# High-resolution multi-modal ultrasound imaging of brain development in Batten disease models

> **NIH NIH R21** · UNIVERSITY OF ROCHESTER · 2022 · $229,809

## Abstract

Batten disease, which has no treatment, is a devastating neurological disorder characterized by severe seizures,
loss of vision, language disabilities, and dementia. The absence of objective neurologic biomarkers of disease
progression is one reason why there has been limited progress in developing effective therapies for Batten and
other genetic neurological disorders. This application will advance a radically new approach to track functional
and structural changes in progressive neurologic disorders by using functional ultrasound (fUS) and shear wave
elastography (SWE). The structural and functional ultrasound imaging data acquired across the brain will provide
a critical link between single-cell properties afforded by optical imaging and large-scale functional organization
afforded by functional magnetic resonance imaging (fMRI). Together, these multi-modal and multi-scale data will
complement the current electroencephalography (EEG)-based neuromarkers of Batten disease and provide a
more detailed and comprehensive view of brain structural and functional changes during disease progression.
Using a Batten disease mouse model, we will measure fUS sensitivity to changes in the functional connectivity
between cortical and subcortical brain structures in response to various sensory stimuli. We will conduct
histological analysis to confirm the validity of the activation maps. To determine how mechanical properties of
the brain change during disease progression and with different stimuli, we will perform studies using a preclinical
SWE system to assess structural and mechanical changes in Batten disease mouse models. In this phase of
the project, we will develop advanced elastography techniques to visualize changes in brain morphology. This
exciting project brings together two experienced researchers, Dr. Marvin Doyley, an expert in ultrasound and
elastography imaging, and Dr. Kuan Hong Wang, an expert in neurobiology and genetic models of brain
disorders, to establish imaging biomarkers for studying progressive neurological disorders such as Batten
disease. This project will demonstrate that it is feasible to use emerging ultrasound imaging techniques to
understand the pathological changes in progressive neurologic disorders and facilitate testing and translation of
different therapeutic interventions.

## Key facts

- **NIH application ID:** 10429881
- **Project number:** 1R21EB033122-01
- **Recipient organization:** UNIVERSITY OF ROCHESTER
- **Principal Investigator:** Marvin M Doyley
- **Activity code:** R21 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2022
- **Award amount:** $229,809
- **Award type:** 1
- **Project period:** 2022-09-07 → 2024-06-30

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/10429881

## Citation

> US National Institutes of Health, RePORTER application 10429881, High-resolution multi-modal ultrasound imaging of brain development in Batten disease models (1R21EB033122-01). Retrieved via AI Analytics 2026-05-25 from https://api.ai-analytics.org/grant/nih/10429881. Licensed CC0.

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