Table Of ContentWHEN AUTISM STRIKES
Families Cope with Childhood
Disintegrative Disorder
WHEN AUTISM STRIKES
Families Cope with Childhood
Disintegrative Disorder
Edited by
ROBERT A. CATALANO, M.D.
Foreword by
FRED R. VOLKMAR, M.D.
Springer Science+Business Media, LLC
Library of Congress Cataloging-in-Publication Data
file
ISBN 978-0-306-45789-0 ISBN 978-1-4899-5964-5 (eBook)
DOI 10.1007/978-1-4899-5964-5
© 1998 Springer Science+Business Media New York
Originally published by Plenum Press, New York in 1998.
http://www.plenum.com
All rights reserved
10987654321
No part of this book may be reproduced, stored in a retrieval system, or transmitted in any
form or by any means, electronic, mechanical, photocopying, microfilming, recording, or
otherwise, without written permission from the Publisher
We dedicate this book in loving memory of
Daniel George Kalmer, and to al1 children with disabilities
and their families.May they find the happiness they deserve.
Contents
Foreword FredR. Volkmar, M.D. ix
Preface xiii
One Per'sPages KjellBerg 1
Two Pickle Sheila Brown 13
Three Searchingfor the Blue Fairy:Thomas'sTale
Madeline Catalano 35
Four ComingHome MarieDay 69
Five Diary ofDavid JennyFairthorne 91
Six Laura'sStory Sue and Stephen Ferris 161
Seven Lookingfor Answers:Chronicles ofan Autistic Child
Candice Goldstein 191
Eight TheRiver Jordan CraigSchulze 221
AuthorBiographies 237
Index 239
vii
Foreword
In his classic description of the autistic syndrome, Leo Kanner (1943)
originallysuggestedthatchildrenwith autismwerebornwiththe disor
der. Subsequent research has modified this impression. It is clear that
most childrenwith autism do exhibitproblems in the first yearor year
anda halfofIife,butasmallgroup ofchildrendevelop autism, orsome
thingverysimilarto it,after whatappears to be 1 or even 2 years ofnor
mal development (Volkmar, Klin, & Cohen, 1997). Of course, various
factors might well act to advance or delaycase detection.Some parents
are more aware ofwhatto expectfrom normallydevelopinginfants and
note problemsthatotherparentsmiss. Insome cases,achildwho goeson
tohavehigher-functioningautismmay speakon timebutasthe childde
velops, more unusualsocialbehaviors or environmentalresponsiveness
are seen.The available data do, on balance, suggest that classical autism
almost neverdevelops after 3 years ofage.However,some childrenwho
develop normallyforaperiodofseveralyears goon to developaclinical
syndromevery similartoautism.Inconsideringhowbest toclassifysuch
disorders, we must turn to the work of the special educator Theodore
Heller.
ix
x WHENAUTISM STRIKES
Working in Vienna in the early years of this century, Heller re
ported on six children who exhibited severe developmental regression
between 3 and 4 years ofage, following aperiod ofapparently normal
development.This condition was initially termed dementia injantiJis
and, subsequently, Heller'ssyndrome,DisintegrativePsychosis,or,most
recently, Childhood Disintegrative Disorder (CDD). In the nearly 90
years since Heller's original report, over 100 cases have been reported
in the literature and the syndrome has undoubtedly been underrecog
nized (Volkmar & Rutter, 1995). The lack ofrecognition has reflected a
presumptionthat the conditionrepresented "late-onset" autism or was
the result ofthe insidious onset ofsome progressive neuropsychologi
cal process.As a result,this condition is unfamiliar to clinicians and
has onlyrecentlybeenrecognizedas an official diagnosis (APA,1994).
Several lines of data have supported the recent recognition of
CDD as a disorder apart from autism. The onset of the condition is
highly distinctive andan essential feature ofthe diagnosis.Consistent
with Heller's (1930) impression, the condition is usually observed af
ter 3 years and before 5 years in children who previously appeared
normal. The condition's onset may be abrupt (over days to weeks) or
more gradual (weeks to months);sometimes there is aperiod ofnon
specific agitationas the childbegins the dramaticregression that is the
hallmark ofthis condition.
The natural historyofthiscondition,once established,also differs
from more classical autism in that the long-termoutcome is apparently
worse than in autism.In about 75% ofthe cases the child's behavioral
and developmental functioning deteriorates to amuchlowerlevelthan
in autism and then plateaus. No further deterioration occurs, but sub
sequentgains are minimal. In othercases, theremaybe some limitedre
covery-s-for example,the child may regain the capacityto speaksingle
words. In only a handful of either of these types of cases has a child
been observed to make a major recovery of developmental skills.In a
small number of cases, deterioration progresses with no plateau and
may result in death. These are extreme cases where the condition is of
late onset and clearly associated with some known progressive neuro
logical disorder (Corbett, 1987). Life expectancy in all other cases ap
pears tobe normal.
Given the dramatic nature ofthe child'sregression,it is not sur
prisingthatformanyyears the presumptionwas thatCDDwas the overt
FOREWORD xi
manifestation ofsameidentifiableneurologicalorothermedicalcondi
tion and, therefore,did not deserve official recognition. Again this has
turnedoutnottobe the case. Althoughsuchcases havebeenobserved,
it usually is the case that exhaustive medical evaluations do not reveal
a specific medical "cause" although nonspecific abnormalities consis
tent with some as yet undiscovered neurobiological process or
processesofseizuresorabnormalbrainwave activityaresometimes ob
served. The lack ofrecognition ofthe condition has limited research,
and only now are systematic studies being conducted.Studies ofepi
demiology, Intervention, and, of course, neurobiology are very much
needed. When Autism Strikes:Families Cope with Childhood Disinte
grative Disorderwill increase awareness ofthis perplexing condition
and, as such, can only facilitate efforts to find its causes.
Thisvolumeis apoignanttestamentto the enormoustoll thatthis
condition takes on afflicted children and their families. Their tremen
doussufferingis intensifiedby theirexperience ofthe children'searlier
normaldevelopment, often documentedin videotapesrecordedpriorto
the regression. I applaud the courage of the families who,in this vol
urne,eloquentlyprovidetheirown accounts ofcopingwiththe human
tragedy that this conditionrepresents.
FredR. Volkmar,M.D.
HarrisAssociateProfessor
Child StudyCenter
Yale University
References
AmericanPsychiatrieAssociation (1994).Diagnostic andstatisticalmanualof
mentaldisorders. (4th ed.). Washington,DC:Author.
Corbett,J. (1987). Development,disintegrationanddementia.JournalofMental
DeficiencyResearch,31(4), 349-356.
Heller,T.(1930).UberDementiaInfantilis.ZeitschriftfuerKinderforschung, 37,
661-667.
Kanner, L. (1943).Autistic disturbances ofaffectivecontact. Nervous Cbild,2,
217-250.
Volkmar, ER., Klin A., & Cohen, D. (1997). Diagnosis and classification of
autism and related issues: Consensus and issues.In D.J. Cohen & F.R.
xii WHENAUTISM STRIKES
Volkmar, (Eds.), Handbook0/autism andpervasive developmentaldis
orders (znded.,pp. 5-59). New York:Wiley.
Volkmar,F.R.,&Rutter,M.(1995).ChildhoodDisintegrativeDisorder:Resultsof
the DSM-IV Autism Field Trial. Journal 0/the American Academy 0/
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