J. Paediatr. Child Health

(2003)

39

, 690–696

The health-related quality of life of childhood epilepsy syndromes

M Sabaz,

1,2

DR Cairns,

1

AF Bleasel,

3

JA Lawson,

2,4

B Grinton,

5

IE Scheffer

5,6

and AME Bye

2,4

1

Departments of Psychology, Macquarie University,

2

Women’s and Children’s Health, University of New South Wales,

3

Neurophysiology, The Children’s Hospital at Westmead,

4

Neurology, Sydney Children’s Hospital, Sydney, New South Wales,

5

Epilepsy Research Institute, University of Melbourne and

6

Monash Medical Centre, Royal Children’s Hospital, Melbourne, Victoria, Australia

Objective:

There is increasing awareness of the importance of assessing physical, psychological, social and behaviouralwell-being in chronic disease. The aim of this study was to examine the health-related quality of life (HRQoL) of childrenwith common epilepsy syndromes and to explore if there are HRQoL differences between those syndromes.

Methods:

Each child had their epilepsy syndrome defined according to the International League Against Epilepsyclassification. Epilepsy syndromes included symptomatic frontal, temporal, parietal/occipital lobe and partial unlocalizedepilepsy, and two idiopathic epilepsies, childhood absence epilepsy (CAE) and benign rolandic epilepsy (BRE). Seizuresemiology and ictal/interictal electroencephalogram (EEG) were determined for symptomatic partial epilepsy syndromes byvideo-EEG monitoring. HRQoL was evaluated with an epilepsy-specific instrument, the Quality of Life in ChildhoodEpilepsy Questionnaire, and two generic instruments, the Child Health Questionnaire and Child Behavior Checklist.

Results:

Children with symptomatic partial epilepsy syndromes were affected by epilepsy in a similar way and did nothave unique HRQoL profiles. However, these children had significantly lower HRQoL scores compared to those with CAEor BRE. All children with epilepsy regardless of syndrome had a higher frequency of behavioural problems compared tonormative data.

Conclusion:

These results indicate that children with epilepsy regardless of syndrome require evaluation of thepsychosocial implications. There is a greater impact on HRQoL in symptomatic epilepsy compared to idiopathic epilepsy.Specific symptomatic partial syndromes did not differ in the degree they affect HRQoL. These findings have importantimplications for clinicians caring for children with epilepsy.

Key words:

children; epilepsy syndromes; quality of life.

In childhood, epilepsy is associated with underachievement atschool, physical restrictions, lower social competence, lesssocial contact and higher rates of psychological and behav-ioural problems.

1–7

There is considerable evidence that thesevere long-term psychosocial consequences of epilepsy havetheir genesis in childhood.

8

These deficits in life function orhealth-related quality of life (HRQoL) have been correlatedwith a number of epilepsy related variables: frequency and/orseverity of the seizures, seizure type, aetiology, age of epilepsyonset, intellectual disability, cognitive deficits, and anti-epileptic drugs (AED).

1,5–16

Epilepsy syndromes are characterized by specific clusters ofsigns and symptoms. The most commonly used classificationof epilepsies is the 1989 proposal from the Commission onClassification and Terminology of the International LeagueAgainst Epilepsy (ILAE).

17

This classification provides acommon language amongst clinicians and is critical for effec-tive patient management. It is the basis of treatment strategies.Childhood epilepsy syndromes differ in their specific clinicalfeatures including aetiology (symptomatic

vs

idiopathic),seizure type, predictability and frequency of seizures, responseto medication and neuropsychological profile.

18–20

Therefore, itis plausible that the HRQoL of children with different epilepsysyndromes is uniquely affected. As diagnosis and managementdecisions are based on syndrome allocation it is appropriate toapproach the question of HRQoL using the same model.Importantly the availability of information on psychosocialfunctioning and HRQoL for each syndrome could be used to

counsel patients and families at the time of diagnosis. In thisway disease impact could potentially be modified.

Attempts have been made to outline functional profiles ofdifferent syndromes including Childhood and Juvenile AbsenceEpilepsy,

21

Benign Rolandic Epilepsy

7,15

and Temporal LobeEpilepsy (TLE).

22–25

Functional comparisons between differentsyndrome groups have also been conducted with the majoritycomparing TLE with FLE and generalized epilepsy syn-dromes.

13,14,26–34

However, these studies were inadequate interms of imprecise syndrome classification based only onhistorical descriptions of seizure semiology and interictal EEGdata. Additionally, the functional profile concentrated onnarrow domains such as behaviour and psychosis.

7,26,29–31

Quality of life assessment allows for a broader analysis of lifefunction.

