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Neurological Assessment

from Birth to 6
Second Edition

Julie Gosselin
Claudine Amiel-Tison
Neurological Assessment
from Birth to 6 years
Second edition
From the same authors

Éditions Elsevier-Masson
Démarche clinique en neurologie du développement, par C. Amiel-Tison, J. Gos-
selin. 2004, 240 pages.
L’infirmité motrice d’origine cérébrale, par C. Amiel-Tison. 2005, 2e édition,
336 pages.
Neurologie périnatale, par C. Amiel-Tison. 2005, 3e édition, 320 pages.
Other publications :
Conduite du bilan neuropsychologique chez l’enfant, par M. Mazeau. 2003,
264 pages.
Électro-encéphalographie de l’enfant, par D. Samson-Dollfus. 2001, 2e édi-
tion, 152 pages.
Fœtus et nouveau-né de faible poids, par F. Gold. Collection de Périnatalité.
2000, 2e édition, 152 pages.
Neurologie pédiatrique, par P. Landrieu, M. Tardieu. Collection Abrégés de
Médecine. 2001, 2e édition, 208 pages.
Neuropédiatrie, par G. Lyon, P. Evrard. 2000, 2e édition, 568 pages.
Pédiatrie en maternité. Réanimation en salle de naissance, par F. Gold, C. Lionnet,
M.-H. Blond. Collection Abrégés de Périnatalité. 2002, 2e édition, 400
pages.
Soins aux nouveaux-nés. Avant, pendant et après la naissance, par J. Laugier,
J.-C. Rozé. 2006, 2e édition, 880 pages.
Soins intensifs et réanimation du nouveau-né, par F. Gold, Y. Aujard, M. Dehan
et coll. Collection de Périnatalité. 2006, 2e édition, 624 pages.
Urgences pédiatriques, par A. Bourrillon, G. Chéron. Collection Urgences.
2005, 3e édition, 752 pages.

Éditions du CHU Sainte-Justine

L’épilepsie chez l’enfant et l’adolescent, par A. Lortie, M. Vanasse et coll, 2006,


208 pages.
Les maladies neuromusculaires chez l’enfant et l’adolescent, par M. Vanasse, H.
Paré, Y. Brousseau, S. D’Arcy, 2004, 376 pages.
Urgences et soins intensifs pédiatriques, par J. Lacroix, M. Gauthier, P. Hubert,
F. Leclerc, P. Gaudreault et coll, en coédition avec Elsevier Masson, 2007,
1368 pages.
Les soins palliatifs pédiatriques, par N. Humbert et coll, 204, 656 pages.
Neurological Assessment
from Birth to 6 years
(Original French Version Published in 2007)
Second edition

Julie Gosselin
Claudine Amiel-Tison

Translated by Lesley Kelley-Régnier


Bibliothèque et Archives nationales du Québec and Library and Archives
Canada cataloguing in publication

Gosselin, Julie, 1961-


Neurological assessment from birth to 6 years [electronic resource]
2nd ed.
(Collection Intervenir)
Translation of: Évaluation neurologique de la naissance à 6 ans, 2e éd.
Includes bibliographical references and index.
ISBN 978-2-89619-447-6
1. Developmental neurobiology. 2. Nervous system - Growth - Evaluation. 3.
Nervous system - Diseases - Diagnosis. 4. Movement disorders in children - Dia-
gnosis. 5. Pediatric neurology. I. Amiel-Tison, Claudine. II. Title. III. Series:
Collection Intervenir.
QP363.5.A4413 2011 612.8 C2011-941578-X

Graphic design : Madeleine Leduc

Cover illustration : Annette Tison

Éditions du CHU Sainte-Justine


3175, chemin de la Côte-Sainte-Catherine
Montreal (Quebec) H3T 1C5
Phone : (514) 345-4671
Fax : (514) 345-4631
www.chu-sainte-justine.org/editions

© Éditions du CHU Sainte-Justine, 1998, 2007, 2011


ISBN 978-2-89619-447-6 (pdf format)
Table of contents

Preface 1st edition - Ann L. Stewart..................................... 7

Preface 2nd edition - Marilee C. Allen................................. 13

Introduction....................................................................... 17

