Case 1: Mr. Smith is a 55-year-old man with the diagnosis of frontotemporal dementia (FTD).

Background Information: There is no psychiatric or dementia history in Mr. Smith's family. Mr. Smith was a healthy child and a good student. He achieved an MBA from Harvard and went on to work for a Fortune 500 company, rising steadily through the ranks. He had never been fired from a position and was considered an even-tempered, reliable worker. He has been married for 28 years, and has 3 children. He lives with his wife in their own house. His family describes him as a devoted husband and father, sensitive to their needs and supportive in all ways. There is no history of substance abuse. He has always managed their finances conservatively.

2. Borderline Type II diabetes mellitus, also successfully treated for many years with diet control

Mr. Smith is on no medications. Prior to the onset of his illness, Mr. Smith had no history of psychiatric symptoms or treatment.

Present Illness: For the very first time at age 51, Mr. Smith began having difficulties at work. He frequently reported to his wife that his supervisor was unfairly critical of his work, and that, "people from Yale just don't like us Harvard grads." He would repeat this phrase on a nearly nightly basis, even when talking about their friends, family or neighbors. Although he claimed that his work was still solid, his supervisor reported that he had become inefficient, condescending to others, and even rude upon a few occasions, once even referring to a client's idea as "silly" (to his face). After being given an ultimatum, Mr. Smith agreed to be evaluated by a psychiatrist through the employee assistance program of the company.

The psychiatric evaluation was inconclusive. There was no evidence of depression or mania, as the patient's mood, energy, sleep, appetite, and libido were all at baseline. There was no evidence of psychotic symptoms as well. The patient's memory, orientation, calculation and visual-spatial perception were found to be normal, although he did get distracted easily. The psychiatrist speculated about the possibility of covert drug use, but a comprehensive toxicology screen was negative, as was a screening battery of blood tests. Ultimately, the patient was referred for interpersonal skills training which he refused, and he was fired from the job.

Over the next year, Mr. Smith bounced from job to job, with similar outcomes as above. Mr. Smith started eating significant amount of sweets while at home, and this was attributed to his frustration at being unemployed, as was his increasing irritability. Over the ensuing months he began to take less interest in his appearance, often refusing to shave even for social engagements. He insisted on wearing the same clothes day after day, and Mrs. Smith found herself surreptitiously washing his clothes at night to prevent them from smelling. His conversation at home became narrowly focused on food, his inability to obtain a job because of "those Yale guys", and TV sitcoms. He took less and less interest in the activities of his wife and children. Although they initially attributed all of this to the stress and embarrassment of being unemployed, they began to suspect that something more was wrong, but out of respect to him, did not initially confront him. When they did raise their concerns, he dismissed them sarcastically.

Mr. Smith began to gain weight. He had a number of hospitalizations for hyperglycemia (elevated blood sugar), but would leave the hospital as soon as he was stabilized, and then refuse to take medications. A competency evaluation found that although he seemed to act impulsively and in poor judgement, he could remember and apparently understand the information presented to him, including the details of the treatment options, and the consequences of not receiving treatment. When asked why he was acting this way he stated that "you only live once, I might as well enjoy myself while I'm still here."

Over the next few months Mrs. Smith noticed that Mr. Smith began having difficulties thinking of the words he wanted to say. Although his comprehension appeared intact, his verbal output became more halting and his sentences became shorter. At this point she insisted that he be evaluated by a neurologist. The neurologist noted that Mr. Smith appeared disheveled, silly and sarcastic at times. His speech was halting and he had difficulty naming simple objects. He had absolutely no insight as to why he was there. A comprehensive physical and neurological examination was normal. On cognitive testing, once again his performance was within the normal range for orientation, calculation, and visual-spatial perception. He did have difficulty with new learning, but it was not severely impaired. Performance on tests of judgement, abstraction, and executive function was relatively worse than the domains listed above. A brain MRI scan revealed atrophy (shrinkage) of the right frontal and left temporal lobes. The diagnosis of FTD was made.

Frontotemporal dementia (FTD) is increasingly recognized as a fairly common type of dementia, and has a clinical course that differs significantly from that of Alzheimer’s Disease. FTD is a degenerative condition primarily affecting the frontal and anterior temporal lobes. These areas primarily control judgement, personality, regulation of behavior, movement, speech, social graces, language, and some aspects of memory. The age of onset of FTD is typically in the 50's and 60's, with equal incidence in men and women. In about one third of patients, a family history of dementia exists, suggesting a genetic component in the etiology of many cases.

FTD is marked by dramatic changes in personality, behavior, and judgement. Changes in personal and social conduct occur in early stages of the disease, and include disinhibition, socially inappropriate behavior, apathy, social withdrawal, hyperorality (greatly increased food intake and mouthing of non-food objects), and ritualistic compulsive behaviors. Profound lack of regard for personal hygiene is common. These symptoms may lead to misdiagnosis as a mood disorder, personality disorder or the product of familial conflict , while in the elderly may be mistaken for withdrawal or eccentricity. Family members often describe these patients as having become "a completely different person." FTD patients often present two seemingly opposite behavioral profiles in the early and middle stages of the disease. Some individuals are overactive, restless, distractible and disinhibited, while others are apathetic, inert, aspontaneous and emotionally blunted. Early in the disease, cognitive functions such as memory, calculation and orientation may be preserved, although eventually, the clinical picture progresses to one of significant cognitive impairment.

Frontotemporal dementia can be fairly accurately diagnosed by a careful neuropsychiatric history, supported by neuroimaging and neuropsychological testing. The length and progression of FTD varies. Some patients decline rapidly over two to three years, while others show only minimal changes over a decade. No medications are known currently to treat or prevent FTD. However, serotonin-boosting or dopamine boosting medications may help to alleviate some behaviors.