Clinical pediatric neurology

Mary’s HospitalAssociate Clinical ProfessorDepartment of PediatricsVirginia Commonwealth University School of Medicine Alpert Medical School of Brown University Providence, Rhode Island

Edited by

Ronald B David, MD

Attending PhysicianDepartment of Pediatrics

St Mary’s HospitalAssociate Clinical ProfessorDepartment of PediatricsVirginia Commonwealth University School of Medicine

Alpert Medical School of Brown University

Providence, Rhode Island

Barbara J Olson, MD

Private PracticePediatric Neurology AssociatesAssistant Clinical ProfessorDepartment of Pediatrics and Neurology

Vanderbilt UniversityNashville, Tennessee

New York

Copyeditor: Joann Woy

Compositor: Patricia Wallenburg

Printer: Bang Printing

Visit our website at www.demosmedpub.com

© 2009 Demos Medical Publishing, LLC All rights reserved This book is protected by copyright No part of it may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, electronic, mechanical, photocopying, recording, or oth- erwise, without the prior written permission of the publisher.

Medicine is an ever-changing science Research and clinical experience are continually expanding our knowledge, in particular our understanding of proper treatment and drug therapy The authors, editors, and publisher have made every effort to ensure that all information in this book is in accordance with the state of knowledge at the time of production of the book Nevertheless, the au- thors, editors, and publisher are not responsible for errors or omissions or for any consequences from application of the informa- tion in this book and make no warranty, express or implied, with respect to the contents of the publication Every reader should examine carefully the package inserts accompanying each drug and should carefully check whether the dosage schedules mentioned therein or the contraindications stated by the manufacturer differ from the statements made in this book Such examination is par- ticularly important with drugs that are either rarely used or have been newly released on the market

Library of Congress Cataloging-in-Publication Data

Clinical pediatric neurology / edited by Ronald B David — 3rd ed.

p ; cm.

Rev ed of: Child and adolescent neurology / edited by Ronald B David 2nd ed 2005.

Includes bibliographical references and index.

ISBN-13: 978-1-933864-22-8 (hardcover : alk paper)

ISBN-10: 1-933864-22-2 (hardcover : alk paper)

1 Pediatric neurology I David, Ronald B II Child and adolescent neurology.

[DNLM: 1 Nervous System Diseases—diagnosis 2 Adolescent 3 Child 4 Diagnostic Techniques, Neurological 5 Infant.

6 Nervous System Diseases—therapy 7 Neurologic Examination WS 340 C6415 2009]

Special Sales Department

Demos Medical Publishing

386 Park Avenue South, Suite 301

To all children but particularly

To the children for whom we care those whose lives have been touched by the misfortune of neurologic disorders.

I Pediatric Neurologic Evaluation

John B Bodensteiner, Editor

John B Bodensteiner and Ronald B David

2 The Neurologic Examination of the Preterm and Full-term Neonate and of the Infant 17

Patricia H Ellison and Donna Kathryn Daily

Ruth D Nass

Ruthmary K Deuel and Amy C Rauchway

Warren T Blume

Laurie Gutmann and Jack E Riggs

John F Kerrigan

Russell J Butterfield, Gary Hedlund, and James F Bale, Jr.

II General Pediatric Neurologic Diseases and Disorders

David E Mandelbaum, Editor

Michael Flink and Doris A Trauner

Amisha Malhotra, William E Bell, and Frederick W Henderson

Richard H Haas and Jennifer Armstrong-Wells

Paul Maertens

16 Inborn Errors of Metabolism II: Disorders of Purine and Amino Acid Metabolism 337

William L Nyhan

Roger J Packer, Tobey J MacDonald, Brian R Rood, Gilbert Vezina, and Robert A Keating

John T Sladky

Emanuel DiCicco-Bloom

20 Disorders of Nervous System Development: Cellular and Molecular Mechanisms 415

John N Gaitanis and David E Mandelbaum

Barry S Russman

Ruthmary K Deuel and Amy C Rauchway

Ruth D Nass and Gail Ross

Max Wiznitzer and Debora L Scheffel

III Common Pediatric Neurologic Problems

Barbara J Olson, Editor

Stavros M Hadjiloizou and James J Riviello, Jr.

28 The Child with Attention Deficit Hyperactivity Disorder 525

Kevin M Antshel, Michelle M Macias, and Russell A Barkley

O’Neill F D’Cruz and Bradley V Vaughn

Traditional textbooks convey knowledge It is the goal of

this text to convey not only essential knowledge but also

the collected wisdom of its many highly regarded

con-tributors To achieve the goal of conveying not only

knowledge but wisdom, each book in this series is built

on a structural framework that was well received by

crit-ics and readers alike in previous editions Our text is

di-vided into three sections:

• Tools for diagnosis

• Diseases and disorders

• Common problems

Also included to facilitate a physician’s use of this

book are:

• Nosologic diagnosis tables,

• “Pearls and Perils” boxes,

• “Consider Consultation When…” boxes,

• Selected annotated bibliographies,

• A complete bibliography,

• And (new in this edition) Key Clinical

Questions

The Nosologic Diagnosis tables are based on a

dis-criminator model to promote clearer understanding and

are superior to a criterion-based model and others that

lack similar specificity (See the Appendix for complete

description of how this system was developed.)

