Frontotemporal Dementia

Original Editor - Kirenga Bamurange Liliane Top Contributors - Kirenga Bamurange Liliane

Description[edit | edit source]

Frontotemporal dementia (FTD) is a group of degenerative diseases that gradually damage or shrink the brain’s frontal and anterior temporal lobes, located in the front of the brain. FTDs include frontotemporal lobar degeneration, Pick's Disease, progressive aphasia and semantic dementia [1]. It represents a group of brain disorders caused by degeneration of the frontal and/or temporal lobes of the brain. It is also frequently referred to as Frontotemporal Dementia, Frontotemporal Lobar Degeneration (FTLD), or Pick’s Disease [2].

The term “Pick’s Disease” (PiD) was named after Arnold Pick for his initial description of focal gross atrophy of the frontotemporal lobes in a patient with progressive language and behavioral disturbances [3]. Pick’s Disease is a rare neurodegenerative disorder, estimated to be 10 times less frequent than Alzheimer’s disease, even in regions with high prevalence. It was first clinically described by Pick in 1892, in a woman with lobar brain atrophy who suffered from pre-senile dementia with dysphasia [4]. Current knowledge indicates that PiD is a member of a group of heterogeneous neurodegenerative disorders with similar or related histopathologic and clinical features [5].

Frontotemporal Dementia age of onset can be as early as the age of 40, with 54 being the average age of onset, and is often misdiagnosed in younger adults as a psychiatric issue and in older adults as Alzheimer’s. Older adults can start to see symptoms all the way into their 80s [6] .

Pick's disease.png

Causes[edit | edit source]

Pick’s Disease, along with other FTDs, is caused by abnormal amounts or types of nerve cell proteins, called tau. These proteins are found in all of our nerve cells. They are usually stored in Pick bodies or Pick cells causing the brain tissue to shrink and leading to dementia [7].

Symptoms[edit | edit source]

FTD is a progressive dementia charaterized by progressive changes leading to impairment of intellect, memory and language [7]. The following symptoms may be experienced:

1. Behavorial and emotional symptoms such as: [7]

  • Abrupt mood changes
  • Compulsive or inappropriate behavior
  • Depression-like symptoms like disinterest in activities of daily life
  • Withdrawal from social interaction
  • Poor social skills
  • Difficulty in keeping a job
  • Poor social
  • Poor personal hygiene
  • Repetitive behavior

2. Language changes such as: [8]

  • Hesitant speech
  • Difficulty articulating
  • Stuttering
  • Ungrammatical speech
  • Difficulty recalling words or names

3. Neurological changes such as: [7]

  • Accelerated memory loss
  • Physical weakness

Diagnosis[edit | edit source]

Many different tools are used to diagnose FTD. The medical history of the patient as well as special imaging tests and lab tests are some of the tools to help make a diagnosis. The diagnosis is made on a clinical basis, although genetic testing can confirm some specific subtypes [9].

  1. Medical history [7]
  • The patient is asked to complete speech and writing tests.
  • Physical and neurological examination is done. Interviews with family members are conducted to learn more about his behavior.
  • Neuropsychological testing to pinpoint and document a person’s cognitive problems and strengths.

2. Special Imaging Test [10]

  • Brain scans examine the brain to detect atrophy and other abnormalities in the frontal and temporal lobes. They can also rule out other conditions such as brain tumors or stroke.
  • Magnetic Resonance Imaging (MRI) to show atrophy of the frontal and temporal lobes; Computer Tomography (CT Scan), Single-photon emission computerized tomography (SPECT) and Positron Emission Tomography (PET) scans are the recommended ones.
  • Electroencephalography (EEG) and Spinal tap (lumbar puncture) can be useful in some cases to rule out other conditions that can cause dementia.