Chapter 1 - Basic Notions Concerning Motor Activity


 Two systems of motor control: brainstem and
cerebral hemispheres............................................................ 21
 Maturation and neurological evaluation: three periods,
three tools........................................................................... 23
 Perinatal hypoxic-ischemic injury: predominantly hemispheric. 27
 Passive muscle tone: physiological and pathological aspects...... 28
 Motor activity..................................................................... 37
 Interpretation difficulties related to muscle shortening............. 40
 Missing signs related to young age......................................... 42
 Special case of unilateral abnormality.................................... 42

Chapter 2 - Presentation of the Method


 A Single tool used throughout infancy and childhood.......... 43
 Structure of the examination............................................ 45
 Recent modifications........................................................ 46
 Training in use of the method........................................... 48

Chapter 3 – Presentation of the Examination Chart


 Description of the examination chart................................. 51
 Choosing the appropriate column....................................... 53
 Corrected age up to 2 years ................................................ 53
 Scoring and recording results ............................................. 53

Chapter 4 - Technical Description: Definitions,


Observations, Maneuvers and Scoring
 Head circumference and growth ....................................... 55
 Cranial examination................................................. 58
 Neurosensory investigations....................................... 60
 Observations and interview data about the period .
since the previous examination................................... 65
 Gross motor development milestones .............................. 67
 Fine motor milestones................................................... 71
 Passive muscle tone ..................................................... 75
 Motor activity ............................................................. 88
 Primitive reflexes.......................................................... 92
 Protective reactions ...................................................... 98
 Deep tendon reflexes..................................................... 100
 Neuromotor abnormalities and acquired deformities........ 101
 Abnormal movements, rigidity, dystonia.......................... 109

Chapter 5 - From Analysis to Synthesis


 Data interpretation at each evaluation........................... 111
 Definition of cerebral palsy (CP)................................... 115
 Spectrum of neuromotor abnormalities at 2 years............. 118
 Categorization of motor disabilities................................ 121
 Expanding the examination to other fields, other tools...... 123

Chapter 6 - Usefullness of the Neurological Assessment


in Research
 Historical background....................................................... 125
 Research results................................................................. 126
 Psychometric characteristics of the assessment................. 127
 Complementary studies................................................. 1 29
 On-going studies.......................................................... 130

Conclusion................................................................... 133

Bibliography................................................................. 137

List of figures and tables.............................................. 147

Appendix I – Examination Chart................................. 153

Index............................................................................. 173
Preface 1st edition
Ann L. Stewart

No single person has made a greater contribution to the


understanding of neonatal neurology than Claudine Amiel-
Tison. I am both honoured and delighted to write the Foreword
to the English edition of this schedule for evaluating the neu-
rological development in children from birth to 6 years.
Shortly after she graduated in Medicine, Dr. Amiel-
Tison decided to specialize in Pædiatrics. Early on, she was
influenced by André-Thomas and his assistant, Suzanne
Saint-Anne Dargassies. On retiring, André-Thomas said he
was going to devote the rest of his life to understanding the
maturation of the central nervous system. He set up a study
of newborns with Julian de Ajuriaguerra and later with
Suzanne Saint-Anne Dargassies. Dr. André-Thomas’s first task
was to define the terms passive tone and active tone, then he
set out to devise reproducible methods for measuring them.
Claudine Amiel-Tison and her collaborator, Julie Gosselin,
use the same definitions and methods in their sophisticated
schedule, presented in this book.
Dr. Amiel-Tison was impressed by the findings of her
study of cerebral damage in full-term infants in the 1960s, and
she published the data in a paper in 1969 (1). She found that if
the infant was fully breast or bottle fed by 10 days of age, the
outlook was good, no matter how seriously compromised the
infant had been at birth; otherwise, one could not be certain
of the outcome until the child was past 9 months of age. We
still cite that paper today. Dr. Amiel-Tison was determined to
devise an assessment for clinicians to use at the cotside that
would be reproducible, predictive and user-friendly.
8 Neurological Assessment from Birth to 6

