Full text of DOI:10.1177/088307380001501004

OriginalArticle
PsychologicalFunctioningin
AdolescentsWith
Children and
Sturge-Weber Syndrome
Ann
Friedman, PhD; DavidLachar,
PhD
LynnChapieski, PhD;
ABSTRACT
Previous studies ofindividuals with
syndromehavefocused onthe medical aspects ofthis
syndrome, but
Sturge-Weber
little has been knownabout the affective andbehavioral correlates. Wecollected
andmedical datafrom
psychological
andteachersfor 79 children andadolescentswith
syndrome and
a
group oftheir siblings. Wealso
parents
obtainedthe results ofintellectual assessmentfor
Sturge-Weber
subset ofthe
a
The
youngpeople with
Sturge-Webersyndrome group.
Sturge-Webersyndromeexhibited
more
problemsthanthegroupofsiblings
across
a
numberofbehavioraldomains:intel-
lectual/academic, socialskills, mood, andcompliance.
withlowerlevels ofintellectual
Thosechildrenmostatriskfor
psychologicalproblems
werethose
those with seizure
andthose withmore
frequentseizures. Larger
olderchildren. Increased
functioning,
disorders,
port-winestains
werealso associatedwithanincrease inmoodand
socialproblems
but
onlyfor
wasnotassociatedwithlowerlevelsofintellectual oracademicfunctioning, butmoodandsocialproblemsweremore
age
commonin older children.
ChildNeurol
(J
2000;15:660-665).
Sturge-Webersyndrome,
characterized
a
rarecongenitalneurocutaneous
combination of
leptomeningealangioma. Glau-
calcifications andcorticalatrophy6,9,10 and
a
highincidence
disorder, is generally
facialport-winenevusand
comaoccursin atleast 30%ofcases,
a
a
of seizures.’,’
50% to 60% of individuals
by
Approximately
a
afflicted with
Sturge-Weber syndrome are mentally
somestud-
retarded.9,11 Hemiparesis, hemiplegia, visual field defects,
and headaches
although
ies have shown
a
prevalence as high as
76%.2-4
also be
Abilateral
Significant
may
present.2,7,8
port-
variations ofthe
also havebeen
reportedin
the
wine stain
may
involvementand
be associated with more extensive brain
syndrome
6
7
cases with nofacial
stain’
literature, including
port-wine
neurologic complications.
and
a
caseinwhichtheintracranial diseasewasnot
Althoughthereis growingbodyofliterature
a
describ-
appar-
as anadult.7In
ent until the
patient beganhaving seizures
the
psychologicalimpactoffacialport-winestainsingen-
little is knownaboutthe affective andbehavioral
ing
response, Roach
hasclassifiedthedisorderintothree
types:
eral,12-15
the most
involves both facial and
include
correlates
ofSturge-Weber syndrome specifically.
ofthe
In addi-
the
type 1,
common,
lep-
2
tomeningeal angiomas and may
glaucoma; type
tion to the social
consequences
port-wine stain,
involves
a
facial
angiomaandthe possibility
of
neurologic impairments associated with this syndrome
glaucoma,
is charac-
but no evidence of intracranial disease; type
3
should
placethis group ofindividuals atincreasedriskfor
terizedbyaleptomeningealangioma,
withnofacialinvolve-
problemsin
anecdotal
behavioral
Theliteratureincludes
functioning.
8
mentand
no
usually
glaucoma.
descriptionsof Sturge-Webersyndromepatients,
from the lep-
and &dquo;behavioral
Neurologic complications stemming
tomeningealangiomaincludetheformationofintracranial
noting &dquo;temper tantrums,&dquo; &dquo;irritability,&dquo;
problems.&dquo;16
Sturge-Webersyndrome,
Lee described three Chinese
twoofwhom
patients
with
presentedwithpara-
depressive pseudode-
noid disorders and the third with
a
mentia.l7SujanskyandConradicollectedinformationabout
emotional andbehavioral problems
childhood and
ReceivedFeb4, 2000. Acceptedforpublication Feb 11, 2000.
Fromthe Department of Pediatrics, Baylor College of Medicine (Dr
Chapieski), DepartmentofEducationalPsychology, University ofHouston
during
the adultyears throughtelephoneinterviews with
of adults with
a
group
(
Dr Friedman), andDepartment ofPsychiatry andBehavioral Science,
2
Sturge-Weber syndrome or their parents.2
University ofTexas-HoustonMedicalSchool(Dr Lachar), Houston, TX.