Whoever having undertaken to speak or write

hath first laid for themselves some [basis] to

their argument such as hot or cold or moist or

dry or whatever else they choose, thus

reduc-ing their subject within a narrow compass

Hippocrates

As Hippocrates has suggested, structure is the key tolearning Unless there is a structure onto which knowl-edge can be built, confusion and disorganization are theinevitable consequences

Classification systems induce orderliness in ing and enhance our ability to communicate effectively

think-A review of the most enduring hierarchical classificationsystems, particularly that of Linnaeus (that is, phyla, gen-era, species), makes clear the value of grouping according

to discriminating features, as well as the value of ity, expandability, and dynamism

simplic-The goal, whatever the classification system, is toseek the most powerful discriminating features that willproduce the greatest diagnostic clarity Discriminating fea-tures should avoid crossing domains Much of the confu-sion that arises in diagnosis may be the result of theclinician who unwittingly crosses the anatomic, pathologic,pathophysiologic, phenomenologic, and etiologic classifi-cation domains used in medicine (for example, the inclu-sion of anatomically oriented “temporal lobe seizures” in

a phenomenologically based classification system that cludes complex partial seizures) Some conditions, such asbrain tumors, are classified according to their histopathol-ogy and lend themselves well to this classification system.Others, such as headaches and movement disorders, areclassified phenomenologically and are therefore much lesseasily classified In other cases, discriminators must en-compass inclusionary as well as exclusionary features Attimes, we can only use a criterion-based system or con-struct tables to compare features

in-Arbitrarily, we label as consistent those features thatoccur more than 75% of the time; features are consideredvariable when they occur less than 75% of the time Thediagnostic tables should be viewed, therefore, only as abeginning in the extremely difficult effort to make diag-nosis more precise and biologically based How well thisbook accomplishes the goals of identifying the most pow-

erful discrimination features for maximum diagnostic

clarity is limited by the current state of the art in child

and adolescent neurology In some areas, several features,

when clustered together, serve to discriminate

This text is designed to be pithy, not exhaustive,

many other books of that ilk are already available Each

text in this series reflects appropriate stylistic differences

among content editors However, each is built upon the

same structural framework; hence the value of this text

to the users

Chapter 16 on “Order and Disorders of Nervous

System Development” is particularly noteworthy because

of its unique treatment of this very important and timely

subject matter

Acknowledgment

I would like to acknowledge some of the people who have

made key contributions to this effort They include Craig

Percy, who initially saw the potential of this effort and

headed the team at Demos; the National Institute of

Neu-rological Disorders and Stroke (NINDS)* for its support

in nosologic research; and the investigators who were volved with this NINDS project; Dr Grover Robinson, along-time friend (who suggested the “Consider Consul-tation When…” boxes); and Ms Laura DeYoung a long-time publishing friend I am also particularly grateful to

in-my associate editor colleagues, Drs John Bodensteiner,David Mandelbaum, and Barbara Olson, for their exten-sive and hands-on contribution to this edition Their help

is reflected, I feel, in the extraordinary quality of the ent effort Lastly, I would thank Dr Susan Pillsbury, aclose friend and trusted colleague, whose advice is alwayscogent and whose personal support is most appreciated This text is therefore in no way a singular effort butrather reflects the expertise of all who contributed in somany different ways It is my hope that this is reflected inthe quality of the effort It is therefore my fondest wishthat this text resides on your desk, rather than on yourbookshelf

pres-Ronald B David, MD

*NINDS 1PO1NS20189–01A1 (Nosology, Higher Cortical Function

Disorders in Children).

Kevin M Antshel, PhD

Assistant Professor of Psychiatry

Department of Psychiatry and Behavioral Sciences

State University of New York Upstate Medical University

Syracuse, New York

Jennifer Armstrong-Wells, MD, MPH

Resident Physician

Department of Neurology

University of California San Francisco

San Francisco, California

James F Bale, Jr., MD

Professor and Associate Chair

Department of Pediatrics

University of Utah School of Medicine

Salt Lake City, Utah

Russell A Barkley, PhD

Clinical Professor of Psychiatry

Department of Psychiatry

Medical University of South Carolina

Charleston, South Carolina

William E Bell, MD

Professor Emeritus

Department of Pediatrics and Neurology

The University of Iowa Hospitals

Iowa City, Iowa

Phoenix, Arizona

Russell J Butterfield, MD, PhD

FellowDepartment of NeurologyUniversity of Utah School of MedicineSalt Lake City, Utah

