Four years earlier, this 58-year-old woman displayed belligerence and foul language, which were unprecedented in her characteristic sophisticated behavior. Several months later, she went on to show difficulties interacting meaningfully with other people. She would occasionally ask what “a fried egg” was, if she had been asked this for breakfast. A year into her illness, she became paranoid, believed her husband was hitting her, and exhibited violent behavioral outbursts. Quetiapine first and risperidone later were given for about 3 months after a hospitalization in the psychiatry ward. With her behavioral outbursts controlled, she became restless and needed to pace about constantly. She also developed stereotypies whereby she would tap her lap or rub her fingers repeatedly. She also exhibited a marked decrease in verbal output with a persistent grin and constant mumbling, occasionally interrupted by echolalia. She needed help to attend all of her personal needs. Her mother is reported to have had Alzheimer disease at the age of 60 years.

In addition to her progressive global aphasia (beyond that shown by the patient of the prior case) and echolalia, this patient displayed a range of abnormal behaviors hinging around a dramatic change in her personality. Her perseverative behaviors and stereotypies associated with disinhibition and early irritability, in the setting of marked atrophy of the frontal and antero-temporal lobes, were highly suggestive of the behavioral variant of
frontotemporal dementia (bvFTD). Akathisia may have emerged as the result of prior neuroleptic exposure but could also have represented underlying motor disinhibition. Given her positive family history, a mutation in tau or progranulin may be the underlying genetic etiology.