Latah: an Indonesian startle syndrome.

The nature of culture-specific startles syndromes such as "Latah" in Indonesia and Malaysia is ill understood. Hypotheses concerning their origin include sociocultural behavior, psychiatric disorders, and neurological syndromes. The various disorders show striking similarities despite occurring in diverse cultural settings and genetically distant populations. They are characterized clinically by exaggerated startle responses and involuntary vocalizations, echolalia, and echopraxia. Quantifying startle reflexes may help define Latah within the 3 groups of startle syndromes: (1) hyperekplexia, (2) startle-induced disorders, and (3) neuropsychiatric startle syndromes. Twelve female Latah patients (mean age, 44.6 years; SD, 7.7 years) and 12 age-, sex- and socioeconomically matched controls (mean age, 42.3 year; SD, 8.0) were studied using structured history taking and neurological examination including provocation of vocalizations, echolalia, and echopraxia. We quantified auditory startle reflexes with electromyographic activity of 6 left-sided muscles following 104-dB tones. We defined 2 phases for the startle response: a short latency motor startle reflex initiated in the lower brain stem <100/120 ms) and a later, second phase more influenced by psychological factors (the "orienting reflex," 100/120-1000 ms after the stimulus). Early as well as late motor startle responses were significantly increased in patients compared with controls (P ≤ .05). Following their startle response, Latah patients showed stereotyped responses including vocalizations and echo phenomena. Startle responses were increased, but clinically these proved insignificant compared with the stereotyped behavioral responses following the startle response. This study supports the classification of Latah as a "neuropsychiatric startle syndrome."

Exaggerated startle reactions.

The origin of the startle reflex lies in the caudal brainstem; it can be elicited by an unexpected stimulus resulting in a bilateral activation of many muscles. Two subsequent responses can be measured during EMG recordings; after the initial motor reflex, lasting until about 150 ms, a second response can occur. The second response contains more emotional and voluntary behavioral responses. Clinically, syndromes with hyperstartling as common feature can be divided into three groups: hyperekplexia, stimulus-induced disorders, and neuropsychiatric disorders. Classification of startle syndromes within these three groups remains challenging. Generalized stiffness at birth, excessive startling and temporary generalized stiffness after being startled point towards hyperekplexia. Stimulus-induced disorders are distinguished by careful clinical and neurophysiological evaluation, including video recordings. Neuropsychiatric disorders usually have additional behavioural and psychiatric symptoms. Polymyographic EMG startle recordings exhibit an exaggeration of the initial motor startle reflex in hyperekplexia, while neuropsychiatric startle syndromes demonstrate a variable response pattern and abnormal behavioural features. Neurophysiological investigation of the startle reflex can help to further delineate between the startle syndromes and unravel the aetiology of neuropsychiatric startle disorders.

Increased auditory startle reflex in children with functional abdominal pain.

OBJECTIVE: To test the hypothesis that children with abdominal pain-related functional gastrointestinal disorders have a general hypersensitivity for sensory stimuli. STUDY DESIGN: Auditory startle reflexes were assessed in 20 children classified according to Rome III classifications of abdominal pain-related functional gastrointestinal disorders (13 irritable bowel syndrome [IBS], 7 functional abdominal pain syndrome; mean age, 12.4 years; 15 girls) and 23 control subjects (14 girls; mean age, 12.3 years) using a case-control design. The activity of 6 left-sided muscles and the sympathetic skin response were obtained by an electromyogram. We presented sudden loud noises to the subjects through headphones. RESULTS: Both the combined response of 6 muscles and the blink response proved to be significantly increased in patients with abdominal pain compared with control subjects. A significant increase of the sympathetic skin response was not found. Comorbid anxiety disorders (8 patients with abdominal pain) or Rome III subclassification did not significantly affect these results. CONCLUSIONS: This study demonstrates an objective hyperresponsivity to nongastrointestinal stimuli. Children with abdominal pain-related functional gastrointestinal disorders may have a generalized hypersensitivity of the central nervous system.

Increased whole-body auditory startle reflex and autonomic reactivity in children with anxiety disorders.