I first met Dr. Amiel-Tison at a conference in 1972, at


which she was speaking on the follow-up of infants presenting
with neurological abnormalities in the first days of life. Shortly
after, she visited our group in the Department of Pædiatrics,
University College London Medical School and demonstrated
her techniques. We were so impressed with these techniques
that when we set up a study into the outcome of brain lesions
detected by ultrasound in very preterm infants, we incorpo-
rated them into our methods.
The assessment schedule has undergone many changes.
Dr. Amiel-Tison published the original assessment, for infants
from birth to one year, in 1976 (2) and then in 1978 she
began a long and fruitful collaboration with Dr. Albert
Grenier. Together, they published the first version of the
assessment schedule in French in 1980 and a revised version
in 1985; the latter was translated into English by Roberta
Goldberg (3-5).
When, in 1979, we began our study in the Department
of Pædiatrics, University College London Medical School, on
the outcome of brain lesions detected by ultrasound in the
newborn, in very preterm infants, we used the assessment
schedule published by Dr. Amiel-Tison in 1976 (2). Over the
following years, we modified the schedule, with the help of
Dr. Amiel-Tison, to include assessments at 30 months and
4 years of age and we published our findings on the prediction
of neurodevelopmental status at four and eight years of age
from neurodevelopmental status at one year of corrected age
(6,7)
. Together with Dr. Amiel-Tison, we recently published
an abstract on the prediction of school performance at 14 to
15 years of age from neurodevelopmental status at one year
of corrected age (8).
Dr. Amiel-Tison had always regretted that the assessment
published in 1976 was applicable only up to one year of age.
She realised that between us, we had collected a lot of data
on 4 year olds and we combined these with the data collected
by Suzanne Saint-Anne Dargassies on two year olds to form
a single database covering birth to 4 years. We discovered, for
Preface 1st edition 9

example, that in measures of passive muscle tone, the angles


changed gradually up to 18 months and little thereafter up to
4 years of age. For the first time, we included a scoring system
strictly for research purposes. Shortly after this analysis, we
were invited to write an «Experience and Reason» article
published in 1989 which included assessments for children
up to five years old (9). In 1990, we made a video in London
(English and French versions) with Dr. Amiel-Tison, demons-
trating the methods on children in our ultrasound cohort (10).
Dr. Amiel-Tison has long been interested in the effect of
subtle neurological signs and their role in determining even-
tual outcome. She was impressed by the paper published by
Cecil Drillien (11) which gave the first description of apparently
transient neurological signs. Dr. Drillien had found that such
signs were associated with poor school performance, and
Dr. Amiel-Tison postulated that these subtle neurological
signs would predict moderately low IQ and sub-optimal
school performance – and she was right (7,8). We have called
the children with this constellation of minor signs «the
apparently normal survivors» (12). Dr. Amiel-Tison believes that
the subtle neurological signs are actually permanent (12) and
that examiners can find them if they look for them, at least
in adolescents.
Neurological development from birth to 6 years is very
clearly written and beautifully illustrated by both Claudine
Amiel-Tison and her sister, Annette Tison. The «evaluation
grids» in the examination chart are clear and ready to use. This
English translation will allow the schedule to be used world-
wide; it is a great achievement and I wish it every success.

Ann Stewart FRCP


Honorary Senior Lecturer
Department of Pædiatrics,
University College London
10 Neurological Assessment from Birth to 6

