Table Of ContentU C L A F O R U M I N M E D I C A L S C I E N C E S
EDITORIAL BOARD
Mary A. B. Brazier, Editor-in-Chief
Nathaniel A. Buchwald Carl M. Pearson
Carmine D. Clemente Charles H. Sawyer
Louise M. Darling Emil L. Smith
John L. Fahey Reidar F. Sognnaes
Victor E. Hall W. Eugene Stern
James V. Maloney Bradley R. Straatsma
Emery Zimmerman
U C L A F O R U M IN M E D I C A L S C I E N C E S
N U M B E R 18
Brain Mechanisms in
Mental Retardation
EDITED BY
N A T H A N I E L A . B U C H W A L D
Mental Retardation Research Center, Brain Research Institute, and
Departments of Anatomy and Psychiatry
University of California Los Angeles
Los Angeles, California
M A R Y A . B . B R A Z I E R
Brain Research Institute
University of California Los Angeles
Los Angeles, California
Proceedings of a Conference in the Series on Mental Retardation
Sponsored by The National Institute of Child Health and Human Development
Mental Retardation Research Centers Series
A C A D E M I C P R E S S N e w Y o r k S a n Franc i sco L o n d o n 1975
A Subsidiary of Harcourt Brace Jovanovich, Publishers
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NO PART OF THIS PUBLICATION MAY B E REPRODUCED OR
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A C A D E M I C P R E S S , I N C .
I l l Fifth Avenue, New York, New York 10003
United Kingdom Edition published by
A C A D E M I C P R E S S , I N C . ( L O N D O N ) L T D .
24/28 Oval Road, London NW1
Library of Congress Cataloging in Publication Data
Main entry under title:
Brain mechanisms in mental retardation.
(UCLA forum in medical sciences ; v. 18)
Includes bibliographies and indexes.
1. Mental deficiency-Congresses. 2. Brain-
Congresses. I. Buchwald, Nathaniel A. II. Brazier,
Mary Agnes Burniston, (date) III. United States.
National Institute of Child Health and Human Develop-
ment. IV. Series. [DNLM: 1. Mental retardation-
Physiopathology-Congresses. 2. Neurophysiology-
Congresses. W3 U17 no. 18 1974 / WM300 B8145 1974]
RC570.B72 616.8 '58 75*3581
ISBN 0 - 1 2 - 1 3 9 0 5 0 - X
PRINTED IN THE UNITED STATES OF AMERICA
List of Contributors and Participants
Names of contributors to this volume are marked with an asterisk.
*Anthony M. Adinolfi, Mental Retardation Research Center and Neuropsychiatric Insti-
tute, University of California Los Angeles, Los Angeles, California 90024
*Joseph Altaian, Department of Biological Sciences, Purdue University, West Lafayette,
Indiana 47906
*June E. Ayling, Mental Retardation Research Center and Department of Biological Chem-
istry, University of California Los Angeles, Los Angeles, California 90024
*Robert E. Barrett, Department of Neurology, College of Physicians and Surgeons,
Columbia University, New York, New York 10032
*John Blass, Mental Retardation Research Center, University of California Los Angeles, Los
Angeles, California 90024
Mary A. B. Brazier, Editor for the UCLA Forum in Medical Sciences, Brain Research
Institute, University of California Los Angeles, Los Angeles, California 90024
*G. A. M. Breen, 1 Mental Retardation Research Center and Department of Anatomy,
University of California Los Angeles, Los Angeles, California 90024
^Jennifer S. Buchwald, Mental Retardation Research Center and Department of Phys-
iology, School of Medicine, University of California Los Angeles, Los Angeles,
California 90024
*N. A. Buchwald, Mental Retardation Research Center, Brain Research Institute, and
Departments of Anatomy and Psychiatry, University of California Los Angeles, Los
Angeles, California 90024
*Gerald Cohen, Department of Neurology, College of Physicians and Surgeons, Columbia
University, New York, New York 10032
*Lucien Cote, Department of Neurology, College of Physicians and Surgeons, Columbia
University, New York, New York 10032
1 Present address; Molecular Biology Department, Roswell Park Memorial Institute,
Buffalo, New York 14203.