Thisresearchwassupportedby grantfromtheSturge-WeberFoundation.
Theirsubjectsreportedexperienceswithdepression, emo-
a
tional
and
orhyper-
that
outbursts,
activebehaviors.
noncompliant, self-abusive,
available datado
Addresscorrespondenceto DrLynnChapieski, BlueBirdCircle Clinic for
PediatricNeurology, 6565Fannin, NB-100,Houston, TX77030. Tel: 713-790-
Althoughthe
suggest
at risk for
5
046 ; fax: 713-793-1313; e-mail: mlchapie@texaschildrenshospital.org.
individuals with Sturge-Weber syndrome are
660
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6
61
with
the conclusions
Table 1.
Medical and DemographicCharacteristics of Participants
problems
psychological functioning,
c.....-,.. 1J1/.....l-..,.....
C:l-.I;,..._,...
tobedrawnfromthesereportsarelimitedbysmallsamples,
failuretousestandardizedmeasures, and/orlackofcontrol
theexistingdatadonotpermitanycon-
groups. Furthermore,
clusions aboutwhichofthese children are at greatestrisk.
Tobetterunderstandthe risk for behavioral andemo-
tional problems in children and adolescents with
Sturge-
Webersyndrome,weaskedparentsandteacherstocomplete
behavioralquestionnairesfor
a
groupofschool-agedchildren
with Sturge-Weber syndrome and their siblings. Psycho-
was assessed in four areas: intellec-
logical functioning
tual/academic, socialskills, mood,
and
compliance. In
order
to identifythose children whoare at
risk, wealso
greatest
numberofpotential
Finally,
assessedtheimpactof
a
and
neurologic
thepossibilitythat
investigated
socialriskfactors.
we
problemsin psychological functioning
nouncedin older children.
become more
pro-
METHODS
Participants
One hundred and four
ADHD attention-deficit hyperactivity disorder.
Numbers in parentheses are standard deviations.
parents of children
and adolescents with
Sturge-Weber syndrome
were contacted bythe national
Sturge-
WeberFoundationforparticipationinthis study. Thechildrenand
adolescents werebetween
and 18 years ofage. Surveypackets
werenumberedandmailedtotheparentsdirectlyfromtheFoun-
anonymityoftheparticipants. TheFoundation
4
child’s nameremovedorto sign
theresearcherto obtaintheinformationdirectly. Theresultsofcog-
nitive testing were obtained for 32 subjects with Sturge-Weber
a
release ofinformationallowing
dation, ensuringthe
madefollow-up telephone calls
parents to complete
encouraging
thepackets. Ultimately, 80 (77%) fullyorpartiallycompletedpack-
ets were
however, one subject with
syndromebut only
uations were received. IQ scores from these 32 subjects were
obtained from
variety of measures of intellectual functioning,
a
small numberofreports ofneurologic eval-
returned;
dromewasexcludedbecauseof
Sturge-Weber syn-
impairments
additionalneurologic
relatedto meningitis. Ifthere was
sibling in the familybetween
the of and18years, parentsandteacherswereaskedto com-
well. Whenthere wasmore
a
a
including the WechslerIntelligence Scale for Children, McCarthy
Scales ofChildren’sAbility, andthe Stanford-Binet. Familystress
was assessed with the Family Inventory of Life Events and
4
ages
plete questionnaires onthat child as
thanonesiblinginthis agerange, parentswereaskedto selectthe
whowasclosestinagetotheirchildwithSturge-Webersyn-
Changes. 18
Psychologicalfunctioninginthe subjectswithSturge-Weber
syndrome and their siblings was assessed with the Personality
sibling
drome. Notall familieshad
a
sibling in the specified agerange so
information wascollected ononly 59 siblings. The group ofsub-
jects withSturge-Webersyndromeandthegroupofsiblings were
demographicallysimilar. Themedicalandsocial characteristics of
the twogroups are presentedin Table 1.
