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European clinical guidelines for hyperkinetic disorder-first upgrade
Taylor, E.; Döpfner, M.; Sergeant, J.A.; Asherson, P.; Banaschewski, T.; Buitelaar, J.;
Coghill, D.; Danckaerts, M.; Rothenberger, A.; Sonuga-Barke, E.; Steinhausen, H.C.;
Zuddas, A.
published in
European Child and Adolescent Psychiatry
2004
DOI (link to publisher)
10.1007/s00787-004-1002-x
document version
Publisher's PDF, also known as Version of record
Link to publication in VU Research Portal
citation for published version (APA)
Taylor, E., Döpfner, M., Sergeant, J. A., Asherson, P., Banaschewski, T., Buitelaar, J., Coghill, D., Danckaerts,
M., Rothenberger, A., Sonuga-Barke, E., Steinhausen, H. C., & Zuddas, A. (2004). European clinical guidelines
for hyperkinetic disorder-first upgrade. European Child and Adolescent Psychiatry, 13(S1), 7-30.
https://doi.org/10.1007/s00787-004-1002-x
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Download date: 09. Aug. 2022
Eur Child Adolesc Psychiatry [Suppl 1]
13:I/7–I/30 (2004) DOI 10.1007/s00787-004-1002-x
ORIGINAL CONTRIBUTION
ECAP 1002
E. Taylor ()
Dept. of Child & Adolescent Psychiatry
Institute of Psychiatry
Kings College London
de Crespigny Park
London, SE5 8AF, UK
E-Mail: e.taylor@iop.kcl.ac.uk
M. Döpfner
Psychiatry and Psychotherapy of Childhood
& Adolescence
University of Cologne
Koeln, Germany
J. Sergeant
Dept. of Clinical Neuropsychology
Faculty of Psychology & Education
Vrije Universitat
Amsterdam,The Netherlands
P. A s he r s o n
MRC Social Genetic & Developmental
Research Centre
Institute of Psychiatry
Kings College London
de Crespigny Park
London, UK
T. Banaschewski · A Rothenberger
Dept. of Child & Adolescent Psychiatry
University of Goettingen
Goettingen, Germany
J. Buitelaar
Dept. of Psychiatry
UMC St Radboud
Nijmegen,The Netherlands
D. Coghill
Dept. of Psychiatry
University of Dundee
Dundee, Scotland
M. Danckaerts
Dept. Child & Adolescent Psychiatry
U. Z. Gasthuisberg
Leuven, Belgium
E. Sonuga-Barke
Dept. of Psychology
University of Southampton
Southampton, UK
H.-C. Steinhausen
Dept. of Child & Adolescent Psychiatry
University of Zurich
Zurich, Switzerland
A. Zuddas
Child Neuropsychiatry
Dept. Neuroscience University of Cagliari
Cagliari, Italy
■ Abstract Background The valid-
ity of clinical guidelines changes
over time, because new evidence-
based knowledge and experience
develop. Objective Hence, the Euro-
pean clinical guidelines on hyper-
kinetic disorder from 1998 had to
be evaluated and modified. Method
Discussions at the European Net-
work for Hyperkinetic Disorders
(EUNETHYDIS) and iterative cri-
tique of each clinical analysis.
Guided by evidence-based infor-
mation and based on evaluation
(rather than metaanalysis) of the
scientific evidence a group of child
psychiatrists and psychologists
from several European countries
updated the guidelines of 1998.
When reliable information is lack-
ing the group gives a clinical con-
sensus when it could be found
among themselves. Results The
group presents here a set of recom-
mendations for the conceptualisa-
tion and management of hyperki-
netic disorder and attention
deficit/hyperactivity disorder
(ADHD). Conclusion A general
scheme for practice in Europe
could be provided, on behalf of the
European Society for Child and
Adolescent Psychiatry (ESCAP).
■ Key words ADHD – HKS –
guidelines – European – children
Eric Taylor
Manfred Döpfner
Joseph Sergeant
Philip Asherson
Tobias Banaschewski
Jan Buitelaar
David Coghill
Marina Danckaerts
Aribert Rothenberger
Edmund Sonuga-Barke
Hans-Christoph Steinhausen
Alessandro Zuddas
European clinical guidelines for
hyperkinetic disorder – first upgrade
Introduction
Hyperkinetic disorder is a persistent and severe impair-
ment of psychological development resulting from a
high level of inattentive, restless and impulsive behav-
iour. Its onset is in early childhood: by definition before
the age of 7, nearly always before the age of 5 and fre-
quently before the age of 2 years. It often persists into
adolescence and adult life, and puts sufferers at risk for
a range of abnormalities in personality development.
