Frontotemporal Dementia

Updated: April 18, 2024

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Background

Frontotemporal dementia is a neurodegenerative disorder characterized by progressive damage to the frontal and temporal lobes of the brain. These brain areas are linked to language, conduct, personality, and making decisions.   

FTD can sometimes be inherited, with specific genetic variations raising the chance of getting the illness.  

Abnormal brain accumulations of TDP-43 are detected in patients with frontotemporal dementia. The regular operation of the brain is disrupted by these protein clumps.  

Modifications in behavior, personality, and social conduct are characteristics of this subtype.  

Epidemiology

FTD though analytically considered can be a primary cause of dementia especially amongst the young people who are even though in minority to other dementia been on the rise. 
 
FTD commonly befalls the middle-age people between ages 40-65 while there are rare instances of cases reported at all ages. 
 
Attempting to estimate the exact prevalence rate of FTD can be affected by three different factors which include the diversified demographic and geographic region as well as the diagnosis standards. 
 
It can be expected that frontotemporal-type dementia is also seen with varying occurrence rates from different locations and nations and causes are also related to genetics or environment or culture for instance.  

Anatomy

Pathophysiology

When tau protein accumulates abnormally in neurons and insoluble clusters known as tauopathies develop in specific types of frontotemporal dementia. These tau aggregates interfere with cellular functions causing harm to neuronal death.  
The primary brain regions affected by FTD are the frontal and temporal lobes which are involved with language behaviour and personality and executive processes.  
Neuronal loss and malfunction occur in these locations, causing unique symptoms such executive failure or language difficulties and odd behaviour.  

Etiology

Numerous gene mutations have been linked to familial forms of FTD.  
These mutations disrupt the cell’s vital pathways and neural processes and protein metabolism.  
Through genome-wide association studies frequent genetic variants associated with an increased risk of sporadic FTD have also been discovered.  

Genetics

Prognostic Factors

The prognosis may vary depending on the subtypes of FTD. For example compared to those with the behavioural variation those with primary progressive aphasia may show a slower deterioration in some cognitive domains.
Age of onset and severity can also affect the prognosis. 

Clinical History

Age Group   

FTD is considered one of the primary causes of dementia in individuals under the age of 65, particularly in the subset of cases known as early-onset FTD.   

This term refers to cases where symptoms begin before the age of 65, often in midlife.  

Physical Examination

Neurological Examination 

Cranial Nerve Assessment 

General Physical Examination 

Head and Neck Examination 

Cardiovascular examination 

Age group

Associated comorbidity

Depression 

Anxiety disorders,  

Psychotic disorders 

Associated activity

Acuity of presentation

In numerous situations FTD symptoms including behavioural character or language impairments are evolving gradually which takes months and years to get deteriorated. 
FTD is a type of dementia that comes with an unnoticeable beginning that means several symptoms show-up but the brain is deteriorating progressively without dramatic changes. This sequential process can possibly be “killer” for families since the initial signals can hardly be identifiable in this period therefore it can result in delayed diagnoses. 
About FTD the disease usually begins in a gradual way, but differential symptoms suddenly appear.  

Differential Diagnoses

Alzheimer’s Disease 

Primary Progressive Aphasia Variants 

Behavioral and Psychiatric Disorders 

Vascular Dementia  

Lewy Body Dementia  

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

Behavioural and Environmental Interventions: These techniques can help control behavioural symptoms and lessen stress for the person with FTD. They include environmental adjustments routines that are set in and education for carers.  
Speech and Language treatment: Speech therapies and language rehabilitation treatments provide assistance in keeping talking skills intact and compensate language inabilities among people with aphasia and FTD variations. 
Occupational Therapy: The functional impairments in an individual’s activities of daily living can be addressed coupled with techniques to help maximize participation in activities of interests and occupational therapists can therefore help the affected person to maintain their independence. 

