ARTICLE IN PRESS

Neuroscience and Biobehavioral Reviews 31 (2007) 11011113

www.elsevier.com/locate/neubiorev

Review

The effect of damage to the cerebellum on sensorimotor and cognitive

function in children and adolescents
Jurgen Konczaka,b,, Dagmar Timmannc
a
Human Sensorimotor Control Laboratory, University of Minnesota, USA
b
Department of Neurology, University of Minnesota, USA
c
Department of Neurology, University of Duisburg-Essen, Germany

Abstract

This review provides a developmental perspective on our current understanding of the role of the cerebellum for sensorimotor and
cognitive function. A synopsis on the contribution of the cerebellum on motor control, learning and cognition based on experiments in
human adults and animals is presented. This knowledge is contrasted to the relevant literature on children and adolescents. Special
attention is given to ndings derived from lesion studies and clinical reports that examined the effect of cerebellar damage during
development. In general, it is established that children may show the same sensorimotor decits as adults as a result of cerebellar damage,
while the ndings of cognitive dysfunction in children are less clear and remain controversial. Younger children do not necessarily
recover better than older children or adolescents. The sparing of the deep cerebellar nuclei and the extent of adjuvant chemo- or radiation
therapy are better predictors of later motor and cognitive function in children and adolescents.
r 2007 Elsevier Ltd. All rights reserved.

Keywords: Cerebellum; Childhood; Cognition; Development; Motor control; Motor learning; Therapy

Contents

1. The compartmentalization of motor control functions in the cerebellum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1102

2. The role of the cerebellum for motor learning . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1102
3. Effect of cerebellar lesions on sensorimotor function during childhood and adolescence. . . . . . . . . . . . . . . . . . . . . . . . . 1103
3.1. Speech motor control . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1103
3.2. Balance control. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1104
3.3. Arm motor control . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1105
4. Cognitive function in children and adolescents with cerebellar lesions. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1106
4.1. Language comprehension and speech production . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1108
4.2. Visuospatial and executive function . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1109
4.3. Effect of age at the time of insult on cognitive function . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1109
4.4. Posterior fossa syndrome and mutism . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1109
5. Concluding remarks. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1110
Acknowledgements . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1110
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1111

Corresponding author. Human Sensorimotor Control Laboratory, University of Minnesota, USA. Tel.: +1 612 624 4370; fax: +1 612 624 1314.
E-mail address: jkonczak@umn.edu (J. Konczak).

