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Chronic Decerebrate State in Infancy: Neurologic Observations in Long Surviving Cases of Hydranencephaly

Chronic Decerebrate State in Infancy: Neurologic Observations in Long Surviving Cases of... Abstract THE TERM hydranencephaly designates the condition in which large portions of the cerebral hemispheres have been reduced to membranous sacs within a cranium of relatively normal size. It may be suspected in the newborn by cranial transillumination, which generally indicates a cerebral thickness of less than 1 cm.1,2 Although this finding implies that such infants have varying levels of decerebration or decortication in a state sometimes permitting long survival, little attention has been paid to the clinical-physiologic features of hydranencephaly and related disorders with severe bilateral cerebral destruction or malformation. Despite many studies of the behavior of the normal newborn3-9 and also of the variable sequences of changes in common spastic diplegia10-12 little can be found concerning the neuroanatomic basis of such common neonatal activity as the Moro and the stepping reflexes. Moreover there are discrepancies between these studies and those in the experimental neurophysiologic literature on References 1. Alexander, E.; Davis, C.H., Jr.; and Kitahata, L.M.: Hydranencephaly: Observations on Transillumination of the Head of Infants , Arch Neurol Psychiat 76:578-584 ( (Dec) ) 1956.Crossref 2. Dodge, P.R., and Porter, P.: Demonstration of Intracranial Pathology by Transillumination , Arch Neurol 5:594-605 ( (Dec) ) 1961.Crossref 3. Peiper, A.: Cerebral Function in Infancy and Childhood , ed 3, Nagler, B., and Nagler, H. (trans) New York: Consultants Bureau, Enterprises, Inc., 1963. 4. Dubowitz, V.: Asymmetrical Moro Response in Neurologically Normal Infants , Develop Med Child Neurol 7:244-248 ( (June) ) 1965.Crossref 5. Mitchell, R.G.: The Moro Reflex , Cerebral Palsy Bull 2:135-142 ( (June) ) 1960. 6. André-Thomás, and Hanon, F.: De la naissance á la march; quelques modes d'activité du nouveau né , Presse Med 56:229-230 ( (March) ) 1948. 7. Paine, R.S.: Neurologic Examination of Infants and Children , Pediat Clin N Amer 7:471-510 ( (Aug) ) 1960. 8. Paine, R.S.: Evolution of Postural Reflexes in Normal Infants and in the Presence of Chronic Brain Syndromes , Neurology 14:1036-1048 ( (Nov) ) 1964.Crossref 9. Yang, D.C.: Neurologic Status of the Newborn Infants on the First and Third Day of Life , Neurology 12:72-77 ( (Jan) ) 1962.Crossref 10. Paine, R.S.: The Evolution of Infantile Postural Reflexes in the Presence of Chronic Brain Syndromes , Develop Med Child Neurol 6:345-361 ( (Aug) ) 1964.Crossref 11. Bobath, K., and Bobath, B.: Diagnosis of Cerebral Palsy in Infancy , Arch Dis Child 31:408-414 ( (Oct) ) 1956.Crossref 12. Crothers, B., and Paine, R.S.: The Natural History of Cerebral Palsy , London: Oxford University Press, Inc., 1959. 13. Rushworth, G., and Denny-Brown, D.: The Two Components of the Grasp Reflex After Ablation of Frontal Cortex in Monkeys , J Neurol Neurosurg Psychiat 22:91-98 ( (May) ) 1959.Crossref 14. Seyffarth, H., and Denny-Brown, D.: The Grasp Reflex and the Instinctive Grasp Reaction , Brain 71:109-183 ( (Nov) ) 1948.Crossref 15. Twitchell, T.E.: The Automatic Grasping Response of Infants , Neuropsychological 3:247-259 ( (Aug) ) 1965.Crossref 16. Kennard, M.D., and Kessler, M.M.: Studies of Motor Performance After Parietal Ablations in Monkeys , J Neurophysiol 3:248-257 ( (May) ) 1940. 