TABLE 1.

Factors Suggestive of TDP-43 Proteinopathies in Patients With Late-onset NPS and Clinical Action Points

Red Flags for Possible TDP-43 Proteinopathy	Clinical Action Points
Late-onset hallucinations and/or delusions	Explore other indicators, cognitive testing, neuroimaging
Nonpsychotic late-onset NPS	Explore other indicators – investigate only if other factors present
Family history of FTD, ALS, or early-onset dementia	Collateral history from family +/− genetic testing and referral to genetic counselor if positive cases
bvFTD features, including hyperorality and stereotypies	Referral to specialty clinics, neuroimaging, neuropsychology, social cognition tests
Language disturbances	Neuropsychological +/− speech therapy assessment, neuroimaging
Cognitive deficits	Neuropsychological testing
Motor weakness	Neurological exam +/− EMG

Notes: ALS: amyotrophic lateral sclerosis; bvFTD: behavioral variant frontotemporal dementia; EMG: electromyography; FTD: frontotemporal dementia; NPS: neuropsychiatrie symptoms; TDP-43: transactive response DNA-binding protein 43.