InventoryforChildren,
Behavior Rating Scale,
a
parentreportmeasure, lgandtheStudent
teacher report measure.20,21 Both
a
ques-
tionnairesarewell-standardizedandvalidatedmeasuresof behav-
ioralfunctioninginchildren. Behavioralfunctioningwasevaluated
in four domains. Intellectual/academic
wasassessed
functioning
Measures
with the Intellectual Screening, Development, and Achievement
(academicperformance) ScalesfromthePersonalityInventoryfor
Children andthe Academic Performance Scale fromthe Student
BehaviorRating Scale. Socialskills wereassessedwiththe Social
Skills Scale fromthe Personality Inventory for Children andthe
SocialProblemsScalefromtheStudentBehaviorRatingScale. Prob-
lemswithmoodwereassessedwiththeDepression, Anxiety, With-
drawal, andSomaticConcernsScalesfromthePersonalityInventory
for Children and the Emotional Distress Scale fromthe Student
BehaviorRatingScale. Noncompliantbehaviorwasassessedwith
Parents
completed a questionnaire
concerning the educational
andmedicalhistoryofthe childwithSturge-Webersyndromeand
demographic characteristics ofthe family. Parents responded to
questionsaboutspecificriskfactorsforproblemswithpsychological
functioning, factorsthatincluded
der, seizure frequency (numberofseizures in the pastyear), and
thepresenceof
a
seizure disor-
the
a
of
a
Other
potentialrisk
factorsincluded
presence
hemiparesis.
the size ofthe
bilateralport-wine stain,
port-wine stain, level of
intellectual
andfamily stress. Informationaboutthe
functioning,
port-wine stain wasobtainedbyaskingparents either to provide
the Hyperactivity and Delinquency Scales from
the Personality
aphotographoftheir child withSturge-Webersyndromeor color
Inventory for Children and the Oppositional-Defiant Disorder,
Conduct Disorder, and Attention-Defici1JHyperactiB’ity Disorder
(ADHD) Scalesfromthe StudentBehaviorRating Scale.
intheport-winestainon
a
drawing. Parentswereaskedtoinclude
copies ofpsychoeducational and/orneurologic reports with the
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662
=
drenwithSturge-Webersyndrome:F(1,80) 56.77,
These not
P
<
.0001.
Analyses
Teacher questionnaires were returned for only 66 subjects with
Sturge-Weber syndrome and38 siblings.
fmdingsare
Sturge-Weber syndrome are
thatindividualswith
surprisinggiven
knownto be at risk for intel-
In addition, wheninfor-
eitherincompleteorambigu-
mationfrom
a
parentorteacherwas
lectual
impairments.
Theresults ofmultiple regression indicatedthatboth
of seizure disorder and the of
ous, thosedatawerenotincludedintheanalyses.As
the number of subjects included for different analyses varied
onthenumberofsubjectsforwhomtherelevantinfor-
a
consequence,
the
presence
a
presence
parentalreportof
a
depending
mation was available.
hemiparesis
were
significantly
relatedto
Group differences in
psychological func-
problemswithintellect andaccountedfor 56%ofthevari-
wereassessedwitheitherananalysis ofvariance
ance
The
ofaseizure disorderandthe
tioning
or
multivariate
(ANOVA)
of
(P
<
.0001).
presence
a
analysis
ofvariance
(MANOVA). The impact
of
a
werealso
relatedto
presence
hemiparesis
significantly
seizure disorder, hemiparesis, bilateral port-wine stain, size of
port-wine stain, and family stress on psychological functioning
of
and
48%
parental report
ofthe variance (P
disorder
developmentaldelay
<
explained
seizure
problems,
and teacher
.0001). Onlythe presence of
a
was assessed with
analyses. Information on
ofacademic
multiple regression
intellectualfunctioningandseizurefrequencywaseitheronlyrel-
evantoravailablefor
smallergroup ofchildren. Theimpacts of
predictedparentalreports
39% of the variance
<
explaining
ofacademic
(P
.0001),
57%ofthe vari-
a
reports
problems, explaining
thesetwovariableswere,therefore, assessedwithindividualPear-
sonproduct-momentcorrelation coefficients.
ance
P <
Not
there was
a
(
.0001).
surprisingly,
significant
inverse correlation between assessed level ofintellectual
and
of intellectual
functioning
accounting
parent report
for59%ofthevariance
problems,
RESULTS
<
(P .0001), developmental
problems, accounting for
and academic
56%
ofthe variance
(P < .0001),
The mean scores and standard deviations on each of the
behavioral scales fromthe Personality Inventoryfor Chil-
dren and Student Behavior
40% of the variance
problems, explaining
P<
Lowerlevelsofintellectual
werealso
(
.0004).
functioning
Rating Scale are presented in
associatedwithteacher
29%ofthevariance
ofacademic
problems,with
reports
Table 2.
explained (P< .004).