BACKGROUND: Young patients with anxiety disorders are thought to have a hypersensitive fear system, including alterations of the early sensorimotor processing of threatening information. However, there is equivocal support in auditory blink response studies for an enlarged auditory startle reflex (ASR) in such patients. We sought to investigate the ASR measured over multiple muscles (whole-body) in children and adolescents with anxiety disorders. METHODS: Between August and December 2006, we assessed ASRs (elicited by 8 consecutive tones of 104 dB, interstimulus interval of about 2 min) in 25 patients and 25 matched controls using a case-control design and in 9 nonaffected siblings. We recorded the electromyographic activity of 6 muscles and the sympathetic skin response. We investigated response occurrence (probability %) and response magnitude (area under the curve in microV x ms) of the combined response of 6 muscles and of the single blink response. RESULTS: In patients (17 girls, mean age 12 years; 13 social phobia, 9 generalized anxiety, 3 other), the combined response probability (p = 0.027) of all muscles, the combined area under the curve of all muscles (p = 0.011) and the sympathetic skin response (p = 0.006) were enlarged compared with matched controls. The response probability (p = 0.48) and area under the curve (p = 0.07) of the blink response were normal in patients compared with controls. The ASR pattern was normal with normal latencies in patients compared with controls. In nonaffected siblings, the sympathetic skin response (p = 0.038), but not the combined response probability of all muscles (p = 0.15), was enlarged compared with controls. LIMITATIONS: Limitations are the sample size and restricted comparison to the psychophysiological ASR paradigm. CONCLUSION: The results point toward a hypersensitive central nervous system (fear system), including early sensorimotor processing alterations and autonomic hyperreactivity. The multiple muscle (whole-body) ASR is suggested to be a better tool to detect ASR abnormalities in patients with anxiety disorders than the blink response alone. Abnormalities in ASR serve as a candidate endophenotype of anxiety disorders.

Quantification of the auditory startle reflex in children.

OBJECTIVE: To find an adequate tool to assess the auditory startle reflex (ASR) in children. METHODS: We investigated the effect of stimulus repetition, gender and age on several quantifications of the ASR. ASR's were elicited by eight consecutive auditory stimuli in 27 healthy children. Electromyographic activity of orbicularis oculi, masseter, sternocleidomastoid, deltoid, abductor pollicis brevis, quadriceps muscles and the sympathetic skin response were recorded. ASR parameters (response probability in % and magnitude in area-under-the-curve) were: (1) combined response of all six muscles (2) blink response. RESULTS: Response probabilities were 78% in orbicularis oculi (median latency 41 ms), 17% in sternocleidomastoid (median latency 66 ms), 10% in masseter (median latency 66 ms) and lower in other muscles. The ASR combined response probability and the sympathetic skin response significantly decreased with the repetitive stimuli, but the blink response probability did not. The magnitude (area-under-the-curve) of both the blink response and the combined response did not decrease with the repetitive stimuli. There were no gender or age effects. CONCLUSION: As in adults, the blink response and the combined response of multiple muscles show different habituation patterns in children. SIGNIFICANCE: Investigation of multiple muscles seems appropriate to quantify the ASR in children.

Startle syndromes.

Startle syndromes consist of three heterogeneous groups of disorders with abnormal responses to startling events. The first is hyperekplexia, which can be split up into the "major" or "minor" form. The major form of hyperekplexia is characterised by excessive startle reflexes, startle-induced falls, and continuous stiffness in the neonatal period. This form has a genetic basis: mutations in the alpha1 subunit of the glycine receptor gene, GLRA1, or related genes. The minor form, which is restricted to excessive startle reflexes with no stiffness, has no known genetic cause or underlying pathophysiological substrate. The second group of startle syndromes are neuropsychiatric, in which excessive startling and various additional behavioural features occur. The third group are disorders in which startling stimuli can induce responses other than startle reflexes, such as startle-induced epilepsy. Diagnosis of startle syndromes depends on clinical history, electromyographic studies, and genetic screening. Further study of these disorders may enable improved discrimination between the different groups.