xi
xii List of Contributors and Participants
*J. de Vellis, Mental Retardation Research Center and Department of Anatomy, University
of California Los Angeles, Los Angeles, California 90024
2
*Marc A. Dichter, Department of Medicine, Beth Israel Hospital, Boston, Massachu-
setts 02215
*John Dobbing, Department of Child Health, University of Manchester, Manchester,
England
*Albert Dorfman, Joseph P. Kennedy, Jr. , Mental Retardation Research Center,
Department of Pediatrics, Pritzker School of Medicine, The University of Chicago,
Chicago, Illinois 60637
*Philip E. Duffy, Department of Neurology, College of Physicians and Surgeons,
Columbia University, New York, New York 10032
*D. R. G. Fuller, Mental Retardation Research Center, University of California Los
Angeles, Los Angeles, California 90024
*John Garcia, Department of Psychology, University of California Los Angeles, Los
Angeles, California 90024
*Patricia S. Goldman, Laboratory of Psychology and Psychopathology, National Institute
of Mental Health, Bethesda, Maryland 20014
*Richard Heikkila, Department of Neurology, College of Physicians and Surgeons,
Columbia University, New York, New York 10032
*Alfred Heller, Department of Pharmacological and Physiological Sciences, The
University of Chicago, Chicago, Illinois 60637
*Philip C. Hoffmann, Department of Pharmacological and Physiological Sciences, The
University of Chicago, Chicago, Illinois 60637
^Chester D. Hull, Mental Retardation Research Center, Brain Research Institute, and
Departments of Anatomy and Psychiatry, University of California Los Angeles, Los
Angeles, California 90024
*Peter R. Huttenlocher, Department of Pediatrics, The University of Chicago, Chicago,
Illinois 60637
David L. Joftes, National Institute of Child Health and Human Development, Bethesda,
Maryland 20014
*Seymour Kaufman, Laboratory of Neurochemistry, National Institute of Mental Health,
Bethesda, Maryland 20014
*Hayato Kihara, The Neuropsychiatric Institute, Pacific State Hospital, Research Group,
Pomona, California
*Anne Kitsikis, Department of Physiology, School of Medicine, Laval University,
University City, Quebec, Canada
James L. Lessard, Children's Hospital Medical Center, Cincinnati, Ohio
*M. S. Levine, Department of Psychiatry, University of California Los Angeles, Los
Angeles, California 90024
*Gary Lynch, Department of Psychobiology, University of California Irvine, Irvine,
California 92664
*J. F. McGinnis, Mental Retardation Research Center and Department of Anatomy,
University of California Los Angeles, Los Angeles, California 90024
*Luis Marco, Department of Neurology, College of Physicians and Surgeons, Columbia
University, New York, New York 10032
*Robert J . Marcus, Mental Retardation Research Center and Department of Psychiatry,
University of California Los Angeles, Los Angeles, California 90024
2
Present address: Department of Neurology, Beth Israel Hospital, Boston, Massachusetts
02215.
List of Contributors and Participants xiii
^Catherine Mytilineou, Department of Neurology, College of Physicians and Surgeons,
Columbia University, New York, New York 10032
"James Olds, Division of Biology, California Institute of Technology, Pasadena, California
91109
*George Popjak, Mental Retardation Research Center and Department of Biochemistry,
University of California Los Angeles, Los Angeles, California 90024
*Dominiek P. Purpura, Rose F. Kennedy Center for Research in Mental Retardation and
Human Development, Albert Einstein College of Medicine, Bronx, New York 10461
*Pasko Rakic, Department of Neuropathology, Harvard Medical School, and Department
of Neuroscience, Children's Hospital Medical Center, Boston, Massachusetts 02115
*Guenter Rose, Mental Retardation Research Center, University of California Los Angeles,
Los Angeles, California 90024
Fred E. Samson, Ralph L. Smith Mental Retardation Research Center, University of
Kansas, Kansas City, Kansas
Richard J . Schain, Mental Retardation Research Center, University of California Los
Angeles, Los Angeles, California 90024
*S. Stefan Soltysik, Mental Retardation Research Center, University of California Los
Angeles, Los Angeles, California 90024
*John F. Tallman, Developmental and Metabolic Neurology Branch, National Institutes of
Health, Bethesda, Maryland 20014
George Tarjan, Director of Mental Retardation and Child Psychiatry Program, Neuro-
psychiatry Institute, University of California Los Angeles, Los Angeles, California 90024
3
T h o m a s A. Tedesco, Department of Human Genetics, University of Pennsylvania School
of Medicine, Philadelphia, Pennsylvania 19104
""Virginia M. Tennyson, Department of Pathology, Division of Neuropathology, and
Department of Neurology, College of Physicians and Surgeons, Columbia University,
New York, New York 10032
*Jaime R. Villablanca, Mental Retardation Research Center and Department of Psychi-
atry, University of California Los Angeles, Los Angeles, California 90024
Lesnick E. Westrum, Child Development and Mental Retardation Center, University of
Washington, Seattle, Washington
*Charles D. Woody, Mental Retardation Research Center, University of California Los
Angeles, Los Angeles, California 90024
Stephen Zamenhof, Department of Microbiology and Immunology, University of Cali-
fornia Los Angeles, Los Angeles, California 90024
3
Present address: Department of Pediatrics, University of South Florida, College of
Medicine, Tampa, Florida 33620.