0149-7634/$ - see front matter r 2007 Elsevier Ltd. All rights reserved.
doi:10.1016/j.neubiorev.2007.04.014
 ARTICLE IN PRESS
1102 J. Konczak, D. Timmann / Neuroscience and Biobehavioral Reviews 31 (2007) 11011113
1. The compartmentalization of motor control functions in
the cerebellum
Lesions to the cerebellum lead to an array of motor
symptoms that collectively can be described as ataxia or the
loss of coordination. The lesions can affect several motor
systems, such as the speech motor system, the oculomotor
system, the postural or the limb motor system. To
understand the role of the cerebellum in motor control, it
is helpful to consider neuroanatomical evidence showing
that distinct regions of the cerebellum are concerned with
specic motor functions, giving rise to concept of
compartments of motor functions within the cerebellum.
From this research we know that the cerebellum of adult
humans and non-human primates follows a mediolateral
organization, effectively dividing the cerebellar cortex and
the underlying deep nuclei into three functional zones
(Jansen and Brodal, 1940). The vermis and nuclei fastigii
form the most medial zone, which is mainly concerned with
the control of posture and locomotion (see Fig. 1). Damage
of the medial zone in humans (Dichgans and Mauritz,
1983; Bastian et al., 1998) and the inactivation of the
fastigial nuclei in monkeys (Thach et al., 1992) will lead to
problems in sitting, standing and walking. The intermediate
zone consists of the interposed nuclei (in humans:
n. emboliformis and n. globosus), adjacent regions of the
dentate nucleus and those portions of the paravermal
cortex that project to these nuclei. Newer neuroanatomical
evidence revealed that adjacent dorsomedial regions of the
dentate nucleus (dND) exhibit similar properties and may
also be part of the intermediate zone. The importance of
both cerebellar outow nuclei for upper limb motor control
is highlighted by the fact that a temporary inactivation of
the nucleus interpositus and the adjacent portion of
the nucleus dentatus in monkeys causes impairments in
reaching and grasping gestures (Growdon et al., 1967;
Mackel, 1987; Mason et al., 1998). The lateral cerebellar
hemisphere and most of the nucleus dentatus form the
lateral cerebellar zone. The ventrolateral part of this zone
Fig. 1. Schematic view of the cerebellum indicating the sagittal zones and the associated motor decits following lesions in these areas. The deep output
cerebellar nuclei are also shown to illustrate their relationship to each respective zone. The results of cerebellar processing leave the cerebellum solely via
the efferent projections from these deep nuclei. SCA: superior cerebellar artery; PICA: posterior inferior cerebellar artery.
seems to participate in higher motor and maybe cognitive
functions such as motor planning or language production
(Leiner et al., 1993; Middleton and Strick, 1998; Dum
et al., 2002). The dorsal portion of the nucleus dentatus
projects to primary and premotor areas of the neocortex
and is thought to mainly modulate output of the motor
cortex (Dum et al., 2002). With respect to cerebellar injury
of the cerebellar output nuclei, animal studies have shown
that a recovery after lesions to the deep nuclei of the
cerebellum is often incomplete (Botterell and Fulton,
1938a,b), even if they were obtained shortly after birth
(Eckmiller and Westheimer, 1983).
In summary, the ndings of neuroanatomical research
combined with the results of lesion studies suggest that the
lateral cerebellar hemispheres have a more prominent role
in higher motor functions and possibly cognitive proces-
sing, whereas the medial parts of the cerebellum (vermis
and paravermis) are more concerned with basic motor
functions such as balance and limb coordination.
2. The role of the cerebellum for motor learning
There is a longstanding debate among neuroscientists
about the specic role of the cerebellum for the motor
control and learning. One position is that the cerebellum is
genuinely important for motor learning, while the opposing
view regards the observed decits in motor learning as a
consequence of a degraded motor performance that
interferes with motor learning. However, there is increasing
evidence that adaptive learning and reex conditioning,
which are two forms of motor learning, are clearly affected
by cerebellar damage.
For example, many authors investigating the involve-
ment of the cerebellum in eyeblink conditioning assume
that the cerebellum is a place where learning-related plastic
changes take place (for recent reviews, Bracha, 2004;
De Zeeuw and Yeo, 2005; Thompson, 2005; Gerwig et al.,
2007). However, contrasting reports show that the failure
in acquiring conditioned responses in animals with
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J. Konczak, D. Timmann / Neuroscience and Biobehavioral Reviews 31 (2007) 11011113
cerebellar lesions was due to disturbed performance of
both learned and unlearned eyeblink responses reecting
an impaired motor performance rather than impaired
motor learning (Welsh and Harvey, 1989).
With respect to adaptive learning, a somewhat similar
controversy has evolved. For example, if one considers the
literature on force control, it is generally accepted that
force production and the scaling of force are impaired in
cerebellar ataxia patients (e.g., Muller and Dichgans,
1994). Yet, we also know that long-term force adaptation
is accompanied by increased activity in the cerebellum
(Nezafat et al., 2001) indicating that the cerebellum is
especially active during force adaptation learning. In
addition, recent reports from our group demonstrate that
a degeneration of the cerebellum leads to a progressive loss
