A 56-year-old man is brought to the office by his wife due to increasingly odd behavior. The patient's wife says that her husband started to become emotionally "cold" toward her about a year ago and that he has since received a number of complaints from women at work, who report that he has been making unwanted advances. She says, "He used to be a very loving and caring husband, but lately he doesn't appear to care about anything." Over the past 6 months the patient's sleep has worsened and he has begun to exhibit memory difficulties; he has gotten lost a few times while driving home. His wife also notes that he never used to have a sweet tooth, but she now cannot buy sweets because he will eat the entire box. The patient has a history of hypertension and hyperlipidemia. Which of the following is the most likely diagnosis?

Question

A 56-year-old man is brought to the office by his wife due to increasingly odd behavior.  The patient's wife says that her husband started to become emotionally "cold" toward her about a year ago and that he has since received a number of complaints from women at work, who report that he has been making unwanted advances.  She says, "He used to be a very loving and caring husband, but lately he doesn't appear to care about anything."  Over the past 6 months the patient's sleep has worsened and he has begun to exhibit memory difficulties; he has gotten lost a few times while driving home.  His wife also notes that he never used to have a sweet tooth, but she now cannot buy sweets because he will eat the entire box.  The patient has a history of hypertension and hyperlipidemia.  Which of the following is the most likely diagnosis?

Quizplus · Accepted Answer

Explanation: 11ec2b69_c18e_1f76_8f63_89d26b61f26e_MD0005_00 This patient's symptoms are consistent with a diagnosis of behavioral variant frontotemporal dementia (bvFTD). bvFTD frequently leads to early-onset dementia and usually presents around age 60 with ≥3 of the following clinical features: Disinhibited behavior, apathy, loss of empathy, perseverative behavior, and hyperorality. Early and marked personality and behavioral changes are typical and can help differentiate bvFTD from other dementias. Memory function is typically spared in the early stages of bvFTD, and neuropsychological testing may be in the normal range. Two-thirds of patients with bvFTD have evidence of frontotemporal atrophy on neuroimaging. Half of patients with bvFTD have an autosomal dominant inheritance pattern evident in family history. bvFTD is typically fatal within 8 years of symptom onset. Other variants of FTD include primary progressive aphasia (PPA), which presents with prominent and relatively isolated language impairment that may progress to impairment of other cognitive domains. (Choices A, B, and E) The differential diagnosis of bvFTD includes Alzheimer disease, dementia with Lewy bodies, and vascular dementia. Alzheimer disease typically presents with early, prominent memory impairment. Dementia with Lewy bodies is characterized by visual hallucinations, fluctuating attention/alertness, rapid-eye movement sleep behavior disorder, and/or parkinsonism. Vascular dementia typically presents in older patients with stepwise decline in cognitive function (temporally related to cerebrovascular events) and evidence of cerebrovascular disease on neuroimaging; early executive dysfunction is classic, and behavioral changes make bvFTD more likely. (Choice D) Huntington disease is an autosomal dominant disorder that usually presents around age 40 with chorea, psychiatric symptoms (eg, depression, anxiety), and abnormal eye movements (eg, abnormal saccades). Symptoms of dementia typically begin later in the course of Huntington disease. Educational objective: Behavioral variant frontotemporal dementia (bvFTD) typically presents with prominent personality and behavioral changes (eg, disinhibition, apathy, loss of empathy, perseverative behavior, and/or hyperorality). Memory may be preserved in early bvFTD, in contrast to the prominent early memory impairment that characterizes Alzheimer disease. References: Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. (http://www.ncbi.nlm.nih.gov/pubmed/21810890) Behavioural-variant frontotemporal dementia: diagnosis, clinical staging, and management. (http://www.ncbi.nlm.nih.gov/pubmed/21147039) Progression in behavioral variant frontotemporal dementia: a longitudinal study. (http://www.ncbi.nlm.nih.gov/pubmed/26501846)

A 56-Year-Old Man Is Brought to the Office by His