Evaluating the electrophysiological signatures of infantile spasms in a model of neonatal hypoxia

Celebrating Physiology in Ireland (Trinity College Dublin, Ireland) (2026) Proc Physiol Soc 77, C18

Poster Communications: Evaluating the electrophysiological signatures of infantile spasms in a model of neonatal hypoxia

Henry Frost1, Jessica Kingston2, Kate Connor2, Eva Jimenez-Mateos2, Mark O. Cunningham1

1Discipline of Physiology, School of Medicine, Trinity College Dublin, University of Dublin, Dublin, Ireland; FutureNeuro Research Ireland Centre for Translational Brain Science, RCSI University of Medicine and Health Sciences, Dublin, Ireland Ireland, 2Discipline of Physiology, School of Medicine, Trinity College Dublin, University of Dublin, Dublin, Ireland Ireland

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Introduction: Infantile spasms (IS), now classified as infantile epileptic spasm syndrome (IESS), is a rare, severe epileptic encephalopathy of infancy with substantial neurodevelopmental morbidity. Its interictal EEG hallmark, hypsarrhythmia, is a chaotic, high-amplitude pattern of irregular slow waves in the delta range (<3 Hz), and hypoxic-ischemic encephalopathy (HIE) is among the most common identifiable causes of IESS, accounting for up to 12% of cases. We propose that HIE-induced stress elevates corticotrophin-releasing factor (CRF) signalling, disrupting GABAergic inhibitory networks and triggering the age-specific onset of IESS; suppression of this pathway likely underlies the efficacy of first-line ACTH therapy. No existing preclinical model combines a clinically realistic HIE aetiology with CRF sensitivity. We therefore developed a novel ex vivo model combining neonatal hypoxic injury, pharmacologically induced delta oscillations as a hypsarrhythmia proxy, and CRF challenge, to test whether hypoxia-exposed networks develop IESS-like excitability and stress-sensitivity.

Aims/objectives: (1) Establish a reproducible ex vivo model of delta-frequency oscillations in developing mouse cortex, (2) determine whether neonatal hypoxic-ischemic injury alters cortical network activity and parvalbumin-positive (PV+) interneuron density, and (3) test whether hypoxia-exposed networks show increased CRF sensitivity across the postnatal window relevant to IESS (P10–P14).

Methods: All procedures accorded with EU Directive 2010/63/EU and were approved by the Trinity College Dublin Animal Welfare Body, licensed by the Health Products Regulatory Authority (AE19136/I775, AE19136/P140). Neonatal hypoxia was induced in C57BL/6J pups (P7) by exposure to 5% O2/95% N2 for 15 min at 34°C; littermate controls received a room-air sham exposure. Motor function was assessed at 24 h and 72 h post-insult. Pups were killed by decapitation for preparation of 400 μm somatosensory cortex slices. Local field potentials were recorded from layer IV/V of the barrel field; delta oscillations were induced with carbachol (2 μM) and SCH23390 (10 μM), followed by CRF (20 nM), and quantified by fast Fourier transform (area under the curve, AUC; peak amplitude; 0.5–4 Hz). Separate cohorts underwent PV immunohistochemistry (P10, P12, P14) and cFos/Arc mRNA quantification (P14). Data are mean ± SEM; comparisons used Mann–Whitney U tests, mixed-effects models, or two-way ANOVA with Šídák’s post hoc test; p<0.05 was significant.

Results: By 72 h, hypoxia-exposed pups (n=6–16 per test) showed reduced ambulation (p=0.0286), increased hindlimb foot angle (p=0.0007), impaired negative geotaxis (p=0.0129) and reduced grip strength (p=0.0122) versus controls. Hypoxia slices (N=4–5/group) showed increased delta-band AUC and amplitude at P10–P14, at baseline and following CRF (p<0.05), with CRF unmasking large, hypsarrhythmia-like discharges in hypoxia-exposed but not control tissue. PV+ interneuron counts in S1BF (N=3/group/age) were reduced at P12 (p<0.05) and P14 (p<0.0001), with layer-specific loss at P14 in layers II/III, IV and V (p<0.01–p<0.001). cFos and Arc mRNA in P14 cortex did not differ between groups (n=5/group).

Conclusions: Neonatal hypoxia produces persistent behavioural deficits, layer-specific PV+ interneuron loss, and enduring cortical hyperexcitability with heightened CRF sensitivity across a developmental window relevant to IESS. This is the first ex vivo model linking a clinically realistic HIE aetiology to CRF-driven, IESS-like network dysfunction, offering a platform for testing CRF-targeted and interneuron-directed interventions.



Where applicable, experiments conform with Society ethical requirements.

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