in force adaptation learning when unknown forces have to
be compensated during multi-joint movements (Maschke
et al., 2004). However, force adaptation learning is less
affected when the unknown force is only exerted in a single-
joint movement (Richter et al., 2004a), which seems to
indicate that motor learning is compromised due to a lack
of motor control.
On the same note, a recent functional imaging study
testing motor skill learning in humans revealed cerebellar
activations, which were correlated more to the motor
control during the learning task rather than to learning
process itself (Seidler et al., 2002). Children may not be
different from adults in this respect. A recent study (Dennis
et al., 2006) investigated motor learning and arm motor
control in a ballistic elbow movement task in children with
spina bida meningomyelocele. Relative to controls, the
children with spina bida had reduced volumes of their
cerebellar hemispheres, yet their motor skill learning was
preserved, indicating that as long as motor control is not
impaired a cerebellar decit may not necessarily lead to
impaired motor learning.
In summary, the picture emerging from this research is
that the cerebellum has a distinct function in implicit motor
learning, although its contributions may well vary between
the various forms of motor learning that range from reex
conditioning to skill acquisition. In addition, it continues
to be difcult to isolate motor learning functions of the
cerebellum from its motor control functions, since success-
ful learning obviously depends on intact control.
3. Effect of cerebellar lesions on sensorimotor function
during childhood and adolescence
It has been a longstanding clinical notion claiming that
lesions of the cerebellum occurring at a young age have
lesser consequences for later functioning than similar
lesions at later stages of life (O Donoghue et al., 1986).
However, until recently systematic clinical studies on
humans were lacking. In addition, the extrapolation from
animal data to humans remains problematic, given that the
size of the cerebellar output nuclei and their projections are
not identical in humans and apes (Matano, 1986; Matano,
1103
2001) and the behavioural expressions of neural systems in
quadruped animals and biped humans are quite different
(Gramsbergen, 1993). With respect to motor function this
review will therefore focus primarily on human data and
will outline how the speech, balance and arm motor control
are affected in children and adolescents with lesions to the
cerebellum.
Sparse data are available on how focal cerebellar lesions
are compensated in infancy. In principle, a model of focal
cerebellar lesions in infancy would be needed to assess the
long-term effect of early damage to the cerebellum. The
recovery after surgical removal of benign tumours (i.e.,
astrocytoma) could serve as such a model. However,
astrocytoma is rare before the age of three years. In
addition, infant motor behaviour, apart from reexes, is
difcult to measure. Finally, infancy is a rather sensitive
period of neural development during which major matura-
tional processes occur. This implies that prenatal and
postnatal development constitutes an extended period of
vulnerability for the cerebellum when insults are capable of
signicantly altering its maturational progress. It is beyond
the scope of this review to outline, in detail, how perinatal
or neonatal insults to the cerebellum affect sensorimotor
function. However, it should be noted that neonatal
malformations of the posterior fossa involving the cere-
bellum, such as Joubert syndrome or DandyWalker
malformation are associated with an array of long-term
motor impairments of multiple motor systems. For
example, symptoms of Joubert syndrome include abnor-
mally rapid breathing (hyperpnea), jerky eye movements,
abnormal muscle tone (hypotonia), and loss of muscle and
limb coordination (ataxia). The prognosis for infants with
Joubert syndrome depends on whether or not the cerebellar
vermis is entirely absent or partially developed. Some
children with a mild form of the disorder show only
minimal motor disability and good mental development,
while others may have severe motor disability and
moderate mental retardation (for recent reviews, see ten
Donkelaar et al., 2003; Limperopoulos and du Plessis,
2006).
3.1. Speech motor control
Cerebellar disorders give rise to speech motor decits
that are collectively termed ataxic dysarthria (Duffy, 2005).
Cases of acquired cerebellar dysarthria in children are rare.
Unless children develop the syndrome of cerebellar
mutism with subsequent dysarthria, speech motor decits
are not common and appear to be mild (Murdoch and
Hudson-Tennent, 1994; Richter et al., 2005a).
Overall characteristics of ataxic dysarthria in children
seem consistent with ndings in adults. Perceived speech
alterations in adults include imprecise consonant and vowel
sounds, irregular articulatory breakdown, excess and
equal stress, i.e., scanning speech rhythm, reduced
speaking rate as well as harsh voice quality (Darley et al.,
1975). Fluctuations of speaking rate, loudness and pitch as
 ARTICLE IN PRESS
1104 J. Konczak, D. Timmann / Neuroscience and Biobehavioral Reviews 31 (2007) 11011113
well as transient changes in voice quality have also been
observed (Kluin et al., 1988). Slow speaking rate is a
prominent symptom in children (Huber et al., 2006;
Richter et al., 2005a; van Mourik et al., 1998). Imprecise
consonants, distorted vowels, excess stress, hoarseness and
decreased pitch variation have also been described (Corn-
well et al., 2003, 2004, 2005; Murdoch and Hudson-
Tennent, 1994; van Mourik et al., 1998). Scanning speech
and irregular articulatory breakdown appear to be less