It is the aim of this study to delineate a comprehensiveHRQoL profile of two common idiopathic (one partial, onegeneralized) and four symptomatic partial childhood epilepsysyndromes. A major methodologic difference in this study, toensure precise syndrome identification, is the utilization of theILAE classification

17

and video-EEG monitoring to documentseizure semiology and ictal and interictal neurophysiology insymptomatic epilepsy syndromes. First, we hypothesized thatall epilepsy syndromes will have a reduced HRQoL comparedto the normal population. Second, we hypothesized that thefour symptomatic partial syndromes will have a worse qualityof life compared to the two idiopathic syndrome groups.Finally, and of unique contribution, we aimed to explore whether

Correspondence: Associate Professor Ann M E Bye, Department of Neurology, Sydney Children’s Hospital, Randwick, NSW 2031, Australia.Fax: + 61 2 9382 1580; email: a.bye@unsw.edu.au

Accepted for publication 13 May 2003.

Epilepsy syndromes and quality of life 691

the two idiopathic syndromes and, separately, whether the foursymptomatic syndromes have different HRQoL profiles.

Health-related quality of life was assessed with the Qualityof Life in Childhood Epilepsy Questionnaire (QOLCE),

35

anepilepsy-specific childhood HRQoL instrument, developed byour group, and two generic instruments previously used toassess life function in childhood epilepsy, the Child BehaviorChecklist (CBCL)

36

and the Child Health Questionnaire (CHQ).

37

We elected to obtain parental-reports of HRQoL only due to theage related problems of paediatric self-report.

38

The QOLCE,CHQ and CBCL are known to be sensitive to importantvariables of interest in the epilepsy population.

1,3,15,35,9

TheCBCL has been shown to be sensitive to detecting differencesin partial epilepsies.

28

Inclusion of the QOLCE and the CHQenables these instruments to be potentially further validated inthe common partial childhood syndromes.

METHODS

Subjects

The majority of the research sample were children with symp-tomatic epilepsy syndromes recruited from two tertiary paedi-atric referral centres in the Sydney metropolitan area: SydneyChildren’s Hospital, Randwick, and the Children’s Hospital,Westmead. Children aged 4–18 years who had their typicalseizure recorded on video-EEG telemetry were included. Wehave previously shown the large effect that intellectual dis-ability can have on the measures included in this study,

1

therefore children with mild to profound mental retardationwere excluded. Children with progressive neurodegenerativedisorders or visual/hearing impairments were also excluded.

An independent sample of children with the syndrome ofCAE consistent with ILAE criteria

17

was recruited from twotertiary paediatric outpatient centres in Melbourne, Australia:the Austin and Repatriation Medical Centre and the RoyalChildren’s Hospital. An additional group of children meetingthe ILAE criteria for benign rolandic epilepsy (BRE) wererecruited from the four aforementioned hospitals. All relevantinstitutional ethics committees approved the study and informed/written consent was obtained from all families involved.

Epilepsy syndrome classification

Clinical classification of patients with symptomatic epilepsysyndromes according to the ILAE

17

was determined independ-ently by three epileptologists (AMEB, JL and AFB). Theclassification was based on four criteria: clinical data, ictalsemiology, interictal EEG epileptogenic activity and ictal EEGabnormality. Clinical data and that of medical history andneurological examination were obtained from all patients at thetime of admission to telemetry. Ictal semiology was determinedand the predominant seizure type classified from continuousvideo-EEG recording (duration 1–5 days). The EEG wasrecorded with the international 10–20 system supplementedwith sphenoidal electrodes and/or closely spaced electrodeswhen necessary to define the potential field of interictal spikesand seizure patterns. The interictal EEG was recorded in sleepand wake cycles and during routine activation procedures(hyperventilation and photic stimulation). It was reviewed forevidence of generalized or focal epileptiform discharges.Localization of the ictal EEG was based on the region of onsetof epileptiform activity. Epilepsy syndromes were classifiedinto idiopathic or symptomatic and generalized or partial.

Symptomatic partial epilepsies were classified as frontal (FLE),temporal (TLE), or parietal/occipital lobe (POLE) epilepsy. Ifthe epilepsy could not be localized to a single region as aboveit was classified as PARTIAL. Both idiopathic epilepsies, CAE(generalized) and BRE (partial), were classified independentlyby three investigators (AMEB, AB, IES) on the basis of ILAEcriteria

16

clinical data and outpatient EEG.

Measures

Families were sent a HRQoL package in the mail containingthe following instruments:1 Child Behavior Checklist (CBCL). The CBCL is a vali-

dated, normed instrument which has been used as a surro-gate quality of life measure in the paediatric epilepsypopulation.

1,6,7,32

It contains scales measuring physical,social and school competence, which are combined toobtain an overall competence score. In addition, the CBCLcontains eight scales measuring types of internalizing andexternalizing behavioural problems. A total behaviouralproblem score can also be calculated. Higher scores on thecompetence scales are indicative of higher levels of func-tioning, whereas higher scores on the behavioural problemscales are indicative of poorer levels of functioning. Thesummary competence and behavioural problem scores wererecorded for the purposes of this study.