17. Peele, T.L.: Acute and Chronic Parietal Lobe Ablations in Monkeys , J Neurophysiol 7:269-286 ( (Sept) ) 1944. 18. Woolsey, C.N., and Bard, P.: Cortical Control of Placing and Hopping Reactions in Macaca Mulatta , Amer J Physiol 116:165 ( (June) ) 1936. 19. Denny-Brown, D., and Chambers, R.A.: " The Parietal Lobe and Behavior ," in Solomon, H.C.; Cobb, S.; and Penfield, W. (eds.): Research Publications: Associations for Research in Nervous and Mental Disease , Baltimore: Williams & Wilkins Co., 1958, vol 36, pp 35-117. 20. Yakovlev, P.I.: Pathoarchitectonic Studies of Cerebral Malformations: III. Arhinencephalies (Holotelencephalies) , J Neuropath Exper Neurol 18:22-55 ( (Jan) ) 1959.Crossref 21. Marburg, O., and Mettler, F.A.: The Diencephalon and Telencephalon in a Human Case of Cyclopia and Arhinia , J Neuropath Exp Neurol 2:111-131 ( (April) ) 1943.Crossref 22. DeMyer, W.; Zeman, W.; and Palmer, C.: The Face Predicts the Brain: Diagnostic Significance of Median Facial Anomalies for Holoprosencephaly (Arhinencephaly) , Pediatrics 34:256-263 ( (Aug) ) 1964. 23. Demyer, W., and White, P.T.: EEG in Holoprosencephaly (Arhinencephaly) , Arch Neurol 11:507-520 ( (Nov) ) 1964.Crossref 24. André-Thomás; Lepage F.; and Sorrel-Dejerine: Examen Anatomo-Clinique de deux anéncephales protubérantiels , Rev Neurol 76:173-193 ( (July) ) 1944. 25. Yakovlev, P.I., and Wadsworth, R.C.: Schizencephalies: A Study of the Congenital Clefts in the Cerebral Mantle: II. Clefts With Hydrocephalus and Lips Separated , J Neuropath Exper Neurol 5:169-206 ( (July) ) 1946.Crossref 26. Denny-Brown, D.: Positive and Negative Aspects of Cortical Function , N Carolina Med J 17:295-303 ( (July) ) 1956. 27. Denny-Brown, D.: " Motor Mechanisms—Introduction: The General Principles of Motor Integration ," in Fields, J.; Magoun, H.W.; and Hall, V.E. (eds.): Handbook of Physiology , Washington: American Physiological Society vol II. 28. Fulton, J.F.: Forced Grasping and Groping in Relation to the Syndrome of the Premotor area , Arch Neurol Psychiat 31:221-235 ( (Feb) ) 1934.Crossref 29. André-Thomás, and de Ajuriaguerra, J.: " Etude anatomo-clinique de l'anencephalie ," in Heuyer, G.; Feld, M.; and Gruner, J. (eds.): Malformations Congenitales du Cerveau , Paris: Masson, 1959. 30. Rushworth, G.: On Postural and Righting Reflexes , Cereb Palsy Bull 3:535-543 ( (Dec) ) 1961. 31. Creed, R.S., et al: Reflex Activity of the Spinal Cord , Oxford: Clarendon Press, 1932. 32. Melchior, J.C., and Tygstrup, I.: Development of Spinal Paraplegia After Breech Presentation , Acta Paediat 52:171-176 ( (March) ) 1963.Crossref 33. Kuhn, R.A., and Macht, M.B.: Some Manifestations of Reflex Activity in Spinal Man With Particular Reference to the Occurrence of Extensor Spasms , Johns Hopkins Med J 84:43-75 ( (Jan) ) 1948. 34. Denny-Brown, D.: The Midbrain and Motor Integration , Proc Roy Soc Med 55:527-538 ( (July) ) 1962. 35. Denny-Brown, D., and Gillman, S.: Dystonic Postures in Relation to Various Levels of Decerebration , Trans Amer Neurol Assoc , 1966. 36. Denny-Brown, D.: The Basal Ganglia , London: Oxford University Press, 1983. 37. Denny-Brown, D.: The Cerebral Control of Movement , Springfield, Ill: Charles C Thomas, Publishers, 1966. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology American Medical Association

