In total, there are thirteen subtypes of Batten disease. Batten disease, also known as neuronal ceroid lipofuscinosis (NCL), is a rare and inherited neurodegenerative disorder that causes visual impairment, behavioral and personality changes, seizures, impaired cognition and motor skills, and dementia.
Researchers at the University of Rochester Medical Center aimed to identify potential neuromarkers specific to juvenile Batten disease caused by CLN3 mutations. Neuromarkers serve as biological indicators, revealing the presence of a disease or indicating its progression, with a specific focus on neuronal function. According to the University of Rochester Medical Center Newsroom, the research team identified auditory sensory memory system issues as a neuromarker of Batten disease.
Can a Neuromarker Transform Batten Disease Research?
The study utilized 21 children with juvenile Batten disease and compared the collected data to 41 age-matched controls. As shared in the Journal of Neurodevelopmental Disorders, the research team explored auditory sensory memory system function with a specific focus on duration processing and memory recall. The researchers note in the research paper that:
“We employed three stimulation rates (fast: 450 ms, medium: 900 ms, slow: 1800 ms), allowing for the assessment of the sustainability of the auditory sensory memory trace. The robustness of mismatch negativity directly relates to the rate at which the regularly occurring stimulus stream is presented [and] as presentation rate slows, robustness of the sensory memory trace diminishes.”
Researchers monitored auditory sensory memory function through EEGs while listening to auditory beeping and watching their favorite movie(s). They found that auditory sensory memory processes were robust and functioning well during the medium stimulation rate (900 ms). Alternately, mismatch negativity was lower at the fastest rate and undetectable at the slowest rate. The control group did not share these results; their auditory sensory memory function was maintained throughout.
Essentially, these findings suggest that the auditory sensory memory system plays a role in poor memory recall and cognitive decline in juvenile Batten disease. More research is needed to determine whether this could be utilized as a meaningful neuromarker. Researchers will evaluate this neuromarker in mice models of Batten disease. If effective, this neuromarker could be used or targeted in eventual human clinical studies to identify how well the therapies are working.