Withinthe
group
ofchildren whohad
was not
a
seizure disorder, seizure frequency
Intellectua1/ Academic Problems
related to either teacher or
significantly
parent
problems.
MANOVArevealeddifferencesbetweenthe two
on
of
oracademic
groups
reports
intellectual, developmental,
parent ratings ofproblems in
demic
the area ofintellectual/aca-
Neitherwasthere
a
betweenthesize
significantrelationship
=
P
<
.0001. Anexami-
ofthe
stain,
functioning: F(3,118)
34.75,
port-winestain,
the
presence
of
of
a
bilateralport-wine
intellectual, develop-
nation of the univariate
analyses indicated that
thechildrenwith
parents
or
family stress
and
reports
more
problemsfor
oracademic
reported
Sturge-Weber
mental,
problems.
syndromein
achievement:
=
P
<
devel-
.0001,
Because
ofpreviousreports
ofanassociationbetween
F(1,120)
51.32,
opment :F(1,120)
71.24,
ANOVA yielded
more academic
P
<
.0001,
andintellect: F(1,120)
similarresultsforteach-
problems for the chil-
the presence of
a
bilateral port-wine stain andneurologic
=
01.59, P<
.0001.
1
complications, wecomparedtheIQscoresofthosechildren
ers who
withbilateraland
unilateralport-winestains.
Therewasno
reported
significantgroupdifferenceinassessedlevelofintellectual
Neitherwerethosechildrenwithbilateralport-
functioning.
Table 2. GroupMeansof Scores onthe Personality Inventory
for Children andthe Student Behavior Rating Scale (SBS)
wine stains more
to have
within the
likely
ofchildren with seizure
seizures, and,
disorders, those withbilat-
group
eralport-wine
stainswerenotmore
tohavemorefre-
likely
quentseizures or earlierage ofonset.
Social Problems
Parents
more
problems with social
skills in the
reported
P
<
.0001.
Sturge-Webersyndromegroup:F(1,120) = 27.85,
Teachersalso
moresocial
problemsinthis group
reported
ofchildren:
25.61,
P
<
.0001.
F(1,103) =
Multiple regression revealed that the presence of
seizure disorder and increased
a
family stress
were
signifi-
cantly related to parent reports of poor social skills and
2
2%ofthevariance
(P< .0001).
The
of
explained
presence
a
seizure disorder andhemiparesis
predicted
34%ofthe
significantly
skills, explaining
teacher
ofpoorsocial
reports
variance
<
Assessed level of intellectual func-
(P
.0001).
wasinverselyrelatedtothelevel ofsocial
tioning
problems
4% of the variance
Scores are t-scores with
Higher scores indicate more problems.
Numbers in parentheses are standard deviations.
mean of 100 and standard deviation of 10.
a a
2
reported by parents, explaining
<
.005), but wasunrelated to teacher reports of social
(P
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6
63
Seizurefrequency, size ofport-winestain, andpres-
bilateral port-wine stain were not significantly
relatedtoeitherparentorteacherreportsofproblemswith
social skills.
thepresenceof
a
seizure disorderremainedinthemodelpre-
problems.
ence of
a
dictingteacherreports ofbehaviorsconsistentwithADHD
with24%ofthevariance explained(P
Novariables
<
.0001).
remainedinthemultipleregressionmodelpredictingteacher
reports ofbehaviors associated with
a conduct disorder.
ProblemswithMood
Within the
of children who had seizure disorders,
group
seizure frequency predicted teacher report of behaviors
consistent with
conduct 34%ofthe
Theresults oftheMANOVAindicatedthat, overall, parents
a
incidenceofmood
problemsinthechil-
a
perceived higher
disorder, explaining
.0001). Seizure frequency did nothave
dren with Sturge-Weber syndrome: F(4,117)
variance (P
<
a
14.05,
sig-
P
<
.0001.