Chronic Decerebrate State in Infancy: Neurologic Observations in Long Surviving Cases of Hydranencephaly

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References (46)

Publisher
American Medical Association
Copyright
Copyright © 1968 American Medical Association. All Rights Reserved.
ISSN
0003-9942
eISSN
1538-3687
DOI
10.1001/archneur.1968.00480030117014
Publisher site
See Article on Publisher Site

Abstract

Abstract THE TERM hydranencephaly designates the condition in which large portions of the cerebral hemispheres have been reduced to membranous sacs within a cranium of relatively normal size. It may be suspected in the newborn by cranial transillumination, which generally indicates a cerebral thickness of less than 1 cm.1,2 Although this finding implies that such infants have varying levels of decerebration or decortication in a state sometimes permitting long survival, little attention has been paid to the clinical-physiologic features of hydranencephaly and related disorders with severe bilateral cerebral destruction or malformation. Despite many studies of the behavior of the normal newborn3-9 and also of the variable sequences of changes in common spastic diplegia10-12 little can be found concerning the neuroanatomic basis of such common neonatal activity as the Moro and the stepping reflexes. Moreover there are discrepancies between these studies and those in the experimental neurophysiologic literature on References 1. Alexander, E.; Davis, C.H., Jr.; and Kitahata, L.M.: Hydranencephaly: Observations on Transillumination of the Head of Infants , Arch Neurol Psychiat 76:578-584 ( (Dec) ) 1956.Crossref 2. Dodge, P.R., and Porter, P.: Demonstration of Intracranial Pathology by Transillumination , Arch Neurol 5:594-605 ( (Dec) ) 1961.Crossref 3. Peiper, A.: Cerebral Function in Infancy and Childhood , ed 3, Nagler, B., and Nagler, H. (trans) New York: Consultants Bureau, Enterprises, Inc., 1963. 4. Dubowitz, V.: Asymmetrical Moro Response in Neurologically Normal Infants , Develop Med Child Neurol 7:244-248 ( (June) ) 1965.Crossref 5. Mitchell, R.G.: The Moro Reflex , Cerebral Palsy Bull 2:135-142 ( (June) ) 1960. 6. André-Thomás, and Hanon, F.: De la naissance á la march; quelques modes d'activité du nouveau né , Presse Med 56:229-230 ( (March) ) 1948. 7. Paine, R.S.: Neurologic Examination of Infants and Children , Pediat Clin N Amer 7:471-510 ( (Aug) ) 1960. 8. Paine, R.S.: Evolution of Postural Reflexes in Normal Infants and in the Presence of Chronic Brain Syndromes , Neurology 14:1036-1048 ( (Nov) ) 1964.Crossref 9. Yang, D.C.: Neurologic Status of the Newborn Infants on the First and Third Day of Life , Neurology 12:72-77 ( (Jan) ) 1962.Crossref 10. Paine, R.S.: The Evolution of Infantile Postural Reflexes in the Presence of Chronic Brain Syndromes , Develop Med Child Neurol 6:345-361 ( (Aug) ) 1964.Crossref 11. Bobath, K., and Bobath, B.: Diagnosis of Cerebral Palsy in Infancy , Arch Dis Child 31:408-414 ( (Oct) ) 1956.Crossref 12. Crothers, B., and Paine, R.S.: The Natural History of Cerebral Palsy , London: Oxford University Press, Inc., 1959. 13. Rushworth, G., and Denny-Brown, D.: The Two Components of the Grasp Reflex After Ablation of Frontal Cortex in Monkeys , J Neurol Neurosurg Psychiat 22:91-98 ( (May) ) 1959.Crossref 14. Seyffarth, H., and Denny-Brown, D.: The Grasp Reflex and the Instinctive Grasp Reaction , Brain 71:109-183 ( (Nov) ) 1948.Crossref 15. Twitchell, T.E.: The Automatic Grasping Response of Infants , Neuropsychological 3:247-259 ( (Aug) ) 1965.Crossref 16. Kennard, M.D., and Kessler, M.M.: Studies of Motor Performance After Parietal Ablations in Monkeys , J Neurophysiol 3:248-257 ( (May) ) 1940. 