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Dysfunction Cerebral Palsy: Implications Therapeutic: Postural in Guidance

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NEURAL PLASTICITY VOLUME 12, NO.

2-3, 2005

Postural Dysfunction in Children with Cerebral Palsy:


Some Implications Therapeutic Guidance
Eva Brogren Carlberg and Mijna Hadders-Algra2

IDepartment of Woman and Child Health, Neuropediatric Research Unit, Astrid Lindgren Children’s
Hospital Stockholm, Sweden," 2University Hospital Groningen, Department ofNeurology,"
Hanzeplein 1, 9713 GZ Groningen, The Netherlands

ABSTRACT on adaptive seating, tilting of the support


surface, and ample, variable training in moti-
Postural problems play a central role in the vating settings. The challenge facing us now is
motor dysfunction of children with cerebral to provide evidence about the efficacy of specific
palsy (CP). Therefore, they spend more time in treatment approaches facilitating that children
sitting than in standing to perform vital tasks of reach an optimal level of functioning in daily life.
daily life. The focus of this article is to describe
the pathophysiology of postural control in
sitting and outline some implications for KEY WORDS
management and treatment. In general, children
with CP exhibit muscular activity counter- training, motor developmem, adaptive seating, EMG
acting forces that disturb equilibrium. Only
’non-sitting’ children with severe CP lack such
’direction-specific’ adjustments, possibly ruling CEREBRAL PALSY
out achievement of independent sitting. Most
frequently, the children display dysfunctions in Cerebral palsy (CP) is the most common
the adaptation of the adjustment. Typical physical disability in childhood, with a prevalence
characteristics of this adaptation in children of 2 to 2.5 per 1000 children in the Western
with CP are a top-down recruitment of pos tural countries. The disorders covered by the term CP
muscles, an excessive degree of antagonistic co- are very heterogeneous, both in clinical symptoms
activation, and an incomplete adaptation of the and in lesions causing these symptoms. Many
EMG-amplitude to task specific constraints. attempts have been made through the years to
Despite our knowledge on the pathophysiology define CP. The most recent consensus definition
underlying the postural problems in children states that CP is "an umbrella term covering a
with CP, little ’high-level’ evidence (according group of non-progressive, but often changing,
to Sackett) exists on how different interventions motor impairment syndromes secondary to lesions
can affect these problems. Therapeutic attention or anomalies of the brain arising in the early
to promote motor performance in sitting focuses stages of its development" (Mutch et al., 1992;
549). This definition addresses primarily the motor
symptoms, whereas other aspects of common co-
Reprint requests to: Neuropediatric Research Unit, Dept of morbidity that significantly influence the children’s
Woman and Child Health, Astrid Lindgren Children’s Hospital day-to-day performance are omitted. Therefore, a
Q2:07, 171 76 Stockholm, e.mail: eva.brogrencarlberg@ chello.se new definition was suggested in July 2004:

(C) 2005 Freund & Pettman, U.K. 221


222 E. BROGREN CARLBERG AND M. HADDERS-ALGRA

Cerebral palsy describes a group of developmental with CP at various functional levels. The authors
disorders of movement and posture, causing activity created ’gross-motor curves’ that provide an
restrictions or disability that are attributed to dis- approximate idea of prognosis. The curves form an
turbances occurring in the fetal or infant brain. important basis for clinical decision-making and
The motor impairment may be accompanied by a for rating change in gross motor function related to
seizure disorder and by impairment of sensation, specific interventions (Ekstr6m Ahl et al., unpub-
cognition, communication, and behavior. This lished). The aim of the present paper is to discuss
definition is currently under debate (www.casting the postural dysfunctions of children with CP and
foundation.net). the implications of these dysfunctions for thera-
peutic guidance.

