Patho Patho #62

Question

The pathophysiology of Amyotrophic lateral sclerosis (ALS) includes:

Answers

  1. autoimmune destruction of peripheral neurons causing neuropathic pain.
    • Rationale:

      This answer choice is not correct as this is a description of Guillain Barre syndrome rather than ALS.

  2. destruction of upper and lower motor neurons causing paralysis.
    • Rationale:

      This answer is correct as ALS results from destruction of upper and lower motor neurons causing muscle paralysis in the late stages of the disease.

  3. an autosomal dominant disorder of insufficient dopamine.
    • Rationale:

      This answer choice is not correct. ALS is a neuromuscular disorder that causes destruction of motor neurons responsible for voluntary muscle movement.

  4. an autoimmune disorder that attacks the neuromuscular junction.
    • Rationale:

      This answer choice is not correct because this is a description of myasthenia gravis and not ALS.

Overview

The focus of this question is asking the nurse to identify the underlying pathophysiology leading to the development of amyotrophic lateral sclerosis (ALS). ALS is a chronic, progressive neurological disorder that disrupts motor neuron function however, clients remain fully intact cognitively. The disease causes destruction of both upper and lower motor neurons eventually causing paralysis and ventilator dependence.

Explanation

Learning Outcomes

ALS, also known as Lou Gerig disease, is a neurodegenerative neuromuscular disease that eventually causes loss of motor neurons that control voluntary muscle movement. Early symptoms include muscle stiffness increasing to weakness and eventually paralysis. There is destruction of both upper and lower motor neurons eventually leading to the inability to walk, speak, swallow, or breathe.

Test Taking Tip

When learning the underlying pathophysiology of disorders, it is helpful to draw out in diagram form what happens at the cellular level that eventually leads to development of the disease.

Video Rationale