Teleneurology Assessment Program for Creutzfeldt-Jakob Disease

NCT07840547 | Recruiting

• 1 other identifier: STUDY20211632
• Study Type: observational
• Target: 200
• Locations: 1 country
• Sites: 1

Brief Summary

This observational study uses secure videoconference visits to follow people with Creuztzfeldt-Jakob disease (CJD) over time. The study collects information about participants' symptoms, neurological function, thinking and memory, and ability to perform daily activities. It also collects information from study partners about the participant and about their own well-being and experiences as caregivers. The goal of the study is to better understand how CJD changes over time and to evaluate the use of remote assessments for CJD research and surveillance.

Conditions

Creutzfeldt-Jakob Disease (CJD) | Prion Disease

Keywords

CJD; Creutzfeldt-Jakob Disease; Prion Disease; Teleneurology; Telemedicine; Natural History; Caregiver Burden; CJD Surveillance

Outcome Measures

Primary Outcomes (1)

• Number of Participants who complete a Teleneurology Assessment

  The number of participants who complete at least on standardized teleneurology assessment by videoconference will be recorded

  Baseline through end of participant follow-up, up to 10 years.

Study Arms (1)

Prion Disease

Adults with definite or probable prion disease, or known PRNP mutation carriers with active disease, who participate in longitudinal videoconference assessments with a study partner.

Other: Teleneurology Assessment

Interventions

Teleneurology Assessment OTHER

Participants undergo standardized videoconference-based assessments of clinical history, cognitive function, neurological function, functional status, and neuropsychiatric symptoms. Study partners provide information about the participant and complete caregiver well-being and burden assessments.

Prion Disease

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Adults with definite or probable prion disease, or known PRNP mutation carriers with active disease, who are referred to TAPCJD and meet study eligibility criteria. Each participant must have an adult study partner who knows the participant well, has regular contact with them, and is present for the videoconference assessments.

You may qualify if:

• Age greater than 18 years.
• Participant must have a study partner who will be present with the participant for all videoconference evaluations (in the same physical location).
• Participant meets criteria for definite or probable prion disease or is a known PRNP mutation carrier with active disease.
• Participant and/or appropriate proxy consents to study participation.
• Age greater than 18 years.
• Study partner must be present with the participant for all videoconference evaluations and must have knowledge of the participant's abilities before the onset of illness. The study partner should be with the participant at least three times per week or for a total of five hours per week as the illness progresses.
• Study partner must have internet access and a computer, smartphone, or tablet with videoconference capabilities.
• Study partner consents to study participation.

You may not qualify if:

• Participant's diagnosis is most likely not prion disease.
• Participant or study partner is less than 18 years of age.
• Participant or study partner does not have internet access or a computer, smartphone, or tablet with videoconference capabilities.
• Participant does not have a study partner.
• Study partner is less than 18 years of age.
• Study partner does not have sufficient knowledge of the participant's abilities prior to the onset of illness or does not have sufficient ongoing contact with the participant.
• Study partner does not have internet access or a computer, smartphone, or tablet with videoconference capabilities.
• Study partner does not consent to study participation.

Sponsors & Collaborators

• CJD Foundation: collaborator
• Case Western Reserve University: collaborator
• University Hospitals Cleveland Medical Center: lead

Study Sites (1)

University Hospitals Cleveland Medical Center

Cleveland, Ohio, 44106, United States

RECRUITING

Related Publications (3)

• Kovacevich A, Martin JT, Glisic K, Ogrocki P, Appleby BS. Caregiver Burden in Prion Disease. Dement Geriatr Cogn Dis Extra. 2025 Nov 24;16(1):4-10. doi: 10.1159/000549584. eCollection 2026 Jan-Dec.

  PMID: 41510146 BACKGROUND

• Leidy L, Yarlas A, Pulido RS, Ludwig J, Glisic K, Appleby BS. Internal consistency, construct validity, and responsiveness of the MRC Prion Disease Rating Scale. J Patient Rep Outcomes. 2025 May 6;9(1):49. doi: 10.1186/s41687-025-00884-3.

  PMID: 40327161 BACKGROUND

• Appleby BS, Glisic K, Rhoads DD, Bizzi A, Cohen ML, Mahajan S. Feasibility of Remote Assessment of Human Prion Diseases for Research and Surveillance. Dement Geriatr Cogn Disord. 2019;47(1-2):79-90. doi: 10.1159/000497055. Epub 2019 Mar 12.

  PMID: 30861521 BACKGROUND

MeSH Terms

Conditions

Creutzfeldt-Jakob Syndrome; Prion Diseases; Caregiver Burden

Condition Hierarchy (Ancestors)

Central Nervous System Infections; Infections; Dementia; Brain Diseases; Central Nervous System Diseases; Nervous System Diseases; Neurocognitive Disorders; Mental Disorders; Neurodegenerative Diseases; Stress, Psychological; Behavioral Symptoms; Behavior

Central Study Contacts

Brian S Appleby, MD

CONTACT

216-368-0587; cjdsurveillance@uhhospitals.org

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Physician

Study Record Dates

• First Submitted: September 15, 2026
• First Posted: September 25, 2026
• Study Start: January 25, 2022
• Primary Completion (Estimated): January 1, 2032
• Study Completion (Estimated): January 25, 2032
• Last Updated: September 25, 2026

Record last verified: 2026-09

Data Sharing

• IPD Sharing: Will share

Locations

US (1)