2 The Child Health Questionnaire (CHQ). The CHQ is a vali-dated, normed instrument that has been used to assess levelof functioning in children with epilepsy.

35,37,39

It contains15 subscales assessing level of well-being. In addition, alinear combination of a subset of these subscales can beused to calculate two summary scores: a physical summaryscore and a psychosocial summary score. Higher scores areindicative of superior levels of functioning.

3 Quality of Life in Childhood Epilepsy Questionnaire(QOLCE). The QOLCE is a 76-item parent-rating instru-ment containing 16 subscales covering five domains of lifefunction: physical function, social function, emotionalwell-being, cognition and behaviour.

1,35

Items are rated ona 5-point Likert scale, which are used to calculate the 16subscale scores ranging from zero (low functioning) to 100(high functioning).

4 The Child Seizure Profile (CSP). The CSP is a supplementto the QOLCE and contains questions for parents recordingfacts and observations about their child’s seizures and medi-cations. Two questions from the CSP have been used in pre-vious investigations:

1,35

the number of AED currently takenand the parent’s perception of the severity of their child’sseizures during the past 6 months (very mild, mild, moder-ate, severe or very severe).If the above package of questionnaires was not returned,

families were given reminder phone calls at 2–4 weeks.Other clinical data collected for the study included: gender,

age at time of quality of life assessment, age of epilepsy onset,time of quality of life assessment relative to epilepsy onset(time since onset in years).

Statistic analysis

All statistical analyses were conducted using the StatisticalPackage for the Social Sciences (SPSS) version 10.

40

Comparison of the syndrome groups in terms of age, age ofepilepsy onset and duration of time since onset of seizures wasperformed using Analysis of Variance (

ANOVA

). Comparing the

692 M Sabaz

et al

.

syndrome groups in terms of gender, number of AED taken andseizure severity was performed using the Fisher exact

χ

2

statistic.In order to determine the extent of behavioural problems in

different syndromes, we used an established methodology toclassify children’s CBCL scores in either the clinical or normalrange.

1

We used the Fisher exact

χ

2

statistic to determine if theproportions falling in the clinical range in the epilepsy sub-groups differed from the published base-rate in the CBCLnormative sample.

36

Eighteen percent of the CBCL normativesample had CBCL Total Behaviour Problem Scores falling inthe clinical range. Five percent of the CBCL normative samplehad a CBCL Total Competence Score falling in the clinicalrange.

In order to control for type I error when determining if therewere different HRQoL profiles for specific syndromes, separateMultivariate Analysis of Variance (

MANOVA

) were conductedusing either the QOLCE or CHQ subscales as the dependentvariables. In addition, the syndromes were compared in termsof their CBCL total problems and total competence scoresusing

ANOVA

. Main effects were judged to be significant at the0.05 level. In order to control for multiple comparisons,posthoc analyses were judged to be significant at the 0.003level.

RESULTS

Response rate and description of sample

Of the 134 families who agreed to participate and met theinclusion criteria, 89% returned the questionnaire package.Therefore, the obtained study-sample was 119 parents of chil-dren with epilepsy. Children with generalized symptomaticepilepsy (

n

= 4) were excluded due to small numbers. Asummary of the clinical data and syndromal classification ofthose children remaining (

n

= 115) is shown in Table 1. Thesyndromes did not significantly differ in gender, mean age ormean age of epilepsy onset (

P

> 0.05). Benign rolandic epi-lepsy patients underwent quality of life assessment signifi-cantly closer to the time of epilepsy onset compared to TLE(

P

< 0.01) and PARTIAL groups (

P

< 0.01). No significantdifference between the time of quality of life assessmentrelative to age of epilepsy onset was evident for other syndrome

groups. There was a significant difference in the number ofmedications taken by the syndrome groups (

P

< 0.05). Childrenwith BRE took significantly less medication than children withFLE (

P

< 0.01). There was a trend for children with BRE totake significantly less medication than children with TLE(

P

= 0.01) or PARTIAL (

P

= 0.03). There was also a trend forchildren with CAE to take less medication than children withFLE (

P

= 0.04). Parental rating of seizure severity during thepast 6 months differed significantly between syndromes(

P

< 0.05). Children with BRE or CAE were significantly morelikely to have no seizures or experience less severe seizures(mild to very mild) compared to children in the PARTIALgroup (

P

< 0.01). There was a statistical trend for children withCAE to have no seizures or less severe seizures than childrenwith FLE (

P

= 0.02) or TLE (

P

= 0.05). No other results weresignificant.

Sixteen (14%) QOLCE, 11 (10%) CHQ and 13 (11%)CBCL questionnaires were returned partially complete. In theseinstances the missing data were replaced by mean imputation.Mean imputation did not result in significant changes from theanalysis of only complete cases and, therefore, the followingresults reflect those with mean imputed data replacing missingvalues.