CLASSIFICATION OF CP
POSTURAL DYSFUNCTION IN
Severity of dysfunction in children with CP CHILDREN WITH CP
can best be classified according to the Gross
Motor Function Classification System (GMFCS; Postural problems play a central role in the
Palisano et al., 1997). The classification system is motor dysfunction of children with CP. The
based on the child’s self-initiated movement with performance of everyday activities is noticeably
an emphasis on controlling sitting and walking influenced by such postural deficits; the extent
abilities, with or without the use of assistive tech- however, varies with the degree of the disability.
nology, such as walkers, crutches, and wheel Apart from severity of disability, biomechanical
chairs. The GMFCS contains five levels; a child constraints, such as the size of the support-base,
classified at Level shows minor gross motor also influence the child’s possibility to control
dysfunction whereas a child at Level V exhibits posture. The small base of support in standing
limited voluntary control of movement. As motor induces a more pronounced deficiency when
function is related to age, the classification has compared with the postural deficit seen in the
four age bands (< 2 years, 2-3 years, 4-5 years, 6- sitting position, which offers larger stability limits.
12 years). Children with CP can also be classified To perform the vital tasks of daily life adequately,
according to diagnosis (i.e. hemiplegia, diplegia, many children therefore spend much time sitting.
tetraplegiathe latter two more recently being In this text, we will therefore largely focus on
classified as bilateral spastic CP to describe the postural control in the sitting position because it
distribution of the impairment). offers good possibilities to investigate the patho-
This categorization, however, only provides a physiology of postural control in a large group of
vague idea about the child’s functional performance. children with CP. Knowledge on the specific
Most children with diplegia are distributed across nature of the postural problems is vial because it
Levels to IV, those with hemiplegia at Levels to can enrich our thinking when choosing therapy and
IIl, and children with tetraplegia and dystonic CP at can be useful when adjusting therapy to the
Levels IV and V (Ostensjo et al., 2003). The difficulties of a specific patient.
GMFCS classification thus offers a possibility to Postural control in children with CP has been
create a functionally more homo-geneous repre- studied using two experimental paradigms: (1) a
sentation of the heterogeneous group of children sudden destabilization by means of a movable
with CP. Rosenbaum and colleagues (2002) longi- support-surface (Nashner et al., 1983; Woollacott et
tudinally followed gross motor function of children al., 1998; Brogren et al., 2001), and (2)disturbing
POSTURAL DYSFUNCTION IN CHILDREN WITH CP 223

forces produced by voluntary movements (Hadders- Only children with severe CP (GMFCS level V),
Algra et al., 1999a; van der Heide et al., 2004). who cannot sit independently, dlsplay a total lack
Destabilization by external forces demands a quick of such ’direction-specific’ postural adjustments
reaction to counteract the forces, whereas destabili- (Hadders-Algra et al., 1999a; 1999b). This severe
zation caused by voluntary movements often can be deficit cannot be attributed to the inability to sit
estimated in advance and thus anticipated, due to without help, as ’non-sitting’, typically developing
experience. At a first glance, the two modes of infants already show direction-specific adjustments
control (compensatory or feed-back control and at a very early age (Hadders-Algra et al., 1996;
anticipatory or feed-forward control) can appear to Hedberg et al., 2004). Two explanations for the
be separate entities but in daily life, they are often lack of direction-specificity in children with severe
combined. When disturbing forces from a voluntary bilateral spastic CP at GMFCS level V can be
movement are not fully anticipated, compensatory offered: (1) the postural synergies cannot be
strategies are called into action. programmed; (2) the sensory pathways cannot
elicit activity in the synergies. We can assume that
Basic level of postural control: direction-specificity children who lack this basic postural building
block will never learn to sit independentlymeven
A primary goal of postural control is efficiently with ample practice. A partial loss of direction-
counteracting the disturbing force by means of specific adjustments at the level of the hip was
direction-specific postural adjustments (see Hadders- found in children at GMFCS level IV and in young
Algra & van der Heide, 2005; Hadders-Algra, children at level III, especially during external
2005). In general, children with CP can produce perturbations (Brogren et al., 1996) (Fig. 1), as
such direction-specific postural muscular activity. well as occasionallyduring successful reaching

a.0tmv
LE

_.10.OlmV
T
Fig. 1: Mean averaged EMG recordings of postural responses to forward platform perturbation while sitting in a
typically developing child (TD) and a child with bilateral spastic CP (Bi-CP), GMFCS- level IV. TD child
shows appropriate direction specific activity in the ventral neck-, triank-, and leg muscle; Bi-CP child" a partial
lack of direction-specific adjustment: activity in HAM precedes activity in RF. Plf=platform signal; NF=neck
flexor; NE=neck extensor; RA=rectus abdominis; LE; lumbar extensor; RF=rectus femoris; HAM=Hamstrings.
Dotted lines indicate baseline muscular activity + 2 SD; vertical line denotes perturbation onset. (Adapted from
Brogren et al., 1996)
224 E. BROGREN CARLBERG AND M. HADDERS-ALGRA

