A 55-year-old man is brought to his physician’s office with a 3-month history of progressive mental deterioration in the form of memory loss, mood changes, and errors in judgment. His gait is unsteady, and he requires assistance to prevent falling. He has no history of seizures, head trauma, or incontinence. Computed tomography (CT) scan and lumbar puncture are unremarkable. Physical examination reveals hypeonicity of all extremities, bilateral equivocal plantar response, ataxic gait, and myoclonic jerks in the lower extremities. What is the mechanism by which this infectious agent causes its pathology?
A 55-year-old man is brought to his physician’s office with a 3-month history of progressive mental deterioration in the form of memory loss, mood changes, and errors in judgment. His gait is unsteady, and he requires assistance to prevent falling. He has no history of seizures, head trauma, or incontinence. Computed tomography (CT) scan and lumbar puncture are unremarkable. Physical examination reveals hypeonicity of all extremities, bilateral equivocal plantar response, ataxic gait, and myoclonic jerks in the lower extremities. What is the mechanism by which this infectious agent causes its pathology?
💡 Explanation
**Core Concept**
The question describes a clinical presentation consistent with subacute sclerosing panencephalitis (SSPE), a rare and chronic neurological disorder caused by a persistent infection with the measles virus. This condition results from a defective measles virus that cannot replicate but still triggers a strong immune response, leading to inflammation and damage in the brain.
**Why the Correct Answer is Right**
The measles virus in SSPE causes its pathology by inducing the production of defective viral proteins that activate the immune system, leading to an autoimmune response against the host's brain tissue. This results in the production of various cytokines and chemokines, which contribute to the inflammation and degeneration of neurons in the brain. The defective viral proteins also interfere with normal cellular processes, further exacerbating the neurological damage.
**Why Each Wrong Option is Incorrect**
**Option A:** This option is incorrect because prion diseases, such as Creutzfeldt-Jakob disease, are caused by misfolded proteins rather than an infectious agent.
**Option B:** This option is incorrect because progressive multifocal leukoencephalopathy (PML) is caused by the JC virus, which primarily affects the white matter of the brain.
**Option C:** This option is incorrect because sporadic Creutzfeldt-Jakob disease is a prion disease that affects the brain, but it does not have a known infectious agent.
**Option D:** This option is incorrect because rabies is a viral infection that primarily affects the central nervous system, but it does not cause the same type of chronic and progressive neurological damage seen in SSPE.
**Clinical Pearl / High-Yield Fact**
One key feature of SSPE is the presence of myoclonus, which is a sudden, involuntary muscle jerk. This symptom is often a sign of underlying neurological damage and can be a key diagnostic clue in patients with a history of measles exposure.
**Correct Answer: A. The measles virus in SSPE causes its pathology by inducing the production of defective viral proteins that activate the immune system, leading to an autoimmune response against the host's brain tissue.**
✓ Correct Answer: A. Amyloid deposition
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