prominent in children compared to adults (van Mourik
et al., 1998). Recent studies revealed reduced maximum
syllable repetition rate and a slowed speaking rate of
sentence utterances in children following cerebellar surgery
(Ozimek et al., 2004; Richter et al., 2005a).
With respect to specic lesion sites associated with
dysarthria, it is known that adults with lesions of the
paramedian regions of the superior cerebellar hemispheres
may develop cerebellar dysarthria (Ackermann et al., 1992;
Lechtenberg and Gilman, 1978; Urban et al., 2003).
Regarding a possible lateralization the reports are less
clear. While early ndings in adults suggest that left
hemispherical cerebellar lesions appear to be more
frequently associated with cerebellar dysarthria than right
hemispherical lesions (Lechtenberg and Gilman, 1978),
later studies found no clear lateralization (Ackermann
et al., 1992) or emphasized the importance of lesions of the
right cerebellar hemisphere in the development of dysar-
thria (Urban et al., 2003). Detailed perceptual and acoustic
speech analyses in children with surgical cerebellar lesions
corroborate these adult data on the topographic distribu-
tion of ataxic dysarthria (Richter et al., 2005a). In this
study, the speech impairments among the examined
children appeared to be rare, because tumours most
commonly affected posteriorinferior and medial parts of
the cerebellum while superior paravermal cerebellar regions
were commonly spared. The results suggest a similar
localization of speech function in the cerebellum in children
and adults.
3.2. Balance control
It is well known that after lesions to the medial portions
of the cerebellum in adult patients exhibit problems with
static and dynamic balance during locomotion. Typical
features of gait ataxia are a reduced step frequency with a
prolonged stance and double limb support time while lower
extremity joints show an almost normal range of motion.
The tandem gait paradigm accentuates all the features of
gait ataxia and is the most sensitive clinical test (Stolze
et al., 2002). With respect to standing, research indicated
that static balance is normal in adult patients with lesions
of the cerebellar hemispheres. However, patients with
vestibulo-cerebellar lesions may exhibit omnidirectional
low frequency sway that is poorly stabilized by vision
(Diener et al., 1984).
Children with developmental coordination disorder
(DCD) or autism have been found to show signs of
postural or gait ataxia, which seems to indicate that both
disorders may be associated with a cerebellar dysfunction.
For example, OHare and Khalid (2002) tested 23 children
with DCD in a standardized motor test. They found that
motor performance and skill level were heterogeneous and
many children showed features of poor cerebellar function,
reected in problems with posture, balance and accurate
control of fast movements. Related studies by Geuze (2003,
2005) come to the conclusion that under normal conditions
static balance control is not a problem for children with
DCD, although they may show higher levels of co-
contraction in postural leg muscles such as the tibialis
anterior and peroneus muscles. Only in difcult, unat-
tended, or novel situations DCD children seemed to exhibit
increased postural sway. Those children with DCD who
had been clinically identied as having balance problems
revealed a weaker coupling between EMG and corrective
force compared with control children (Geuze, 2005), which
was likely due to a more variable timing of their EMG
responses. Such loss of the timing of muscular synergies is
known to be a main feature of cerebellar ataxia (e.g., Wild
et al., 1993). However, one needs to be cognizant to the
notion that although children with DCD may show signs of
cerebellar dysfunction, the etiology of DCD is still not fully
understood and DCD has not been classied as a cerebellar
disorder per se.
Until recently, little was known about how focal lesions
in the cerebellum specically affect postural control in
children and how well these children recovered balance
function. A recent study from our group (Konczak et al.,
2005) examined in 22 children and adolescents after
resection of a cerebellar tumour (age-at-surgery: 117 yrs.,
minimum 3 years past surgery). During clinical examina-
tion the patients showed only mild postural problems.
When vision was present measured postural sway was
essentially normal in all patients. However, when vision
was occluded and ankle proprioceptive signals were not
indicative of body orientation (sway-referenced), postural
sway was enhanced in 64% of the patients with some
patients losing balance completely (see Fig. 2). A brain
imaging analysis revealed that 8/8 patients who had
exhibited severe postural dyscontrol and who had not
received chemo or radiation therapy (CRT), the lesion site
included the nuclei fastigii (NF) (see Fig. 3). This nding is
consistent with results from experiments with monkeys
showing that NF inactivation will lead to a loss of
equilibrium control during sitting, standing and walking
(Thach et al., 1992). In monkeys such loss in postural
control becomes understandable, knowing that neurons in
the rostral part of the fastigial nucleus respond to
vestibular stimulation (Thach, 1970; Siebold et al., 1999)
and are not modulated by individual eye movements
(Buttner et al., 1991). Assuming that fastigial neurons play
a similar role in human posture, it then becomes plausible,
why patients with lesioned fastigial nuclei lose postural
control when they have to rely exclusively on vestibular
signals to maintain equilibrium.
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J. Konczak, D. Timmann / Neuroscience and Biobehavioral Reviews 31 (2007) 11011113 1105

