Cardiac Startle Response and Clinical Outcomes in Preschool Children With Fragile X Syndrome and Autism Spectrum Disorder

Objective: Poor physiological regulation in response to threat is linked to multiple negative developmental outcomes including anxiety, which is highly prevalent and impairing in young children with neurodevelopmental disabilities like fragile X syndrome (FXS) and autism spectrum disorder (ASD). The present study contrasted cardiac startle response in pre-school-aged children with FXS, with and without ASD, to children with non-syndromic ASD (nsASD) and neurotypical controls (NT). The relationship of cardiac startle to non-verbal mental age (NVMA), ASD severity, and parent-reported anxiety was also examined.Method: Four age-matched groups of pre-school children participated including those with FXS without ASD (FXS-Only, n = 21), FXS with ASD (FXS+ASD, n = 17), nsASD (n = 42), and NT children (n = 27). Participants viewed a silent movie during which a single 200 ms 98-decibel white noise burst occurred. Cardiac activity was analyzed for pre-stimulus respiratory sinus arrhythmia (RSA) and the inter-beat intervals (IBI) at the auditory stimulus and 10 s post-stimulus. The Spence Pre-school Anxiety Scale, Autism Diagnostic Observation Schedule-2nd Edition, and Mullen Scales of Early Learning were examined in relation to startle response.Results: The nsASD group demonstrated heightened cardiac activity at the auditory stimulus and 10 s post-stimulus compared to the NT controls. Neither of the FXS groups showed differences from any other group. Higher pre-stimulus RSA was associated with reduced cardiac response across groups, while the relationship between cognitive ability and ASD severity to cardiac response varied between groups. Parent-reported anxiety was not associated with cardiac response for any group.Conclusion: These findings demonstrate group distinctions in cardiac responses to auditory startle. Although FXS and ASD share behavioral characteristics, the nsASD group showed a heightened cardiac startle response compared to the NT group that was not present in the FXS groups with or without ASD. Non-verbal mental age was associated with greater stimulus or post-stimulus reactivity for all groups except the FXS+ASD group, which showed no association between startle response and any clinical outcomes. Increased understanding of the relationship between physiological regulation and clinical outcomes will assist in identifying the timing and targets for effective interventions for individuals with neurodevelopmental disabilities.

Looming Effects on Attentional Modulation of Prepulse Inhibition Paradigm

In a hazardous environment, it is fundamentally important to successfully evaluate the motion of sounds. Previous studies demonstrated “auditory looming bias” in both macaques and humans, as looming sounds that increased in intensity were processed preferentially by the brain. In this study on rats, we used a prepulse inhibition (PPI) of the acoustic startle response paradigm to investigate whether auditory looming sound with intrinsic warning value could draw attention of the animals and dampen the startle reflex caused by the startling noise. We showed looming sound with a duration of 120 ms enhanced PPI compared with receding sound with the same duration; however, when both sound types were at shorter duration/higher change rate (i.e., 30 ms) or longer duration/lower rate (i.e., more than 160 ms), there was no PPI difference. This indicates that looming sound–induced PPI enhancement was duration dependent. We further showed that isolation rearing impaired the abilities of animals to differentiate looming and receding prepulse stimuli, although it did not abolish their discrimination between looming and stationary prepulse stimuli. This suggests that isolation rearing compromised their assessment of potential threats from approaching objects and receding objects.

The Mere Sight of Loved Ones Does Not Inhibit Psychophysiological Defense Mechanisms When Threatened

Looking at pictures of loved ones, such as one's romantic partner or good friends, has been shown to alleviate the experience of pain and reduce defensive reactions. However, little is known about such modulatory effects on threat and safety learning and the psychophysiological processes involved. Here, we explored the hypothesis that beloved faces serve as implicit safety cues and attenuate the expression of fear responses and/or accelerate extinction learning in a threatening context. Thirty-two participants viewed pictures of their loved ones (romantic partner, parents, and best friend) as well as of unknown individuals within contextual background colors indicating threat-of-shock or safety. Focusing on the extinction of non-reinforced threat associations (no shocks were given), the experiment was repeated on two more test days while the defensive startle-EMG, SCR, and threat ratings were obtained. Results confirmed pronounced defensive responding to instructed threat-of-shock relative to safety context (e.g., threat-enhanced startle reflex and SCR). Moreover, threat-potentiated startle response slowly declined across test days indicating passive extinction learning in the absence of shocks. Importantly, neither a main effect of face category (loved vs. unknown) nor a significant interaction with threat/safety instructions was observed. Thus, a long-term learning history of beneficial relations (e.g., with supportive parents) did not interfere with verbal threat learning and aversive apprehensions.