Subsequentexaminationoftheunivariate analy-
nificant correlation with behaviors associated with either
ses, however, only revealed
a
effect for
ADHDor
oppositional-defiant disorder.
Assessed level of
correlated with
significant group
.0001,
.05. There were no
somatic
P
<
and
intellectual
was
concerns, F(1,120)
29.72,
depres-
functioning
negatively
=
P
<
behaviorsassociatedwithconduct
disorder, accountingfor
oppositional-defiant dis-
sion, F(1,120)
4.05,
significant
differencesforeitherwithdrawnbehavioror
18%ofthevariance
(P< .03),
and
group
Teachers
anxiety.
general
morebehaviorsassociatedwith
for28%of thevariance
(P<
Assessed
reported
emotional distress in the
order, accounting
levelofintellectual
.005).
Sturge-Weber syndrome group,
functioning
didnothave
a
significant
rela-
F(1,80) = 16.82,
P
<
.0001).
tionship
the
withbehaviorsassociatedwithADHD.Thesize of
revealedthat
the
of a bilateral
Multipleregressionanalyses
parentreports
port-wine stain,
presence
noneofthe
port-wine
ofnon-
ofdepressed
enceof
1%ofthevariance
moodwere
significantlypredictedbythepres-
and
familystress predicted
stain,
types
a
seizuredisorderand
familystress, accountingfor
compliantbehavior
reportedbyteachers.
3
P< .0001). Only
familystressremained
parentreports
(
inthe
model
Effect ofAgeonPsychological Functioning
The
ofthe child wasnot relatedto either
multiple regression
predicting
ofsomatic
for 11%ofthevariance
complaints, accounting
age
parentorteacherreportsofintellectual, developmental, or
academic problems. Neither wasthere significant asso-
significantly
P<
Novariablesremainedinthemultiple
regression
(
.007).
teacher
of
general emotional distress.
a
predicting
reports
Within the group of children who had seizure disorders,
ciationbetweenageandassessedlevelofintellectualfunc-
tioning. Both parents and teachers, however, were more
likelytoreportproblemswithsocialskills inolderchildren
withSturge-Webersyndrome. Ageofthechildexplained7%
seizure frequencywas
correlatedwithparent
significantly
ofsomatic
12%ofthe vari-
reports
ance(P
complaints, explaining
<
.01), butwasnotsignificantlyrelatedtoeitherpar-
ent
of
depression or
teacher
of
ofthevariance
in
reportsofsocialproblems
reports
reports
general
(P< .04)
parent
inteacher
emotionaldistress. Assessedlevel ofintellectual function-
and6%ofthevariance (P< .05)
problems.
inolderchildren.
reports ofsocial
had
a
inversecorrelationwith
Problemswithmoodwerealsomoreprominent
ing
significant
parentreports
ofdepression
with21%ofthevariance
explained(P< .009)
was
correlatedwith
par-
22%of the variance
Age
significantly
andparent reports ofsomatic complaints with 13%ofthe
ent reports of depression,
andteacher
explaining
variance
explained(P< . 05).
Assessedlevel ofintellectual
(P <
of
general emotional dis-
Therewas
.0001),
reports
25%ofthe
was also
correlated with teacher
variance(P< .0001).
functioning
negatively
tress, explaining
reportsofgeneralemotionaldistress, accounting
for 18%of
hemipare-
no
significant
with
increase in
parent reports
ofsomatic com-
thevariance
P<
Neitherthepresenceof
northe
a
age. Ageofthe
reports
for9%ofthevariance
childwasalso
cor-
(
.03).
plaints
related with teacher
significantly
thesize ofthe
of
a
bilat-
of
sis,
port-winestain,
presence
oppositional behavior,
eral
port-wine stain
was associated with either
parent or
(P<
Teachersdidnot
accounting
.02).
teacher
ofmood
morebehaviorsassociatedwitheitherADHDorcon-
reports
problems.
report
ductdisorder in older children.
