Teleneurology Assessment Program for Creutzfeldt-Jakob Disease

TAPCJD is a longitudinal observational study designed to collect standardized clinical information from individuals with Prion Disease using remote videoconference assessments. The study builds upon an earlier TAPCJD pilot study and is intended to improve understanding of the clinical course of Prion Disease and the use of remote assessments in prion disease research and surveillance.

Participants take part in videoconference visits with a study partner who knows them well. Assessments include clinical history, cognitive assessment, modified neurological examination, functional assessment, and evaluation of neuropsychiatric symptoms. Study partners also complete measures related to caregiver well-being and burden.

Videoconference evaluations occur at baseline and at approximately 1, 2, 4, and 6 months, followed by evaluations every three months for the duration of the participant's survival. Assessments may be modified or omitted depending on the participant's clinical condition. Participation ends at death, if the clinical diagnosis changes and no longer includes prion disease, or if the participant or study partner chooses to discontinue participation.

The study also examines the reliability and validity of standardized remote assessments, caregiver burden and well-being, and the potential benefits of caregiver support-group participation.

Inclusion criteria

• Study Participant Inclusion Criteria:
• 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.

Study Partner Inclusion Criteria:

• 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.

Exclusion criteria

Study Participant Exclusion Criteria:

• 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 Exclusion Criteria:

• 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.