(van der Heide et al., 2004). A partial loss of adaptation in children with CP are
direction-specificity is often accompanied by 1. top-down recruitment of postural muscles
difficulties in sitting independently, difficulties (Nashner et al., 1983; Brogren et al., 1996),
that seem possible to overcome with training 2. excessive degree of antagonistic co-activation
(Butler et al., 1998). during external perturbations (but not during
reaching) (Woollacott et al., 1998; Brogren et
al., 2001; Van der Heide et al., 2004), and
Second level of postural control--adaptation of 3. lack or an incomplete modulation of the EMG-
the adjustment amplitude to task specific constraints (Brogren
et al., 2001).
The most frequently occurring dysfunctions in The predominant early recruitment of neck
children with CP are in the adaptation of postural muscles in children with CP forms a good basis for
muscular activity. This adaptation involves a fine- training of head control (Fig. 2). Improved control
tuning of the basic direction-specific adjustment to of the head is a vital goal of intervention for
environmental conditions, based on experience and children with moderate to severe disabilities, since
concurrent sensory input from somatosensory, visual, it is a prerequisite for communication, feeding and
and vestibular systems. Typical characteristics of this eating, and successful reaching.

A B

100 %

50 %

O%

NF RA RF NF RA RF

Fig. 2: Differences in postural activity during backward body sway in sitting position induced by forward perturbations
from a moving support surface between typically developing children and children with CP. Panel A: latencies
(msec) to EMG responses in NF=neck flexors, RA=rectus abdominis, and RF=rectus femoris. Panel B: rate of
response (%) during.which a specific muscle started the adjustment. Filled boxes represent children with CP
th th
and open boxes represent typically developing children. Boxes indicate 25 and 75 centiles, vertical bars the
total range, and black horizontal bars denote the median value. Asterisks indicate statistically significant
differences * p<0.05, ** p<0.01 (Wilcoxon). (Adapted from Brogren et al., 1996 and Brogren et ai., 1998).
POSTURAL DYSFUNCTION IN CHILDREN WITH CP 225

A high degree of antagonistic co-activation has possibly ruling out the achievement of independent
been demonstrated in children with CP, especially sitting. Virtually all children with CP display
during backward body sway induced by a movable dysfunctions in the adaptation of the adjustment.
support-surface (Brogren et al., 1998; Brogren et Typical characteristics of this adaptation in sitting
al., 2001). During forward body sway induced by a children with CP are a top-down recruitment of
backward moving support-surface, the degree of postural muscles, an excessive degree of antagonistic
co-activation decreases. This lower degree of co-activation, and an incomplete adaptation of the
antagonistic activation could be related to the EMG-amplitude to task specific constraints.
larger stability limits in forward direction but might
also reflect differences in the supraspinal control
of flexor muscles and extensor muscles (Dietz et SITTING POSITION AND ARM-HAND
al., 1989, Hadders-Algra et al., 1998). During self- FUNCTION
paced voluntary reaching, the antagonistic muscles
are rarely active (van der Heide & Hadders-Algra, Stimulation of motor development, including
2005). Thus, the degree of co-activation in postural development results in better functional
children with CP seems task-specific and cannot performance of activities of daily life. It is,
be explained solely by altered spinal circuitry like however, far from clear what the best ways are to
reduced reciprocal inhibition (Leonard et al., stimulate motor development in children with CP.
1990). Two questions often asked in clinical practice are
A high degree of antagonistic co-activation 1. Is there a best sitting position for children with
provides stability but reduces flexibility. The CP?
strategy is commonly used in the cognitive phase 2. Does a specific sitting position result in good
of learning when forces linked to a specific task arm-hand function?
have not yet been fully integrated into the motor
behavior. A high degree of co-activation could Special seating plays a significant role in the
therefore be viewed as a strategy to cope With management of children with CP. Various studies
deficient postural control rather than a problem per have attempted to elucidate which sitting position
se. Providing support and thereby decreasing the can be considered optimal. There are advocates of
degrees of freedom might be one therapeutic an erect posture (Nwaobi 1986., 1987; Green &
solution that can facilitate learning in children with Nelham., 1991), of a straddle position sometimes
CP as they gain control over various motor tasks combined with a forward leaning of the trunk
that challenge the control of posture. The support (Myhr & von Wendt., 1991; Pope et al., 1994;
can then gradually be decreased to a level that the Reid, 1996), and a few promoters of a reclined
child can cope with. posture (McClenaghan et al., 1992; Hadders-Algra
The deficient modulation of EMG-amplitude et al., 1999; Brogren et al., 2001). The confusing
seen in a majority of children with CP could results can be attributed to many factors, the
represent difficulties in implicit learning, leaving substantial heterogeneity of the study groups being
them with co-activation as one solution to this one. A primary goal in habilitation is to find a
problem (Gentile, 1998). sitting position that gives the child an opportunity
In conclusion" children with CP exhibit in to control the arm and the hand in an optimal way
general muscular activity counteracting forces that in such activities as eating, communication, and
disturb equilibrium. Only ’non-sitting’ children with dressing. Few studies, however, have evaluated
severe CP lack such ’direction-specific’ adjustments, whether adaptive seating leads to better arm-hand
226 E. BROGREN CARLBERG AND M. HADDERS-ALGRA