The regulatory role of GABAA receptor in Actinia equina nervous system and the possible effect of global ocean acidification

Global warming and connected acidification of the world ocean attract a substantial amount of research efforts, in particular in a context of their impact on behaviour and metabolism of marine organisms, such as Cnidaria. Nevertheless, mechanisms underlying Cnidarians’ neural signalling and behaviour and their (possible) alterations due to the world ocean acidification remain poorly understood. Here we researched for the first time modulation of GABAA receptors (GABAARs) in Actinia equina (Cnidaria: Anthozoa) by pH fluctuations within a range predicted by the world ocean acidification scenarios for the next 80–100 years and by selective pharmacological activation. We found that in line with earlier studies on vertebrates, both changes of pH and activation of GABAARs with a selective allosteric agonist (diazepam) modulate electrical charge transfer through GABAAR and the whole-cell excitability. On top of that, diazepam modifies the animal behavioural reaction on startle response. However, despite behavioural reactions displayed by living animals are controlled by GABAARs, changes of pH do not alter them significantly. Possible mechanisms underlying the species resistance to acidification impact are discussed.

The Effect of Sound and Stimulus Expectation on Transcranial Magnetic Stimulation-Elicited Motor Evoked Potentials

The amplitude of motor-evoked potentials (MEPs) elicited by transcranial magnetic stimulation (TMS) over the motor cortex is influenced by multiple factors. TMS delivery is accompanied by an abrupt clicking noise which can induce a startle response. This study investigated how masking/attenuating the sound produced by the TMS system discharging influences MEP amplitudes. In addition, the effects of increasing the time between consecutive stimuli and of making participants aware of the time at which they would be stimulated were studied. MEPs were recorded from the Flexor Carpi Radialis (FCR) muscle at rest by stimulation at motor threshold (MT), 120% MT and 140% MT intensity. Participants (N = 23) received stimulation under normal (NORMAL) conditions and while: wearing sound-attenuating earmuffs (EAR); listening to white noise (NOISE); the interval between stimuli were prolonged (LONG); stimulation timing was presented on a screen (READY). The results showed that masking (p = 0.020) and attenuating (p = 0.004) the incoming sound significantly reduced the amplitude of MEPs recorded across the intensities of stimulation. Increasing the interval between pulses had no effect on the recorded traces if a jitter was introduced (p = 1), but making participants aware of stimulation timing decreased MEP amplitudes (p = 0.049). These findings suggest that the sound produced by TMS at discharging increases MEP amplitudes and that MEP amplitudes are influenced by stimulus expectation. These confounding factors need to be considered when using TMS to assess corticospinal excitability.

“I’m Nervous about the Jump Scares”

Most horror films contain several jump scares, which are sudden audiovisual stimuli that elicit a startle response. Many people who are nervous about horror films point to the jump scare as a dreaded element. The jump scare usually follows a predictable formula, but jump scares can be complex and artful, and the science of the startle response reveals an ancient defensive system designed by evolution for survival. While it is difficult to protect oneself from the jump scare, the chapter offers science-based advice on how to attenuate its effect—including coping strategies—and suggests some horror movies with low jump-scare frequency.

Teaching Video Neuroimages: Hereditary Hyperekplexia Mimicking Tonic Seizures in an Infant

Hereditary hyperekplexia is a rare neurological disorder characterized by an exaggerated startle response with profound muscle stiffness. Given the nature of the spells, this condition is often misdiagnosed as epilepsy. Mutations in glycine receptors and transporters are the primary cause of this syndrome. We present an example of stimulus induced hyperekplexia captured on video EEG in a 7-week-old girl with compound heterozygous variants in the presynaptic glycine transporter gene SLC6A5.