II. Pathophysiology
IV. Clinical Findings
- Findings are specific to the Frontotemporal Dementia type
- All types are of insidious onset and gradual progression
- Progressive changes in personality and behavior
- Apathy (e.g. sits and watches television constantly)
- Disinhibition (e.g. tells jokes in bad taste)
- Agitation
- Judgement, reasoning, problem solving difficulty
- Memory may be maintained
- Progressive language Impairment
V. Types
- Behavioral Variant Frontotemporal Dementia
- Early decline socially, interpersonally, as well as in conduct, emotional range and insight
- May be associated with distractibility, repetitive behaviors (e.g. Echolalia), and decreased hygiene
- Semantic Dementia
- Effortless speach that lacks meaning
- Uses broad terms instead of detailed specific words
- Failure to recognize familiar faces such as famous people
- Progressive Nonfluent Aphasia
- Nonfluent Aphasia, Stuttering and poor grammar despite significant effort
- Difficult word finding and anomia
- Decreased comprehension
VI. Lab: Pathology
- Pick bodies (when present FTD is known as Pick Disease)
- Ubiquitin-positive inclusions (55% of Frontotemporal Dementia)
- Associated with TAR DNA-binding Protein (TDP-43)
- Associated with progranulin gene mutations
- Associated with Behavioral Variant Frontotemporal Dementia
- Associated with Semantic Dementia
- Tau-positive inclusions (45% of Frontotemporal Dementia)
- Associated with tau gene mutations
- Associated with Behavioral Variant Frontotemporal Dementia
- Associated with Progressive Nonfluent Aphasia
VII. Management
- Behavioral symptoms
- Consider Selective Serotonin Reuptake Inhibitors (SSRI)
- Aggressive behaviors
- Consider low dose Antipsychotics
- Progressive Nonfluent Aphasia
-
General Dementia progression
- NMDA Receptor Antagonists (experimental)
- Acetylcholinesterase Inhibitors (experimental)
VIII. Prognosis
IX. Resources
- Association for Frontotemporal Dementia