3. Other Tests [7] [10]

1. Possible causes of Dementia in older adults such as Hypothyroidism, Vitamin B-12 deficiency and Syphilis, Kidney or Liver problems

2. Infections

3. Blood disorders

4. Hormonal disorders

5. Chemical abnormalities

Treatment[edit | edit source]

As for Alzheimer's Disease, there is no cure for FTD. The treatment aims to relieve and/or improve symptoms. There are no specific medications for FTDs. they are symptom-oriented rather than disease-modifying or curative [9].

1. Non Drug Treatment [10]

  • Interventions for helping or adjusting behaviors.
  • Speech Therapy to improve communication abilities.

2. Drug Treatment [7][10]

  • Antidepressant and antipsychotic medications for emotional and behavioral changes.
  • Cholinesterase inhibitors for memory and attention.
  • Antioxidant tocopherol (vitamin E) to counteract the damage in brain cells that causes PiD/ FTD and slow the worsening of the disease.
  • Anti-inflammatory drugs and hormone replacement therapy are also being used by some specialists who treat PiD/ FTD as experimental treatments and are therefore not widely accepted.

Life with FTD - Home Care Program[edit | edit source]

It is recommended for People with FTD to remain physically, mentally and socially active as long as they can. In order to this, they need to:

  • Daily physical activity such as walking for at least 20 minutes.
  • Mental activity and stimulation such as puzzles, games, reading to slow the progression of the disease.
  • Social interaction is recommended as a stimulating and enjoyable activity.
  • Have balanced diet to maintain a healthy weight and prevent malnutrition and constipation.
  • Smoking is prohibited for health and safety reasons.
















References[edit | edit source]

  1. Frontotemporal Dementia. Available from: https://www.ucsfhealth.org/conditions/frontotemporal-dementia (Accessed, 09/08/2021).
  2. Fast Facts about FTD. Available from: https://www.theaftd.org/wp-content/uploads/2018/03/Fast-Facts-about-FTD-for-web.pdf (Accessed, 09/08/2021).
  3. Irwin DJ, Brettschneider J, McMillan CT, Cooper F, Olm C, Arnold SE, Van Deerlin VM, Seeley WW, Miller BL, Lee EB, Lee VM. Deep clinical and neuropathological phenotyping of Pick disease. Annals of neurology. 2016 Feb;79(2):272-87.
  4. Dickson DW. Pick's disease: a modern approach. Brain pathology. 1998 Apr;8(2):339-54.
  5. Hu W, Luo JJ. Research Advances in Pick’s Disease: A New Biomarker Candidate. J Neurol Neurophysiol. 2013;4:e112.
  6. Stages of Frontotemporal Dementia: Symptoms, Age of Onset, Risk Factors, Life Expectancy. Available from://www.griswoldhomecare.com/blog/2018/june/stages-of-frontotemporal-dementia-symptoms-age-o/ ( Accessed, 09/08/2021).
  7. 7.0 7.1 7.2 7.3 7.4 7.5 7.6 Pick Disease of the Brain: Causes, Symptoms, and Diagnosis. Available from: https://www.healthline.com/health/picks-disease#symptoms (Accessed, 01/08/2021).
  8. What is Pick’s Disease? Available from: https://www.alzheimers.net/what-is-picks-disease (Accessed, 04/08/2021).
  9. 9.0 9.1 What is Pick’s Disease? Available from: https://www.brightfocus.org/alzheimers-disease/article/what-picks-disease (Accessed, 01/08/2021).
  10. 10.0 10.1 10.2 10.3 Pick Disease. Available from: https://www.emedicinehealth.com/pick_disease/article_em.htm (Accessed, 05/08/2021).
  11. The Audiopedia. What is Pick’s DISEASE? What does PICK’S DISEASE mean? PICK’S DISEASE meaning & explanation. Available from: https://www.youtube.com/watch?v=ILn2LNzevdc [Accessed, 06/08/2021]
  12. Brain and Beyond. What is Frontotemporal Dementia and Pick's Disease? Available from: https://www.youtube.com/watch?v=fbLeQXi4RW8[Accessed, 06/08/2021]