Foreword
Th i s v o l u m e has b e e n sponsored jo in t ly b y the Men ta l Retarda t ion
Program of the Nat iona l Ins t i tu te of Chi ld Heal th and H u m a n Deve l -
o p m e n t and b y the Un ive r s i t y of Cal i fornia Los Ange l e s F o r u m in M e d -
ical Sc i ences . For the N . I . C . H . D . Men ta l Re ta rda t ion Prog ram, it is the
fourth b o o k b a s e d on conferences organ ized b y one of the twelve
Menta l Re ta rda t ion Resea rch Cen te r s . T h e staff of the Men ta l Retarda-
t ion Program has enthus ias t ica l ly suppor ted the Cen te r s ' Resea rch
Ser ies vo lumes . In part icular , Dr. Theodo re T j o s s e m , Direc tor of the
Menta l Re ta rda t ion Prog ram, Dr . M i c h a e l J . B e g a b , H e a d , Menta l R e -
tardat ion Resea rch Cen te r s , and Dr . D a v i d Jof tes , B i o m e d i c a l Sc i ences
Admin is t ra to r , prov ided e n c o u r a g e m e n t and suppor t for th is vo lume .
Dr . Joftes , in addi t ion , devoted m u c h hard work prior to and dur ing
the conference w h i c h prov ided the mater ia l for our b o o k .
T h e Menta l Re ta rda t ion Resea rch Cente rs have b e e n set up at major
univers i t i es th roughou t the U n i t e d Sta tes . T h e y w e r e des igned to con-
duct research direc ted toward an unde r s t and ing of menta l retardat ion
and related aspects of h u m a n deve lopment . T h e Menta l Retarda t ion
Research Cente r at U C L A is a mul t id i sc ip l inary organiza t ion w h o s e
interes ts extend from anthropolog ica l and sociologica l inves t iga t ions
of cross-cul tural aspects of men ta l retardat ion to molecular chemica l
studies of m e m b r a n e p h e n o m e n a related to metabo l i c def ic iencies
w h i c h under l ie the m o s t severe cogni t ive abnormal i t i e s . T h e U C L A
Cente r is h o u s e d in a four-story addi t ion to the preexis t ing U C L A
Neuropsych ia t r i c Ins t i tu te . It has an impor tan t adjunct research group
at Pacific State Hospi ta l in P o m o n a , Cal i fornia , w h e r e ep idemio log ica l
xiv
Foreword xv
s tudies of men ta l re tardat ion and b i o c h e m i c a l and neurophys io log ica l
research , requ i r ing the p re sence of a popula t ion of re tarded pat ien ts ,
are carr ied out. T h e Resea rch Cen te r is closely a l igned wi th a sister
un i t devo ted to cl inical aspec ts of menta l re tardat ion and to interd is -
c ip l inary t ra in ing of profess ionals in the field. T h e Cl in ica l U n i t and
the Resea rch Cen te r compr i s e a broad ly b a s e d Men ta l Reta rda t ion Pro-
gram at U C L A . T h i s p rog ram, founded and organ ized u n d e r the lead-
ersh ip of Dr . G e o r g e Tar jan , represen t s U C L A ' s approach to the un-
ders tand ing , preven t ion , and trea tment of the cogni t ive def ic iencies
w h i c h afflict several mi l l ions of A m e r i c a n c i t i zens .
