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Large Electroencephalographic Responses and Their Relationship to Cleido-Cranial Dysplasia

Published online by Cambridge University Press:  18 September 2015

Adrian Upton ,
Sarah Bundey  and
Susan Sanders
Adrian Upton*
Affiliation:
The National Hospitals for Nervous Diseases, Queen Square, London, W.C.1.
Sarah Bundey
Affiliation:
The National Hospitals for Nervous Diseases, Queen Square, London, W.C.1.
Susan Sanders*
Affiliation:
The National Hospitals for Nervous Diseases, Queen Square, London, W.C.1.
*
Department of Medicine (Neurology), McMaster University, 1200 Main St. West, Hamilton L8S 4J9, Ontario, Canada
Department of Medicine (Neurology), McMaster University, 1200 Main St. West, Hamilton L8S 4J9, Ontario, Canada
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Summary:

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We have reported six individuals (five certain heterozygotes for cleido-cranial-dysostosis and one possible heterozygote) who have unusual EEG findings, consisting of very large responses to photic flash stimulation at very low stimulus rates.

Such visual responses are extremely rare and have not been seen before in the experience of an EEG department over 12 years and they were not seen in 98 control subjects. It is likely that these responses are an irregular mani-festation of the gene for cleido-cranial-dysplasia, and that the responses are independent of skull deformity. One importance of these responses is their demonstration in neurologically normal individuals for previously such large responses have only been reported in association with neurolipidosis. They may have neurophysiological significance in that they may reflect an unusual balance between inhibitory and excitatory mechanisms in the nervous system.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 1 , Issue 4 , November 1974 , pp. 242 - 249
Copyright
Copyright © Canadian Neurological Sciences Federation 1974