Prevalence of behavioural and social problems in the different epilepsy syndromes

All syndrome groups (68% TLE, 89% FLE, 38% POLE,100% PARTIAL, 62% CAE, 57% BRE) had a significantlyhigher proportion of children with an Overall Social Com-petence Score falling within the clinically abnormal rangecompared to the base-rate normative data (i.e. 5%) (

P

< 0.05).With regard to the Overall Behavioural Problem Score, therewas a significantly higher proportion of children scoring inthe clinical range in TLE (48%), FLE (44%), PARTIAL(56%) and CAE (44%) syndromes (

P

< 0.05). However,children with POLE (38%) or BRE (29%) did not have asignificantly higher likelihood of scoring in the clinical rangecompared to the normative base-rate (i.e. 18%) (

P

> 0.05).The proportion of children with behavioural problems did notdiffer significantly between the symptomatic (FLE, TLE,POLE, PARTIAL) (

P

> 0.01) or idiopathic (BRE, CAE)(

P

> 0.01) groups.

Table 1

Summary of clinical data by epilepsy syndrome group

TLE FLE POLE PARTIAL CAE BRE

n

25 17 8 16 27 21Female gender 13 6 3 9 20 12Mean age (SD) 11.57 (4.04) 11.58 (4.01) 10.92 (2.40) 11.15 (4.29) 9.96 (3.78) 9.53 (2.59)Mean age of onset (SD) 4.52 (4.14) 5.59 (3.83) 3.25 (3.06) 4.19 (3.56) 5.33 (4.09) 7.19 (1.72)Time since onset in years 7.05 (4.11) 5.79 (4.90) 7.67 (4.13) 6.96 (4.26) 4.74 (3.52) 2.34 (2.81)Number of AED:

zero or one 11 5 5 7 17 18two 10 5 2 5 6 2three or more 3 7 1 4 4 1

Seizure severity:very severe to severe 7 4 5 4 3 1moderate 7 6 2 3 3 4mild to very mild 6 2 1 3 14 7no seizures 4 5 0 5 7 9

AED, antiepileptic drugs; BRE, benign rolandic epilepsy; CRE, childhood absence epilepsy; FLE, frontal lobe epilepsy; POLE, parietal/occipitallobe epilepsy; SD, standard deviation; TLE, temporal lobe epilepsy. Two families (one in the TLE group and one in the PARTIAL group) did not ratethe severity of their child’s seizures. One family in the TLE group did not specify the number of AED.

Epilepsy syndromes and quality of life 693

HRQoL profile of unique epilepsy syndrome groups

Multivariate analysis of the symptomatic partial syndromes(TLE, FLE, POLE, PARTIAL) found no significant differencesin the scores of the CBCL, CHQ and QOLCE. Similarly, nosignificant differences on any of the scores from the threequestionnaires was detected on comparison of the idiopathicgeneralized epilepsy group (CAE) with the idiopathic partialepilepsy group (BRE). Therefore, for the purposes of subse-quent analyses the four symptomatic partial epilepsy groupswere collapsed into a group labeled ‘symptomatic epilepsy’ andthe two idiopathic epilepsy syndromes were collapsed into agroup labeled ‘idiopathic epilepsy’.

HRQoL comparison of idiopathic and symptomatic epilepsy

The mean CBCL Total Behaviour Problem score for the sympto-matic and idiopathic epilepsy groups were 59.60 (SD = 10.62)and 53.90 (SD = 13.24), respectively. The mean CBCL Compe-tence scores for the symptomatic and idiopathic epilepsy groups

were 38.42 (SD = 8.47) and 41.61 (SD = 11.40), respectively.

ANOVA

demonstrated that syndrome type had a significant effecton CBCL Total Behaviour Problem scores (

P

< 0.05) but notCompetence scores (

P

> 0.05).The

MANOVA

of the symptomatic and idiopathic epilepsygroups against a linear combination of the 14 CHQ subscaleswas not significant (

P

> 0.05). Follow up pairwise comparisonsshown in Table 2 demonstrate that the symptomatic epilepsygroup had significantly lower physical function, social limita-tions due to behavioural difficulties, social limitations due tophysical health, self-esteem and emotional impact on parentscores compared to the idiopathic epilepsy group (

P

< = 0.003).Children with idiopathic epilepsy (mean = 48.01, SD = 5.83)had significantly higher CHQ physical summary scores com-pared to children with symptomatic epilepsy (mean = 40.54,SD = 11.56) (

P

< 0.003). Children with idiopathic epilepsy(mean = 39.94, SD = 9.17) had significantly higher CHQ psycho-social summary scores compared to children with symptomaticepilepsy (mean = 33.83, SD = 10.85) (

P

< 0.003).A

MANOVA

of the symptomatic and idiopathic groupsagainst the 16 QOLCE subscales was significant (

P

< 0.05).Follow up pairwise comparisons shown in Table 3 reveal that

Table 2

Univariate comparisons symptomatic and idiopathic epilepsy syndrome groups against CHQ subscales.