The adverse outcomes include delinquency and other
antisocial behaviour and underachievement in school.
Longitudinal studies indicate that the inattentive and
restless behaviour is a developmental risk.It is not just a
marker to some more fundamental kind of disadvan-
I/8 European Child & Adolescent Psychiatry, Vol. 13, Supplement 1 (2004)
© Steinkopff Verlag 2004
tage.The treatment of severely hyperactive behaviour is
therefore a major target for child mental health services.
The importance of treating hyperactivity is estab-
lished.It has been hard to establish European guidelines,
because a variety of different clinical traditions have
evolved within Europe [143], and there has been wide-
spread public controversy about the disorder and possi-
ble over-prescription in the United States.In 1998 an ap-
proach to European guidelines was produced by a group
of experts, including some of the present authors [169].
Since then, however, a good deal has changed. Profes-
sional consensus has emerged in most countries, re-
flected in published national protocols [25, 33, 70]. The
use of treatments for hyperactivity and attention deficits
has increased markedly, especially for stimulant med-
ication [22, 139]. Scientific knowledge has increased
rapidly [134]. There is therefore a need for revision and
update of these “European guidelines” and we have
aimed at achieving this on behalf of ESCAP (European
Society for Child and Adolescent Psychiatry). The revi-
sion has been greatly helped by discussions at the Euro-
pean Network for Hyperkinetic Disorders (EUNETHY-
DIS). Practice does, of course, vary between countries
and centres; and these guidelines are therefore intended
as a statement of evidence-based or consensus-driven
general principles rather than detailed protocols for
clinical management. The development of specific pro-
tocols will need to take place at the local level, involving
other stakeholders such as users and purchasers of ser-
vice; we hope that these guidelines will form a useful
framework.
Influences on pathogenesis
The exact aetiological pathways of AD/HD are un-
known. AD/HD aggregates within families with a 3–5
times increased risk in first-degree relatives [45]. Twin
studies have found considerable heritability with ge-
netic factors contributing 65% to 90% of the phenotypic
variance in the population [173]. High heritability must
not be confused with genetic determinism and some
caution should be adopted in counselling families about
causes.
Molecular genetic studies have found associations
with variations in genes for the dopamine receptors 4
(DRD4 7-repeat allele) and 5 (DRD5 148bp-allele), and
the dopamine transporter (DAT1 10-repeat allele) [30,
46, 94]. While the 10-repeat allele of DAT1 has been as-
sociated with an increased expression of the transporter
[49, 99], the 7-repeat allele of DRD4 seems to encode a
receptor that is subsensitive to dopamine [5].
Preliminary evidence suggests aetiological influ-
ences of the receptor genes DRD1 [101] and 5-HT(1B)
[66,118],the Taq 1 polymorphism of the dopamine beta
hydroxylase gene [31,148],and the SNAP-25 gene,which
is involved in the regulation of neurotransmitter release
[12, 100].
Each of these risk alleles increases the relative risk for
AD/HD only slightly (odds ratios: 1.2–1.9), consistent
with the hypothesis that,in most cases,AD/HD is a com-
plex disorder influenced by the interaction of multiple
aetiological factors,each of minor effect.However,some
rarer genes,including fragile X and resistance to thyroid
hormone, may show large effects. Some genes involved
may have pleiotropic effects.Thus,it has been suggested
that AD/HD and reading disability [6, 147, 185], and
AD/HD and autism [6, 147] share genetic susceptibility
factors.
There are also associations with a variety of environ-
mental risks, including prenatal and perinatal obstetric
complications, low birth weight, prenatal exposure to
benzodiazepines, alcohol, or nicotine, and brain dis-
eases and injuries [24, 51, 97, 98, 166, 171]. Severe early
deprivation, institutional rearing, idiosyncratic reac-
tions to food,and exposure to toxic levels of lead are also
considered to have aetiological importance [17,127,130,
170]. The quality of relationships within the family and
at school can be considered as maintaining or protective
factors [17, 130]. Gene-environment interactions seem
likely (e. g. DAT-10 allele multiplies the risk of maternal
prenatal smoking [77]), but have not yet been studied
extensively.
Studies using structural and functional brain imag-
ing, electrophysiology and transcranial magnetic stim-
ulation have shown various abnormalities in frontal,
temporal, and parietal cortical regions, basal ganglia
(striatum), callosal areas, and cerebellum [13, 14,23, 28,
29, 47, 102, 111, 126, 157,163, 176, 190]. The morpholog-
ical abnormalities seem to be evident early, non-pro-
gressive, and not a result of stimulant treatment [29].