function. No advantage on the smoothness and can activate the arm and trunk muscles
precision of the arm-hand movement was reported independently, better control can be gained in
in changing the .seat angle (Seeger et al., 1984; various activities, but this means that the child has
McPherson et al., 1991), whereas anterior tilting of to learn to deal with many degrees of freedom.
the support surface decreased the speed of arm How could this be done? One suggestion could be
movement (Nwaobi, 1987). to restrain the trunk loosely to make it possible for
Van der Heide et al. (unpublished) recently the child to start the reaching movement with both
investigated the effect of seat surface inclination the arm and the trunk, but in order to reach a
on postural stability and quality of reaching in desired object, the arm has to travel the path to the
freely sitting children with CP. The authors found end-point isolated from the trunk. This would
that in children with spastic hemiplegia and in provide a more relevant somatosensory input from
children with bilateral spastic CP, tilting of the the arm that can be used to modulate the reaching
seat surface differentially affected postural adjust- pattern. Reaches beyond arm length could also
ments and the quality of reaching. In children with provide a possibility to experience a freely moving
spastic hemiplegia, forward tilting of the seat arm detached from the trunk.
surface improved postural efficiency and quality of Another way to influence the control of
reaching, whereas back-ward tilting was associated posture could be to augment the intensity of the
with increased postural muscle activity and less somatosensory input by putting a bracelet with a
stability of the head. In children with bilateral weight on the moving arm (Hadders-Algra et al.,
spastic CP, both forward and back-ward tilting of 1999). From functional goal-directed training
the seat surface was associated with postural (Ketelaar et al., 2001; Ekstr6m-Ahl et al.,
instability. The results of these studies suggest that unpublished), we now know that ample, variable
in children with spastic hemiplegia, the forward- training in motivating settings is an important
tilted position is the optimal sitting condition, prerequisite for learning. Trial and error can thus
whereas in children with bilateral spastic CP, the form the basis for selecting efficient movement
horizontal sitting position seems to be optimal. patterns (Hadders-Algra, 2000).
Children with CP move their trunks during
reaching just as much as typically developing
children do (Van der Heide et al., unpublished). In CONCLUDING REMARKS
typically developing children, movements of the
trunk are not related to the quality of reaching. In Postural problems in children with CP and the
children with CP however, a positive link exists pathophysiology underlying these problems are
between trunk movements and reaching quality. presently fairly well described. On the other hand,
Thus, it seems that the arm, hand, and trunk are we have little ’high-level’ evidence on how
programmed together in a fixed temporal order different interventions can affect these problems.
during the reaching movement to assist trans- Therapeutic attention to promote motor perfor-
porting the hand to the target in a precise way. mance in sitting focuses on adaptive seating, tilting
This program strategy can be useful in movement of the support surface, and ample, variable training
coordination but requires stable control of the in motivating settings. The challenge facing us
trunk through a longer movement path. This now is to provide evidence about the efficacy of
control, in turn, may decrease the child’s ability to specific treatment approaches facilitating that
function optimally in daily life. From a clinical children reach an optimal level of functioning in
perspective, we presume that if a child with CP daily life.
POSTURAL DYSFUNCTION IN CHILDREN WITH CP 227

ACKNOWLEDGMENTS Postural adjustments due to external perturbations


during sitting in 1-month-old infants: evidence for
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