Problemswith Compliance
TheMANOVAdidnotreveal
Becausetheolderchildreninoursamplewereexhibit-
ing more problems with behavioral and emotional func-
a
difference
significantgroup
across scales for
of
and that association could not be
by
parent report
compliance problems.
tioning
deterioratingcognitive
explained
skills, wedecidedto reconsiderthe
did
report higherincidenceof
a
com-
Teachers, conversely,
plianceproblemsinchildrenwithSturge-Webersyndrome,
effectofthesize of theport-winestainatdifferentages. The
P
<
.0001. Anexaminationoftheunivariate
analyses indicated that teachers reported morebehaviors
for
associatedwith
size ofthe port-wine stain wasnot
related to
F(3,100)
9.17,
significantly
teacherreportofoppositional
behavioror
parentreports
of
theSturge-Webersyndromegroup
socialproblemsat anyage. Thesize ofthe port-wine stain
oppo-
.0001; ADHD,
disorder, F(1,102)
sitional-defiantdisorder, F(1,102)
=
20.64,
P
<
did, however, have
of
a
significant relationship with parent
andteacher
emo-
23.10, P< .0001;
.03).
andconduct
reports ofgeneral
of
Inthis older
F(1,102)
reports
depression
P
<
tionaldistressinchildrenoverthe
9
years.
=
5.30,
age
Onlythepresenceof
a
seizure disorderremainedinthe
group of children, the size ofthe port-wine stain wassig-
nificantly correlated with parent reports of depression,
multiple regression model predicting teacher reports of
behaviors consistent with
accounting for
reports
of
9% of the variance
emotional
(P <
and teacher
25%of
oppositional-defiant disorder,
.05),
accountingfor 11%ofthevariance (P
distress, accountingfor
<
.05). Similarly, only
general
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664
thevariance
P<
Teacherreports of
socialproblems
donot
appear
to beworsein
whohavebilat-
(
.002).
Psychological problems
thosechildrenwith
werealso associatedwiththe size ofthe
port-winestain in
Sturge-Webersyndrome
this older
ance
ofchildren,
accountingfor
of
11%ofthevari-
eralport-winestains.
Neitherdolevels ofintellectualfunc-
tobelowerinthis
Parentsandteachers
group
P<
Teacher
socialproblems
wereasso-
(
.04).
reports
tioningappear
group.
cognitive problems and, within the
ofchildrenforwhomIQscoreswereavailable, there
group
ciated withmood
problems as well, accountingfor
40%of
did not
more
report
thevarianceinparentreports ofdepressedmood
and62%ofthevarianceinteacher
P<
(
.05)
ofemotionaldis-
was no
difference in intellectual
reports
significant
functioning
port-
winestains. Neitherwerethosechildrenwithbilateralport-
winestains more to have seizure disorders or, when
tress
P<
Thesize of
theport-wine stain
wasnot
betweenthosewithbilateralandthosewithunilateral
(
.002).
related to either
sig-
parent or teacher reports of
nificantly
emotional or social
problems in children
9
old or
years
likely
younger. Furthermore,the
stain didnotincrease
size ofthe effectoftheport-wine
theyhadthem,haveanearlier
ofonsetormorefrequent
thatany asso-
age
appreciably after
the
of 10years.
seizures. Taken
these
age
together,
findingssuggest
ciationbetweenbilateralport-winestainsandseverityofneu-
involvementmaybe
a
weakone.
Because of the social consequences of
that the size of the port-wine stain
rologic
DISCUSSION
a
port-wine
we
stain,
anticipated
predictor of emotional andbehavioral
Youngpeoplewith
Sturge-Webersyndromeappearto beat
would be
a
strong
in
riskfor
a
varietyofproblemswithbehavioralandemotional
Theparents andteachers whoparticipated in
people with
problems
young
Sturge-Weber
syndrome.
Weweresurprised, therefore, tofindthat, in ourtotalsam-
functioning.
this study reported
more
of children and adolescents with
significantly
cognitive, develop-
the children and ado-
ple
Sturge-Weber
syn-
and academic
problems for
drome, there
was no association between the size of the
mental,
lescents with
syndrome than they did for
a
port-winestainandemotionalorbehavioralproblems.After
Sturge-Weber
These
oftheir
group
of
under-
further
wefound
asthe areaofthe
siblings.
findingsare,
course,
analysis, however,
that,
standablein
oftheintellectual
impairmentsthat
condition.
arecom-
face coveredwith
a
port-wine stain
so did
par-
light
increased,
monin individuals withthis
ent andteacher
ofemotional distress andteacher
neurologic
reports
Parents andteachersalso
a
incidence
reports of
socialproblems
butonlyforchildren whowere
at least 10 years old. Theliterature onthe developmentof
reported higher
ofsocial problems, emotional distress, andproblemswith
with
complianceinthegroup
entsandteacherswere
Sturge-Webersyndrome. Par-
self-concept
forthis
in normal children
an
explanation
suggests
ingeneralagreement, although
only
Thereis
a
between
per-
finding.