Donna Kathryn Daily, MD, MA

Associate Professor of PediatricsDepartment of PediatricsVanderbilt University Medical SchoolNashville, Tennessee

Ronald B David, MD

Attending PhysicianDepartment of Pediatrics

St Mary’s HospitalAssociate Clinical ProfessorDepartment of PediatricsVirginia Commonwealth University School of MedicineRichmond, Virginia

O’Neill F D’Cruz, MD, MBA

Clinical Research PhysicianDepartment of Clinical ScienceActelion Parmaceuticals Ltd

Cherry Hill, New Jersey

Ruthmary K Deuel, MD

Professor Emeritus

Department of Pediatrics and Neurology

Washington University School of Medicine

Robert Wood Johnson School of Medicine

Piscataway, New Jersey

Warren Alpert School of Medicine at Brown University

Providence, Rhode Island

Laurie Gutmann, MD

Professor of Neurology and Exercise Physiology

Department of Neurology

West Virginia University

Morgantown, West Virginia

Richard H Haas, MB, B Chir

Professor

Department of Neurosciences and Pediatrics

University of California San Diego

La Jolla, California

Stavros M Hadjiloizou, MD

Child Neurologist

Department of Epilepsy and Clinical Neurophysiology

Cyprus Paediatric Neurology Institute

The Cyprus Institute of Neurology and Genetics

Nicosia, Cyprus

Gary Hedlund, DO

Adjunct Professor RadiologyDepartment of Medical ImagingUniversity of Utah School of MedicineSalt Lake City, Utah

Andrew D Hershey, MD, PhD, FAHS

Professor of Pediatrics and NeurologyDepartment of Pediatrics

Cincinnati Children’s Hospital Medical CenterUniversity of Cincinnati College of MedicineCincinnati, Ohio

Deborah G Hirtz, MD

Program DirectorOffice of Clinical TrialsNational Institute of Neurological Disorders and StrokeNational Institutes of Health

Rockville, Maryland

H Terry Hutchinson, MD, PhD

Clinical ProfessorDepartment of Child NeurologyUniversity of California San FranciscoSan Francisco, California

Robert A Keating, MD

ChiefDepartment of Pediatric NeurosurgeryChildren’s National Medical CenterProfessor

Department of NeurosurgeryThe George Washington UniversityWashington, DC

John F Kerrigan, MD

DirectorPediatric Epilepsy ProgramCo-Director

Hypothalamic Hamartoma ProgramBarrow Neurologic Institute of St Joseph’s Hospitaland Medical Center

Assistant ProfessorDepartment of Clinical Pediatrics and NeurologyUniversity of Arizona College of Medicine PhoenixPhoenix, Arizona

Center for Cancer and Blood Disorders

Children’s National Medical Center

Associate Professor

Department of Neurology and Pediatrics

The George Washington University

Washington, DC

Michelle M Macias, MD

Associate Professor of Pediatrics

Department of Pediatrics

Medical University of South Carolina

Charleston, South Carolina

Kenneth J Mack, MD, PhD

Medical Director

Departments of Neurology and Pediatrics

Mayo Clinic Pediatric Center

Rochester, Minnesota

Paul Maertens, MD

Associate Professor Child Neurology

Department of Neurology and Pediatrics

University of South Alabama

Mobile, Alabama

Amisha Malhotra, MD

Assistant Professor of Pediatrics

Department of Pediatrics

Robert Wood Johnson School of Medicine

University of Medicine and Dentistry of New Jersey

New Brunswick, New Jersey

David E Mandelbaum, MD, PhD

Professor

Department of Clinical Neurosciences and Pediatrics

Alpert Medical School of Brown University

Providence, Rhode Island

Ruth D Nass, MD

Professor of Clinical Neurology (Pediatrics)

Department of Neurology

New York University School of Medicine

New York, New York

Karin B Nelson, MD

Scientist EmeritusNational Institute of Neurological Disorders and StrokeNational Institutes of Health

Bethesda, Maryland

William L Nyhan, MD, PhD

Professor of PediatricsDepartment of PediatricsUniversity of California San DiegoSan Diego, California

Barbara J Olson, MD

Private PracticePediatric Neurology AssociatesAssistant Clinical ProfessorDepartment of Pediatrics and NeurologyVanderbilt University

Nashville, Tennessee

Roger J Packer, MD

Executive DirectorDepartment of Neuroscience and Behavioral MedicineDirector

Brain Tumor InstituteChildren’s National Medical CenterProfessor

Department of Neurology and PediatricsThe George Washington UniversityWashington, DC

Amy C Rauchway, DO

Assistant Professor of NeurologyDepartments of Neurology and PsychiatrySaint Louis University School of Medicine

St Louis, Missouri

Jack E Riggs, MD

Professor of NeurologyDepartment of NeurologyWest Virginia UniversityMorgantown, West Virginia