References
1. Amiel-Tison, C. Cerebral damage in full-term newborns: ætiolo-
gical factors, neonatal status and long term follow-up. Biological
Neonatorum 1969; 14: 234-250.
2. Amiel-Tison, C. A method for neurologic evaluation within the
first year of life. In: Current problems in pediatrics, VII, no 1,
pp. 1-50. Year Book Medical Publishers, Chicago, 1976.
3. Amiel-Tison, C., Grenier, A. Évaluation neurologique du nouveau-né
et du nourrisson. Paris, Masson, 1980.
4. Amiel-Tison, C., Grenier, A. La surveillance neurologique au cours
de la première année de la vie. Paris, Masson, 1985.
5. Amiel-Tison, C., Grenier, A. Neurological assessment during the first
year of life. New York, Oxford University Press, 1986.
6. Stewart, A.L., Costello, AmdeL, Hamilton, P.A., Baudin, J., Brad-
ford, B.C., Reynolds, E.O.R. Relation between neurodevelop-
mental status at one and four years in very preterm infants. Dev
Med Child Neurol 1989; 33: 756-765.
7. Roth, S.C., Baudin, J., Pezzani-Goldsmith, M., Townsend, J.,
Reynolds E.O.R., Stewart, A.L. Relation between neurodevelop-
mental status of very preterm infants at one and eight years. Dev
Med Child Neurol 1994; 36: 1049-1062.
8. Roth, S., Baudin, J., Townsend, J., Rifkin, L., Rushe, T., Amiel-
Tison, C., Stewart, A. Prediction of extra educational provision
at 14-15 years from neurodevelopmental status at one year of
corrected age in subjects born before 33 weeks gestation. Ped Res
1999: 45: 904 (abstr).
9. Amiel-Tison, C., Stewart, A. Follow-up studies during the first five
years of life: a pervasive assessment of neurological function.
Arch Dis Child 1989; 64: 496-502.
10. Amiel-Tison, C. and Stewart, A. Neuromotor assessment during the
first five years of life. (video, English and French versions) UCL
Images, London, 1990.
Preface 1st edition 11

11. Drillien, C.M. Abnormal neurologic signs in the first year of


life in low birth weight infants: possible prognostic significance.
Dev Med Child Neurol 1972; 14: 572-584.
12. A miel -T ison , C. and S tewart , A. Apparently normal survi-
vors: neuromotor and cognitive function as they grow
older. In: A miel -T ison ,C., S tewart , A. ( eds .) The Newborn
Infant: one brain for life. Les Éditions INSERM, Paris, 1994, pp.
227-237.
Preface 2nd edition
Marilee C. Allen

The most remarkable aspect of this book is its simplicity. It is


the culmination of Claudine Amiel-Tison’s lifetime of work
examining infants and children. She has integrated her clinical
experience with knowledge of neurophysiologic maturation
to develop an elegant clinical examination. Julie Gosselin has
contributed her extensive clinical experience with the exam
and described its psychometric properties. The result of this
collaboration is a very practical book that describes a concise,
elegant and valuable examination.
Claudine Amiel-Tison represents a distinguished heritage
in the study of the development of central nervous system
(CNS) function, initiated in the middle of the 20th century
by French neurologist André-Thomas.1 He tackled the very
difficult problem of describing one of the most important
aspects of the neurological examination: muscle tone, which
changes as infants mature. Muscle tone is controlled by the
brain, and thereby provides an important window on brain
maturation. His pupils, Suzanne Saint-Anne Dargassies and
Claudine Amiel-Tison, have furnished meticulous descrip-
tions of how muscle tone, reflexes and responses evolve over
time in preterm and fullterm infant.2-6 Detailed descriptions
of the neuromaturation of infants with CNS injury provide
clues as to which clinical findings best predict long-term
neurodevelopmental disabilities, as well as insight into
causes of brain injury and the relationships between struc-
ture and function of the developing brain.6-8 The examination
described in this book by Claudine Amiel-Tison and Julie
Gosselin is a distillation of these years of work: a practical,
efficient and elegant method for assessing neurodevelopment
in newborns, infants, and children to age 6 years. Thorough
descriptions of examination maneuvers make this exam
14 Neurological Assessment from Birth to 6

accessible and easy to master for any clinician who works with
young children. Its great value lies in the continuity it pro-
vides for following high risk infants through early childhood.
During this critical time, brain injury can be detected and its
recovery followed, allowing for early intervention strategies to
minimize neurodevelopmental disabilities.
This book, first published in English in 2001, clearly
filled a clinical need, a need great enough to warrant publi-
cation of this second edition. Neonatologists, pediatricians,
midwives, nurse practitioners and primary care providers
in clinical practice have used the examination method des-
cribed in this book. This examination fills a need in clinical
outcomes research, by providing a continuous outcome
measure for neuromotor abilities from birth to 6 years. This
characteristic also makes it ideal for use as a more immediate
measure of early CNS function in randomized clinical trials
of medications and treatment strategies used in neonatal
intensive care. The ability to recognize adverse drug effects
on early CNS function improves the safety of clinical drug
trials. Furthermore, the ability to identify neonates at high
risk for neurodevelopmental disability facilitates selection
of the best candidates for clinical trials and early inter-
vention programs.
To better illuminate the background and context for their
examination, the authors have revised and added to this
second edition. They expanded the section describing the
neurophysiological basis for the examination. The most
important new section incorporates the results of clinical
research by Julie Gosselin and her graduate students in Mon-
tréal and a description of the psychometric properties of the
examination. The authors describe a spectrum of neuromotor
abnormalities at 2 years corrected for degree of prematurity,
ranging from disabling cerebral palsy to mild cerebral palsy
to minor neuromotor dysfunction. The spectrum of neuro-
motor abnormalities significantly correlates with neuroco-
gnitive outcome at school age. This work underscores the
Preface 2nd edition 15

