14 research outputs found

    Hyperekplexia

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    Latah

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    Jumping Frenchmen of Maine

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    Latah:An indonesian startle syndrome

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    <p>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</p>

    Exaggerated startle reactions

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    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. (C) 2011 International Federation of Clinical Neurophysiology. Published by Elsevier Ireland Ltd. All rights reserve

    Increased Auditory Startle Reflex in Children with Functional Abdominal Pain

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    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. (J Pediatr 2010; 156: 285-91)
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