American Board of Psychiatry and Neurology (ABPN) Practice Exam 2025 - Free ABPN Practice Questions and Study Guide

Question: 1 / 1275

Which syndrome is characterized by rapidly progressing dementia and myoclonus?

Progressive multifocal leukoencephalopathy

Subacute sclerosing panencephalitis

Spongiform encephalopathy

The syndrome characterized by rapidly progressing dementia and myoclonus is indeed linked to spongiform encephalopathies, which include conditions such as Creutzfeldt-Jakob disease. These diseases are associated with prion proteins, which lead to the degeneration of brain tissue, resulting in a variety of neurological symptoms.

Patients often present with rapidly progressive cognitive decline, predominantly affecting memory and executive function, alongside myoclonic jerks—sudden involuntary muscle contractions. The combination of these symptoms, along with specific findings on neuroimaging and cerebrospinal fluid analysis, helps to identify spongiform encephalopathies.

The characteristic features of this condition, such as the rapid progression of dementia, strong association with myoclonus, and distinctive pathophysiological changes in the brain, make it the correct choice in the context of the symptoms described.

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Cytomegalovirus encephalitis

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