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Familial Startle Disease (Hyperexplexia): Electrophysiologic Studies

Familial Startle Disease (Hyperexplexia): Electrophysiologic Studies Abstract • Six affected members from a family of 15 patients with familial startle disease (hyperexplexia) underwent extensive electrophysiologic evaluation. The most marked abnormality consisted of prominent C response 60 to 75 ms after median and peroneal nerve stimulation. The somatosensory evoked responses were also relatively high in amplitude. These findings suggest that hyperactive long-loop reflexes may constitute the physiologic basis of startle disease. References 1. Suhren O, Bruyn GW, Tuynman JA: Hyperexplexia: A hereditary startle syndrome . J Neurol Sci 1966;3:577-605.Crossref 2. Morley DJ, Weaver DD, Garg BP, et al: Hyperexplexia: An inherited disorder of the startle response . Clin Genet 1982;21:308-316. 3. Conrad B, Aschoff JC: Effects of voluntary isometric and isotonic activity on late transcortical reflex components in normal subjects and hemiparetic patients . Electroencephalogr Clin Neurophysiol 1977;42:107-116.Crossref 4. Kelly JJ, Sharbrough FW, Daube JR: A clinical and electrophysiological evaluation of myoclonus . Neurology 1981;31:581-589.Crossref 5. Gaustaut H, Villeneuve A: The startle disease or hyperekplexia: Pathological surprise reaction . J Neurol Sci 1967;5:523-542.Crossref 6. Andermann F, Keene DL, Andermann E, et al: Startle disease or hyperekplexia: Further delineation of the syndrome . Brain 1980;103:985-997.Crossref 7. Lee RG, Tatton WG: Long loop reflexes in man: Clinical application . Prog Clin Neurophysiol 1978;4:320-333. 8. Upton ARM, McComas AJ, Sica REP: Potentiation of 'late' response evoked in muscles during effort . J Neurol Neurosurg Psychiatry 1971;34:699-711.Crossref 9. Conrad B: The motor center as a primary device for fast adjustment of programmed motor patterns to afferent signals . Prog Clin Neurophysiol 1978;4:123-140. 10. Delwaide PJ, Toulouse P, Crenna P: Hypothetical role of long loop reflex pathways . Appl Neurophysiol 1980;44:171-176. 11. Shibasaki H, Yamashita J, Kuroiwa Y: Electroencephalographic studies of myoclonus . Brain 1978;101:447-460.Crossref 12. Sutton GG, Mayer RF: Focal reflex myoclonus . J Neurol Neurosurg Psychiatry 1974;37:207-217.Crossref 13. Hallett M, Chadwick D, Adam J, et al: Reticular reflex myoclonus: A physiological type of human post-hypoxic myoclonus . J Neurol Neurosurg Psychiatry 1977;40:253-264.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology American Medical Association

Familial Startle Disease (Hyperexplexia): Electrophysiologic Studies

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References (14)

Publisher
American Medical Association
Copyright
Copyright © 1984 American Medical Association. All Rights Reserved.
ISSN
0003-9942
eISSN
1538-3687
DOI
10.1001/archneur.1984.04050130077028
Publisher site
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Abstract

Abstract • Six affected members from a family of 15 patients with familial startle disease (hyperexplexia) underwent extensive electrophysiologic evaluation. The most marked abnormality consisted of prominent C response 60 to 75 ms after median and peroneal nerve stimulation. The somatosensory evoked responses were also relatively high in amplitude. These findings suggest that hyperactive long-loop reflexes may constitute the physiologic basis of startle disease. References 1. Suhren O, Bruyn GW, Tuynman JA: Hyperexplexia: A hereditary startle syndrome . J Neurol Sci 1966;3:577-605.Crossref 2. Morley DJ, Weaver DD, Garg BP, et al: Hyperexplexia: An inherited disorder of the startle response . Clin Genet 1982;21:308-316. 3. Conrad B, Aschoff JC: Effects of voluntary isometric and isotonic activity on late transcortical reflex components in normal subjects and hemiparetic patients . Electroencephalogr Clin Neurophysiol 1977;42:107-116.Crossref 4. Kelly JJ, Sharbrough FW, Daube JR: A clinical and electrophysiological evaluation of myoclonus . Neurology 1981;31:581-589.Crossref 5. Gaustaut H, Villeneuve A: The startle disease or hyperekplexia: Pathological surprise reaction . J Neurol Sci 1967;5:523-542.Crossref 6. Andermann F, Keene DL, Andermann E, et al: Startle disease or hyperekplexia: Further delineation of the syndrome . Brain 1980;103:985-997.Crossref 7. Lee RG, Tatton WG: Long loop reflexes in man: Clinical application . Prog Clin Neurophysiol 1978;4:320-333. 8. Upton ARM, McComas AJ, Sica REP: Potentiation of 'late' response evoked in muscles during effort . J Neurol Neurosurg Psychiatry 1971;34:699-711.Crossref 9. Conrad B: The motor center as a primary device for fast adjustment of programmed motor patterns to afferent signals . Prog Clin Neurophysiol 1978;4:123-140. 10. Delwaide PJ, Toulouse P, Crenna P: Hypothetical role of long loop reflex pathways . Appl Neurophysiol 1980;44:171-176. 11. Shibasaki H, Yamashita J, Kuroiwa Y: Electroencephalographic studies of myoclonus . Brain 1978;101:447-460.Crossref 12. Sutton GG, Mayer RF: Focal reflex myoclonus . J Neurol Neurosurg Psychiatry 1974;37:207-217.Crossref 13. Hallett M, Chadwick D, Adam J, et al: Reticular reflex myoclonus: A physiological type of human post-hypoxic myoclonus . J Neurol Neurosurg Psychiatry 1977;40:253-264.Crossref

Journal

Archives of Neurology – American Medical Association

Published: Jan 1, 1984

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