A 67-year-old man comes to the office with his wife due to increasing difficulty performing his job over the past year. He has become more irritable at work and has gotten into arguments with his boss for the first time in 20 years. His office has become increasingly cluttered as he refuses to throw things out, and he has forgotten to submit numerous recent assignments. His wife describes him as withdrawn, emotionally detached, and "not the man I married." She says, "Recently, he wants to eat the same meal every day and has lost interest in golf, which used to be one of his favorite pastimes." The patient has a history of hypertension and hyperlipidemia. He has a 30-pack-year smoking history. The patient scores 25/30 on the Montreal Cognitive Assessment (normal: ≥26). The remainder of the physical examination is normal. Which of the following is the most likely diagnosis in this patient?

Question

A 67-year-old man comes to the office with his wife due to increasing difficulty performing his job over the past year.  He has become more irritable at work and has gotten into arguments with his boss for the first time in 20 years.  His office has become increasingly cluttered as he refuses to throw things out, and he has forgotten to submit numerous recent assignments.  His wife describes him as withdrawn, emotionally detached, and "not the man I married."  She says, "Recently, he wants to eat the same meal every day and has lost interest in golf, which used to be one of his favorite pastimes."  The patient has a history of hypertension and hyperlipidemia.  He has a 30-pack-year smoking history.  The patient scores 25/30 on the Montreal Cognitive Assessment (normal: ≥26).  The remainder of the physical examination is normal.  Which of the following is the most likely diagnosis in this patient?

Quizplus · Accepted Answer

Explanation: This patient's symptoms and course of illness are most consistent with the behavioral variant of frontotemporal dementia (FTD), also known as Pick disease, which is characterized by focal degeneration of the frontal and/or temporal lobes. Clinical features of this condition are: early personality and behavioral changes (eg, disinhibition, apathy). compulsive behaviors (eg, hoarding, hyperorality). executive dysfunction. Memory deficits tend to appear later in the disease course. FTD involves an earlier onset than other dementing illnesses, and those afflicted are usually in their 50s or 60s. Up to 40% of patients with FTD have a family history of the disease. Early symptoms such as apathy and personality changes may be mistakenly attributed to depression or another psychiatric disorder. FTD must also be differentiated from other dementia syndromes (eg, Alzheimer disease, dementia with Lewy bodies). dementia syndromes (Choice A) Alzheimer disease is the most common type of dementia and presents with early, prominent memory impairment. This patient's memory impairment is mild, and his overall presentation is better explained by FTD. (Choice B) Dementia with Lewy bodies presents with visual hallucinations, parkinsonism, REM sleep behavior disorder, and fluctuating cognition with variations in attention and alertness that may be misdiagnosed as delirium. (Choice D) Hoarding disorder typically presents in the teenage years and is characterized by an inability to dispose of items that may have no actual worth (eg, magazines, paper towels), resulting in significant and often dangerous clutter in the home environment. Hoarding behavior tends to worsen with age. This patient's recent onset of hoarding behavior, associated marked personality changes, and cognitive impairment make this diagnosis unlikely. (Choice E) Although patients with major depressive disorder may show withdrawn, apathetic behavior and reversible cognitive impairment, this patient lacks other key features of depression (eg, sadness, feelings of worthlessness). His symptom constellation and marked change in personality are better explained by FTD. (Choice F) Vascular dementia presents with cognitive impairment and focal neurologic findings that correspond anatomically with cerebrovascular pathology. This patient does not have any focal neurologic findings on examination. Educational objective: The behavioral variant of frontotemporal dementia (FTD) is characterized by early personality changes, compulsive behaviors, and executive dysfunction; memory difficulties tend to develop later in the disease course. FTD has an early age of onset (ie, 50s-60s) and a strong hereditary component. References: Frontotemporal dementia. (http://www.ncbi.nlm.nih.gov/pubmed/26595641) Behavioral-variant frontotemporal dementia: diagnosis, clinical staging, and management. (http://www.ncbi.nlm.nih.gov/pubmed/21147039) Sensitivity of revised diagnostic criteria for the behavioral variant of frontotemporal dementia. (http://www.ncbi.nlm.nih.gov/pubmed/21810890)

A 67-Year-Old Man Comes to the Office with His Wife