James J Riviello, Jr., MD

George Peterkin Endowed Chair in PediatricsProfessor of Pediatrics and Neurology/NeurophysiologyDepartments of Pediatrics and Neurology

Texas Children’s HospitalHouston, Texas

Associate Professor of Psychology

Departments of Pediatrics and Psychiatry

Weill Medical Center of Cornell University

New York, New York

Barry S Russman, MD

Professor Pediatrics and Neurology

Oregon Health & Science University

Department of Pediatric Neurology

Children’s Healthcare of Atlanta at Egleston

Chief

Department of Pediatric Neurology

Emory University School of Medicine

Doris A Trauner, MD

Professor and Chief, Pediatric NeurologyDepartment of Neurosciences and PediatricsRady Children’s Hospital

University of California San Diego School of Medicine

Department of RadiologyThe George Washington UniversityWashington, DC

Max Wiznitzer, MD

Division Of Pediatric NeurologyRainbow Babies & Children’s HospitalAssociate Professor of Pediatrics and NeurologyCase Western Reserve University

Cleveland, Ohio

SECTION 1

PEDIATRIC NEUROLOGIC

EVALUATION

John B Bodensteiner

Some clinicians have suggested that the taking of the

neu-rologic history is as important as, or potentially more

im-portant than, the neurologic examination itself Other

clinicians have suggested that the neurologic history

iden-tifies the nature of the disorder or disease, and the

neu-rologic examination confirms or pinpoints its location

The history itself may be a narrative recapitulation of

in-formation provided by a child’s primary caregiver(s), or it

may be generated in response to a questionnaire or

check-list Experienced clinicians realize that the key to making

a successful diagnosis often lies in asking the right

ques-tions and listening carefully to the answers Responses to

questionnaires or checklists can be used as part of a

for-mal structured interview Diagnostically, they can be both

reliable and valid For example, a patient may be asked

the following questions with respect to headaches: Are

your headaches confined to one side of your head? Are

your headaches associated with vomiting or a desire to

sleep? Do you have visual symptoms, such as dancing

lights or other phenomena? An affirmative response to all

three questions would permit accuracy of close to 100%

for the diagnosis of migraine No other questions or

lab-oratory investigations may be necessary Other questions

provide clinical rather than diagnostic information,

use-ful in practicing the art as well as the science of medicine

The reliability of the information gained from a

questionnaire depends to some extent on the ability of the

respondent to understand the questions being asked The

questions that follow are those used by many clinicians toaccomplish this end Some are also valuable in answeringresearch questions They are all designed to be useful inthe practice of pediatric neurology Note: This form may

be reproduced for clinical use without further permissionfrom the author or publisher In order to make the ques-tions more useful, we have collected them into groupsbased on the three most common presenting complaints

in the pediatric neurology outpatient setting, namelyseizures, headache, and developmental delay This is not

a copyrighted section of this text and clinicians shouldfeel free to lift any or all questions or formats for repro-duction and clinical use

3

Neurologic History

John B Bodensteiner and Ronald B David

Pearls and Perils

The diagnosis can often be determined or inferred from one or two key questions.

Willingness to comply with treatment can be probed by use of key questions.

Willingness to accept diagnosis can be probed through key questions.

M

8 Child’s siblings (please list oldest first)

9 Marital status of parents

13 Please check if the parent was or is considered to have difficulty with any of the following:

c Overactivity, restlessness, hyperactivity

1 Please check if your child has ever experienced any of the following: Yes No

c Visual difficulty requiring either glasses or visual training _ _

d Hearing difficulty requiring the use of a hearing aid _ _

e Movement problems requiring the use of special shoes, splints, braces,

or a wheelchair or a specialized program of motor training _ _

s Picked last or close to last in games where children pick sides _ _

u Headaches not relieved by nonprescription pain medicine _ _

w Headaches occurring in the middle of the night or upon awakening _ _

cc Lost once-attained skills (speech, language, or motor) _ _

2 Has your child ever been diagnosed as

Yes No

3 Has your child ever

e Received special education services, grades K through 12 _ _

g Been suspended or discharged from day care, kindergarten or school _ _

C Treatment information

1 Has your child ever been evaluated by a

3 Has your child ever had any unusual reaction to any of the medications listed above?

Please list and describe reaction

_

4 Describe each of your child’s emergency room visits or hospitalizations Begin with the most recent

_ / _ / _ / _ /

D Pregnancy, birth, and development information/history

1 How many pregnancies did the child’s mother have?

3 Were any medicines prescribed during your (her) pregnancy with this child, such as

Yes No

4 Were any of the following used during this child’s pregnancy?

5 Did you (she) have any of the following complications during this pregnancy?

9 How long was it from the time your (her) water broke

10 During this pregnancy

f If so, were there any abnormalities in the amniocentesis?