CHQ subscale Symptomatic Idiopathic

P

Physical function 84.21 (18.77) 95.61 (8.26) < 0.001Social limits

†

62.77 (37.11) 81.43 (25.55) 0.003Social limits

‡

20.65 (35.31) 90.13 (17.55) 0.001Global behaviour 69.23 (28.52) 74.93 (22.74) 0.254Behaviour 64.23 (19.25) 73.82 (14.85) 0.005Mental health 69.84 (17.81) 76.22 (15.43) 0.048Self-esteem 62.65 (22.86) 74.88 (16.23) 0.002Bodily pain/discomfort 77.33 (23.78) 85.25 (17.24) 0.052Global health 66.37 (25.72) 77.57 (20.19) 0.013General health perceptions 57.89 (18.92) 67.42 (16.12) 0.006Change in health 3.22 (0.95) 3.54 (1.05) 0.094Emotional impact on parent 42.55 (28.44) 58.87 (26.81) 0.002Time impact on parent 63.81 (30.76) 78.88 (23.05) 0.005Family activities 66.94 (26.30) 77.81 (22.39) 0.022Family cohesion 72.34 (20.27) 75.11 (20.81) 0.476

Table shows the mean score (standard deviation) on each subscale for each epilepsy group (

n

= 115).

†

Social limitations due to behavioural difficul-ties;

‡

Social limitations due to physical health; CHQ, Child Health Questionnaire.

Table 3

Univariate comparisons symptomatic and idiopathic epilepsy syndrome groups against QOLCE subscales

QOLCE subscale Symptomatic Idiopathic

P

Attention 50.78 (22.96) 69.27 (22.22) < 0.001Memory 47.60 (22.89) 71.04 (21.20) < 0.001Language 54.29 (21.96) 74.28 (22.74) < 0.001Other cognitive 48.71 (22.79) 67.10 (26.69) < 0.001Social interactions 59.40 (30.32) 85.43 (20.13) < 0.001Social activities 58.72 (30.90) 86.56 (18.16) < 0.001Stigma 72.71 (26.57) 86.24 (22.30) 0.005Depression 69.56 (15.33) 80.00 (13.80) < 0.001Anxiety 65.12 (18.73) 73.85 (19.02) 0.016Control/helplessness 64.72 (19.58) 74.61 (19.94) 0.009Self-esteem 62.63 (18.92) 74.95 (18.54) 0.001Physical restrictions 56.36 (19.61) 69.07 (19.46) 0.001Energy/fatigue 46.11 (20.90) 58.34 (19.00) 0.002Behaviour 62.04 (14.65) 70.64 (14.36) 0.002Quality of life 57.85 (28.83) 77.96 (21.71) < 0.001General health 53.51 (29.81) 67.71 (26.78) 0.010

Table shows the mean score (standard deviation) on each subscale for each epilepsy group (

n

= 115). QOLCE, Quality of Life in ChildhoodEpilepsy Questionnaire.

694 M Sabaz

et al

.

the symptomatic group had significantly lower scores thanchildren with idiopathic epilepsy on 12 of the 16 QOLCEsubscales (

P

< = 0.003). The overall QOLCE quality of lifescore was significantly lower for the symptomatic (mean =58.63, sd = 14.41) compared to the idiopathic group (mean =73.61, SD = 12.54) (

P

< 0.003).

DISCUSSION

In this study we have confirmed that epilepsy regardless ofsyndromal diagnosis is associated with a reduced HRQoL. Inaddition, symptomatic partial epilepsy syndromes are associ-ated with poorer HRQoL compared to a common idiopathicpartial epilepsy syndrome, BRE, or a well recognized general-ized idiopathic epilepsy syndrome, CAE. However, within thesymptomatic and idiopathic syndromes, there was no differencein HRQoL between the specific syndromal groups. The syn-dromes did not differ with regard to age, age of epilepsy onsetand gender. However, compared to the symptomatic syndromeschildren with the idiopathic syndromes, CAE and BRE, weremore likely to take fewer or no AED and have less severe or noseizures during the past 6 months. In addition, children withBRE underwent HRQoL assessment closer to the time ofepilepsy onset compared to symptomatic epilepsy groups. Theresponse-rate of the study was 89 percent. Given this excellentresponse-rate it is unlikely that our results reflect response bias.

Regardless of syndrome type all children with epilepsydemonstrated high rates of psychosocial problems. In childrenwith symptomatic epilepsy and CAE, the proportion of thosewith behavioural problems that fell within the clinical rangewas two to three times that of published norms. These behav-ioural problems did not occur more frequently in children withBRE. Nevertheless the latter group were not completelyunaffected by their epilepsy as shown by their high rate ofsocial problems compared to the published norms. This findingcontributes to a growing literature demonstrating the cognitiveand functional impact of BRE despite the ‘benign’ label.

