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Development of Real-World Motor Outcome Measures in Ataxia-Telangiectasia

Award Number: R01NS134597
ORGANIZATION: NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE
OPDIV: NIH
AWARD CLASS: DISCRETIONARY
AWARD ACTIVITY TYPE: SCIENTIFIC/HEALTH RESEARCH (INCLUDES SURVEYS)
PERIOD OF PERFORMANCE START DATE: 12/15/2023
PERIOD OF PERFORMANCE END DATE: 11/30/2028

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Development of Real-World Motor Outcome Measures in Ataxia-Telangiectasia - Objective, sensitive, and clinically meaningful assessments of disease severity are needed in ataxia-telangiectasia (A-T) and other neurological disorders to support drug development efforts, facilitate clinical care, and provide caregivers and patients with real-time information about disease status. This need has become even more critical as the number of disease-modifying therapies under evaluation rapidly expands. Daily fluctuation in symptoms, superimposed changes related to child development, and variable participation in task-based assessments pose challenges to precisely measuring disease severity. Passive and continuous (i.e., task-free) collection of movement data at-home using consumer-grade wearable sensors, combined with motor control theory-inspired signal processing and machine learning methods, provides an opportunity to produce reliable, interpretable, and meaningful motor measures that can sensitively capture disease change. We have recently made substantial preliminary progress in creating algorithms to extract and characterize movement building blocks called “submovements” from a single wrist or ankle sensor worn continuously at home. These sensor-based features show strong potential as novel disease measures: they demonstrate high reliability, correlate with clinician assessments of motor severity and caregiver-reported motor function, and show potential to sensitively quantify disease progression. By passively measuring everyday activity, the information obtained can be more ecologically valid and comprehensive than task-specific measurements and is applicable in young children as well in older, non-ambulatory individuals. We propose to further develop and expand this technology over a wide age range and in a longitudinal cohort of A-T and age- and sex-matched control participants to establish reliability, age relationships, and sensitivity to disease change. Additional clinical, caregiver-reported, and molecular data will be obtained alongside wrist and ankle sensor data to increase the interpretability and clinical meaningfulness of the measures and move toward clinical trial-ready outcome measures. The overall goal of this project is to develop accessible technologies that produce highly sensitive, meaningful, and ecologically-valid measures of motor function for use in clinical trials and clinical practice in A-T as well as other pediatric and adult neurodegenerative disorders.


Issue Date FY	Funding FY	Legal Entity Name	Legal Entity Address	Legal Entity City	Legal Entity State	Legal Entity Zip Code	Legal Entity COUNTY	Legal Entity COUNTRY	Assistance Listing	Award Code	Budget Year	Action Date	Action Type	Action Amount

Issue Date FY: 2026 ( Subtotal = $624,776 )
2026	2026	THE GENERAL HOSPITAL CORPORATION	55 FRUIT ST	BOSTON	MA	02114	SUFFOLK	USA	Extramural Research Programs in the Neurosciences and Neurological Disorders	000	3	3/25/2026	NON-COMPETING CONTINUATION	$624,776
														Subtotal = $624,776

Issue Date FY: 2025 ( Subtotal = $584,256 )
2025	2025	THE GENERAL HOSPITAL CORPORATION	55 FRUIT ST	BOSTON	MA	02114	SUFFOLK	USA	Extramural Research Programs in the Neurosciences and Neurological Disorders	000	2	11/29/2024	NON-COMPETING CONTINUATION	$584,256
														Subtotal = $584,256

Issue Date FY: 2024 ( Subtotal = $691,502 )
2024	2024	THE GENERAL HOSPITAL CORPORATION	55 FRUIT ST	BOSTON	MA	02114	SUFFOLK	USA	Extramural Research Programs in the Neurosciences and Neurological Disorders	000	1	12/14/2023	NEW	$691,502
														Subtotal = $691,502

Grand Total All Awards = $1,900,534

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Development of Real-World Motor Outcome Measures in Ataxia-Telangiectasia

Award Number: R01NS134597

ORGANIZATION: NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE

OPDIV: NIH

AWARD CLASS: DISCRETIONARY

AWARD ACTIVITY TYPE: SCIENTIFIC/HEALTH RESEARCH (INCLUDES SURVEYS)

PERIOD OF PERFORMANCE START DATE: 12/15/2023

PERIOD OF PERFORMANCE END DATE: 11/30/2028

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