strongrelationship
and
teachers
more
problems with noncompliance.
ceptions ofphysical attractiveness
self-esteem, butit
reported
Teacher reports of
behaviors, conduct problems, and behaviors associated
with ADHD. Thefailure of
noncompliance included oppositional
is
onlyolder
childrenwhocomparethemselvesto othersin
evaluating themselves.25 In addition, an enduring state of
parents to report higherlevels
ismorecommon
middlechildhoodand
unhappiness
later than it is at
during
ofbehaviorsassociatedwithADHDwas
surprisinggiventhat
The social
problems
correlatedwithboth
younger ages.26
22%ofthe
participantsinthis study
drome had been
with
reportedbyteachers
were
Sturge-Webersyn-
highly
moodandteacher
par-
ofemo-
with ADHD. The incidence of
entreportsofdepressed
diagnosed
reports
at least in
part,
diagnosed cases
Sturge-Weber
lenceof3%to 5%thatis reported
ofADHDin our sample of
youngpeople
thanthepreva-
tional
distress, suggesting
that
they are,
a
with
syndromeis much
of
a
mood
Teachers also
higher
consequence
more
problem.
behaviorintheolderchildrenwith
reported
Sturge-
forthegeneralpopulation
oppositional
of
children.22
Weber
syndrome but that
behaviorwasnot related to the
evenin older children.
school-aged
The
young people with Sturge-Weber syndrome
who
size ofthe
port-wine stain,
appeartobeatmostriskfor
emotional
a
broadrangeofbehavioraland
A
surprisingfindingwasthelackofassociationbetween
problems are those with lower levels of intel-
lectualfunctioning, thosewhohaveseizuresdisorders, and
those with more
andbothassessed andteacher
and
parent
age
reportsofintellectual/academicproblems.Thatis, theolder
children with
intelligence
frequent seizures. The identification of
Sturge-Weber syndrome in
clearanswertothe
problems.
question ofwhetherthese children deteriorate cognitively
our
sample did
theseriskfactors in this
sampleis
notparticularly
notexhibit
A
surpris-
greatercognitive
ing given that
associated with an increased incidence of
seizures and mental retardation are often
overtime will depend on
sectional datacollected as
data, but the cross-
study
leastwithinthe
psychological
longitudinal
part
notion, at
inindividualswithout
a
ofthis
do not
problems
The
Sturge-Webersyndrome. 23,24
pro-
of
a
hemiparesis
and
family stress
wererisk
vide
support
years. Thedatado
forthat
school-aged
presence
factorsin somesituations. Intellectual/academicproblems
peoplewith
however, thatthe social conse-
suggest,
syndromeincrease overtime.
weremorecommonly
reportedforthoseyoung
weremorelikelytoreportsocial
quences
ofSturge-Weber
a
hemiparesis, and
parents
problems whentheir children had ahemiparesis. Parents
were also morelikely to report social problemsandprob-
References
1
.
IwachA, Hoskins, HetheringtonJ, ShafferR: Analysisofsurgical
andmedical of in
syndrome.
lemswithmoodwhenthefamilieswere
experiencinggreater
management glaucoma Sturge-Weber
Opthalmology 1990;97:904-909.
levels ofstress.
Downloaded from jcn.sagepub.com at GEORGIAN COURT UNIV on March 11, 2015

6
65
2
.
SujanskyE, ConradiS: OutcomeofSturge-Webersyndromein 52
adults. AmJ Med Genet 1995;57:35-45.
Sullivan TJ, Clarke MP, MorinJD: The ocularmanifestations of
16.
Falconer M, Rushworth R: Treatment of encephalotrigeminal
angiomatosis(Sturge-Weberdisease) byhemispherectomy.Arch
Dis Child 1960;35:443-447.
3
.
Sturge-Webersyndrome. J Pediatr
OpthalmolStrabismus 1992;29:
17. Lee S: Psychopathologyin Sturge-Weber syndrome. Can
chiatry 1990;35:674-678.
J
Psy-
3
49-356.