7,15

No significant differences were found between children withCAE and BRE on any of the HRQoL measures. In contrast,Loiseau

et al

.

26

found that children with typical absenceseizures were more likely to have psychiatric problems andpoor social adaptability compared to children with BRE. Thereare two possible reasons for these discrepant results. First,Loiseau

et al

. included patients with a variety of seizure types –classical simple absences, myoclonic absences and absenceswith significant atonic components. In these patients withidiopathic generalized seizures it is probable that patients ofdiffering syndromal diagnoses with varying prognosis wereincluded in the one group. Second, Loiseau

et al

. definedpsychiatric problems and poor adaptability informally on thebasis of conversations with families and doctors compared toour use of psychometrically valid, standardized measures.

Children with CAE and BRE did have significantly higherscores on a number of psychosocial, HRQoL measures com-pared to children with symptomatic epilepsy. Sillanpaa studiedchildren with partial epilepsy and found that those with symp-tomatic partial epilepsy had more adjustment difficulties thanpatients with idiopathic partial epilepsy.

16

Our findings extendthese results by comparing symptomatic partial syndromes notonly with an idiopathic partial group but also an idiopathicgeneralized epilepsy, CAE. Given the relatively good prognosisin CAE and BRE

20,25

with lower medication load and usualgood response to treatment the finding of a better HRQoL inthe idiopathic groups is not surprising. The known degree ofcollinearity between medication load, seizure severity and

epilepsy syndrome diagnosis makes it difficult to disentanglethe individual contribution of these variables.

18

Comparison of the common symptomatic partial epilepsiesof childhood revealed no significant difference in any of thepsychosocial, HRQoL measures. It has been hypothesized thattemporal lobe dysfunction is associated with higher rates ofpsychological and behavioural problems because of this lobe’shigh connectivity with the limbic (emotional) system. Conflict-ing results have been achieved in adult research that hasattempted to define a TLE personality or behavioural syn-drome. Some demonstrated that TLE is associated with higherrates of psychosis and behavioural problems,

29,30

whereasothers found no group differences.

7,31

A recent review of thisliterature concluded there was no evidence for a unique constel-lation of personality and behavioural traits with any specificepilepsy syndrome.

33

In all of these studies, classification of thelocalization syndrome groups was based on historical descrip-tions of seizure semiology and interictal EEG data only. Thecurrent study uses a superior methodology with localizationsyndrome classification based on the ILAE criteria and ictalvideo-EEG data. We, along with others have shown thatspecific symptomatic partial epilepsy syndromes may be asso-ciated with unique clinical and cognitive features,

19,20,35

butthis does not translate into different HRQoL profiles forchildren. Overall there appears to be a generic psychosocialdecline that is related to epilepsy severity. This is an importantcritical finding that should avoid discriminatory labelling of achild as having a TLE personality and this information shouldbe given to parents early in the course of the disease. Factorscommon to all symptomatic epilepsies such as the severity ofthe seizures and response to medication may determine theeffect on HRQoL.

An important methodological question that arises in HRQoLresearch relates to the value of quantitative tools currentlyavailable. There is an assumption in the literature that disease-specific HRQoL questionnaires are more sensitive than genericquestionnaires to the psychosocial impact of epilepsy.

10,35

However, minimal evidence is available to evaluate thisassumption. Interestingly, an adult study provided support thata generic instrument was more sensitive to the psychosocialimpact of epilepsy surgery than an epilepsy-specific instru-ment.

41

No data have been published evaluating this hypothesisin children. The current study provides data on the relativesensitivity of the CBCL, CHQ and QOLCE in detectingpsychosocial differences. The summary and a number of sub-scale scores of the CBCL, CHQ and QOLCE were sensitive tothe higher functional level of children with idiopathic syn-dromes compared to those children with symptomatic syndromes.No instrument was sensitive to differences between the foursymptomatic groups or the two idiopathic groups included inthis study. Within their respective idiopathic and symptomaticcategories the syndromes were well controlled on a number ofclinical variables relevant to epilepsy. This suggests that theCBCL, CHQ and QOLCE are sensitive to clinical variablesused to reach a syndromal diagnosis (for example age of onset,seizure type and frequency, medication and cognitive function-ing) but they are relatively insensitive to these syndromalcategories themselves. This is likely due to the high degreeof collinearity between syndromal group and other clinicalvariables.