Fig. 2. Postural dyscontrol after lesioning of the nucleus fastigius. A. Subject stood erect on a force platform. The visual surround could move in reference
to the persons body sway. B. The tilt of the platform could also be coupled to the persons sway reducing posture-relevant input from ankle
proprioception (sway referenced). C. Exemplar recording from patients and controls. Shown are the COG sway paths over 20 s. Units are in centimeter.
Test condition was no vision/platform sway referenced. In patients CD and SA the nuclei fastigii were intact, while lesioned in patients SS and JO, who
demonstrated excessive postural sway. Excessive sway was dened as sway outside the range of the control group or falls. Adapted from Konczak et al.
(2005).

In a follow up study, the inuence of adjuvant CRT on
the balance function of children after removal of a
cerebellar tumour was assessed (Schoch et al., 2006). The
main nding of this analysis was that damage to the
cerebellar nuclei had more impact on neurological impair-
ment than concomitant tumour therapy. Balance abnorm-
alities were most pronounced when a lesion affected the
fastigial nucleus, which underlines the claim that the
sparing of the fastigial nuclei is critical for the recovery
of balance function after the surgical removal of a
cerebellar tumour in children.
3.3. Arm motor control
Upper limb control may become compromised after
cerebellar injury in adults and children. Typical signs of
upper limb ataxia are dysmetria and an intention tremor at
the end of reaching or pointing movements. The dysmetria
usually becomes more pronounced when movement speed
increases. The underlying reason for this speed dependent
loss of limb coordination seems the inability of cerebellar
patients to correctly predict the magnitude of the interac-
tion torques that arise during multi-joint motion (Bastian
et al., 1996; Topka et al., 1998). Since the interaction
torques increase with movement velocity and acceleration,
this predictive ability becomes critical in fast movements,
which could explain why the limb ataxia becomes
exacerbated during high speed multi-joint motion. Because
cerebellar patients are usually cognizant of this decit they
tend to move slower than healthy controls during goal-
directed arm movements, which is an adapted strategy to
avoid dysmetric movements and not a genuine decit due
to cerebellar dysfunction.
A recent study from our group examined the long term
recovery of upper limb function during single- and multi-
joint nger and arm movements in 22 children and
adolescents after cerebellar tumour removal (Konczak
et al., 2005). The participants were at minimum three
years past surgery. During clinical examination 54% of the
patients showed moderate signs of upper limb ataxia with
only two patients exhibiting a marked intention tremor.
We found that single-joint nger movements were intact in
the large majority of the patients (91%). However, twelve
patients exhibited abnormal deceleration times of the hand
during the three-dimensional multi-joint arm pointing task
(see Fig. 4). The fact that the patients showed abnormally
slow decelerations implies that they likely had adapted a
compensation strategy to avoid hypermetric movements.
A corresponding MRI analysis revealed that all 12
patients, who had not received chemo- or radiation therapy
but revealed abnormal deceleration times, had lesions of
the interposed nucleus (NI). In addition, the region of the
highest lesion overlap included dorsomedial portions of
nucleus dentatus in 10/12 patients. This nding is
consistent with neuroanatomical studies on monkeys
showing that the dorsal portion of the nucleus dentatus
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1106 J. Konczak, D. Timmann / Neuroscience and Biobehavioral Reviews 31 (2007) 11011113
Fig. 3. Superimposition of MRI images of child and adolescent patients
who exhibited excessive postural sway. Excessive sway was dened as sway
outside the range of the control group or falls. Shown are transverse,
coronal and sagittal views of the region of overlap. A. Patients who
exhibited abnormal posture (N 14). The region of highest overlap
included 13 patients and was located mainly in the medial zone including
the nuclei fastigii. B. All patients who exhibited normal postural sway
(N 8). The region of highest overlap included only 3 patients. Colours
represent the degree of overlap between patients (purple 1 patient,
red all patients, i.e., 14 patients in A. and 8 patients in B.). From
Konczak et al. (2005).
projects to primary and premotor areas of the neocortex
(Dum and Strick, 2003). It is further known that
substantial output of the intermediate cerebellar zone is
channelled through the nucleus interpositus. Both nuclear
regions (NI and dND) have large projections to the
magnocellular red nucleus (Kennedy et al., 1986) and to
the primary motor cortex via the ventrolateral thalamus
(Middleton and Strick, 1997). That is, the efferent
projections from the intermediate cerebellar zone can
indirectly inuence the control of reaching and grasping.
With respect to the long-term recovery of upper limb
function it seems essential that the cerebellar output via NI
and dND is not impaired. The sparing of these nuclei better
predicted recovery than age-at-surgery or time-since-
surgery.
4. Cognitive function in children and adolescents with
cerebellar lesions
In the last two decades cerebellar involvement in a wide
range of cognitive tasks as well as in affective behaviour is
much discussed (Leiner et al., 1986). The neocerebellum
and cerebral hemispheres appear to have evolved in
parallel in mammals, hominoids and fossil humans
suggesting a functional dependence of the one upon the
other (MacLeod et al., 2003). Neuroanatomical studies in
monkeys have shown that reciprocal bre connections do
not only exist between the neocerebellum and the primary
motor and premotor areas of the cerebral cortex, but also
extend to prefrontal and posterior parietal associative
cortical areas (Dum and Strick, 2003; Middleton and
Strick, 2001; Ramnani et al., 2006). An increasing number
of human lesion and functional brain imaging studies
support the hypothesis that the cerebellum contributes to
non-motor functions (Chen and Desmond, 2005; Ravizza
et al., 2006; Schmahmann, 2004). Specically, it has been
proposed that the lateral cerebellar hemispheres participate
in cognitive processing. Cerebello-cerebral diaschisis as a
possible pathomechanism has been suggested by others
(Ackermann et al., in press; Marien et al., 2001).
In an inuential cerebellar lesion study with adults
Schmahmann and Sherman (1998) introduced the cere-
bellar cognitive affective syndrome, a constellation of
ndings that includes disturbances of executive functions,
impaired spatial cognition, linguistic difculties, and
personality changes. The lateral cerebellar hemispheres
have been related to executive function including verbal
working memory, and neocerebellar areas of both the
vermis and hemispheres have been related to attention
(Courchesne et al., 1994). Because of the known connec-
tions of the cerebellar hemispheres with the contralateral
cerebrum, it has been suggested that the right cerebellar
hemisphere is associated with language and the left
cerebellar hemisphere with visuospatial functions (Middleton
and Strick, 2001; Schmahmann, 2004), while the vermis
may be part of a limbic cerebellum that is involved in
modulating affective behaviour (see Fig. 5).
There is a longstanding interest in cognitive function in
children with focal surgical cerebellar lesions, which are the
most frequent cause of brain lesions in children (Ries et al.,
2004). Because of the known side effects of the adjuvant
chemo- and radiation therapies, there was an early interest
in cognitive function in children with malign tumours
(Copeland et al., 1999; Duffner et al., 1983; Packer et al.,
1987). Within the scope of the ongoing discussion of
possible involvement of the cerebellum in non-motor
function cognitive ndings in children and adolescents
with benign tumours became of increasing interest. Various
abnormalities have been reported in standard neuropsy-
chological tests (Aarsen et al., 2004; Beebe et al., 2005;
Scott et al., 2001; Levisohn et al., 2000; Riva and Giorgi,
2000; Ronning et al., 2005; Steinlin et al., 2003). Likewise
cognitive and behavioural changes in neuropsychiatric
 ARTICLE IN PRESS
J. Konczak, D. Timmann / Neuroscience and Biobehavioral Reviews 31 (2007) 11011113 1107

