Families should expect to receive a comprehensive evaluation for their child or children with diagnosed or suspected Batten disease when they visit the University of Rochester Batten Center (URBC). The visit will begin with a comprehensive medical history. We will review results of previous diagnostic testing and treatments. A Child Neurologist will perform a complete physical and neurological examination. The Unified Batten Disease Rating ScaleUnified Batten Disease Rating Scale (UBDRS) and other appropriate clinical rating scales will be administered.
The cognitive and behavioral difficulties experienced by many children with Batten disease can have a significant impact on their day-to-day functioning. Understanding a child’s cognitive and behavioral needs and adapting their environment accordingly may help reduce frustration. The URBC offers neuropsychological and behavioral assessment to help clarify the patient’s pattern of cognitive strengths and weaknesses, and develop recommendations for educational services and behavior management. Neuropsychological assessment is available to all children able to provide verbal responses to questions - vision is not required. Children able to participate in neuropsychological testing will receive a brief (20-30 minutes maximum) assessment of cognitive abilities, including attention, memory, and language skills. In addition, the neuropsychologist will meet with parents to discuss their specific concerns about their child’s learning and behavior. Parents will receive a written report that summarizes test results and recommendations. Educational consultation is also available, including review of school records, providing schools with information on Batten disease, and assisting schools with planning their own evaluations and services for the child with Batten’s. Follow-up assessment is also available to track changes over time and help adjust services as needed.
Evaluation by other health care professionals is available depending on individual needs. This may include evaluation by a Pediatric Ophthalmologist, other Pediatric specialists, and/or a genetic counselor. If the diagnosis has not yet been confirmed, the URBC will offer genetic testing; other recommended diagnostic testing will be arranged either at the University of Rochester or at a medical center near the family’s home. If the diagnosis is confirmed after the visit to the center, the URBC will arrange genetic counseling at a medical center near the family’s home, if desired.
Upon conclusion of the evaluation, the URBC will prepare a formal report. This will be sent to the patient’s primary care physician and to the patient’s family.
A major focus of URBC is clinical research on Batten disease. Therefore, current research protocols will be discussed prior to the visit. Each family has the opportunity to participate in our research. However, participation in research is not a requirement to be evaluated at the center. There is no charge for participation in any of the URBC’s research activities.
Travel and Lodging
The University of Rochester is located in Rochester, NY. The following airlines service the Greater Rochester International Airport:
- Air Canada
- Jet Blue
- US Airways
Since most patients come to the URBC from outside of western New York, visits usually entail an overnight stay in Rochester and up to a full day of evaluation. Lodging can be arranged at the Ronald McDonald House or at a nearby hotel that offers discounted rates to families with medical appointments, depending on the family’s preference. Our staff will assist with lodging arrangements.
Accommodations at the Ronald MacDonald House cost $10 per day. A shuttle is available for transportation to the Medical Center 4 times daily, at 9:15 am, 12 noon, 1:00 pm and 7:00 pm. Reservations must be scheduled through our office at (585) 275-4762. The house is located several blocks from the Medical Center.
Batten Study Group
601 Elmwood Ave
Rochester, NY 14642
(directed to Amy Vierhile)