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

use-of-non-pharmacological-approach-for-frontotemporal-dementia

Minimize Environmental Stimuli: Minimize the clutter and noise and distractions to ensure that sensory overloads will be minimized which agitates and confuses needed people. 
Ensure Safety: Provide safety norms that are geared to preventing accidents and injuries. Set aside or make safe any little wrist-breakers chemical contaminants or trip-and-fall hazards.  
Optimize Lighting and Visibility:  Ensure proper lighting in all areas of the house to avoid accidents and to facilitate the prominence of the outlines and reduce the intensity of the confusion especially during the night time. 
Create Safe Zones: Assign defined space within the home for the area of safety where they can enjoy some of the activities and don’t face the risk of getting themselves to harm by mistake. 
Provide Meaningful Activities: Make areas for commitment and outlets that support cognitive ability as well as communication between people.  

Use of Antipsychotic agents

Risperidone: The drug is used for psychotic symptoms as it struggles with the prohibiting dopamine receptors in the mind resulting to a minimized hallucinations delusions and other imaging in the brain. 
 
Risperidone can suppress the activity of dopamine and serotonin receptors in the brain thereby it can bring back stability in mood and behaviour of the patients with FTD which helps to diminish aggressive behaviour or unpredictability and impulsiveness. 

use-of-intervention-with-a-procedure-in-treating-frontotemporal-dementia

Deep Brain Stimulation: DBS is certainty a nonstandard treatments against FTD however it has been studied as a therapeutic approach in order to relieve with some particular symptoms such as behavioural disturbances or motor manifestations that shows up in a process of shape. 
Electroconvulsive Therapy:  ECT is a medical practice that is usually used for the treatment of extreme depression; however, it has also been utilized as the procedure for the same symptoms that dementia exhibits such as FTD.  

use-of-phases-in-managing-frontotemporal-dementia

Early Detection and Diagnosis: This step starts with diagnosing the initial indicators of FTD that could be diversity of emotional and behavioural changes and problems with executive performance as well as speaking disturbances and endogenous dysfunction all the way up to cognitive impairment. 
 
Genetic Counselling and Testing: Nevertheless knowing about families with this type of an FTD may trigger genetic counselling and evaluation of the known mutated genes contribution to development of the disease; this will be vital in enabling you take the right decision regarding your family planning. 
 
Monitoring and Surveillance: People living with FTD being under personally monitored for finding symptoms and cognitive function and functional abilities, are tracking progress of disease and those management strategies are adopted which are changed according to the circumstances. 
Palliative Care: Supportive care principles are used in helping FTD as a way of easing symptoms maxing on comfort and quality of life of FTD people who are at the end stages of their illness. 

Medication

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Frontotemporal Dementia

Updated : April 18, 2024

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Frontotemporal dementia is a neurodegenerative disorder characterized by progressive damage to the frontal and temporal lobes of the brain. These brain areas are linked to language, conduct, personality, and making decisions.   

FTD can sometimes be inherited, with specific genetic variations raising the chance of getting the illness.  

Abnormal brain accumulations of TDP-43 are detected in patients with frontotemporal dementia. The regular operation of the brain is disrupted by these protein clumps.  

Modifications in behavior, personality, and social conduct are characteristics of this subtype.  

FTD though analytically considered can be a primary cause of dementia especially amongst the young people who are even though in minority to other dementia been on the rise. 
 
FTD commonly befalls the middle-age people between ages 40-65 while there are rare instances of cases reported at all ages. 
 
Attempting to estimate the exact prevalence rate of FTD can be affected by three different factors which include the diversified demographic and geographic region as well as the diagnosis standards. 
 
It can be expected that frontotemporal-type dementia is also seen with varying occurrence rates from different locations and nations and causes are also related to genetics or environment or culture for instance.  

When tau protein accumulates abnormally in neurons and insoluble clusters known as tauopathies develop in specific types of frontotemporal dementia. These tau aggregates interfere with cellular functions causing harm to neuronal death.  
The primary brain regions affected by FTD are the frontal and temporal lobes which are involved with language behaviour and personality and executive processes.  
Neuronal loss and malfunction occur in these locations, causing unique symptoms such executive failure or language difficulties and odd behaviour.  

Numerous gene mutations have been linked to familial forms of FTD.  
These mutations disrupt the cell’s vital pathways and neural processes and protein metabolism.  
Through genome-wide association studies frequent genetic variants associated with an increased risk of sporadic FTD have also been discovered.  

The prognosis may vary depending on the subtypes of FTD. For example compared to those with the behavioural variation those with primary progressive aphasia may show a slower deterioration in some cognitive domains.
Age of onset and severity can also affect the prognosis. 