Converging evidence from a variety of sources sug-
gest that catecholaminergic dysregulations are centrally
involved. The molecular genetic findings above, the ef-
fectiveness of stimulants and noradrenergic substances
[4], some animal models [e.g., 128, 138], and functional
imaging studies – which suggest altered DOPA decar-
boxylase activity in the striatum and prefrontal areas [42,
43], as well as increased striatal dopamine transporter
binding capacity [37,84] – are all in keeping with this.
Neuropsychological and electrophysiological studies
have found various alterations in higher-order cognitive
functions [9,114,142],motivational processes [129],and
more basic information processing stages [13, 83, 135,
149, 174, 188]. The tests used are summarised in the Ap-
pendix.
Some research evidence suggests heterogeneity at
several levels: phenotypic [e.g., 16], neuropsychological
[e.g., 150],psychophysiological [e.g., 7,23,125], and ge-
netic [e. g., 107, 175, 186]. There may be multiple devel-
opmental pathways from aetiological factors to behav-
ioural symptoms [151, 152].
E. Taylor et al. I/9
European clinical guidelines for hyperkinetic disorder – first upgrade
Prevalence
Several studies converge on a point prevalence for hy-
perkinetic disorder of about 1.5% in the primary school
age population – which is the age at which the problem
is most likely to be referred for specialist attention [163].
In the UK,the rates are similar in studies stemming from
the 1970s and 1990s – a period during which the recog-
nition of the disorder changed greatly. The administra-
tive prevalence – the rate at which the disorders are in
practice recognised – varies vary greatly between diffe-
rent European countries, from approximately zero to
nearly 2.5% [81].Attention deficit without hyperactivity
has received less research attention, but is troublesome
for something like another 1% of the school age popu-
lation [166].
The prevalence of the broader category of AD/HD is,
obviously, higher. The estimates vary from about 4% to
19% but the usual figures adopted are those of the DSM
IV estimates of 3–5% [26]. The exact figures found in
studies probably depend more upon the cut-off that is
chosen than on any major differences between popula-
tions [163].Hyperactive behaviour is distributed contin-
uously in the population, and the exact cut-off taken is
somewhat arbitrary.In some populations – for instance,
the Chinese population in Hong Kong – careful diagnos-
tic measures have suggested that there may be a lower
prevalence of disorder than in Europe [88].Interestingly,
however, the apparent prevalence taken from rating
scales was higher in Hong Kong than in Western popula-
tions – perhaps illustrating the extent to which the disor-
der is socially defined and influenced by the concern that
adults feel for these behaviours in different cultural set-
tings.Methodological differences,however,usually make
it hard to compare rates between countries.
Diagnosis
The diagnosis of hyperkinetic disorders follows the
ICD-10 criteria.
The DSM-IV category of AD/HD is more broadly de-
fined and is a commoner diagnosis.In both schemes,the
behaviours to be recognised are very much the same.
The differences come in the ways that the symptoms are
weighted and combined into categories:
The ICD-10 diagnosis of hyperkinetic disorder is the
narrower category,and it appears that nearly all cases of
hyperkinetic disorder should be included within
AD/HD. The additional criteria of ICD-10 are that all
three problems of attention, hyperactivity and impul-
siveness should be present; that more stringent criteria
for pervasiveness across situations are met; and that the
presence of another disorder such as anxiety state is in
itself an exclusion criterion – the expectation is that
most cases will have a single diagnosis.
Both these diagnostic schemes have their advantages
and disadvantages and a narrower or a broader defini-
tion will be suitable for different purposes. Many Euro-
pean clinicians prefer to use the wider definition of
AD/HD.We see no contradictions involved. Indeed, it is
helpful to use both concepts.In the assessment of an in-
dividual child, the first question then becomes whether
the criteria for AD/HD are met, i. e. whether there is any
target problem for investigation and management. If
there is,then one should proceed to a finer grain of clas-
sification. Does the child also meet the criteria for hy-
perkinetic disorder; or,if they fall only into the wider de-
finition, is it possible to assign an alternative type of
psychopathology?
Impairment
Diagnosis requires that there should be clear evidence of
clinically significant impairment in social, academic, or
occupational functioning. This requirement is essential
not only for AD/HD but for all mental disorders, in or-
der to differentiate disorders from ubiquitous symp-
toms and variations of behaviour. Impairment implies
not only a higher severity or frequency of symptoms but
also interference with functioning in the major life do-
mains of the child, e.g. at home, at school, with friends
or elsewhere.