Fig. 4. Hand deceleration time during a pointing task. A. Subjects pointed to a target at shoulder height. The two graphs show the three-dimensional hand
velocity proles of a healthy control and of an ataxic adolescent during this task. Note the reduced peak velocity and longer deceleration time (DT) of the
patient. B. Individual mean deceleration time as a function of MRI identied nuclear lesions. The horizontal dotted lines serve to indicate the upper limit
of the control group. In the control group deceleration times of the dominant right arm were overall lower. Two 11-year-old boys exhibited the highest left
arm deceleration times. Such age effect is missing in the patient groups, because most young patients fell in the lesioned group. N.I.: nucleus interpositus,
N.D.: nucleus dentatus. Adapted from Konczak et al. (2005).

of cerebellar disease are primarily on the motor side.

Fig. 5. Proposed compartmentalization of cognitive function within the
neocerebellum.
disorders, such as autism, dyslexia and schizophrenia, have
been linked to structural cerebellar abnormalities (Andrea-
sen et al., 1998; Courchesne et al., 1994; Nicolson and
Fawcett, 2005).
Although there is increasing evidence that the cerebellum
has a role in non-motor functioning, it is still an open
question which cognitive functions are involved, why and
to what extent (Daum and Ackermann, 1997; Frank et al.,
in press; Glickstein, 1993, 2006). Despite the fact that the
cerebellum might be involved in cognition, the symptoms
Unless more extensive damage or degeneration occurs
beyond the cerebellum, cerebellar disorders in both adults
and children are not associated with general intellectual
impairment, such as dementia (Aarsen et al., 2004; Globas
et al., 2003; Malm et al., 1998; Steinlin et al., 2003).
Overall, disorders appear to be mild and far less frequent
than disorders observed following lesions of cerebral areas.
Limitations of lesion studies and negative ndings in
children and adolescents with focal cerebellar lesions are
frequently overlooked. Firstly, the effects of additional
extracerebellar lesions have to be considered. Cerebellar
tumours are rarely restricted to the cerebellum alone. The
brainstem may also be affected either directly by tumour
invasion or indirectly by pressure or obliteration of vessels.
Children and adolescents either with malign or benign
tumours may present with signs of increased intracranial
pressure. Accompanying hydrocephalus frequently occurs.
The long-term effects of increased intracranial pressure and
sudden surgical decompression on cognitive function are
difcult to control. Likewise effects of hospitalization and
depression have to be considered.
Another important problem is the effect of associated
motor impairments. Most neuropsychological tasks have
to be performed with some kind of motion. Even internal
speech or the imagination of movements is affected by
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1108 J. Konczak, D. Timmann / Neuroscience and Biobehavioral Reviews 31 (2007) 11011113

cerebellar ataxia (Ackermann et al., 1998; Kagerer et al.,
1998). However, motor control conditions specic for a
given neuropsychological task are commonly not included
in standard neuropsychological test batteries. For example,
a study of our group failed to replicate Akshoomoff and
Courchesnes (1992) frequently cited initial ndings of
impaired rapid shifts of attention in a larger group of
children with acute focal cerebellar lesions (Schoch et al.,
2004). Introducing specic control conditions, Ivry and co-
workers showed that decits seen in tasks requiring the
shifting of attention are likely due to motor performance
decits in cerebellar patients (Bischoff-Grethe et al., 2002;
Ravizza and Ivry, 2001). Finally, non-motor impairments
may also be a consequence of poor motor abilities in
development in patients suffering from lesions acquired in
childhood (Lehnung et al., 2003; Riviere and Lecuyer,
2002). Likewise, cognitive impairments in patients with
congenital non-progressive cerebellar ataxia (Steinlin et al.,
1999) and cerebellar agenesis (Richter et al., 2005b;
Timmann et al., 2003) may at least in part be related to
motor abnormalities during development. In congenital
cerebellar disorders motor impairment is comparably
weak; however, compensation by extracerebellar areas
may impede cognitive development.
Aside from cognitive and behavioural decits, motor
abnormalities (clumsiness) are frequently observed in
neuropsychiatric disorders including autism, dyslexia and
schizophrenia (Gowen and Miall, in press). Although the
underlying neural bases of clumsiness are likely hetero-
geneous, cerebellar dysfunction appears to contribute.
Therefore structural abnormalities of the cerebellum
observed in these disorders may be related to motor but
not necessarily to cognitive and behavioural dysfunction
(Ivry, 2003).
4.1. Language comprehension and speech production
In cerebellar diseases impairments in several areas of
language are postulated, particularly in semantics and
syntax (Justus, 2004; Marien et al., 2001). In an inuential
single-case study Fiez et al. (1992) reported disorders in a
verb generation task in an adult patient with a right-sided
infarction of the posteriorinferior cerebellar artery. Our
group investigated verb generation in adults with degen-
erative (Richter et al., 2004b) and ischemic cerebellar
lesions (Richter et al., 2007) as well as in children with
acute and chronic surgical lesions (Frank et al., 2007;
Richter et al., 2005c). Overall ndings were negative, that is
both the quality of answers and the reduction of response
times across blocks were not signicantly reduced in any of
the patient groups (see Fig. 6). In particular, in patients
with lesions affecting the right cerebellar hemisphere
ndings were not different compared to patients with
lesions affecting the left cerebellar hemisphere.
If the right cerebellar hemisphere contributes to language
function of the left cerebral hemisphere (Marien et al.,
2001; Schmahmann, 2004), one may expect signs of
aphasia in children and adolescents with cerebellar
disorders. Findings in the literature, however, are contra-
dictory. In a study of our group no signs of aphasia were
observed in children and adolescents with chronic cerebel-
lar lesions (Richter et al., 2005c), while others reported
expressive language problems, including word nding
difculties and non-uent speech (Aarsen et al., 2004;

