The primary objective of this clinical study is to provide the initial validation for monitoring biomarkers of symptoms and functioning for individuals with FTLD syndromes.

Researchers at BioSensics and Johns Hopkins School of Medicine will use wearable sensors, computerized speech, psychomotor, and cognitive assessments to create outcome measures and digital biomarkers for FTLD syndromes. Researchers will deploy this digital health solution to monitor 20 patients with FTLD syndromes for 12 months with study visits every 3 months.

Frontotemporal degeneration (FTD) spans the spectrum of rare neurodegenerative disorders affecting movement, behavior, and cognitive function. FTD represents a group of disorders including progressive supranuclear palsy (PSP - a severe and rapidly progressive FTD disorder estimated to affect at least 20,000 Americans), frontotemporal dementia (FTD, the second most common cause of early-onset (<65) dementia), primary progressive aphasia, semantic dementia, and corticobasal syndrome.

The primary objective of this clinical study is to provide the initial validation for monitoring biomarkers of symptoms and functioning for individuals with FTLD syndromes.

Twenty patients with FTLD syndromes will be recruited from Johns Hopkins University School of medicine to participate in this 12-month study with visits every 3 months. The investigators aim to assess the correlation between outcomes as measured by the PAMSys pendant and PAMSys ULM wrist monitoring sensors, and clinical/functional assessments used for FTLD syndromes (bvFTD, nfPPA, SD, and CBS), including the FTD Rating Scale (FTDRS), Cortical Basal ganglia Functional Scale (CBFS), PSP Rating Scale (PSPRS), Frontal Assessment Battery (FAB) (lexical fluency and Go-No-Go tasks), FTLD-CDR, modified Interpersonal Reactivity Index (mIRI Perspective Taking and Empathic Concern subscales summed to obtain a total Empathy score), the Pyramids and Palm Trees test (PPT), the Bedside Western Aphasia Battery-Revised (BWAB-R), digit span, phonemic fluency, category fluency, color trails, and trails A and B in individuals with different stages of FTLD disease severity. The results will be used to develop and validate monitoring symptoms and function in FTLD syndromes. In an exploratory aim, the caregiver burden (Zarit Burden Inventory) and its relationship to patients' empathy levels (mIRI) will be assessed.

The secondary objective of this study is to conduct free-flowing interviews with participants and clinical experts using the Technology Acceptance Model (TAM). This approach will help us examine perceptions of benefit, technology acceptance, technological anxiety, trust, facilitating conditions, perceived risk, and attitudes towards use from the perspectives of both patients and clinicians.

Inclusion Criteria:

• Male and female participants aged 40 years or older with a clinical diagnosis of possible or probable FTLD syndrome phenotype.
• Participants must be fluent in reading and speaking English and must be capable of providing informed consent based on the principal investigator's judgment.
• Individuals eligible for inclusion must be able to comply with the protocol per the investigator's judgment and must have a caregiver or study partner who is willing and able to assist with all study-related procedures.

Exclusion Criteria:

• Any neurological, medical, or psychiatric condition that would preclude or confound participation in study activities based on the investigator's judgment.
• Individuals who have a history of frequent falls defined as more than 5 falls per month.

The study intends to enroll a diverse population of individuals representative of the general FTLD syndrome population. There are no restrictions based on race or ethnic origin. This study will enroll both male and female participants aged 40 years or older with a clinical diagnosis of possible or probable FTLD syndrome phenotype.