Yes No

21 If so, was the cord wrapped more than once around the baby’s neck or

28 Was the baby placed in an isolette, incubator, or intensive special care unit? _ _

31 Was the baby placed on a respirator (breathing machine)? _ _

33 Was the fluid stained with the baby’s meconium (bowel movement)? _ _

List if known _

35 Did the baby have physical features that were unusual or very _ _much unlike baby’s relatives?

37 How long after birth did the parents take the baby home? _days

38 During the first 2 weeks after the birth of the baby

39 During the first year of life, did the baby

c Show any unusual trembling or unusual movements of arms, legs, or head? _ _

Questions 40 to 50 should be considered in the evaluation of the complaint of developmental abnormalities Section

E could be added to further assess children somewhat older, such as school-age children

40 How old was the baby (your best guess) when he or she first

< 6 mo 6–12 mo 12–18 mo 18–24 mo 24–36 mo 36–48 mo 48+ mo

e Walked without assistance

g Was toilet trained–bowel

h Was toilet trained–urine

i Began to vocalize (babble)

k Began to talk in sentences

Yes No

42 Does your child

e Turn head to distinguish from where a sound is coming? _ _

43 General language skills

a Does your child

(2) Omit words from sentences (i.e., do his sentences sound telegraphic)? _ _

(4) Have trouble with verbs, such as is, am, was, and were? _ _

(7) Have difficulty responding appropriately to questions? _ _(8) Have problems asking questions beginning with who,

(9) Have trouble using present and past tense verbs correctly? _ _(10) Show little or no progress in speech and language in the

(12) Do you feel your child’s speech is more difficult to understand

(13) Does it seem that your child uses t, d, k or g in place of most

44 Receptive language skills

a Does your child

(3) Follow two-step commands two times out of three? _ _

(6) Point to spoon and ball and show how a cup is used? _ _

(8) Know three out of four prepositions (on, under, in front, behind, etc.)? _ _

45 Expressive language skills

a Does your child

(8) Name opposite analogies two times out of three (up/down,

(9) Comprehend senses (taste, feel, smell, see, hear)? _ _(10) Define words correctly six out of nine times (ball, desk,

house, banana, curtain, ceiling, bush, sidewalk)? _ _

46 Other language skills

a Does your child

(1) Have difficulty finding the correct words in conversation? _ _

Yes No(2) Have difficulty in getting the correct word out to use in conversation? _ _

(6) Hesitate or stop before he or she completes sentences? _ _

(11) Label actions (walk, run, sleep, ride, jump, read, write)? _ _

47 Is your child

48 Social skill development and idiosyncratic behaviors:

a Does your child

(2) Like to be held or played with as much as other children? _ _(3) Share or take turns with other children readily? _ _(4) Tend to be bossy or attempt to dominate other children? _ _(5) When compared with other children, show decreased eye contact? _ _(6) When with a group of children his or her age, stand

(9) Flap his hands or arms when excited or stressed? _ _(10) Exhibit other repetitive movements when excited or stressed? _ _

50 Basic educational skills:

a Can your child

(8) Point to basic colors (red, green, blue, yellow, black, white)? _ _

(13) Include at least six body parts (head, arms, body, legs, eyes,

ears, nose, fingers, hair) when drawing a person? _ _

Yes No

b Does your child have problems in

(6) Reasoning and problem solving (personal or in school)? _ _(7) Science, social studies, humanities, foreign languages? _ _

E Attention/activity/behavior/habits

1 Does your child

a Sit still for a fascinating activity, such as television or being read to

b Sit and listen to a story when being read to individually? _ _

j Appear overly frightened or anxious about new experiences? _ _

l Produce sloppy work, even though he or she tries hard? _ _

n Insist on being in charge or he or she will not play? _ _

Yes No

cc Frequently place his or her hands over ears to block out sound? _ _

gg Seem preoccupied with strange creatures or monsters? _ _

kk When observed with a group of children, seem to be apart

2 What type of school does your child attend? Public Private

3 At what age did your child begin preschool or day care? _

6 If in a regular grade (class), does your child receive special help? _ _

7 Has your child ever been absent from school for 2 weeks or longer at one time? _ _

8 Has your child had frequent short absences from school, resulting in

10 Has your child ever been retained by either your decision or the school’s? _ _

7 In what skill or ability area(s) does your child seem to excel over most children his or her age?

G Signs and symptoms

b Does your child have staring spells or spells where you cannot

c Do the convulsions occur only when the child is ill? Or febrile? _ _

d Does your child have convulsions (seizures) without fever? _ _

e Has your doctor ever used the term epilepsy to refer to your child? _ _

h Is the child aware at all before the seizure, during the seizure or

i How long is it before the child returns to normal after

j Have the spells changed since they started or are they all the same? _ _

k Has the child had an electroencephalogram (EEG) to evaluate the spells? _ _

l Has the child had an imaging study of the brain to evaluate

m Has your child taken any medications to prevent seizures? _ _

n Do you have medications you are to administer when a seizure occurs? _ _

o Has your child had side effects from the medications? _ _

p Which medications have been used? Please list them and doses if possible _ _

q Have you had blood levels of the antiepileptic medications

r Have you discussed therapies other than AEDs with your doctor? _ _

Yes No

s Have you considered dietary therapies for the prevention of seizures? _ _

t Have you considered surgical therapy for the prevention of seizures? _ _

2 Headaches

d Do you recognize any stress or environmental factor that

g Is the pain in the front or back of the head or both? _ _

i What does the child do when they have a headache?