Fig. 6. Verb generation task: children and adolescents with acute and chronic focal lesions of the cerebellum showed no signicant decits (Richter et al.,
2005c; Frank et al., 2007). A. Superimposition of lesions in children with acute and chronic left- and right-sided affection of the cerebellar hemisphere on
horizontal sections (8 mm apart) of the cerebellum of a healthy adult subject. The number of overlapping lesions is illustrated by colour, from violet
(n 1) to red (n 46). B. Means and standard deviations [s] of the reaction times across the six subsequent blocks for subjects with acute right- (open
black triangle) and left-sided (open grey triangle) lesions, and for subjects with chronic right- (lled black triangle) and left-sided (lled grey triangle)
lesions compared to control subjects (squares). The rst and the last block are naming blocks (Nam1, Nam2), the second to fth block are verb generation
blocks (Vg1Vg4). From Frank et al. (in press).
 ARTICLE IN PRESS
J. Konczak, D. Timmann / Neuroscience and Biobehavioral Reviews 31 (2007) 11011113
Levisohn et al., 2000). The study by Riva and Giorgi (2000)
examined expressive language function in children with
acute lesions and found mild abnormalities in patients with
lesions of the right cerebellar hemisphere.
Likewise, ndings in adults are partly contradictory.
Whereas no signs of aphasia are reported in two studies in
adults with cerebellar infarcts (Gomez-Beldarrain et al.,
1997; Richter et al., 2007), agrammatism as a sign of
expressive aphasia has been described by others in
particular following right-hemispherical ischemic lesions
(Kalashnikova et al., 2005; Marien et al., 2001;
Schmahmann and Sherman, 1998; Silveri et al., 1994;
Zettin et al., 1997). Specic problems in grammar function
and temporal aspects of both speech production and
perception have been shown by others (Ackermann et al.,
2004; Gasparini et al., 1999; Justus, 2004). Most studies
investigating linguistic functions in adults emphasize that
only minor decits in grammar are present after cerebellar
damage (Justus, 2004; Marien et al., 2001).
4.2. Visuospatial and executive function
To examine visuospatial function in children with
cerebellar disorders, numerous tests have been used such
as the Wechsler intelligence scale (block design, object
assembly), copying and recall of the ReyOsterrieth
complex gure or mental rotations of objects. Visuospatial
decits in various combinations and to different degrees
have been reported (Aarsen et al., 2004; Levisohn et al.,
2000; Steinlin et al., 2003). In a study by our group,
however, no signicant abnormalities in neglect and
extinction tasks were observed in children with acute and
chronic cerebellar lesions (Richter et al., 2005c).
Various abnormalities have been reported in different
tests of executive function (for example, verbal uency,
reverse digit span, Wisconsin card sorting test, Stroop test)
in children and adolescents with surgical lesions (Aarsen et
al., 2004; Karatekin et al., 2000; Levisohn et al., 2000; Riva
and Giorgi, 2000; Steinlin et al., 2003). Both fMRI and
cerebellar lesion studies in adults suggest a specic
involvement of the cerebellum in verbal working memory
tasks (Chen and Desmond, 2005; Ravizza et al., 2006). In
standard tests, however, digit spans are relatively pre-
served, especially in comparison to the dramatic reductions
typically observed in classic short-term memory patients
with frontal lobe damage (Ravizza et al., 2006; Riva and
Giorgi, 2000; Steinlin et al., 2003). Studies by our group
both in children and adults with pure cerebellar lesions did
not observe signicant differences compared to controls
(Drepper et al., 1999; Konczak et al., 2005). Patients
appear to be more impaired in working memory tasks with
long delays (Ravizza et al., 2006). Several authors proposed
that the cerebellum may be involved in covert articulatory
rehearsal or it contributes to a phonological storage system
during the initial phonological encoding or by strengthen-
ing memory traces (Chen and Desmond, 2005; Ravizza
et al., 2006).
1109
Finally, acute and chronic vermal lesions in children
have been associated with behavioural and affective
changes, characterized by a attening or blunting of affect
and disinhibited, inappropriate behaviour (Aarsen et al.,
2004; Levisohn et al., 2000; Steinlin et al., 2003). They
appear to be similar to those changes, which are
characteristic of prefrontal or limbic lesions. In a study
of our group mild symptoms of both negative (increased
anxiousness, insecurity, aggressive and depressive beha-
viour) and positive character (decreased aggressiveness,
thoughtful behaviour) were observed in chronic cerebellar
children (Richter et al., 2005d).
4.3. Effect of age at the time of insult on cognitive function
Cognitive and behavioural abnormalities both in pre-
and perinatal insults (for example, cerebellar atrophy and
hemorrhage in preterm children; Krageloh-Mann et al.,
1999; Limperopoulos et al., 2005) and congenital disorders
of the cerebellum appear to be more marked compared to
cognitive dysfunction reported in children who have been
treated for cerebellar tumours (Levisohn et al., 2000;
Steinlin et al., 2003; Aarsen et al., 2004). In the latter,
children who were 3 years or older at the time of surgery
have been investigated. One may argue that cerebellar
lesions acquired very early in life are followed by more
severe cognitive decits. Furthermore, long-term cognitive
impairments might result from changes during the further
postnatal development of the cerebellum, leading to
disruption in pathways that fail to develop properly as a
result of the lesion (growing into a decit; Scott et al.,
2001). However, brain abnormalities in premature children
and children with congenital cerebellar abnormalities are
rarely restricted to the cerebellum. Accompanying cerebral
lesions likely play a role.
Steinlin et al. (2003) compared cognitive function in
children with cerebellar tumours who were 3.56.5 years
(preschool age), 79.5 years (early school age) and 1015
years at the time of diagnosis. They found that cognitive
abnormalities were most marked in the second group.
Likewise, Levisohn et al. (2000) found a higher incidence of
cognitive abnormalities in children older than 7 years at the
time of surgery. Findings suggest a vulnerable age around
