability, and eventually death.

What is the median survival time for a patient diagnosed with ALS?

$3-5$ years, though $10\%$ can live $10$ years or more.

What is the most common clinical presentation of ALS regarding limb weakness?

Asymmetric limb weakness.

How does lower extremity weakness often begin in ALS patients?

It often begins with weakness of foot dorsiflexion, also known as foot drop.

What are some of the life-threatening clinical manifestations of ALS?

Respiratory muscle weakness.

Which specific clinical manifestation refers to the twitching of the face or tongue in ALS?

Fasciculations.

What is the cognitive status of a patient during the illness trajectory of ALS?

The patient remains cognitively intact while wasting away.

What is the only FDA approved medication for ALS and what is its primary benefit?

riluzole (Rilutek); it is not a cure but extends survival time.

Why is it important to maintain NPO status for an ALS patient?

Because there is a major risk for aspiration.

How is Guillain-Barre Syndrome (GBS) defined?

An acute, immune-mediated polyneuritis characterized by progressive, usually ascending symmetric muscle weakness and absent or depressed deep tendon reflexes.

What are common signs of dysautonomia in Guillain-Barre Syndrome?

Tachycardia (most common), labile blood pressure, orthostatic hypotension, ileus, and urinary retention.

What percentage of Guillain-Barre Syndrome patients experience paresthesia in the hands and feet?

$$80\%$$

What common events often precede the onset of Guillain-Barre Syndrome?

Viral infection, immunization, trauma, or surgery.

By what point in the disease progression have $90\%$ of Guillain-Barre patients reached their nadir?

By $4$ weeks.

What are the characteristic findings in the Cerebrospinal fluid (CSF) for a GBS diagnosis after one week of onset?

Increased protein and normal WBC count.

What percentage of Guillain-Barre Syndrome patients require mechanical ventilation?

$$15-30\%$$

What is the role of Plasmapheresis in treating GBS?

It removes circulating antibodies, complement, and soluble biological response modifiers via $4-6$ treatments over $8-10$ days.

What is the protocol for administering IV immune globulin (IVIG) in GBS patients?

Administer for $5$ days within $4$ weeks of onset.

What is the prognosis for recovery in patients with Guillain-Barre Syndrome?

$95\%$ survive, $75\%$ completely recover, but $25-30\%$ have residual weakness after $3$ years.