Sandhoff disease is a GM2 gangliosidosis that is rare in Thailand. The authors report a Thai family with two children known to have infantile form of Sandhoff disease. The index case exhibited mitral valve prolapse with mitral regurgitation as an early sign, which is a rare presentation in Sandhoff disease. Thereafter the patient had developmental regression, startle reaction, and cherry red sp...

Previous studies have shown alterations of eyeblink reflex in patients with various psychiatric disorders. It has previously been demonstrated by our group that EEG measures of the reactivity to eye opening could effectively predict patient-reported startle response in patients with acute stress reaction. In our present study, EEG spectral power analysis and eyeblink electrical startle response...

An experiment is reported that investigated the effects of an auditory startling stimulus on a compound movement task. Previous findings have shown that in a targeting task, a secondary movement can be initiated based on the proprioceptive information provided by a primary movement (Cordo 1990). Studies involving the presentation of a startling stimulus have shown that in reaction-time (RT) tas...

Habituation is a form of non-associative learning that enables animals to reduce their reaction to repeated harmless stimuli. When exposed to ethanol vapor, Drosophila show an olfactory-mediated startle response characterized by a transient increase in locomotor activity. Upon repeated exposures, this olfactory startle attenuates with the characteristics of habituation. Here we describe the res...

Pictures of emotional facial expressions or natural scenes are often used as cues in emotion research. We examined the extent to which these different stimuli engage emotion and attention, and whether the presence of social anxiety symptoms influences responding to facial cues. Sixty participants reporting high or low social anxiety viewed pictures of angry, neutral, and happy faces, as well as...

Hyperekplexia (startle disease) is a rare non-epileptic disorder characterised by an exaggerated persistent startle reaction to unexpected auditory, somatosensory and visual stimuli, generalised muscular rigidity, and nocturnal myoclonus. The genetic basis is a mutation usually of the arginine residue 271 leading to neuronal hyperexcitability by impairing glycinergic inhibition. Hyperekplexia i...

Fast reaction times and the ability to develop a high rate of force development (RFD) are crucial for sports performance. However, little is known regarding the relationship between these parameters. The aim of this study was to investigate the effects of auditory stimuli of different intensities on the performance of a concentric bench-press exercise. Concentric bench-presses were performed by...

We investigated the eyeblink component of acoustic startle reactions in maltreated children. Previous research indicates that acoustic startle is enhanced in adult males with posttraumatic stress disorders (PTSD) whereas findings on women with PTSD have been inconsistent. In accord with the only previous report for children with PTSD, we found that maltreated boys, particularly those who had be...

INTRODUCTION Loud sounds can decrease reaction time (RT) and increase force generated during voluntary contractions. Accordingly, we hypothesized that the loud starter's pistol at the Olympic Games allows runners closer to the starter to react sooner and stronger than runners farther away. METHODS RT for the 100/110 m athletics events at the 2004 Olympics were obtained from International Asso...

Measures of acoustic startle such as prepulse inhibition (PPI) and startle latency have been found to be impaired in schizophrenia, and are commonly thought to be related to cognitive deficits in this disease. However, findings about the relationship between startle variables and cognitive performance have been equivocal. In this study, we examined correlations between startle measures (baselin...