Witschel H, Font RL: Hemangioma of the choroid:
casesand reviewof the literature. Surv
4
.
A
clinico-
18.
GrotevantH, Carlson C: FamilyAssessment:
ods andMeasures. NewYork, Guilford Press, 1989.
Wirt Lachar SeatP. Multidimensional
A
Guide to Meth-
pathologicalstudyof71
Ophthalmol 1976;20:415-431.
a
19.
R,
tion ofChildPersonality:
D, KlinedinstJ,
Descrip-
Manual forthePersonalityInven-
5
.
Pascual-Castroviejo I, Pascual-Pascual I, Viano J, et al: Sturge-
Webersyndromewithoutfacial nevus.Neuropediatrics 1995;26:
A
tory for Children, Revised 1984. Los Angeles, Western
Psychological Services, 1984.
2
20-222.
6
.
OakesJ: ThenaturalhistoryofpatientswiththeSturge-Webersyn-
drome.PediatrNeurosurg 1992;18:287-290.
20. Wingenfeld S, LacharD, Gruber C, Kline R: Development ofthe
teacher-informant Student Behavior Survey. PsychoedAssess
998; 16:226-249.
21. PiseccoA, LacharD, Gruber,
of disruptive behavior scales for the Student Behavior Survey
SBS).
PsychoedAssess 1999;17:314-331.
J
1
7
.
SujanskyE, Conradi S: Sturge-Webersyndrome: Age ofonset of
seizures and
glaucomaand
C1 9h 9i 5 l; d1 0 N: 4e 9u -r5 o8 l.
the
prognosisfor
affected children.
J
C
etal: Developmentandvalidation
Roach E: Neurocutaneous syndromes. Pediatr Clin NorthAm
992;39:591-620.
(
J
8
.
.
2
2. American Psychiatric Association: Diagnostic and Statistical
1
Manualof MentalDisorders, 4thed.
Psychiatric Association, 1994.
DC,American
Washington,
9
Pascual-Castroviejo I, Diaz-Gonzales C, Garcia-Melian R, et al:
Sturge-Weber syndrome: Study of 40 patients. Pediatr Neurol
2
3. SzymanskiL: Theretardedchild andadolescent, in Kestenbaum
C, Williams,
(eds): HandbookofClinicalAssessment ofChil-
dren andAdolescents, vol I. NewYork, University Press, 1988,
46-468.
Curley A: Behavioral disturbance in children with seizures, in
1993;9:283-288.
D
1
1
0.
Griffiths P: Sturge-Webersyndromerevisited: Therole of neuro-
radiology. Neuropediatrics 1996;27:284-294.
4
1. BebinE, GomezM: Prognosisin Sturge-Weberdisease: Compar-
2
4.
ison ofunihemispheric andbihemispheric involvement. Child
J
Tramontana M, Hooper
S
(eds): Advances in Child Neuropsy-
N1 98 e8 ; u3 :1 r8 1 -o1 8 l4 .
chology, vol 1. NewYork, Springer-Verlag, 1992, 109-136.
Harter S: The development of self-representation, in Damon
1
1
1
1
2. ShawW: Folkloresurroundingfacial deformityandthe originsof
2
5.
6.
W,
facial prejudice. Br PlastSurg 1988;34:237-246.
J
N
HandbookofChild
Psychology,
5thed, vol 3,
Eisenberg
(eds):
3. MalmM, Calberg M: Port-wine stain:
A
surgical andpsychologi-
Social, Emotional and Personality Development. NewYork,
calproblem. AnnPlastSurg 1988;20:512-516.
JohnWiley
&
Sons, 1998, 553-618.
4.
5.
Kalick S: Towards an interdisciplinary psychology of appear-
ances. Psychiatry 1978;41:243-252.
2
Bemporad J,LeeK: Affectivedisorders, inKestenbaumC, Williams
D
HandbookofClinicalAssessmentofChildrenandAdo-
(eds):
Lanigan
S, CotterillJ:
Psychologicaldisabilities amongstpatients
lescents,
626-649.
vol. 2. New
York,
NewYork
University Press, 1988,
withportwinestains. Br
J1 98 9D; 1e 2 r1 : m2 0a9 t- 2 o1 5l.
Downloaded from jcn.sagepub.com at GEORGIAN COURT UNIV on March 11, 2015