FRONTOTEMPORAL (FTD) DEMENTIA

By Lynne Ploetz, R.N., C., CCMPresented November 14, 2001

Definition of FTD Dementia A degenerative condition of the anterior brain

Definition of FTD Dementia A degenerative condition of the anterior brain Different from Alzheimer's, Pick's and

Creutzfeldt Jakob's diseases

Definition of FTD Dementia A degenerative condition of anterior brain Different from Alzheimer's, Pick's and

Creutzfeldt Jakob's diseases Areas affected by FTD—the frontal &

anterior temporal lobes—control reasoning, speech, personality, movement, social graces, language and some aspects of memory.

Definition of FTD Dementia A degenerative condition of anterior brain Different from Alzheimer's, Pick's and

Creutzfeldt Jakob's diseases Areas affected by FTD—the frontal &

anterior temporal lobes—control reasoning, speech, personality, movement, social graces, language and some aspects of memory.

Significant impairment in “theory of mind.”

Facts about FTD FTD may account for 2-5% or 140,000 - 350,000

dementia cases & 25% of pre-senile dementias

Facts about FTD

FTD may account for 2-5% or 140,000 - 350,000 dementia cases & 25% of pre-senile dementias

FTD occurs after age 40 and usually before age 65, with equal incidence in men and women.

Facts about FTD FTD may account for 2-5% or 140,000 - 350,000

dementia cases & 25% of pre-senile dementias FTD occurs after age 40 and usually before age

65, with equal incidence in men and women. In half of patients, family hx of dementia exists in

a first degree relative (parent or sibling).

Facts about FTD FTD may account for 2-5% or 140,000 - 350,000

dementia cases & 25% of pre-senile dementias FTD occurs after age 40 and usually before age

65, with equal incidence in men and women. In almost half of FTD patients, family hx of

dementia exists in first degree relative suggesting genetic component.

A form of dementia found in persons with ALS may be associated with FTD.

Major symptoms of FTDEarly Stage

Dramatic change in personal & social conduct with lack of initiative, neglect of domestic, financial and occupational responsibilities.

Major symptoms of FTDEarly Stage

Dramatic change in personal & social conduct with lack of initiative, neglect of domestic, financial and occupational responsibilities.

Loss of empathy toward others (impaired “theory of mind.”)

Major symptoms of FTDEarly Stage

Dramatic change in personal & social conduct with lack of initiative, neglect of domestic, financial and occupational responsibilities.

Loss of empathy toward others (impaired “theory of mind.”)

Shallow affect or inappropriate jocular behavior, sing, dance, clap or recite phrases repeatedly.

Major symptoms of FTDEarly Stage

Dramatic change in personal & social conduct with lack of initiative, neglect of domestic, financial and occupational responsibilities.

Loss of empathy toward others (impaired “theory of mind.”)

Shallow affect or inappropriate jocular behavior, sing, dance, clap or recite phrases repeatedly.

Rigid, inflexible thinking and impaired judgment.

Major symptoms of FTDEarly Stage

Dramatic change in personal & social conduct with lack of initiative, neglect of domestic, financial and occupational responsibilities.

Loss of empathy toward others (impaired “theory of mind.”)

Shallow affect or inappropriate jocular behavior, sing, dance, clap or recite phrases repeatedly.

Rigid, inflexible thinking and impaired judgment. Disinhibition, loss of insight into personal and

social misconduct, sexual/moral transgressions.

Major symptoms of FTDEarly Stage

Repetitive or compulsive behavior

Major symptoms of FTDEarly Stage

Repetitive or compulsive behavior Hypochondriasis, bizarre somatic complaints.

Major symptoms of FTDEarly Stage

Repetitive or compulsive behavior Hypochondriasis, bizarre somatic complaints. Excessive eating, gluttony, food fads (especially

craving for sweets) excessive alcohol intake.

Major symptoms of FTDEarly Stage

Repetitive or compulsive behavior Hypochondriasis, bizarre somatic complaints. Excessive eating, gluttony, food fads (especially

craving for sweets) excessive alcohol intake. Refusal to eat due to "negativism" or inability to

use motor skills needed for eating.