In summary, this is the first study to utilize a methodology toaccurately identify syndromes in order to outline a comprehen-sive HRQoL profile for the common childhood epilepsies. Thisstudy showed that all children with epilepsy regardless ofsyndrome experience a higher frequency of functional prob-lems compared to the children in the normal population. The

Epilepsy syndromes and quality of life 695

result indicates that all children with epilepsy require attentionto physical activities, social interactions, academic achieve-ment, cognition and behaviour, domains that collectivelyreflect HRQoL. Children with BRE and CAE have a similarHRQoL profile and both do functionally better than childrenwith symptomatic partial syndromes who also do not differ intheir HRQoL profile. This information is of great practicalimportance to clinicians. To recognize epilepsy has a signifi-cant impact on HRQOL and to give an accurate picture of thepsychosocial consequences associated with specific epilepsysyndromal diagnoses is a critical part of patient management. Inaddition, it dispels myths that particular psychosocial problemsare associated with specific syndromal diagnoses.

This study further supports the sensitivity of two genericinstruments, the CBCL and CHQ, and an epilepsy-specificinstrument, the QOLCE, in detecting HRQoL limitations inchildren with epilepsy. In particular, it extends the properties ofthe latter tool building on previous work relating the QOLCE toepilepsy variables of seizure burden and medication as well asthe patient’s intellectual status. Validation of such tools isessential for further research into the implications of thediagnosis of epilepsy.

ACKNOWLEDGEMENTS

This work was supported by the National Health & MedicalResearch Council (NH & MRC) and the Movement DisordersFoundation. We thank the patients and their families forparticipation in this study.

REFERENCES

1 Sabaz M, Cairns DR, Lawson JA, Bleasel AF, Bye AME. Thehealth-related quality of life of children with refractory epilepsy:a comparison of those with and without intellectual disability.

Epilepsia

2001;

42

: 621–8.2 Caplan R, Guthrie D, Komo S

et al.

Social communication inchildren with epilepsy.

J. Child Psychol. Psychiatry

2002;

43

:245–53.

3 Austin JK, Harezlak J, Dunn DW, Huster GA, Rose DF,Ambrosius WT. Behavior problems in children before first recog-nized seizures.

Pediatrics

2001;

107

: 115–22.4 Dunn D, Austin J, Huster G. Behaviour problems in children with

new onset epilepsy.

Seizure

1997;

6

: 283–7.5 Caplan R, Arbelle S, Guthrie D

et al.

Formal thought disorder andpsychopathology in pediatric primary generalised and complexpartial epilepsy.

J. Am. Acad. Child Adolesc. Psychiatry

1997;

36

:1286–94.

6 Schoenfeld J, Seidenberg M, Woodard A

et al.

Neuropsycho-logical and behavioral status of children with complex partialseizures.

Dev. Med. Child Neurol.

1999;

41: 724–31.7 Weglage J, Demsky A, Pietsch M, Kurlemann G. Neuropsycho-

logical, intellectual, and behavioral findings in patients withcentrotemporal spikes with and without seizures. Dev. Med. ChildNeurol. 1997; 29: 646–51.

8 Jalava M, Sillanpaa M. Concurrent illness in adults with childhood-onset epilepsy: a population-based 35-year follow-up study.Epilepsia 1996; 37: 1155–63.

9 Aman MG, Werry JS, Turbott SH. Behavior of children withseizures: Comparison with norms and effect of seizure type.J. Nervous Mental Dis. 1992; 180: 124–9.

10 Devinsky O, Westbrook L, Cramer J, Glassman M, Perrine K,Camfield C. Risk factors for poor health-related quality of life inadolescents with epilepsy. Epilepsia 1999; 40: 1715–20.

11 Austin JK, Huster GA, Dunn DW, Risinger MW. Adolescentswith active or inactive epilepsy or asthma: a comparison of qualityof life. Epilepsia 1996; 37: 1228–38.

12 Carpay HA, Vermeulen J, Stroink H et al. Disability due to restric-tions in childhood epilepsy. Dev. Med. Child Neurol. 1997; 39:521–6.

13 Ott D, Caplan R, Guthrie D et al. Measures of psychopathology inchildren with complex partial seizures and primary generalisedepilepsy with absence. J. Am. Acad. Child Adolesc. Psychiatry2001; 40: 907–14.

14 Mandelbaum DE, Burack GD. The effect of seizure type andmedication on cognitive and behavioral functioning in childrenwith idiopathic epilepsy. Dev. Med. Child Neurol. 1997; 39:731–5.

15 Croona C, Kihlgren M, Lundberg S, Eeg-Olofsson O, Eeg-Olofsson K. Neuropsychological findings in children with benignchildhood epilepsy with centrotemporal spikes. Dev. Med. ChildNeurol. 1999; 41: 813–18.

16 Sillanpaa M. Disabilities in Children with Focal Epilepsies. In:Tuxhorn I, Holthausen H, Boenigk H, eds. Paediatric, epilepsysyndromes and their surgical treatment. London, UK: JohnLibbey, 1997; 33–40.