j Is there a change in behavior before the onset of the headache? (an aura) _ _

k How long does it take the child to get back to normal after the headache?

l Headaches not relieved by nonprescription pain medicine

m Headaches not relieved by prescription pain medicine

n Headaches occurring in the middle of the night or upon awakening

Does the neurologic examination of infants still

con-tribute to the diagnosis and treatment of neurologic

dis-orders? Can it be used as a measure of improvement from

either systemic disease or neurologic injury? Does it have

other attributes, such as reassurance to the parents and

clinicians, or to identify the need for early intervention

services? Has imaging technology changed the need for

clinical examination?

The considerable diversity that exists in the

num-bers and types of items recorded by physicians as part of

the neonatal neurologic examination makes it difficult to

answer these questions Generally, a report is modest,

with notes often consisting of brief phrases such as “alert,

moves all extremities.” The most detailed examinations

are often those of the physical or occupational therapist

or those of a developmental pediatrician or pediatric

neu-rologist, if consulted The neurologic abnormality may be

first noted after a clinical event, such as a seizure, an

ab-normal imaging study indicating cerebral hemorrhage, or

after the observation of significant lack of response

fol-lowing birth or failure to suck well

Single-item abnormalities, such as a facial palsy or

brachial plexus injury, appear to be noted fairly soon, if

not in the delivery room, then in the initial newborn

ex-amination Other neurologic abnormalities, such as

de-creased alertness or even fairly diffuse hypotonia, may not

be identified in the current brief newborn hospitalization,

thus placing an increased obligation on physicians viding primary care or specialty services

pro-Who should do the neurologic examination?

It is obvious in reading charts that neurologic tions are being done by clinicians and therapists withvarying levels and types of training The documentation

examina-of the neurologic examination as performed by otherhealthcare professionals, such as nurses or rehabilitationtherapists, can complement that of the treating physician

What should be part of the neurologic examination?

The traditional newborn and infant neurologic tion can be divided into four main areas: general descrip-tion, cranial nerves, special situations such as alteredmental status and spinal lesions, and data from the Pre-mie-Neuro, NeoNeuro & Up, and Infanib scoring sheetsdescribed later The traditional examination is described

examina-in more detail examina-in the Appendix of this chapter In addition,some other basic information needs to be gathered Ma-ternal, fetal, and perinatal history may be helpful, as well

as the current medical history of the infant A generalphysical examination may provide information to supportthe neurologic examination Growth patterns are particu-larly important Serial head circumferences seem so basicthat this measurement would not need to be mentioned in

a learned chapter Yet circumferences have been missing

in charts under review from the initial newborn tion; serial evaluations have been missing in newborns al-ready identified with brain abnormality and in infantswith a chief complaint that could refer to the brain

evalua-17

The Neurologic Examination of the Preterm

and Full-term Neonate and of the Infant

Patricia H Ellison and Donna Kathryn Daily

Outline

Who should do the neurologic examination?

What should be part of the neurologic examination?

Scored assessment instruments

What is the prognosis for neurologic abnormality?

Appendix: Neonatal and infant neurologic examination

M

The emphasis of this chapter is on scored

assess-ment instruassess-ments Fortunately, a number of clinicians

have keenly observed newborns and infants and have

cre-ated a large pool of items that could be used for

neuro-logic examinations The French angles are an excellent

example, forming a part of the measure of gestational age

by assessment when both physical and neurologic items

are combined (Amiel-Tison 1976) The progressions are

described from extreme immaturity to full term Reversed

progressions occur from full term to approximately 9–10

months in infancy The scarf sign, heel-to-ear, popliteal

angle, and leg abduction look similar in the preterm

neonate who has a gestational age of 28 weeks and in the

9- to 10-month-old infant Significant deviations are

in-dicative of hypotonia or hypertonia

Most of these clinicians have described and

recom-mended a far larger number of items than can be done

due to limitations of time for the clinician or tolerance of

the sick newborn Our first consideration has been to find

some method of limiting the number of items Second, the

examination needs to be reliable, using scientific

defini-tions for clinical measurement In short, the examination

method should have a mathematical cohesiveness of

reli-ability, should be highly correlated when used from one

time of examination to another, and should be highly

cor-related when used from one examiner to another To this

end, we have developed instruments of measurement for

the neurologic examination of three age groups: the

Pre-mie-Neuro for gestational ages 23–37 weeks (Daily and

Ellison 2005); the NeoNeuro & Up for the gestational

ages 38 weeks to age 4 months (Sheridan-Pereira and

El-lison 1991); and the Infanib for infants, ages 4–18 months

(Ellison and Horn 1985a; Ellison 1994) The details of

the methodology have been described previously (Ellison

1990) These three examinations assess aspects at the

different ages, each of which has a number of items ficient to assure validity (Table 2.1)

