II. Pathophysiology

  1. Prototype: Pick's Disease
  2. Tau accumulation in Neurons and glia
  3. Results in atrophy in Amygdala and frontal cortex

III. Epidemiology

  1. Accounts for 20-50% of Dementia in age <65 years
  2. Incidence: 15 per 100,000

IV. Clinical Findings

  1. Findings are specific to the Frontotemporal Dementia type
  2. All types are of insidious onset and gradual progression
  3. Progressive changes in personality and behavior
    1. Apathy (e.g. sits and watches television constantly)
    2. Disinhibition (e.g. tells jokes in bad taste)
    3. Agitation
  4. Judgement, reasoning, problem solving difficulty
  5. Memory may be maintained
  6. Progressive language Impairment

V. Types

  1. Behavioral Variant Frontotemporal Dementia
    1. Early decline socially, interpersonally, as well as in conduct, emotional range and insight
    2. May be associated with distractibility, repetitive behaviors (e.g. Echolalia), and decreased hygiene
  2. Semantic Dementia
    1. Effortless speach that lacks meaning
    2. Uses broad terms instead of detailed specific words
    3. Failure to recognize familiar faces such as famous people
  3. Progressive Nonfluent Aphasia
    1. Nonfluent Aphasia, Stuttering and poor grammar despite significant effort
    2. Difficult word finding and anomia
    3. Decreased comprehension

VI. Lab: Pathology

  1. Pick bodies (when present FTD is known as Pick Disease)
  2. Ubiquitin-positive inclusions (55% of Frontotemporal Dementia)
    1. Associated with TAR DNA-binding Protein (TDP-43)
    2. Associated with progranulin gene mutations
    3. Associated with Behavioral Variant Frontotemporal Dementia
    4. Associated with Semantic Dementia
  3. Tau-positive inclusions (45% of Frontotemporal Dementia)
    1. Associated with tau gene mutations
    2. Associated with Behavioral Variant Frontotemporal Dementia
    3. Associated with Progressive Nonfluent Aphasia

VII. Management

  1. Behavioral symptoms
    1. Consider Selective Serotonin Reuptake Inhibitors (SSRI)
  2. Aggressive behaviors
    1. Consider low dose Antipsychotics
  3. Progressive Nonfluent Aphasia
    1. Galantamine (Razadyne)
  4. General Dementia progression
    1. NMDA Receptor Antagonists (experimental)
    2. Acetylcholinesterase Inhibitors (experimental)

VIII. Prognosis

  1. Median survival: 8.7 years
    1. Contrast with alzheimer's Dementia where survival is 11.8 years
    2. Semantic Dementia has a median survival closer to alzheimer's Dementia

IX. Resources

  1. Association for Frontotemporal Dementia
    1. http://www.ftd-picks.org

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Related Studies

Ontology: Frontotemporal dementia (C0338451)

