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Frontotemporal dementia
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Pathogenesis:
TDP-43 mutation
Tau protein inclusions (eg, Pick bodies)
Signs / symptoms:
Early onset (age 50s-60s)
Initial behavior or personality changes (3 of 6 required)
Disinhibition
Apathy
Loss of empathy
Compulsions or rituals
Hyperorality
Executive dysfunction
20%: motor neuron disease (upper: hyperreflexia; lower: fasciculations)
Diagnosis:
Primarily clinical
Neuroimaging (CT, MRI)
Rule out other disorders
Look for frontal/anterior temporal lobe atrophy
Treatment / management:
Nonpharm: behavior modification
Pharmacologic (symptomatic): SSRI, atypical antipsychotic agent
TDP-43 mutation
Tau protein inclusions (eg, Pick bodies)
Signs / symptoms:
Early onset (age 50s-60s)
Initial behavior or personality changes (3 of 6 required)
Disinhibition
Apathy
Loss of empathy
Compulsions or rituals
Hyperorality
Executive dysfunction
20%: motor neuron disease (upper: hyperreflexia; lower: fasciculations)
Diagnosis:
Primarily clinical
Neuroimaging (CT, MRI)
Rule out other disorders
Look for frontal/anterior temporal lobe atrophy
Treatment / management:
Nonpharm: behavior modification
Pharmacologic (symptomatic): SSRI, atypical antipsychotic agent
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