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Difficulty: MediumNeurocognitive Disorders

A 58-year-old man is brought to the physician by his wife due to profound changes in his behavior over the past 12 months. His wife states that he was previously a reserved and empathetic middle school teacher, but he recently began making sexually explicit comments to strangers at the grocery store. He has also developed a habit of eating exclusively sugary cereals and frequently takes food from the plates of other patrons at restaurants. When confronted about these actions, the patient is apathetic and demonstrates no remorse. His medical history is significant for hyperlipidemia. Vital signs are within normal limits. On mental status examination, he is alert and oriented to person, place, and time. He scores 26/30 on the Montreal Cognitive Assessment, primarily losing points on verbal fluency and abstraction. Memory recall is intact. Neurological examination reveals a positive grasp reflex but is otherwise unremarkable. Which of the following is the most likely diagnosis?

  1. A
    Alzheimer disease
  2. B
    Lewy body dementia
  3. Frontotemporal dementiaAnswer
  4. D
    Major depressive disorder
  5. E
    Vascular dementia

Answer

Frontotemporal dementia
The correct answer is frontotemporal dementia. This patient's presentation of early-onset personality changes, marked disinhibition (making sexually explicit comments), apathy, hyperorality (exclusive diet of sugary cereals, taking food from others), and relative sparing of memory is highly characteristic of the behavioral variant of frontotemporal dementia (bvFTD). The presence of frontal release signs (such as a positive grasp reflex) further supports frontal lobe dysfunction. Memory is classically preserved early in the disease, whereas executive dysfunction (impaired verbal fluency and abstraction) is prominent.

Step-by-Step Solution

1
Identify the primary clinical manifestations in the vignette.
The patient has early-onset personality changes, marked disinhibition, apathy, hyperorality, and frontal release signs.
Recognizing the core symptoms is essential for differentiating neurocognitive disorders.
2
Evaluate the patient's cognitive profile.
The patient has intact memory recall and orientation but exhibits executive dysfunction (poor verbal fluency and abstraction).
The pattern of cognitive deficits helps rule out memory-predominant dementias like Alzheimer disease.
3
Synthesize the behavioral and cognitive findings to determine the diagnosis.
The combination of severe behavioral variant symptoms with executive dysfunction and spared memory strongly indicates frontotemporal dementia.
These findings strictly meet the clinical diagnostic criteria for the behavioral variant of frontotemporal dementia (bvFTD).

Key Concept

The behavioral variant of frontotemporal dementia presents with early and prominent personality changes, disinhibition, apathy, hyperorality, and executive dysfunction, with relative sparing of memory and visuospatial skills.
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Topics

Neurocognitive Disorders

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