Definition (MSH) The most common clinical form of FRONTOTEMPORAL LOBAR DEGENERATION, this dementia presents with personality and behavioral changes often associated with disinhibition, apathy, and lack of insight.
Definition (NCI) A syndrome caused by progressive degeneration of the frontal or temporal lobes of the brain. It is manifested with personality changes and deterioration of the language skills.
Concepts Disease or Syndrome (T047)
MSH D057180
ICD9 331.1
ICD10 G31.0
SnomedCT 230270009
English MSTD, FRONTOTEMPORAL DEMENTIA, PALLIDOPONTONIGRAL DEGENERATION, DEMENTIA, FRONTOTEMPORAL, WITH PARKINSONISM, FLDEM, MULTIPLE SYSTEM TAUOPATHY WITH PRESENILE DEMENTIA, FRONTOTEMPORAL LOBE DEMENTIA, WILHELMSEN-LYNCH DISEASE, WLD, DISINHIBITION-DEMENTIA-PARKINSONISM-AMYOTROPHY COMPLEX, FTDP17, PPND, FRONTOTEMPORAL DEMENTIA WITH PARKINSONISM, FTD, frontotemporal dementia (diagnosis), frontotemporal dementia, Dementia, Frontotemporal Lobe (FLDEM), Dementias, Frontotemporal Lobe (FLDEM), Frontotemporal Lobe Dementias (FLDEM), Frontotemporal Dementia, Frontotemporal Dementias, Dementias, Frontotemporal, Frontotemporal Lobe Dementia (FLDEM), Frontotemporal Dementia [Disease/Finding], dementia frontotemporal, FTLD WITH TAU INCLUSIONS, FRONTOTEMPORAL LOBAR DEGENERATION WITH TAU INCLUSIONS, Wilhelmsen-Lynch Disease, Dementia, Frontotemporal, Frontotemporal Lobe Dementia, Frontotemporal Dementia with Parkinsonism, Familial Pick Disease, Ubiquitin-Positive Frontotemporal Dementias, FTDP-17, Frontotemporal Dementia, GRN-Related, Lobe Dementia, Frontotemporal, FTD-GRN, FTD-PGRN, DDPAC, Disease, Familial Pick's, Pick's Disease, Familial, Dementias, Frontotemporal Lobe, Dementias, GRN-Related Frontotemporal, Complex, Disinhibition-Dementia-Parkinsonism-Amyotrophy, Disinhibition Dementia Parkinsonism Amytrophy Complex, Dementia, GRN-Related Frontotemporal, Dementia, Frontotemporal Lobe, Disinhibition-Dementia-Parkinsonism-Amytrophy Complex, FTLD with TDP-43 Pathology, FTLD-TDP, Familial Picks Disease, Pick's Diseases, Familial, Frontotemporal Dementia, Ubiquitin-Positive, Familial Pick's Disease, Disinhibition-Dementia-Parkinsonism-Amytrophy Complices, FTLD-17 GRN, Dementia, Hereditary Dysphasic Disinhibition, Dementias, Ubiquitin-Positive Frontotemporal, Disinhibition-Dementia-Parkinsonism-Amyotrophy Complices, HDDD1, Dementia, Frontotemporal, with Parkinsonism, Disinhibition-Dementia-Parkinsonism-Amyotrophy Complex, Frontotemporal Dementia, Ubiquitin Positive, Complex, Disinhibition-Dementia-Parkinsonism-Amytrophy, Frontotemporal Lobar Degeneration With Ubiquitin Positive Inclusions, Multiple System Tauopathy with Presenile Dementia, Hereditary Dysphasic Disinhibition Dementia, Diseases, Familial Pick's, Frontotemporal Dementias, Ubiquitin-Positive, Frontotemporal Lobar Degeneration With Ubiquitin-Positive Inclusions, Frontotemporal Lobe Dementias, Lobe Dementias, Frontotemporal, Diseases, Wilhelmsen-Lynch, GRN-Related Frontotemporal Dementias, Complices, Disinhibition-Dementia-Parkinsonism-Amyotrophy, Familial Pick's Diseases, Frontotemporal Dementia with Parkinsonism-17, Disease, Wilhelmsen-Lynch, Frontotemporal Dementias, GRN-Related, GRN Related Frontotemporal Dementia, Dementia, Ubiquitin-Positive Frontotemporal, FTLD with TDP 43 Pathology, GRN-Related Frontotemporal Dementia, HDDD2, Disinhibition Dementia Parkinsonism Amyotrophy Complex, Complices, Disinhibition-Dementia-Parkinsonism-Amytrophy, Frontotemporal Dementia with Parkinsonism 17, Ubiquitin-Positive Frontotemporal Dementia, Wilhelmsen Lynch Disease, Wilhelmsen-Lynch Diseases, Frontotemporal dementia, Frontotemporal dementia (disorder)
Spanish Demencia frontotemporal, Demencia Frontotemporal, demencia frontotemporal (trastorno), demencia frontotemporal
Portuguese Demência fronto-temporal, Demência Frontotemporal
Dutch frontotemporale dementie
French Démence frontotemporelle, Démence fronto-temporale, Démence frontotemporale, DFT (Démence FrontoTemporale), DFTP-17, Démence frontotemporale avec parkinsonisme liée au chromosome 17
German frontotemporale Demenz, FTD, Frontotemporale Demenz
Czech Frontotemporální demence, frontotemporální demence
Japanese ゼントウソクトウガタニンチショウ, 認知症-前頭側頭型, 前頭側頭型認知症, 痴呆-前頭側頭型
Italian Demenza frontotemporale
Swedish Frontotemporal demens
Polish Demencja czołowo-skroniowa, Otępienie czołowo-skroniowe
Hungarian Frontotemporalis dementia
Norwegian Demens, frontotemporal, Frontotemporal demens