concept of a continuum of developmental abnormalities


described by Arnold Capute and Pasquale Accardo.9 Unders-
tanding this relationship helps optimize the use of early
intervention resources, especially with regard to language
acquisition.
As modern medicine continues to develop and employ
diagnostic technologies, much of medical training is devoted
to learning how to use this technology. The art of medicine
is often overlooked. Medical schools still teach physical
examination, but fewer physicians than in the past are given
the time for comprehensive histories and thorough physical
examinations. If physicians do not hone their own expertise,
who will teach the next generation?
By providing a simple, intelligent guide to examining
central nervous system function in infants and children, this
book takes an important step towards ensuring that future
clinicians can develop into skilled examiners. There is no
doubt that mastering the exam described in this text is
something to which clinicians who work with neonates should
aspire, and that it should be part of the core curriculum for
pediatricians. Only by adding expertise in neurological exa-
mination to our state-of-the-art neuroimaging and other dia-
gnostic technologies will we realize our potential for critical
insight into how the developing brain recovers from injury,
and the miracle of healthy neuromaturation.

Marilee C. Allen, M.D.


Professor of Pediatrics
The Johns Hopkins School of Medicine
Baltimore, MD, U.S.
16 Neurological Assessment from Birth to 6

References
1. Andre-Thomas, Chesni, Y., & Saint-Anne Dargassies, S. The Neurolo-
gical Examination of the Infant. Little Club Clinics in Developmental
Medicine No. 1. London: National Spastics Society, 1960.
2. Saint-Anne Dargassies, S. Neurological Development of the Full-
Term and Premature Neonate. Amsterdam: Elsevier/North-
Holland Biomedical Press; 1977.
3. Amiel-Tison, C. A method for neurologic examination within the
first year of life. Curr Probl Pediatr 1976; 7(1):1-50.
4. Amiel-Tison, C. & Grenier, A. Neurologic Assessment During the First
Year of Life. New York: Oxford University Press; 1986.
5. Amiel-Tison, C. (2003). Neurologic maturation of the neonate.
NeoReviews; 2003; 4: e199-e206.
6. Amiel-Tison, C., Clinical assessment of the infant nervous system.
In M.I.Levene, F. A. Chervenak, M. J. Whittle, M. J. Bennett, & J.
Punt (Eds.), Fetal and Neonatal Neurology and Neurosurgery (Third
ed., pp. 99-120). London: Churchill Livingstone; 2002.
7. Amiel-Tison, C., Cabrol, D., Denver, R., Jarreau, P. H., Papiernik, E.,
& Piazza, P. V. (2004). Fetal adaptation to stress. Part I: accelera-
tion of fetal maturation and earlier birth triggered by placental
insufficiency in humans. Early Hum.Dev, 2004; 78: 15-27.
8. Amiel-Tison C, Cabrol D, Denver R et al. Fetal adaptation to
stress: Part II. Evolutionary aspects; stress-induced hippocampal
damage; long-term effects on behavior; consequences on adult
health. Early Hum Dev; 2004; 78(2):81-94.
9. Capute, A.J., & Accardo, P.J. (Eds.). Developmental Disabilities in
Infancy and Childhood. Second edition. Baltimore: Paul H. Brookes
Publishing Co.; 1996.
Introduction

