The Form of Dementia that Often Goes Unrecognized

Dear Dr. Pat,

There is a terrible form of dementia that often goes unrecognized until too many bad things have happened to the patient, personally and professionally. My sister was a brilliant entrepreneur with several successful businesses. She had a great marriage to an attorney and had two perfect children and a charmed life.  She was generous, grateful for her success and happiness and very kind to everyone in her orbit. For example, she provided at-home care for our aging mother, which none of her siblings could have done.

About six years ago, at the age of 48, she complained that she was having a terrible menopause. She became short tempered and was not sleeping much. Then, she became oddly preoccupied with financial issues, sensitive to how much everything cost in the care of our mother for example, almost to the point of paranoia. She unilaterally fired the round-the-clock help and left us with little notice to work to find another team, which was almost impossible. Our mother was concerned, confused and frightened that the people who had cared for her for years had been fired abruptly.  This was not an isolated event, unfortunately. Over the next three years, she became hostile at work and in her relationships with the investors in her company to the point that the board ousted her from the company she created and nurtured. This further fueled her paranoia and she became estranged from her husband and children as well. She refused to attend counseling sessions and left for a “trip around the world.” We assumed that she had started using drugs or was drinking but her husband and children denied this was possible. Weeks after her “trip around the world” she was hospitalized in a psychiatric hospital in another country for uncontrolled volatility and anger. They were concerned that she had developed some type of acute psychosis. Her family arranged for her to be urgently brought back to the U.S. where she was evaluated at a major medical center.  She hadn’t developed a psychiatric illness.  She wasn’t using drugs or alcohol. Menopause hadn’t caused this.  She had a form of dementia that affects the fronto-temporal area of the brain and often causes significant behavior change that initially can be confused with mid-life change, drug or alcohol use or even a kind of sudden psychiatric breakdown.

My sister was finally diagnosed properly at 54.  We who had always loved her, understood what had happened to our loving generous sister.  Her husband and children were united in their determination to care for her.  She is sadly unable to function without 24-hour care, but is able to live at home. This was not menopause or a mid-life crisis.  This was a terrible form of dementia.

If you notice that someone you know well has an  unexplained personality or behavior change, be aware of this condition as a diagnosis and work with others to get the person properly evaluated.  It is always important to insist that a patient who has a change in behavior undergo a thorough medical and neurological evaluation as well as consultation with a psychiatrist. I know this may not be easy, but the fallout from this kind of undiagnosed dementia can ruin the lives of more than the person who is terribly sick. I have chosen to inform people about this illness as a way of giving back to the memory of the sister I once knew.



Dear Constance,

I know it must be difficult for you to write about your your sister’s terrible illness and its severe impact on her life along with the uncertainty and suffering experienced by those in her professional and personal life.  But, you are remembering your sister in a positive way by sharing this personal story of loss in order to educate and inform others about this form of dementia often seen in early mid-life. We have asked Dr. Baxter Allen to discuss dementia in general and this form, frontotemporal lobar degeneration, in particular. I send my deepest sympathy to you and your family.

Dr. Pat


Dr. Baxter Allen Responds:

Dementia is a catchall term that describes cognitive impairment and loss of independent function due to problems with memory, judgment, thinking (executive function), language processing, and ability to function appropriately in social situations. In general, these symptoms progress over many years, although the speed varies significantly depending on the underlying cause of the dementia. The most common causes of dementia are Alzheimer’s disease, vascular dementia, and Parkinson’s/Lewy Body dementia. These forms of dementia are more common in the elderly, with diagnoses generally occurring after age 65.

There is another subset of dementias that affect younger people (45-65), which are collectively known as early onset dementias, even though Alzheimer’s and vascular dementia are still the most common causes of dementia in these younger patients, accounting for 34 percent and 18 percent of diagnoses, respectively. However, the third most common cause of dementia in this younger population is frontotemporal lobar degeneration, also known as frontotemporal dementia (FTD), accounting for 12 percent of cases. (Lancet Neurology) For every 100,000 people, there are 3 to 4 who receive a diagnosis of FTD per year.

Alzheimer’s dementia patients have memory loss as the prevailing symptom. Patients with FTD often have a significant decline in behavior (the behavior variant) or in speech and language (the primary progressive aphasia variant). The behavioral variant, not surprisingly, is characterized by prominent personality change and the loss of normal interpersonal behavior along with bizarre and out-of-character personal conduct.

It is not uncommon for these symptoms to be entirely thought of as just increasingly erratic and inappropriate behavior. Family members, work colleagues and friends may all assume that the person may have begun to use drugs, abuse alcohol or has just decided to become hedonistic, self centered and unaware that there could be anything wrong with these new behaviors. It is unfortunate that consideration of an organic medical problem often does not happen until later in this disease process when much damage may have been done to family structure and financial health of the family unit.

The primary progressive aphasia variant is subdivided into three separate problems that are language based: the semantic variant, in which individuals lose their ability to understand or formulate nouns in a spoken sentence; the nonfluent variant, in which speech becomes hesitant, labored, and/or lacking proper grammar; and the logopenic variant, where there are mostly word-retrieval issues while understanding of words appears to remain intact during the early stages.

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