Frontotemporal Dementia
Everything about the symptoms, progression, prognosis, and treatments for impairment of the frontal and temporal lobes of the brain.
Symptoms and causes
Frontotemporal dementia is a disease caused by progressive degeneration of the frontal and temporal lobes of the brain. This condition leads to deterioration of brain function, specifically affecting behavior, personality, decision-making, language, and emotional processing, as these functions are governed by this brain region.
This disease is often confused with Alzheimer’s, but there are two significant differences:
- Frontotemporal dementia begins with changes in personality and behavior, while Alzheimer’s initially manifests as memory loss or impaired reasoning.
- This type of dementia affects individuals between 45 and 60 years old, whereas Alzheimer’s typically occurs after age 65.
Frontotemporal dementia is a progressive disease with no current cure, resulting in a poor prognosis. The rate of progression varies depending on multiple factors, but life expectancy is estimated at approximately eight years from diagnosis.
Symptoms
The symptoms of frontotemporal dementia may be mistaken for Alzheimer’s, other types of dementia, or certain psychological disorders, especially schizophrenia or depression. They also tend to manifest differently from one person to another. The most common symptoms are:
- Behavioral and personality changes:
- Lack of empathy
- Loss of inhibition
- Socially inappropriate behavior
- Impaired decision-making
- Impulsive actions
- Apathy
- Neglect of personal hygiene
- Overeating or consumption of unhealthy foods
- Ingesting objects
- Repetitive behaviors
- Increased sexual interest
- Language impairments:
- Difficulty speaking
- Use of generic words to replace more specific ones
- Loss of semantic knowledge
- Language errors
- Aphasia: difficulty reading, writing, and expressing ideas
- Movement disorders:
- Balance problems
- Muscle weakness
- Tremors
- Muscle spasms
- Rigidity
- Lack of coordination
- Difficulty swallowing (dysphagia)
- Inappropriate laughter or crying
- Urinary incontinence
Symptoms worsen over time as the disease progresses. Generally, three stages are identified:
- Mild stage: difficulties performing complex tasks
- Moderate stage: difficulties performing simple tasks
- Severe stage: inability to complete simple tasks, loss of speech, and impaired walking
Causes
The exact causes of frontotemporal dementia are unknown, but it is believed that the frontal and temporal lobes shrink due to a genetic component involving mutations in the C9orf72, GRN, or MAPT genes. Between 10% and 30% of patients have abnormal or malformed TAU and TDP-43 proteins in brain nerve cells. When these proteins accumulate, neurons are damaged and die over the years.
Risk Factors
The only identified risk factor for frontotemporal dementia is having a family history of the disease.
Complications
The most common complications of frontotemporal dementia include:
- Respiratory infections due to dysphagia
- Urinary infections due to incontinence
- Impairment of other brain regions; for example, patients who initially show language difficulties may later develop behavioral problems, and vice versa
- Amyotrophic lateral sclerosis (ALS)
- Parkinson’s disease
Prevention
Frontotemporal dementia cannot be prevented.
Which Doctor Treats Frontotemporal Dementia?
Neurology specialists diagnose and treat frontotemporal dementia. Clinical psychology and Speech-language therapy also play a role in treatment.
Diagnosis
Various tests are conducted for diagnosis, some to rule out other diseases with similar symptoms:
- Medical history: evaluation of the patient’s personal and family medical history and assessment of presenting symptoms
- Blood Tests: provide information about the patient’s overall health
- Neuropsychological tests
- Brain Magnetic Resonance Imaging (MRIBrain Magnetic Resonance Imaging (MRI))Brain Magnetic Resonance Imaging (MRI)) ): shows brain structure, identifying changes in shape and size
- PET Scan of the Brain (PET-FDG): a positron emission tomography scan using a radiopharmaceutical to highlight areas with reduced metabolic activity; effective for ruling out Alzheimer’s and determining the type of dementia
Treatment
Currently, there is no treatment for frontotemporal dementia, and medications used for similar conditions are generally ineffective.
To alleviate symptoms and improve quality of life, antidepressants or antipsychotics may be administered. Cognitive stimulation therapy is recommended to slow functional decline. Speech therapy is also useful to help patients maintain language abilities for as long as possible.