T h i s v o l u m e is the ou tg rowth of a confe rence he ld at O x n a r d , Cali -
fornia , in January , 1974 . T h e pu rpose of th is confe rence was to presen t
and to discuss neurob io log ica l f indings w h i c h m i g h t lead to an under -
s tand ing of b a s i c processes under ly ing the p h e n o m e n a of menta l
defic iency. M o s t of the data repor ted at O x n a r d were der ived from
labora tory researches . H o w e v e r , several of the speakers , all wi th long
his to r ies of a c c o m p l i s h m e n t in the labora tory , concen t ra ted thei r pre-
sen ta t ions on research on h u m a n b e i n g s or examina t ion of h u m a n
pathologica l mater ia l .
T h e c osponso r of this v o l u m e , the U C L A F o r u m in Medica l Sc i -
e n c e s , w a s set up b y Dr. S h e r m a n Mell inkoff , D e a n of the S c h o o l of
M e d i c i n e , to enab le pub l i ca t ion of research mater ia l reflective of the
var ie ty of inves t iga t ive efforts at the U C L A Cen te r for the Heal th Sc i -
ences . T h i s is the e igh teen th v o l u m e in the F o r u m Ser i e s . W e are
grateful to D e a n Mel l inkof f and to the Edi tor ia l Boa rd of the F o r u m for
thei r part in the produc t ion of th is b o o k . M o s t especia l ly , thanks go to
Doc to r M a r y A. B . Braz ie r , the efficient and unde r s t and ing Edi tor - in-
C h i e f of the U C L A forum, for he r e n c o u r a g e m e n t and exper t i se dur ing
the p lann ing and carrying out of the ent i re project .
NATHANIEL A. BUCHWALD
G E O R G E T A R J A N
Professor of Psychiatry and Director of Mental Retardation
and Child Psychiatry Program, Neuropsychiatric Institute
University of California Los Angeles
Los Angeles, California
Welcoming Remarks
This confe rence seeks to e x a m i n e wha t w e k n o w , at presen t , abou t
the b ra in m e c h a n i s m s under ly ing menta l retardat ion. Progress in the
study of men ta l re tardat ion and h u m a n deve lopmen t has , of course ,
accelera ted marked ly s ince Congres s legis la ted the es t ab l i shmen t of
the Menta l Re ta rda t ion Resea rch Cen te r s . T h e s e Cente r s and the avail-
abi l i ty of research suppor t in deve lopmenta l b i o l o g y and chemis t ry for
sc ient i s t s not affiliated wi th the Cente rs have brough t a w h o l e n e w
popula t ion of researchers and a w h o l e n e w set of approaches to
studies i m p i n g i n g on p r o b l e m s of men ta l retardat ion. T h e excel lent
qual i ty of the workers at t racted to this field is a t tes ted to b y the peop le
represen ted at th is conference and b y the difficulty in c h o o s i n g t h e m
from the large pool of dedica ted researchers in the field.
T h e r e is an old idea abou t sc ien t i s t s b e i n g un in te re s t ed in the real
p r o b l e m s of the wor ld , the idea that sc i ence is good for its o w n sake ,
and that if it is useful , so m u c h the bet te r , bu t that uti l i ty is not a
requ i remen t . I don ' t th ink m o s t good sc ient i s t s accept this v i e w any-
more . T h e fact that so m a n y of you are devot ing your efforts to
prob lems of s igni f icance to h u m a n b e i n g s nega tes this idea . E v e n
m o r e impor tan t , I th ink , is the fact that you h a v e b e e n able to do so
wi thou t sacrif icing the level of your scientif ic efforts. L o w grade sc ien-
tific research , even i f specif ical ly a i m e d at a target such as men ta l re-
tardat ion, has little va lue . It is hea r t en ing , i n d e e d , that fine sc ient i s t s
have b e c o m e in te res ted in p r o b l e m s of men ta l re tarda t ion and related
aspects of deve lopmen t . I look forward to your confe rence and to the
t ime w h e n the resul ts of you r efforts wil l impac t u p o n the p r o b l e m s of
the menta l ly retarded.
GEORGE TARJAN
