Actively Recruiting

All Genders
ID01873924

Clinical and Neuropsychological Investigations in Batten Disease

Led by University of Rochester · Updated on 2025-09-12

500

Participants Needed

1

Research Sites

N/A

Total Duration

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AI-Summary

What this Trial Is About

Researchers are investigating the natural history of Batten disease, also known as Neuronal Ceroid Lipofuscinosis, a genetic disorder that causes progressive cognitive and behavioral decline in children and adults. The study aims to gather detailed information about the motor, behavioral, and functional abilities of people with Batten disease and to improve the Unified Batten Disease Rating Scale (UBDRS), a clinical tool designed to measure disease severity and progression. Participants with any form of Batten disease will be assessed using the UBDRS approximately once a year for up to 20 years. The study involves clinical and neuropsychological evaluations, including cognitive and behavioral assessments, adaptive function, quality of life measures, and vision tests. Assessments may take place at the University of Rochester Batten Center, annual meetings of the Batten Disease Support and Research Association, or remotely through televideo. During their involvement, participants will provide medical history, symptom details, medication use, and diagnostic test information. Researchers will monitor changes in physical health, seizures, behavior, and function annually using the UBDRS, along with cognitive and vision-related measures. The study may last up to 20 years, with some assessments continuing for up to 10 years, and aims to better understand disease progression and its impact on daily life.

CONDITIONS

Brief Title

Clinical and Neuropsychological Investigations in Batten Disease

Who Can Participate

All Genders

Eligibility Criteria

Eligible

You may qualify if you...

  • Child or adult with any form of Batten disease
  • Parent or legal guardian of a child or adult with any form of Batten disease
Not Eligible

You will not qualify if you...

  • Parent or guardian unable or unwilling to provide permission for the affected individual

AI-Screening

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Your Study Journey

Screening

Duration - 2 to 4 weeks

Participants are screened for eligibility to participate in the trial.

Long-term Monitoring

Duration - Up to 20 years

Participants undergo annual assessments to track disease progression and assess cognitive, behavioral, and functional changes related to Batten Disease.

Approximately 1 visit per year (in-person or remote)

Trial Site Locations

Total: 1 location

1

University of Rochester

Rochester, New York, United States, 14642

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Research Team

A

Amy Vierhile, RN PNP

How is the study designed?

Study Type

OBSERVATIONAL

Masking

N/A

Allocation

N/A

Model

N/A

Primary Purpose

N/A

Number of Arms

1

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