suf-Scored assessment instruments

The Premie-Neuro scoring sheet

The Premie-Neuro is a neurologic examination of preterminfants between the ages of 23 and 37 weeks of gesta-tional age It consists of 24 items divided into three fac-tors (Neurological, Movement, Responsiveness), eachwith eight items Only the first 16 items are scored if theinfant is very immature or on the ventilator because thesechecks can be done with minimal disturbance of the in-fant The items in Factor 1 (Neurological) address reflex-ive behavior, progression of muscle tone, and movementtype The items in Factor 2 (Movement) document rateper minute of behaviors and limb movement Last, theitems in Factor 3 (Responsiveness) address head andtrunk control as well as alertness and responsiveness Theexamination should be scheduled 5 to 1 hour before afeeding Asymmetry of findings should be noted for scor-ing The examination consists of techniques commonlyused for more mature infants but criteria for describingthe very immature infant’s responses differ (see photo-graphs in NeoNeuro examination)

both hands and extend them alongside the trunk,hold 3 seconds and release Note the amount of flex-ion at the elbow that is observed within 5 seconds.(a) >180° (b) 100–180° (c) 60–100° (d) <60°

grasp the wrist slowly and pull arms to vertical

Table 2.1 Scored neurologic assessments of the newborn and infant—comparative characteristics

Premie-Neuro NeoNeuro & Up Infanib

Age group to be tested 23–37 weeks gestational age 38 weeks gestation or 4–18 months of age

or post menstrual age post menstrual age to 16 weeks of age Diagnostic category for Abnormal, questionable, normal Severely abnormal, moderately Abnormal, transient, total score abnormal, mildly abnormal, normal normal

Factors (elements which Neurologic, movement, responsiveness Hypertonus, primitive reflexes, limb Spasticity, vestibular comprise the total score) tone, neck support, reflexes and function, head and trunk,

tremor, alertness, fussiness French angles, legs Number of items 16 ( ⬍28 weeks/on respirator) 32 20

24 ( ⭓28 weeks/off respirator) Behavioral measures

2 items

8 items

0 items

Score the amount of elbow flexion and resistance

that is noted at the moment the infant is initially

lifted off the surface

(a) >180° (b) 160–180° (c) 120–160° (d) 100–120°

(e) <100°

insert index finger into hand and gently press

pal-mar surface Grade according to strength of finger

flexion

(a) absent (b) weak flexion (c) medium flexion (d)

strong flexion spread to forearm (e) very strong–lifts

off bed

give pressure to the ball of the infant’s foot Grade

according to strength of toe flexion

(a) absent (b) weak (c) medium (d) strong (e) very

strong

move the arm across the infant’s chest until

resist-ance is met Observe the angle formed by the upper

arm and a line parallel to the trunk

(a) >85° (b) 60–85° (c) 45–60° (d) 15–45° (e) 0–15°

approx-imate knees and thighs to abdomen; extend legs by

gentle pressure with index finger behind each ankle

at the same time until resistance is met When

scor-ing this test, measure the angle of extension such

that 180° equals a fully extended knee

(a) >180° (b) 150–180° (c) 130–150° (d) 110–130°

(e) 90–110° (f) <90°

grasp both thighs and flex hips with knees extended

until resistance is met Measure the angle between

the infant’s trunk and legs

(a) <10° (b) 10–40° (c) 40–60° (d) 60–90° (e) 90–

100° (f) >100°

movement: sluggish, uncoordinated, jerky, athetoid,

stretching, smooth, alternating, both spontaneous

and elicited seen throughout the examination

(a) mostly sluggish (b) mostly stretching or smooth

(c) smooth alternating (d) markedly asymmetrical

(e) mostly tremulous

(trembling, shaking) observed in any part of the

body, including face, and extremities

flail-ing movements, which could involve head and

trunk, whole body, or single extremity

move-ments (frowns, grimaces, quizzical) seen during theexamination

re-sponse of the arms in rere-sponse to a loud noise,bright light, or if one is elicited spontaneously

dur-ing the examination

notice-able color change that is observed during the amination, including mottling, duskiness, pallor, orincreased redness anywhere on the body

arm movements observed during the examination

sponta-neous leg movements observed during the nation

grasp both wrists and by applying gently traction,elevate the shoulders about 45° Note the flexion re-sponse at the elbows (Done simultaneously with

No 18)

