Certified Neuroscience Registered Nurse (CNRN) Practice Exam

How does ALD primarily affect the nervous system?

1. Causes peripheral nerve damage

2. Leads to neuron death

3. Results in myelin degeneration

4. Ischemic stroke

The correct answer is: Results in myelin degeneration

ALD, or Adrenoleukodystrophy, primarily impacts the nervous system by causing myelin degeneration. Myelin is a fatty substance that serves as an insulating layer around nerve fibers, facilitating the efficient transmission of electrical impulses. In ALD, there is a disruption in the metabolism of very long-chain fatty acids, which leads to the accumulation of these fatty acids in the body, particularly affecting the brain and spinal cord. This accumulation causes damage to oligodendrocytes, the cells responsible for producing and maintaining myelin. As a result, the loss of myelin compromises neural communication, leading to a range of neurological symptoms. Understanding this mechanism is crucial for recognizing how ALD manifests neurologically, explaining why myelin degeneration is the primary concern in this condition. Other processes, such as peripheral nerve damage or neuron death, may occur secondary to myelin destruction, but the focal point of ALD's impact on the nervous system is fundamentally rooted in the degeneration of myelin, leading to neurological dysfunction.