17 Commission on Classification and Terminology of the Inter-national League Against Epilepsy: Proposal for revised classifica-tion of epilepsies and epileptic syndromes. Epilepsia 1989; 30:389–99.

18 Aicardi J. Epileptic syndromes in childhood. Epilepsia 1988; 29(Suppl. 3): S1–S5.

19 Hernandez MT, Sauerwein HC, Jambaque I. Deficits in executivefunctions and motor coordination in children with frontal lobeepilepsy. Neuropsychologia 2002; 40: 384–400.

20 Hermann B, Seidenberg M, Schoenfeld J, Davies K. Neuro-psychological characteristics of the syndrome of mesial temporallobe epilepsy. Arch. Neurol. 1997; 54: 369–76.

21 Wirrell EC, Camfield CS, Camfield PR, Dooley JM, Gordon KE,Smith B. Long-term psychosocial outcome in typical absenceepilepsy: Sometimes a wolf in sheeps’ clothing. Arch. Pediatr.Adolesc. Med. 1997; 151: 152–8.

22 Bear DM, Fedio P. Quantitative analysis of interictal behavior intemporal lobe epilepsy. Arch. Neurol. 1997; 34: 454–67.

23 Naugle RI, Rodgers DA, Stagno SJ, Lalli J. Unilateral temporallobe epilepsy: an examination of psychopathology and psycho-social behavior. J. Epilepsy 1991; 4: 157–64.

24 Pritchard PB, Lombroso CT, McIntyre M. Psychologicalcomplications of temporal lobe epilepsy. Neurology 1980; 30:227–32.

25 Apter A, Aviv A, Kaminer Y, Weizman A, Lerman P, Tyano S.Behavioral profile and social competence in temporal lobeepilepsy of adolescence. J. Am. Acad. Child Adolesc. Psychiatry1991; 30: 887–92.

26 Loiseau P, Pestre M, Dartigues JF, Commenges D, Gateau-Barberger C, Cohadon S. Long-term prognosis in two forms ofchildhood epilepsy: Typical absence seizures and epilepsy withrolandic (centrotemporal) EEG foci. Ann. Neurol. 1983; 13:642–8.

27 Paradiso S, Hermann BP, Robinson RG. The heterogeneity oftemporal lobe epilepsy: Neurology, neuropsychology and psychi-atry. J. Nervous Mental Dis. 1995; 183: 538–47.

28 Hermann BP, Black RB, Chhabria S. Behavioral problems andsocial competence in children with epilepsy. Epilepsia 1981; 22:703–10.

29 Hermann BP, Riel P. Interictal personality and behavioral traits intemporal lobe and generalised epilepsy. Cortex 1981; 17: 125–8.

30 Stores G. School children with epilepsy at risk for learning andbehavior problems. Dev. Med. Child Neurol. 1978; 20: 502–8.

31 Whitman S, Hermann BP, Black RB, Chhabria S. Psycho-pathology and seizure type in children with epilepsy. Psychol.Med. 1982; 12: 843–53.

32 Perini GI, Tosin C, Carraro C et al. Interictal mood and personal-ity disorders in temporal lobe epilepsy and juvenile myoclonicepilepsy. J. Neurol. Neurosurg. Psychiatry 1996; 61: 601–5.

33 Devinsky O, Najjar S. Evidence against the existence of a tem-poral lobe epilepsy personality syndrome. Neurology 1999; 53(Suppl. 2): S13–S25.

34 Blumer D. Evidence supporting the temporal lobe epilepsy person-ality syndrome. Neurology 1999; 53 (Suppl. 2): S9–S12.

696 M Sabaz et al.

35 Sabaz M, Cairns DR, Lawson JA, Nheu N, Bleasel AF, Bye AME.Validation of a new quality of life measure for children withepilepsy. Epilepsia 2000; 41: 765–74.

36 Achenbach TM. Manual for the Child Behavior Checklist/4–18and 1991 Profile. Burlington, VT, USA: University of VermontDepartment of Psychiatry, 1991.

37 Landgraf JM, Abetz L, Ware JE. Child Health Questionnaire(CHQ): a user’s manual, 1st edn. Boston, MA, USA: The HealthInstitute, New England Medical Center, 1996.

38 Theunissen NC, Vogels TG, Koopman HM et al. The proxyproblem: Child report versus parent report in health-related qualityof life research. Qual. Life Res. 1998; 7: 387–97.

39 Gilliam F, Wyllie E, Kashden J et al. Epilepsy surgery outcome:Comprehensive assessment in children. Neurology 1997; 48:1368–74.

40 Bryman A, Cramer D. Quantitative Data Analysis with SPSSRelease 10 for Windows. London, UK: Routledge, 2001.

41 Wiebe S, Rose K, Derry P et al. Outcome assessment in epilepsy:comparative responsiveness of quality of life and psychosocialinstruments. Epilepsia 1997; 38: 430–8.