Nothing is ever cast in stone, even a neurological examina-


tion technique. And fortunately so, because everything is in
constant flux. Since the publication of the first edition of our
book, Neurological Development from Birth to Six Years (Amiel-
Tison & Gosselin, 1998), a number of essential questions have
been answered.
ƒƒ A more precise study of neurological signs has brought
to light the notion of a spectrum of neuromotor disor-
ders, from disabling cerebral palsy (CP) to isolated signs.
Classifying minor forms has allowed a little order to
be introduced where confusion abounded before.
ƒƒ Patient clinical research validated the earlier intuitions
as described in the first edition of 1998. As a result, the
neurological and cranial signs that had been detected and
classified have at last lost their status as ‘soft signs’ and
become the markers that can be tracked from the perinatal
period to school age.
ƒƒ This development opens up several new perspectives:
first, a more precise and earlier identification of the dif-
ficulties that at-risk neonates have to face and, second, a
better targeted early intervention. (We never imagined
back in 1998 that finding a bilateral or right stretch
reflex at the level of the triceps surae would justify early
speech therapy). And along with this improvement in the
identification process come the hope for a more precise
definition of selection criteria for sampling in the context
of evaluative studies to successfully demonstrate the
effectiveness of early intervention: for that, we had to start
by separating the wheat from the chaff before conducting
clinical tests.
18 Neurological Assessment from Birth to 6

Is this boasting? Absolutely not. It is rather the satisfaction


of better understanding so that we can help these children
with difficulties that weigh so heavily on their learning and
that they themselves certainly do not consider minor. As early
as 1947, Gesell and Amatruda had written:
“In the case of major brain damage, the child is usually so
obviously handicapped that his/her difficulties naturally draw
offers of help and sympathy. In the case of minor cerebral
damage, the child has exactly the same need for considera-
tion and understanding. He also has a greater than-average
need to be protected from stress and competition… In all these
cases, we are dealing with an extremely complex interaction
between development potential and dynamic forces. Despite
the minor nature of initial motor dysfunction, the damage to
the personality can be considerable and more or less perma-
nent. Psychiatric concepts are often less useful for interpreting
these pathologies than a thorough understanding of the neuro-
logical development.” (Gesell and Amatruda, 1947, p. 248).
This neuromotor examination and its application derive
from the French school of André-Thomas and Saint-Anne
Dargassies (1952; Saint-Anne Dargassies 1982). The method
became the heritage of Claudine Amiel-Tison, the first author
of the 1998 edition of this book. As a result of Julie Gosselin’s
exacting measurements and ten years of patient work vali-
dating the signs, this method has now become hers and the
signatures have been inverted in this second edition, a reflec-
tion of their fruitful collaboration and friendship. Their hope
is to renew the pediatric community’s interest in this clinical
study of signs using the keys that a thorough understanding
of the neuromotor maturation and other brain functions pro-
vides. Being easy to perform and fast enough to be included
in their routine practice, such a screening assessment is aimed
to pediatricians. In case of abnormalities, the child will be
referred to a neurologist.
The authors are passionately committed to sharing this
clinical method with others so that it can be used in daily
clinical practice as well as in research and in regional health-
care networks. The core of this work is a basic study of signs,
How to make simple in clinical neurology in the
first years of life? Perinatal insults occur on a brain
which is in rapid development. The clinical presenta-
tion of these pathologies is thus changing, particu­larly
in the neuromotor domain during the first two years.
Therefore, the clinician needs precise landmarks to
detect possible deviancy at each stage of development.
In the first edition, published in 1998, we proposed
a single and unique assessment tool applicable from
birth to 6 years of life. This first edition already included
the technical description for each maneuvers, scoring
and coding system.
After 10 years of use in clinical research as well as
in regular follow-up, the current edition is improved:
• research data collected at the corrected age of
2 years have allowed to identify a spectrum of
neurological disabilities, from disabling cerebral
palsy to more minor signs;
• the proposed 5-level categorization of neuromo-
tor disabilities has shown significant correlation
with developmental and intellectual performances
both at preschool and school age, providing a
link between early perinatal insults and different
neurodevelopmental disabilities of late emergence.
This assessment tool should be systematically used
with high risk infants to better understand the course
of their development in order to implement early inter-
vention when neurocranial signs are present. It should
also be used to assess current perinatal practices.

ISBN 978-2-89619-447-6