References

REFERENCES

Adrian, E.D. and Matthews, B.H.C. (1934). The Berger Rhythm: Potential Changes from the Occipital Lobes in Man. Brain, 57: 355.CrossRefGoogle Scholar
Alajouanine, T., Nehlil, J., and Gabersek, V. (1959). A propos d’une cas d’epilepsie declenche par la lecture. Revue Neurologique, 101: 463–467.Google Scholar
Cernaceck, J. and Ciganek, L. (1962). The Cortical Electroencephalographic Response to Light Stimulation in Epilepsy. Epilepsia (Amst.), 3: 303–314.CrossRefGoogle Scholar
Cobb, W.A. and Dawson, (1960). The Form and Latency in Man of the Occipital Potentials Evoked by Bright Flashes. Journal of Physiology (London), 152: 108–121.CrossRefGoogle ScholarPubMed
Cobb, W.A., Ettlinger, G. and Morton, H.B. (1968). Cerebral Potentials Evoked in Man by Pattern Reversal and their Suppression in Visual Rivalry. Journal of Physiology (Lond.), 195: 33–34P.Google Scholar
Donchin, E. and Lindsley, D.B. (1966). Averaged evoked potentials and reaction times to visual stimuli. Electroencephalography and Clinical Neurophysiology, 20: 217–223.CrossRefGoogle ScholarPubMed
Dustman, R.E. and Beck, E.C. (1966). Visual Evoked Potentials. Amplitude Changes with Age. Science, 151:1013–1015.CrossRefGoogle Scholar
Dustman, R.E. and Beck, E.C. (1969). The Effects of Maturation on the Wave Form of Visually Evoked Potentials. Electroencephalography and Clinical Neurophysiology, 26: 2–11.CrossRefGoogle ScholarPubMed
Eccles, J.C. (1969). Excitatory and Inhibatory Mechanisms in the Brain. In Jasper, H.H.et al. (Eds.), Basic Mechanism of the Epilepsies. Little, Brown, Boston: 229–252.Google Scholar
Fenwick, P.B.C. and Dollimore, J. (1969). Mathematical Model Providing a Synthesis between Evoked and Spontaneous Phenomena of the EEG. Electroencephalography and Clinical Neurophysiology. Supp. 2: 60.Google ScholarPubMed
Forland, M. (1962). Cleido-Cranial Dysostosis. American Journal of Medicine, 33: 792–799.CrossRefGoogle Scholar
Giblin, D.R. (1964). Somatosensory Evoked Potentials in Healthy Subjects and in Patients with Lesions of the Nervous System. Annals of the New York Academy of Science, 112: 93–142.CrossRefGoogle ScholarPubMed
Grusser, O.J. and Creutzfeldt, O. (1957). Maxima der Impulsfrequenz Retinaler und Corticaler Neurone bei Flimmerlight Mittlerer Frequenzen Pflugers Arch. Ges. Physiol., 263: 668–681.Google Scholar
Grusser, O.J. and Rebelo, C. (1959). Die Wirkung von Flimmerreizen mit Lichtbutzen an Einselnen Corticalen Neuronen. I.V. Int. EEG Congr., Brussels. Pergamon, London, 371–375.Google Scholar
Halliday, A.M. (1965). Cerebral Evoked Potentials in Familial Progressive Myoclonus Epilepsy. Journal Royal College of Physicians, London, 1: 123–134.Google Scholar
Hirsch, J.F., Pertuiset, B., Calvet, J., Buisson-Ferey, J., Fischgold, H., et Scherrer, J. (1961). Etude des responses electrocorticales obtenues chez l’homme par des stimulations somesthetiques et visuelles. Electroencephalography and Clinical Neurophysiology, 13: 411–424.CrossRefGoogle Scholar
Hubel, D.H. and Wiesel, T.N. (1971). Receptive Fields of Convergence and Accommodation on Visually Evoked Cortical Responses and Visual Acuity. Electroencephalography and Clinical Neurophysiology, 31: 287–288.Google Scholar
Jasper, J., Lende, R. and Rasmussen, T. (1960). Evoked Potentials from the Exposed Somatosensory Cortex in Man. Journal of Nervous and Mental Diseases, 130: 526–537.CrossRefGoogle ScholarPubMed
Jonkmann, E.J. (1967). The Average Cortical Response to Photic Stimulation. Doctoral Thesis. University of Amsterdam, 160p.Google Scholar
Jung, R., and Tonnies, J.F. (1950). Hirnelektrische Untersuchung uber Enstehung und Erhaltung von Krampfent-ladungen. Arch. Psychiat. Nervenkr., 185: 701–735.CrossRefGoogle Scholar
Katzman, R. (1964). The Validity of the Visual Evoked Response in Man. Annals of the New York Academy of Science, 112: 238–240.CrossRefGoogle ScholarPubMed
Koloh, L.G. and Osselton, J.W. (1966). Clinical Electroencephalography. 2nd Edition, London. Butterworths.Google Scholar
Kooi, K.A. and Bagchi, B.K. (1964). Visual Evoked Responses in Man: Normative Data. Annals of the New York Academy of Science, 112: 254–269.CrossRefGoogle ScholarPubMed
Kuhnt, U. and Creutzfeldt, D.D. (1971). Decreased Postsynaptic Inhibition in the Visual Cortex during Flicker Stimulation. Electroencephalography and Clinical Neurophysiology, 30: 79–82.CrossRefGoogle ScholarPubMed
Leissner, P., Lindholm, L.E., and Petersen, I. (1970). Alpha Amplitude Dependence on Skull Thickness as Measured by Ultrasound Technique. Electroencephalography and Clinical Neurophysiology, 29: 392–399.CrossRefGoogle ScholarPubMed
Levin, E.J., Sonnenschein, H. (1963). Cleido-Cranial-Dysostosis. New York State Journal of Medicine, 63: 1562–1566.Google Scholar
Lucking, C.H., Creutzfeldt, O.D. and Heinemann, U. (1970). Visual Evoked Potentials of Patients with Epilepsy and of a Control Group. Electroencephalography and Clinical Neurophysiology, 29: 557–566.CrossRefGoogle ScholarPubMed
Morocutti, C., Sommer-Smith, J. and Creutzfeldt, O.D. (1966). Das Visuelle Reaktionspontential bei Normalen Versuchspersonen und Characteristische Veranderungen bei Epileptikern. Archives Psychiat., Nervenkr. 208: 234–254.CrossRefGoogle Scholar
Pampiglione, G. and Lehovsky, M. (1968). The Evolution of EEG Features in 26 Children with Proven Neuronal Lipidosis. Electroencephalography and Clinical Neurophysiology, 25: 508.Google ScholarPubMed
Penrose, L.S. (1963). The Biology of Mental Defect. Published by Sidgwick and Jackson, London.Google Scholar
Reiger, H., Lesèvre, N., Rémond, A. and Lairy, G.D. (1970). Responses evo–quees visuelles chez les enfants voyants et mal noyants. Electroencephalography and Clinical Neurophysiology, 28: 437–448.CrossRefGoogle Scholar
Rémond, A., Lesèvre, N. and Torres, F. (1965). Etude Chronotoporgraphique de l’activite occipitale moyenne receullie sur le scalp chez l’homme en relation avec les deplacements due regard (complex Lambda). Revue Neurologique, 113: 193–226.Google Scholar
Rhodes, L.E., Dustman, R.E. and Beck, E.C. (1969). The Visual Evoked Response: A Comparison of Bright and Dull Children. Electroencephalography and Clinical Neurophysiology, 27: 364–372.CrossRefGoogle Scholar
Richards, B.W. (1969). Personal Communication.CrossRefGoogle Scholar
Russell Harter, M. and Salmon, L.E. (1971). Effects of Convergence and Accommodation on Visually Evoked Responses and Visual Acuity. Electroencephalography and Clinical Neurophysiology, 31: 287–288.Google Scholar
Vitova, Z. and Hrbek, A. (1970). Ontogeny of Photic Driving in Infants. Electroencephalography and Clinical Neurophysiology, 28: 391–398.Google Scholar
Walter, W.G. (1950). The Functions of Electrical Rythm in the Brain. Journal of Mental Science, 96: 1.CrossRefGoogle Scholar
Walter, W.G. (1951). The Effect of Physical Stimuli on the EEG. Electroencephalography and Clinical Neurophysiology, Supp. 2, 60.Google Scholar
Warkany, J., Kirkpatrick, J.A. (1969) in Textbook of Pediatrics. Nelson, W.E., Vaughan, V.C, Mckay, R.J., Saunders, W.B., Toronto.Google Scholar
Weinman, H., Creutzfeldt, O.D. and Heyde, G. (1965). Die Entwicklung der Visuellen Reizantwort bei Kindern. Archives Psychiat. Nervenkr, 207: 323–341.CrossRefGoogle Scholar