# Clinical-Res-Project2

> **NIH NIH U54** · CHILDREN'S RESEARCH INSTITUTE · 2021 · $154,881

## Abstract

PROJECT 2 - ABSTRACT
Epilepsy has been considered an infrequent manifestation in UCD (1). However, we found the
frequent occurrence of sub-clinical electrographic seizures ((ES); seizures detected on EEG
without accompanying clinical manifestations) during acute hyperammonemic (HA) episodes in
patients, especially neonates, enrolled in the UCDC longitudinal study (LS). This new finding
raises the question of whether seizures play an important role in the pathophysiology of
neurocognitive deficits in UCD patients and/or can represent a biomarker that correlates with
brain damage in these disorders. In this project, we propose to systematically study the scope
and role of ES in our LS UCD population, characterizing the onset, frequency, duration,
localization (focal or generalized) EEG background features, and biochemical and clinical
correlates, as well as any association of ES or EEG background with the subsequent
development of epilepsy.
We hypothesize that:
1) There is a correlation between significant increases in plasma ammonia and/or glutamine
levels and the development of ES, and
2) ES play a role in the adverse neurocognitive outcome after HA encephalopathy, especially in
the neonate with UCD.
If ES are in fact associated with adverse neurocognitive outcome, whether as a cause or as an
associated biomarker, this project will provide clinical trial readiness data to subsequently test
the role of anti-epileptic drugs as neuroprotection agents during HA in UCD, and presents both
an opportunity and critical need to develop new therapeutic strategies that are targeted to the
brain. The UCDC multisite consortium represents an ideal venue to capture this information.

## Key facts

- **NIH application ID:** 10241409
- **Project number:** 5U54HD061221-18
- **Recipient organization:** CHILDREN'S RESEARCH INSTITUTE
- **Principal Investigator:** Andrea Lynne Gropman
- **Activity code:** U54 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2021
- **Award amount:** $154,881
- **Award type:** 5
- **Project period:** 2003-09-30 → 2024-07-31

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/10241409

## Citation

> US National Institutes of Health, RePORTER application 10241409, Clinical-Res-Project2 (5U54HD061221-18). Retrieved via AI Analytics 2026-05-22 from https://api.ai-analytics.org/grant/nih/10241409. Licensed CC0.

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