Pick’s Disease
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CASE PRESENTATION
Mr. J is a 59-year-old married man who is the vice president of a large accounting firm. He comes to see his internist accompanied by his wife. Mrs. J tells Dr. G that she is extremely concerned about her husband. He has been forgetful at times, such as eating dinner and then coming to her an hour later asking, “What’s for dinner?” She reports that her husband, a usually quiet man, has been talking to strangers, often carrying on long conversations about small details, such as whether the price of postage stamps is too high. Mr. J has been socially inappropriate at times, often kissing and grabbing his wife in restaurants or in front of friends. Mrs. J reports that he often gets up at night and eats large amounts of food, something he never did in the past. Mr. J seems to be performing well on his job and has recently acquired several large clients for the firm. At a recent office party, several coworkers joked with Mrs. J about her husband’s new habit of coming to work without socks and walking barefoot in his office.
Mr. J has a history of hypertension that has been well-controlled on benazepril 10 mg with hydrochlorothiazide 25 mg once daily. He drinks alcohol occasionally.
Dr. G observes that Mr. J appears unusually talkative, commenting at length about the doctor’s blue striped necktie. He walks around the exam room, tells sexually related jokes, and uses several obscenities. This is a marked change from the reserved, business-like man Dr. G has cared for over the past 10 years. Dr. G starts thinking of a differential diagnosis, including a possible stroke or psychiatric illness. He undertakes a physical examination, electrocardiogram (ECG), and fingerstick glucose, all of which are normal. On the Mini-Mental State Examination, Mr. J scores 28 out of 30. Of concern was that he is unable to name the items “pencil” and “watch,” instead giving a definition of “a writing instrument used for centuries to provide information for the masses.” Dr. G orders laboratory tests, including complete blood count, chemistry panel, thyroid profile, and vitamin B12, folate, and urine toxicology levels. He decides to ask Mr. J to sign a consent for HIV testing, which prompts another series of sexually explicit jokes.
Dr. G refers Mr. J to a neurologist who reviews his laboratory tests, finding no abnormalities. Mr. J undergoes a lumbar puncture, with normal results, including testing for Creutzfeldt-Jakob proteins. The neurologist sends Mr. J for magnetic resonance imaging (MRI) and positron emission tomography (PET) scans of the brain, and asks Mrs. J and her two sons to submit blood samples for genetic testing. The neurologist tells Mr. and Mrs. J that he has a serious disorder and recommends a brain biopsy. Mr. J insists that he is fine and refuses further testing. Mrs. J calls Dr. G in a highly anxious state. She asks for help clarifying her husband’s diagnosis and how they should proceed. Dr. G arranges to meet with the family to discuss the findings and recommendations.
DISCUSSION
Pick’s disease is a type of dementia marked by prominent changes in behavior, mood, social skills, and personality, followed by progressive decline in memory and loss of executive functioning.1 Cortical atrophy selective to the frontal and temporal lobes makes this disorder distinct from dementia of the Alzheimer’s type, as does the early age of onset, which is typically before the age of 60 years. The Czechoslovakian neurologist Arnold Pick was the first to describe this syndrome in 1892. His colleague, Dr.
REFERENCES
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