A valid instrument for the assessment of impairment
is the Children’s Global Assessment Scale (CGAS) by
Shaffer etal. [144]. The CGAS runs from 0 to 100 with 0
indicating a child with the most severe disorder and im-
pairment and 100 the most healthy and well functioning
child. Cut-offs have been proposed in order to differen-
tiate normal functioning from severe problems in need
of treatment [144, 161]. The multiaxial classification of
child and adolescent psychiatric disorders by the WHO
has introduced a similar scale to measure the level of
psychosocial functioning [187].Axis six of the multiax-
ial scheme (MAS) runs from 0 indicating superior/good
social functioning to 8 reflecting gross and pervasive so-
cial disability. Both CGAS and Axis six of the MAS are
suitable for the clinical assessment of psychosocial im-
pairment resulting from mental disorders in children
and adolescents.
The CGAS has also been influential for the definition
of AD/HD according to DSM-IV.A cut-off point of 60 on
the CGAS – indicating a level of clinical impairment that
requires treatment – has been used in order to define the
number of symptom criteria for AD/HD. In a field trial
it was found that 5 symptoms of AD/HD had to be pres-
ent in order to arrive at the CGAS cut-off point of 60
[85]. In order to be conservative and avoid false-posi-
tives the numbers were increased by one to 6 (or more)
symptoms of inattention and hyperactivity-impulsivity.
I/10 European Child & Adolescent Psychiatry, Vol. 13, Supplement 1 (2004)
© Steinkopff Verlag 2004
Comorbidity
The co-existence of other types of psychopathology is
very common. The reasons appear to be different in dif-
ferent forms [54].
■ Conduct disorder
Oppositional defiant and conduct disorders are very
common in hyperactivity, and genetic influences over-
lap. They should often be seen, not necessarily as a dif-
ferential diagnosis or a comorbid condition, but as a
complication. Longitudinal studies indicate that in pri-
mary school-aged children hyperactive behaviour is a
risk factor for conduct disorder,that it appears over time
even in children who showed a pure pattern of hyperac-
tivity without conduct disorder at the beginning of their
problems, and that conduct disorder does not give rise
to hyperactivity in the same way [164].
■ Emotional disorders
Much less is known about the reasons for the frequent
coexistence of hyperactivity and problems of anxiety
and depression. Some children may develop low self es-
teem and insecurity as a result of failures at school and
interpersonal relationships
■ Specific learning disorders
Children with hyperkinetic disorders are more likely to
show neurodevelopmental delays of various types. Lan-
guage milestones are achieved later than normal, ex-
pressive language is unduly simple,sensory motor coor-
dination is often impaired, handwriting is poor, and
reading ability is behind that expected for chronological
age [166].
McGee and Share [91] suggest that children with on-
set of hyperactivity after school entry are more likely to
have behaviour problems confined to school, and to
show specific learning difficulties. The suggestion is
therefore that some children may enter school with their
cognitive function compromised by neuropsychological
deviations. The attendant stress in adjusting to class-
room demands leads to disturbances in the control of
activity and attention.For other children,a primary dis-
turbance of attention and impulse may give rise to sec-
ondary academic problems, either through inability to
cope with the work or aversion to it.
■ Pervasive developmental disorders
Children with autism often show hyperactive behaviour,
and autistic symptoms are sometimes seen in the hy-
peractive. Research is now on its way to clarify the rela-
tionship between them. Clinically, children with hyper-
kinetic disorder and an autistic type of social
impairment will sometimes show a partial response to
stimulants (though caution is needed in view of possible
adverse effects). It is therefore desirable to recognise
both disorders when they are present. The ICD-10 (but
not the DSM) scheme includes a diagnosis of “Hyperki-
nesis with stereotypies”. This is in our experience usu-
ally accompanied by social impairment and best seen as
a part of the autism spectrum.
■ Tic disorders
A number of children with AD/HD develop comorbid tic
disorders during their early school years [124]. In these
cases, the degree of psychosocial impairment is usually
determined by AD/HD and it may be the target for ther-
apy.
■ Developmental coordination disorder
AD/HD is often accompanied by problems in sensory
motor coordination, especially seen as poor handwrit-
ing,clumsiness,poor performance in sports and marked
delays in achieving motor milestones [53, 76]. If signifi-
cant interference with academic achievements or activ-
ities of daily living is observed, treatment with stimu-
lants seems to be indicated: it may improve motor
coordination and increase the motivation of the child
for further sensorimotor training.
■ Bipolar disorder
The main DSM-IV inclusion criteria for mania are elated
mood and/or grandiosity. There is still some contro-
versy about the existence and definition of preadoles-
cent mania [65,67,79].More work is needed on the phe-
nomenology and diagnosis of mania in children, on its
natural history and on its familial correlates. Neverthe-
less, some studies which describe high rates of overlap
between AD/HD and mania exist. This might have im-
plications for treatment approaches in such cases, but
consensus is not reached yet.
