Frontotemporal Dementia

Frontotemporal Dementia or Pick’s Disease belongs to the rare dementias associated with focal brain atrophy in the frontal and temporal lobes.

It is a dementia that can occur even in people who are only 20 years old and, in most cases, gives symptoms at the age of 45 to 64 years.

Patients with the behavioral variant of frontotemporal degeneration are usually mistakenly diagnosed with psychiatric disorders or other neurological diseases since the onset of this condition is characterized by abnormalities in behavior or personality. Apathy and lack of interest, both early symptoms, can be misdiagnosed as signs of depression. The lifting of inhibitions can be misinterpreted as a manic episode, obsessive-compulsive disorder, or personality disorder.

While Alzheimer’s disease can affect many different areas of the brain, Pick’s disease causes damage only to specific areas of the frontal and temporal lobe. It manifests itself not so much with a decrease in memory, but also mainly with changes in behavior. Other less common variants may affect speech production and understanding.

What is the cause of frontotemporal dementia?

Frontotemporal dementia is due to an abnormal amount or function of specific nerve cell proteins called “t” (Tau). These proteins can normally be detected in all nerve cells. In Pick’s disease, there is usually an increased amount of these proteins or a pathological structure, resulting in them being agglomerated and creating large spherical masses. These masses are called Pick bodies and when they are formed in nerve cells, they cause their death. This phenomenon is observed mainly in the areas of the temporal and frontal cerebral lobe and causes shrinkage of brain tissue and symptoms of dementia.
The exact causes of the disease have not been identified but there is some hereditary predisposition.

This condition is not reversible.

Frontotemporal Dementia Symptoms

Patients face problems in language, communication, memory, judgment, logical thinking as well as disturbances in emotional reaction and behavior, sometimes completely changing their personality and making them antisocial.

Usual symptoms are:

The diagnosis is made based on clinical and neurological / neuropsychological examination findings and blood tests, which measure the levels of thyroid hormones and vitamin B12, as lack of these can cause symptoms of dementia.
Imaging tests such as Brain MRI are performed.

In addition, the doctor can investigate the possibility of other diseases or health problems that coexist and may aggravate the patient’s symptoms such as depression, anemia, headaches, thyroid disorders, decreased oxygen levels, eating disorders, insufficiency of kidney function, liver, heart. As of now there is no etiological therapy. There are only drugs that treat individual causes and symptoms.

The rate of progression of symptoms and the duration of the disease vary greatly from person to person. In general, symptoms become more pronounced over time and cause disability over a course of 8-10 years.

Picture of Christos Sidiropoulos, MD, PhD, FAAN

Christos Sidiropoulos, MD, PhD, FAAN

Associate Professor of Neurology, Michigan State University, USA
Specialist in Parkinson’s Disease, Movement Disorders & Dementias.
16 years of training and specialization in the USA, Germany & Canada.
Specialist in the use of Botulinum Toxin for neurological disorders.
Tremendous experience in the assessment for patient candidacy for Deep Brain Stimulation (DBS) for Movement Disorders and patient follow up.

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