Frontotemporal Dementia: Language, Behavior, and Movement


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Also called "Pick's Disease" after its founder, Dr. Arnold Pick, Frontotemporal Dementia (FTD) exhibits specific characteristics that can help the diagnostician differentiate FTD from Alzheimer's Disease. Not actually a single disease, FTD affects the frontal and temporal lobes of the brain, which are the primary developers and transmitters for personality, language, and behavior & controlling inhibition--sometimes referred to as being our moral control center.


There are three subtypes associated with FTD, focused on the attributes mentioned above:

  1. Behavior variant FTD (bvFTD)

  2. Primary progressive aphasia (PPA)

  3. Motor function disturbances

In order, the three subtypes above affect (1) behavior, (2) language, and (3) muscle or motor functions. Sometimes misdiagnosed still as being Alzheimer's, FTD may occur at a younger age--even as young as 40.


Symptoms

There are key indicators that differentiate FTD from Alzheimer's. First, FTD occurs typically at a younger age than Alzheimer's, but Alzheimer's more commonly has memory loss, confusion, hallucinations, and delusions. In FTD, some of the distinct symptoms that appear include:

  • Prominent or dramatic changes in behavior--especially in personality & relationships

  • Problems with speech and communications--Semantic Dementia (SD)

  • Often accompanied by Amyotrophic Lateral Sclerosis (ALS)--muscle weakness

  • Corticobasal Syndrome--Stiff and uncoordinated arms and leg muscles

  • Progressive Supranuclear Palsy (PSP)--abnormal eye, walking, posture changes

  • Compusive behaviors--especially regarding eating

  • Cognitive deteriorization--especially concerning planning and/or organizing

These symptoms are focused on the frontal and temporal lobes of the brain. Because if this, as opposed to other dementia such as Alzheimer's, memory, spatial orientation, and perception often do not suffer from marked decreases in FTD.


Much like other dementia, FTD is an outcome of protein buildups in the brain. In this case, the proteins are the protein tau and the protein TDP-43. These are sometimes referred to as Pick's bodies, but it is not clear why they localize to the frontal and temporal lobes of the brain. These two areas of the brain, when afflicted with FTD, shrink noticeably over time.


Diagnosis

Frontotemporal Dementia (FTD) is a challenge to diagnose. not only are there some similarities to other dementia--well, let's face it, most dementia have properties that are shared with other forms--FTD is not as common as Alzheimer's Disease, Vascular Dementia, or Lewy Body Dementia. Because FTD can occur at a much earlier age, it often does not enter the realm of possibilities when an examination and diagnosis is accomplished. Although there is no direct evaluative tests for FTD, a battery of medical history, current tests and scans, and assessment of cognitive and neurological functions. If there is the possibility that FTD is a result of genetic variation, genetic testing may be included in the battery of diagnostic measures.


Recently-developed measure for diagnosis focus on behavioral FTD (bvFTD), of which at least three must be present to make a clinical diagnosis. These diagnostic markers include:

  • Disinhibition

  • Apatha/Inertia

  • Loss or lack of sympathy or empathy

  • Perseverative or compulsive behaviors

  • Hyperorality

  • Dysexecutive neuropsychological profile

Other diagnostic tools include the Iowa Gambling Task and the Faux Pas Recognition Test, both of which have sensitivity to changes in the orbitofrontal cortex--where the early degeneration occurs.


Treatment and Prognosis

Like the forms of dementia discussed previously in this series, Frontotemporal Dementia (FTD) is progressive--which means that the symptoms and their effects grow worse over time. Some of the symptoms--primarily those dealing with depression, disinhibition, and compulsive behaviors--may often be controlled with medications. Therapy may also help some patients as they gradually lose speech and motor functions; however, the biggest challenge to caregivers of patients with FTD is when they lose the ability to empathize or foster concern for others.


When FTD reaches a severe state, muscle weakness and lack of coordination are likely to land the patient in a wheelchair (or electric scooters--they are fun...right?). Associated severe symptoms include trouble swallowing (a Parkinson's symptom as well), chewing control (food falling out of mouth, drooling, for example), and bladder and/or bowel control problems. At this stage, death may occur as the result of a lung infection or urinary tract infection because of the weakened state of the patient. One may optimistically look at about 8-10 years as the average that someone lives with FTD after diagnosis...it is fatal.


Lists in this article adapted from Earlstein, F. (2016). Dementia Facts & Information. NRB Publishing: Nevada.


For more resources and references on Frontotemporal Dementia, see the following:

Frontotemporal Dementia News & Research: https://www.drcarlforkner.com/dementia-memory-news