(a) >170° (b) 140–170° (c) 110–140° (d) 70–110°(e) <70°

gen-tle traction, elevate the shoulders about 45° serve the amount of head lag

examiner’s hands used to support the infant’s ders Observe the length of time the head is held in

shoul-an upright position

(a) head stays forward or backward (b) head up <3seconds (c) head up 3–10 seconds (d) head up >10seconds

sit-ting Allow head to fall forward as you hold theshoulders, wait 15 seconds Grade according toability to lift head and maintain it upright

(a) no attempt to raise head (b) tries but cannot raisehead (c) head upright by 30 seconds, drops head (d)head upright by 30 seconds, maintained (e) exam-iner cannot extend head

Allow head to drop backward as you hold theshoulders, wait 15 seconds Grade according toability to lift head and maintain it upright

(a) no attempt to raise head (b) tries but cannot raisehead (c) head upright by 30 seconds, drops head (d)head upright by 30 seconds, maintained (e) exam-iner cannot flex head

22 Alert Estimate the amount of time the infant is in

the quiet, alert state, i.e alert, with a bright look,

minimal motor activity, and regular respirations

(a) 0–4 sec (b) 5–10 sec (c) 11–30 sec (d) 31–60

sec (e) > 60 sec

abdomen in prone position and suspend

horizon-tally Observe curvature of back, flexion of limbs,

and relationship of head to trunk

throughout the examination, a subjective and

qual-itative assessment of the infant’s response to

move-ment, touching, handling, noise, hunger, etc

(a) not very responsive (b) average (c) very

respon-sive

Using the Premie-Neuro scoring sheet

The Premie-Neuro scoring sheet lists the test items and

their descriptions on the left side of the examination sheet

Each item should be evaluated and the appropriate

de-scription letter circled at that time On the right-hand side

of the page is the scoring for gestational ages 23–37

weeks When scoring items 1–7, record a score for both

the right and left extremities When an asymmetry is

pres-ent, score the lower value if there is a one-letter

differ-ence When the asymmetry is greater than or equal to two

levels, score the letter indicated in the central column and

its corresponding value for the postmenstrual age Enter

the points that correspond to the letter circled in the

scor-ing columns at the far right For items 9–16, determine

rate based on number of observations divided by total

time taken to complete the examination Each column is

summed to yield a factor score Factor scores are then

summed to yield a total score Scoring ranges for three

categories (normal, questionable, and abnormal) are

in-dicated for neonates of less than 28 weeks/on a respirator

and more than 28 weeks/off a respirator The scoring

sheet and manual are available from the authors

NeoNeuro & Up scoring sheet

Items 1–4 These four questions are asked of the main

caretaker by the examiner They make a nice introduction

to the baby and immediately give the examiner helpful

in-formation about apathy/irritability

(a) rarely (b) sometimes (c) often

(a) none (b) 1 (c) 2 (d) 3 (e) 4 (f) 5 (g) 6 or more

(a) too easy (b) easy (c) not so easy (d) difficult

(a) 1–3 min (b) 4–7 min (c) 8–12 min (d) 13–18 min(e) 19–24 min (f) 25 min or more

Make separate note of extension, semiflexion, ion, or strong flexion for arms and for legs Alsonote recurrent asymmetry The normal position for

flex-a full-term neonflex-ate is one of semiflexion or flexion

of both arms and legs (Figure 2.1)

ex-amination for decorticate, decerebrate, or tonic posturing In Figure 2.2A, there is flexion ofthe arms and extension of the legs (decorticate).There is also some neck retraction In Figure 2.2B,there is extension of the arms and extension of thelegs (decerebrate) In Figure 2.2C, the neonate as-sumes an opisthotonic posture Note also extension

opistho-of the arms and the clenched hands

are clenched, clenched with stress, closed, times closed, open In Figure 2.3A, the hands of anormal newborn are shown In Figure 2.3B, thehands are persistently clenched Note also theopisthotonic posturing

the little finger side of the hand Observe the degree

of flexion of the fingers and arm The normal degree

of flexion for a newborn is shown in Figure 2.4

balls of the feet and observe the degree of plantarflexion of the toes The normal degree of flexion for

a newborn is shown in Figure 2.5

Figure 2.1 Normal posture of full-term infant.

10 Asymmetric Tonic Neck Reflex Turn the head slowly

to one side, and hold it Observe for a fencing

posi-tion: extension of the arm near the face and flexion

of the opposite arm Repeat on the other side

Ob-serve whether this response is absent or present If

present, observe for ability of the infant to

over-come the position and for persistence of the

posi-tion The position for a normal newborn is shown

in Figure 2.6

move the arm across the chest Observe the angle

formed by the upper arm and a line parallel to the

body In Figure 2.7A, the angle is shown for a

nor-mal neonate In Figure 2.7B, the infant

demon-strates the excessive excursion of hypotonia or of

Figure 2.3 (A) Hand position of normal newborn (B) Hands

clenched in abnormal pattern.

A

B

Figure 2.4 Palmar grasp