early school age in children with focal cerebellar lesions.
The lower incidence of impairment in younger children
may reect neural plasticity. However, effects of tumour
type, lesion side, incidence of accompanying hydrocephalus
and surgical complications have also to be considered. For
example, in the Levisohn study the incidence of malign
tumours was higher in the older compared to the younger
group.
4.4. Posterior fossa syndrome and mutism
Studies of cognitive function in children with cerebellar
tumours is of great interest because of the occurrence of
the so-called posterior fossa syndrome, a specic
 ARTICLE IN PRESS
1110 J. Konczak, D. Timmann / Neuroscience and Biobehavioral Reviews 31 (2007) 11011113
complication of tumour surgery in children (Pollack et al.,
1995; Riva and Giorgi, 2000). During the last two decades,
more than 160 cases have been reported (Ozgur et al.,
2006). The posterior fossa syndrome is most likely to
occur after removal of large tumours of the cerebellar
vermis (Catsman-Berrevoets et al., 1999). In the majority
of cases, symptoms develop after an interval of
relatively normal functioning in the immediate postopera-
tive period (most commonly one to two days following
surgery) and the decits are largely reversible within
a few weeks to months. Mutism is the central element
of this syndrome in the majority of patients. Accompany-
ing emotional and behavioural changes ranging from
irritability to behaviours reminiscent of autism are
frequently observed. Cases with behavioural alterations in
the foreground occur (Ozimek et al., 2004; Riva and
Giorgi, 2000). Decreased spontaneous initiation of move-
ments, poor oral intake and impaired eye opening as well
as long tract signs and urinary retention are other
accompanying signs.
The causes and anatomic basis for this syndrome in
children with posterior fossa lesions remain controversial.
The conditions in which it develops suggest an extracer-
ebellar component of cerebellar mutism (van Dongen et al.,
1994). A complex interaction of cerebellar lesion, brain-
stem affection and hydrocephalus appears likely (Gordon,
1996; van Dongen et al., 1994). In addition, the incomplete
development of speech motor control and language in
childhood has to be considered (Murdoch and Hudson-
Tennent, 1994).
On the one hand, mutism is considered an extreme form
of ataxic dysarthria, that is, anarthria (Ackermann and
Ziegler, 1994; van Calenbergh et al., 1995). This hypothesis
is supported by descriptions of a period of dysarthria
following the mute phase (Ozimek et al., 2004; Riva and
Giorgi, 2000). This picture has been designated as the
syndrome of cerebellar mutism and subsequent dysar-
thria (van Dongen et al., 1994). Lesions of the dentate
nuclei may play a role (Ozimek et al., 2004). Pollack et al.
(1995) proposed that mutism is related to transient
impairment of the afferent and/or efferent pathways of
the dentate nuclei that are involved in initiating of complex
volitional movements (Pollack et al., 1995).
On the other hand, mutism is assumed to be a language
disorder (Riva, 1998), since in some children language
impairment similar to agrammatism but not dysarthria has
been reported in the postmutistic phase (Riva and Giorgi,
2000). The same authors proposed that lesions restricted to
the vermis are followed by mutism and subsequent
dysarthria and behavioural alterations, whereas alterations
in linguistic processes including language organization are
due to an additional partial excision of the right cerebellar
hemisphere. However, emotional and behavioural changes
have also been related to accompanying dysfunction of the
brainstem tegmentum (Pollack et al., 1995), which again
speaks for the notion that mutism is not exclusively of
cerebellar nature.
5. Concluding remarks
This review examined our current knowledge on how
damage to the cerebellum affects motor and cognitive
function in children and adolescents. There are the key
points to be made:
First, children and adolescents generally show the
same motor symptoms to cerebellar damage as adults.
The ataxia may present itself by a dysarthric speech,
unstable gait and posture, and dysmetric upper limb
movements.
Second, the children and adolescents may reveal a
nearly complete recovery of motor function after
cerebellar injury as long as the deep cerebellar nuclei
are not lesioned and extracerebellar systems are not
affected by the injury.
Third, age-at-surgery is not good predictor of
recovery. This is to say, that young children will not
necessarily show a more complete recovery than
adolescents.
Fourth, successful motor learning will depend on the
level of motor control. Severe ataxia will impede motor
learning in children and adults.
Fifth, while there is increasing evidence that the
cerebellum has a role in cognition, the observed
symptoms with respect to changes in working memory,
affect, and language production are mostly mild. In
addition, many ndings, which are frequently cited
today to support cerebellar involvement in cognition,
are insufcient to prove the hypothesis. There is ongoing
need of well-controlled studies, which show that
disorders are due to cerebellar lesions independent of
motor dysfunction and other confounding factors.
Sixth, standard neuropsychological tests are of limited
use to explore the contribution of the cerebellum to non-
motor function.
In future studies neuropsychological tests need to be
developed which control for effects of motor dysfunction
specic for a given task. Furthermore, better control
groups are needed that include patients after brain surgery
with increased intracranial pressure, which is common in
children and adolescents with cerebellar tumours. Any
learning or motor rehabilitation study ought to consider
the patients level of motor control when evaluating
learning progress, since motor control necessarily con-
founds any learning effect.
Acknowledgements
This work was supported by a grant of the Deutsche
Forschungsgemeinschaft (DFG Ti 239/5-2). The idea for
the review originated from a presentation given at the
international workshop on Lesions of the Developing
Nervous System in Groningen, Holland, in May 2006.
 ARTICLE IN PRESS
J. Konczak, D. Timmann / Neuroscience and Biobehavioral Reviews 31 (2007) 11011113
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