Cognitive

Frontotemporal Dementia

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Frontotemporal Dementia, Pick Disease, Behavioral Variant Frontotemporal Dementia, Semantic Dementia, Progressive Nonfluent Aphasia

  • See Also
  1. Dementia
  • Pathophysiology
  1. Prototype: Pick's Disease
  2. Tau accumulation in Neurons and glia
  3. Results in atrophy in Amygdala and frontal cortex
  • Epidemiology
  1. Accounts for 20-50% of Dementia in age <65 years
  2. Incidence: 15 per 100,000
  • 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
  • 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
  • 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
  • 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)
  • 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
  • Resources
  1. Association for Frontotemporal Dementia
    1. http://www.ftd-picks.org
  • References
  1. Cardarelli (2010) Am Fam Physician 82(11): 1372-7 [PubMed]
  2. Josephs (2008) Ann Neurol 64(1): 4-14 [PubMed]
  3. Wittenberg (2008) Neuropsychol Rev 18(1): 91-102 [PubMed]