17. Peele, T.L.: Acute and Chronic Parietal Lobe Ablations in Monkeys , J Neurophysiol 7:269-286 ( (Sept) ) 1944. 18. Woolsey, C.N., and Bard, P.: Cortical Control of Placing and Hopping Reactions in Macaca Mulatta , Amer J Physiol 116:165 ( (June) ) 1936. 19. Denny-Brown, D., and Chambers, R.A.: " The Parietal Lobe and Behavior ," in Solomon, H.C.; Cobb, S.; and Penfield, W. (eds.): Research Publications: Associations for Research in Nervous and Mental Disease , Baltimore: Williams & Wilkins Co., 1958, vol 36, pp 35-117. 20. Yakovlev, P.I.: Pathoarchitectonic Studies of Cerebral Malformations: III. Arhinencephalies (Holotelencephalies) , J Neuropath Exper Neurol 18:22-55 ( (Jan) ) 1959.Crossref 21. Marburg, O., and Mettler, F.A.: The Diencephalon and Telencephalon in a Human Case of Cyclopia and Arhinia , J Neuropath Exp Neurol 2:111-131 ( (April) ) 1943.Crossref 22. DeMyer, W.; Zeman, W.; and Palmer, C.: The Face Predicts the Brain: Diagnostic Significance of Median Facial Anomalies for Holoprosencephaly (Arhinencephaly) , Pediatrics 34:256-263 ( (Aug) ) 1964. 23. Demyer, W., and White, P.T.: EEG in Holoprosencephaly (Arhinencephaly) , Arch Neurol 11:507-520 ( (Nov) ) 1964.Crossref 24. André-Thomás; Lepage F.; and Sorrel-Dejerine: Examen Anatomo-Clinique de deux anéncephales protubérantiels , Rev Neurol 76:173-193 ( (July) ) 1944. 25. Yakovlev, P.I., and Wadsworth, R.C.: Schizencephalies: A Study of the Congenital Clefts in the Cerebral Mantle: II. Clefts With Hydrocephalus and Lips Separated , J Neuropath Exper Neurol 5:169-206 ( (July) ) 1946.Crossref 26. Denny-Brown, D.: Positive and Negative Aspects of Cortical Function , N Carolina Med J 17:295-303 ( (July) ) 1956. 27. Denny-Brown, D.: " Motor Mechanisms—Introduction: The General Principles of Motor Integration ," in Fields, J.; Magoun, H.W.; and Hall, V.E. (eds.): Handbook of Physiology , Washington: American Physiological Society vol II. 28. Fulton, J.F.: Forced Grasping and Groping in Relation to the Syndrome of the Premotor area , Arch Neurol Psychiat 31:221-235 ( (Feb) ) 1934.Crossref 29. André-Thomás, and de Ajuriaguerra, J.: " Etude anatomo-clinique de l'anencephalie ," in Heuyer, G.; Feld, M.; and Gruner, J. (eds.): Malformations Congenitales du Cerveau , Paris: Masson, 1959. 30. Rushworth, G.: On Postural and Righting Reflexes , Cereb Palsy Bull 3:535-543 ( (Dec) ) 1961. 31. Creed, R.S., et al: Reflex Activity of the Spinal Cord , Oxford: Clarendon Press, 1932. 32. Melchior, J.C., and Tygstrup, I.: Development of Spinal Paraplegia After Breech Presentation , Acta Paediat 52:171-176 ( (March) ) 1963.Crossref 33. Kuhn, R.A., and Macht, M.B.: Some Manifestations of Reflex Activity in Spinal Man With Particular Reference to the Occurrence of Extensor Spasms , Johns Hopkins Med J 84:43-75 ( (Jan) ) 1948. 34. Denny-Brown, D.: The Midbrain and Motor Integration , Proc Roy Soc Med 55:527-538 ( (July) ) 1962. 35. Denny-Brown, D., and Gillman, S.: Dystonic Postures in Relation to Various Levels of Decerebration , Trans Amer Neurol Assoc , 1966. 36. Denny-Brown, D.: The Basal Ganglia , London: Oxford University Press, 1983. 37. Denny-Brown, D.: The Cerebral Control of Movement , Springfield, Ill: Charles C Thomas, Publishers, 1966.

Journal

Archives of NeurologyAmerican Medical Association

Published: Sep 1, 1968

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