Age Group   

FTD is considered one of the primary causes of dementia in individuals under the age of 65, particularly in the subset of cases known as early-onset FTD.   

This term refers to cases where symptoms begin before the age of 65, often in midlife.  

Neurological Examination 

Cranial Nerve Assessment 

General Physical Examination 

Head and Neck Examination 

Cardiovascular examination 

Depression 

Anxiety disorders,  

Psychotic disorders 

In numerous situations FTD symptoms including behavioural character or language impairments are evolving gradually which takes months and years to get deteriorated. 
FTD is a type of dementia that comes with an unnoticeable beginning that means several symptoms show-up but the brain is deteriorating progressively without dramatic changes. This sequential process can possibly be “killer” for families since the initial signals can hardly be identifiable in this period therefore it can result in delayed diagnoses. 
About FTD the disease usually begins in a gradual way, but differential symptoms suddenly appear.  

Alzheimer’s Disease 

Primary Progressive Aphasia Variants 

Behavioral and Psychiatric Disorders 

Vascular Dementia  

Lewy Body Dementia  

Behavioural and Environmental Interventions: These techniques can help control behavioural symptoms and lessen stress for the person with FTD. They include environmental adjustments routines that are set in and education for carers.  
Speech and Language treatment: Speech therapies and language rehabilitation treatments provide assistance in keeping talking skills intact and compensate language inabilities among people with aphasia and FTD variations. 
Occupational Therapy: The functional impairments in an individual’s activities of daily living can be addressed coupled with techniques to help maximize participation in activities of interests and occupational therapists can therefore help the affected person to maintain their independence. 

Psychiatry/Mental Health

Minimize Environmental Stimuli: Minimize the clutter and noise and distractions to ensure that sensory overloads will be minimized which agitates and confuses needed people. 
Ensure Safety: Provide safety norms that are geared to preventing accidents and injuries. Set aside or make safe any little wrist-breakers chemical contaminants or trip-and-fall hazards.  
Optimize Lighting and Visibility:  Ensure proper lighting in all areas of the house to avoid accidents and to facilitate the prominence of the outlines and reduce the intensity of the confusion especially during the night time. 
Create Safe Zones: Assign defined space within the home for the area of safety where they can enjoy some of the activities and don’t face the risk of getting themselves to harm by mistake. 
Provide Meaningful Activities: Make areas for commitment and outlets that support cognitive ability as well as communication between people.  

Neurology

Risperidone: The drug is used for psychotic symptoms as it struggles with the prohibiting dopamine receptors in the mind resulting to a minimized hallucinations delusions and other imaging in the brain. 
 
Risperidone can suppress the activity of dopamine and serotonin receptors in the brain thereby it can bring back stability in mood and behaviour of the patients with FTD which helps to diminish aggressive behaviour or unpredictability and impulsiveness. 

Neurology

Neurosurgery

Deep Brain Stimulation: DBS is certainty a nonstandard treatments against FTD however it has been studied as a therapeutic approach in order to relieve with some particular symptoms such as behavioural disturbances or motor manifestations that shows up in a process of shape. 
Electroconvulsive Therapy:  ECT is a medical practice that is usually used for the treatment of extreme depression; however, it has also been utilized as the procedure for the same symptoms that dementia exhibits such as FTD.  

Neurology

Psychiatry/Mental Health

Early Detection and Diagnosis: This step starts with diagnosing the initial indicators of FTD that could be diversity of emotional and behavioural changes and problems with executive performance as well as speaking disturbances and endogenous dysfunction all the way up to cognitive impairment. 
 
Genetic Counselling and Testing: Nevertheless knowing about families with this type of an FTD may trigger genetic counselling and evaluation of the known mutated genes contribution to development of the disease; this will be vital in enabling you take the right decision regarding your family planning. 
 
Monitoring and Surveillance: People living with FTD being under personally monitored for finding symptoms and cognitive function and functional abilities, are tracking progress of disease and those management strategies are adopted which are changed according to the circumstances. 
Palliative Care: Supportive care principles are used in helping FTD as a way of easing symptoms maxing on comfort and quality of life of FTD people who are at the end stages of their illness. 

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