A 69-year-old man is brought to the office by his wife due to recent changes in behavior. Despite years of managing the family's finances, over the previous 12 months the patient has failed to pay a number of important bills. He has become more withdrawn and no longer enjoys social gatherings. When he does socialize now, he is often irritable and offensive. The patient used to smoke a quarter pack of cigarettes a day but has increased to a pack a day over the previous 12 months. He also now insists on eating the same thing for breakfast every morning and gets very upset if a different meal is prepared. The patient denies having any problems and seems indifferent to his wife's concern. Medical history includes hypertension and type 2 diabetes mellitus. There is a family history of Alzheimer disease. Temperature is 36.7 C (98 F), blood pressure is 144/86 mm Hg, pulse is 62/min, and respirations are 14/min. The patient scores a 24 out of 30 on the Montreal Cognitive Assessment (normal ≥26). Which of the following is the most likely diagnosis in this patient?

Question

A 69-year-old man is brought to the office by his wife due to recent changes in behavior.  Despite years of managing the family's finances, over the previous 12 months the patient has failed to pay a number of important bills.  He has become more withdrawn and no longer enjoys social gatherings.  When he does socialize now, he is often irritable and offensive.  The patient used to smoke a quarter pack of cigarettes a day but has increased to a pack a day over the previous 12 months.  He also now insists on eating the same thing for breakfast every morning and gets very upset if a different meal is prepared.  The patient denies having any problems and seems indifferent to his wife's concern.  Medical history includes hypertension and type 2 diabetes mellitus.  There is a family history of Alzheimer disease.  Temperature is 36.7 C (98 F), blood pressure is 144/86 mm Hg, pulse is 62/min, and respirations are 14/min.  The patient scores a 24 out of 30 on the Montreal Cognitive Assessment (normal ≥26).  Which of the following is the most likely diagnosis in this patient?

Quizplus · Accepted Answer

11ec25c8_d584_7ff6_bc81_b5ed5477fb53_MD0003_00 This patient's behavioral changes (eg, socially offensive behavior, social withdrawal), compulsive behavior (eg, consuming the same meal daily), hyperorality (eg, recently increased cigarette consumption), apathy, lack of insight, and executive dysfunction (eg, difficulty with tasks that require planning) are consistent with a diagnosis of behavioral-variant frontotemporal dementia (FTD). Disinhibition is often a prominent component of the behavioral changes seen in FTD. Unlike in Alzheimer disease, memory impairment is generally mild in early FTD but worsens as the disease progresses. FTD typically presents around age 60, affects men and women at equal rates, and follows an autosomal dominant pattern of inheritance in up to 25% of cases. The degenerative process of FTD progresses more rapidly than other dementing syndromes (eg, Alzheimer disease), and the average length of survival following diagnosis is 5-10 years. Behavioral-variant FTD is most often associated with the accumulation of tau protein inclusions known as Pick bodies in the hippocampi, temporal lobes, and frontal lobes. Selective serotonin reuptake inhibitors (eg, sertraline, escitalopram) or trazodone have demonstrated some benefit in the management of neuropsychiatric symptoms of FTD. (Choice A) Alzheimer disease is characterized by early memory, executive, and visuospatial dysfunction. Personality change generally does not occur until later stages of the disease. (Choice B) Creutzfeldt-Jakob disease is a prion disease that presents with rapidly progressive dementia, myoclonus, hypokinesia, and cerebellar dysfunction. (Choice C) Dementia with Lewy bodies presents with dementia plus ≥2 of the following features: visual hallucinations, parkinsonism, fluctuating cognition, and REM sleep behavior disorder. (Choice E) Mild cognitive impairment can be diagnosed only in patients whose cognitive impairment has not impaired activities of daily living (eg, managing finances). This patient meets criteria for FTD. (Choice F) Vascular dementia classically presents with mild memory impairments, prominent executive dysfunction, and focal neurologic deficits corresponding to cerebrovascular pathology. This patient's behavioral symptoms and overall presentation are better explained by FTD. Educational objective: Behavioral-variant frontotemporal dementia presents with behavioral changes (eg, disinhibition), compulsive behavior, hyperorality, apathy, and executive dysfunction. Unlike Alzheimer disease, memory impairment is normally mild early in the disease. References: Frontotemporal dementia. (http://www.ncbi.nlm.nih.gov/pubmed/26595641) Behavioural-variant frontotemporal dementia: diagnosis, clinical staging, and management. (http://www.ncbi.nlm.nih.gov/pubmed/21147039) Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. (http://www.ncbi.nlm.nih.gov/pubmed/21810890)

A 69-Year-Old Man Is Brought to the Office by His