Major symptoms of FTDEarly Stage

Repetitive or compulsive behavior Hypochondriasis, bizarre somatic complaints. Excessive eating, gluttony, food fads (especially

craving for sweets) excessive alcohol intake. Refusal to eat due to "negativism" or inability to

use motor skills needed for eating. Change in sleep patterns, with prolonged

sleepiness, especially in those with apathetic behaviors.

Major symptoms of FTDMiddle Stage

Some individuals are overactive, restless, distractible and disinhibited.

Major symptoms of FTDMiddle Stage

Some individuals are overactive, restless, distractible and disinhibited.

Others are apathetic, inert, aspontaneous and emotionally blunted

Major symptoms of FTDMiddle Stage

Some individuals are overactive, restless, distractible and disinhibited.

Others are apathetic, inert, aspontaneous and emotionally blunted

These differences in outward activity disappear in the late stages of the disease.

Major symptoms of FTDLate Stage

A gradual reduction in speech, culminating in mutism.

Major symptoms of FTDLate Stage

A gradual reduction in speech, culminating in mutism.

Hyperoral traits.

Major symptoms of FTDLate Stage

A gradual reduction in speech, culminating in mutism.

Hyperoral traits. Failure or inability to make motor responses to

verbal commands.

Major symptoms of FTDLate Stage

A gradual reduction in speech, culminating in mutism.

Hyperoral traits. Failure or inability to make motor

responses to verbal commands. Akinesia (loss of muscle movement) and

rigidity with death due to complications of immobility.

Differences Between FTD and Alzheimer’s Disease

Differences in tissue pathology Differences in memory loss Differences in orientation Differences in visual-spatial skills Differences in intellectual abilities Differences in life expectancy Differences in genetics

Testing and Diagnosis Imaging and physical testing

CT and MRI Functional brain imaging and single

PET EEG Autopsy

TESTING AND DIAGNOSISNeuropsychological testing

Responses may be impulsive and tasks readily abandoned, while other patients may be slow, inert and persistent.

• May show poor mental effort and unconcern. Conversation not spontaneous; responses are brief

without elaboration, May make mechanical, repetitive remarks, echo

words spoken by others, or repeat responses. May have a weak voice and/or an odd or halting

speaking pattern. Overactive patients may be opposite, with.

unconstrained speech.

TESTING AND DIAGNOSISNeuropsychological testing

Visual-spatial skills remain normal except for those compromised by behavioral abnormalities.

Memory problems do not occur except as a result of ineffective use of memory.

Thought processes show impaired powers of abstraction, verbal response and design fluency.

Failure to respond or inappropriate responses not incomprehension, but concreteness of thinking or inattention.

Duration of FTD

Some patients decline rapidly over two to three years

Others show only minimal changes over a decade.

Studies have shown persons with FTD to live with the disease an average of 8 years, with a range from 3 to 17 years.

Management and Treatment of FTD

No medications are known currently to treat or prevent FTD

Behavior modification o       Review driving competence o       Monitor for eating non-food items o       Caregiver education and

counseling

Management and Treatment of FTD

Environmental adjustmento       Institutional care needed earlier

than in Alzheimer’so       Structure area for pacing/ wanderingo       Secure exitso       Medical alert bracelet with

name/address/phone.

Management and Treatment of FTD

Pharmacological treatment of major types of frontal lobe pathologyo  Irritable/disinhibited typeo  Aggressive/psychotic typeo  Apathetic/unmotivated type

      o  Depression/emotional incontinenceo  Compulsive behaviorso  Sexual disinhibition

EFC and relationship to FTD Frontal brain areas is seat of EFC. 30% of brain weight is responsible for

EFC. Not fully developed in children. Responsible for complex, goal-directed

actions. Responsible for initiation, execution and

monitoring of complex behaviors

Executive discontrol

Disorganized thoughts, behaviors and/or emotions.

Results in problem behaviors. Caused by many factors Is part of BPSD (behavioral and

psychological symptoms of dementia)

Treatment of Executive Discontrol

Psychosis and aggressiono   Rule out

    Medical disorders     Medication side-effects     

Pre-existing psychiatric illnessbefore treating symptoms