A 40-year-old male patient is complaining of chronic progressive deterioration and is admitted to the unit. The nurse recognizes that these characteristics indicate a disease that results in degeneration of the basal ganglia and cerebral cortex. The disease is called?

1. A

   GBS (Guillain-Barré syndrome)

2. B

   Huntington's disease

3. C

   Multiple sclerosis

4. D

   Myasthenia gravis

Appeared in: HPSSC - 2015

Explanation

• Huntington's disease is a genetic neurodegenerative disorder.
• It is specifically characterized by the progressive degeneration of nerve cells in the basal ganglia (striatum) and the cerebral cortex.
• This neuronal loss is responsible for the classic triad of symptoms: involuntary choreiform movements, cognitive decline (dementia), and psychiatric disturbances.
• The age of onset is typically between 30 and 50 years, which matches the patient's age of 40.

Why Other Options Were Wrong

• Option A: Guillain-Barré syndrome (GBS) is an acute inflammatory demyelinating polyneuropathy, affecting the peripheral nervous system, not the basal ganglia or cerebral cortex. It typically presents with rapidly ascending muscle weakness.
• Option C: Multiple sclerosis is an autoimmune disease characterized by the demyelination of the central nervous system (brain and spinal cord), primarily affecting the white matter. It does not cause the specific degeneration of the basal ganglia described in the question.
• Option D: Myasthenia gravis is an autoimmune disorder that affects the neuromuscular junction, leading to fatigable muscle weakness. It does not involve degeneration of the central nervous system structures like the basal ganglia or cortex.

Related Visual

Clinical Relevance

• Nursing practice connection: Use the key finding related to Pathophysiology of Neurodegenerative and Neuromuscular Disorders to guide bedside assessment, documentation, and the next nursing action.
• Nurses must prioritize patient safety for individuals with Huntington's disease due to the high risk of falls from chorea and aspiration from dysphagia.
• Providing comprehensive psychosocial support and education is critical, as the disease has a profound impact on patients and their families, including the need for genetic counseling for at-risk relatives.
• What if? If the patient presented with bradykinesia (slowness of movement), rigidity, and a resting tremor, the primary diagnosis to consider would be Parkinson's disease, which also involves the basal ganglia but through a different mechanism (loss of dopaminergic neurons).

How to Approach the Question

• First, identify the key pathological features described in the question stem: 'degeneration of the basal ganglia and cerebral cortex'.
• Next, systematically review the pathophysiology of each option provided.
• Recall or deduce that Huntington's disease is specifically known for causing atrophy and neuronal loss in the basal ganglia and cortex.
• Eliminate Guillain-Barré syndrome because it affects the peripheral nervous system.
• Eliminate Myasthenia gravis as it is a disorder of the neuromuscular junction.
• Eliminate Multiple sclerosis, which is a demyelinating disease of CNS white matter, a different pathology than the neuronal degeneration described.

Concept Tested & Keywords

• Concept Tested: Pathophysiology of Neurodegenerative and Neuromuscular Disorders
• Stem keywords: chronic progressive deterioration, degeneration, basal ganglia, cerebral cortex
• Lead-in keywords: The disease is called?
• Clinical cues: 40-year-old male
• Clinical cues: chronic progressive deterioration

Question ID

QcDQNXw6giUaNs7Xz7YmHL

Copy ID

Reference Book

E6 Pathology- ROBBINS & COTRAN PATHOLOGIC BASIS OF DISEASE 10TH Ed p. 1283-1285

E6 Physiology Ganong 27e Vol 1 Part 2 p. 22-24

E6 Medicine Davidson Principles Practice 24e p. 1135-1137