Dreaming of Clooney and Costner: Frontotemporal dementia

Alzheimer´s disease is probably the best known of the progressive dementias. A recent survey conducted by the Harvard School of Public Health and Alzheimer Europe also showed that this is a disease that very many fear. But there are other neurodegenerative illnesses that are possibly more devastating than Alzheimers´s disease. Consider the following case described by Sarah Kremen and colleagues in Cognitive and Behavioral Neurology in 2010:
Charles Bell: Anatomy of the Brain, c. 1802

A 53-year-old woman presented to a clinic at the University of California with a 5 to 6 years history of personality and behavioral changes. Initially she had become withdrawn and passive and lacked motivation to do household chores which in her case was quite out of character. Later she started having trouble executing simple tasks, such as turning on the shower. She also made inappropriate comments to strangers, asked them silly questions and behaved childishly. She became impulsive and exhibited compulsive and repetitive behaviors. She would, for example, not step on cracks on sidewalks, she rubbed her knee repetitively and swayed in a particular way. There was emotional blunting as she appeared unconcerned when her brain-damaged daughter was placed in a nursing home. Her symptoms progressed and her behavior became more disinhibited. She started asking strangers inappropriate sexual questions, told her family she was thinking about becoming gay and made obscene gestures in public. She had vivid fantasies about movie stars. She claimed they were her friends or that she was married to them and frequently conversed with her imaginary husbands. She spoke about her relationship with Kevin Costner and claimed being married to George Clooney and having two children with him. As she deteriorated even further her inappropriate behavior decreased and she became less responsive. At age 58, eleven years after the first symptoms, the woman passed away. At that time she had become mute, very rigid in her movements and had problems walking.

Frontotemporal dementia: Behavioral variant
The patient described was diagnosed with a particular sup-type of frontotemporal dementia (FTD), behavioral variant FTD (bvFTD). FTD is an umbrella term and is generally thought to encompass three sub-types. Two of them have language problems as their main feature early in the disease and the third one has personality changes as the presenting feature as in the case previously described. Therefore this is called the behavioral variant FTD (bvFTD). In contrast to Alzheimer´s disease memory can be relatively spared for some time even though behavior is grossly abnormal and inappropriate.
In FTD there is disproportionate bilateral atrophy, or loss of tissue, in the frontal and temporal lobes of the brain. The etiology, or the cause, of the disease is not known and the underlying pathology in the brain can be of various types. There is no disease-specific treatment available and life-expectancy after diagnosis is 6 to 13 years.

FTD is under-recognized
In spite of being the second most common early-onset dementia after Alzheimer´s disease the diagnosis of bvFTD is often missed. Because of the personality changes and relatively unimpaired memory the possibility of dementia is overlooked. The patients are sometimes diganosed with psychiatric illness and may end up in psychiatric wards where they are unsuccfessfully treated. The patient described here was initially treated for depression because of her withdrawal, lack of motivation and loss of appetite. Patients with bvFTD may fulfill diagnostic criteria for bipolar disease, obsessive-compulsive disorders, and even schizophrenia. Because of this overlap between the symptoms of bvFTD and psychiatric illnesses there is often a lag between the initial symptoms and definite diagnosis.

Caregiver burden
BvFTD causes dramatic changes in behavior and personality and thus attacks a person´s identity and core characteristics in a way that Alzheimer´s disease does not. Patients may burst out singing when not appropriate, walk around naked, steal, neglect personal hygiene, hoard and become childish and ego-centered, showing little or no empathy. They also typically have no insight into their behavior. It is therefore not surprising that caregiver burden is greater for those who care for loved ones with FTD than those who have Alzheimer´s disease. Even though patients with Alzheimer´s disease generally have very severe memory problems they can preserve their personality and social graces for a long time and enjoy spending time with their loved ones. An added source of stress is the fact that FTD affects younger people more often than Alzheimer´s disease, symptoms often appearing between the ages of 40 and 65.

Here is a short video on frontotemporal dementia

Kremen, S.A., Solis, O.E., Shapira, J.S., Vinters, H.V. & Mendez, M. F. (2010). ‘‘Fantastic Thinking’’ in Pathologically Proven Pick Disease. Cognitive and Behavioral Neurology, 23, 130-134.

Loy, C.T., Kril, J.J., Trollor, J.N. et al. (2012). The case of a 48 year-old woman with bizarre and complex delusions. Nature Reviews, Neurology, 6, 175-179.

Rascovsky, K. et al. (2011). Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dmentia. Brain, 134, 2456-2477.

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