From Movement To Action A New Framework For Cerebral Palsy

European Journal of Physical and Rehabilitation Medicine
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From movement to action: a new framework for cerebral

palsy
Adriano FERRARI
European Journal of Physical and Rehabilitation Medicine 2019 Sep 24

DOI: 10.23736/S1973-9087.19.05845-3
Article type: Special Article
© 2019 EDIZIONI MINERVA MEDICA
Article first published online: September 24, 2019

Manuscript accepted: September 17, 2019
Manuscript revised: July 9, 2019
Manuscript received: May 9, 2019
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From movement to action: a new framework for cerebral palsy

Adriano Ferrari1,2*
1
Physical and Rehabilitation Medicine, CHIMOMO Department, University of Modena and Reggio Emilia, Italy
2
UDGEE Mother-child department, S. Maria Nuova Hospital, IRCCS AUSL Reggio Emilia, Reggio Emilia, Italy
*Corresponding author: Adriano Ferrari, UDGEE, Arcispedale S. Maria Nuova, viale Risorgimento 80, 42123 Reggio
Emilia. E-mail: adriano.ferrari@unimore.it
ABSTRACT
The interpretation of Cerebral Palsy (CP) is closely linked to points of view that are no longer acceptable:
- the idea that it is primarily a motor problem (posture and movement disorder),
- the idea that it is only a central (cerebral) pathology,
- the idea that it is a non-progressive disease (fixed encephalopathy).
Actually, the problems that contribute to producing the CP clinical picture are several and complex. First of all, building
of the action, starting from subject motivation, through motor imagery and subsequent project elaboration. Sequentially,
executive planning, disorder often hidden under the most remarkable alteration of motor patterns and muscle tone.
Finally, realization, conditioned by the idea that the locomotor apparatus is only and always the victim of an incapable
central nervous system. Little known and very neglected perceptive components can contribute to compromising subject
motor control. The influences that primitive changes of musculoskeletal system, often depending on site, nature, size
and time of the lesion, exert on the possible choices of the central nervous system are often overlooked. Peripheral
structures can in fact modify considerably the expression of palsy (understood as the form of adaptive functions)
primitively.
At least six different sources of error can be identified in the cerebral palsied child. For a rehabilitative intervention with
greater possibilities of effectiveness, it is necessary to recognize and evaluate each of them. Especially as regards the
prevention of secondary deformities, the responsibility attributed to physiotherapy must be re-evaluated.
Key words: Cerebral palsy – ideomotor model – possible errors – principal alteration – perceptive problems.
INTRODUCTION
By nature, a definition characterizes and circumscribes an entity on a conceptual level. In other words, to highlight its
"core".
For over 60 years, the definition of Cerebral Palsy (CP) has been centered on the concept of disturbance of posture and
movement1. Even assuming that this may be the true nature of CP, to be precise, as any definition requires, both these
terms must be reconsidered because the posture we are talking about is actually a position, since it refers to a body
subject to the gravitational field, and as such already included in the more general term of movement. If we want to
conceptually distinguish position from movement, the latter should be coined gesture, intended as a passage from one
position to another. So, CP could be considered a disturbance of position and gesture created by non-progressive
damage (fixed encephalopathy) suffered by Central Nervous System (CSN) of a child during pregnancy, at delivery or
shortly after birth. However, is the disorder of position and gesture really the "core" of CP? Is the adjective "cerebral"
appropriate in characterizing CP nature? Altered movement is surely the first and most explicit sign of the presence of
CP. Nevertheless, should this sign be considered exclusive or, in any case, the most important aspect of CP?
More than a hundred years ago (1897), Sigmund Freud questioned the nature of Cerebral Palsy, an expression
introduced into the scientific world by John Little in 1862 as "Cerebral Paresis": The term infantile cerebral palsy ... in
fact is a nomen proprium. It characterizes not only what is implicit in the combination of these words, namely paralysis
in childhood due to cerebral causes (as a result of cerebral damage), but what has long been applied to pathological
conditions in which the paralysis is overshadowed or replaced by muscle stiffness or spontaneous muscular spasms ...
The term infantile paralysis simply represents a clinical picture of the illness ... it is not equivalent or comparable to
either an anatomopathological picture or an etiological entity. It is therefore likely that, from a clinical point of view,
the term can only obtain the value of a temporary entity that has to be soon abandoned in favor of a more coherent and
possibly etiologically better-defined disease description.
The current CP definition is still the one coined by Balf and Ingram in 1955 1, re-proposed in 1959 by Mac Keith,
MacKenzie and Polani2 (members of the Little Club) and later disseminated by Bax 3 (1964). Cerebral palsy is a
"persistent but not unchanging disorder of movement and posture". Fifty years later, a review of this definition, written
by Rosenbaum et al.4 (2006), again states: Cerebral palsy describes a group of disorders of the development of
movement and posture, causing activity limitation, which are attributed to non-progressive disturbances that occurred
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in the developing fetal or infant brain. The reference to ICF (International Classification of Functioning, WHO, 2002) is
a mandatory act, as well as the emphasizing of the "infantile" nature of CP. The most important change introduced by
the 2006 revision is contained in the admission that: The motor disorders of cerebral palsy are often accompanied by
disturbances of sensation, cognition, communication, perception, and / or behavior, and by epilepsy and secondary
musculoskeletal problems. In fact, Rosenbaum (2017), only ten years later, re-evaluating the observations of Freud,
wrote “CP remains an idea – a concept – rather than a specific disease entity”, thus joining the list of authors who have
patiently sought a clearer and more encompassing definition of CP 5, 6.
To understand which relationships exist between disorders of posture and movement (position and gesture) and sensory
and perceptive disturbances, deficits or alterations in cognition, communication and behavior, it is necessary to consider
which and how many possible errors are committed by a CP child. To guide and simplify this reasoning, the ideomotor
model7 will be utilized (see fig.1).
Fig. 1 Ideomotor model
THE IDEOMOTOR MODEL
Projection
Analyzing the left column first, the initial point is projection. This term includes motivation, ideation and programming
of the movement, or rather, as we will soon see, of the action to be performed.
1) Motivation is what drives individuals to satisfy their needs and desires. It is a complex of internal states of the
organism that determines capacity for action and global and selective reactivity to endogenous stimuli: a specific state
that neurophysiologists call arousal. Every motivational state activates the organism and pushes individuals to identify
possible goals in order to direct their actions. Motivating behaviors can be induced by contingent needs or by a simple
prediction of the pleasure that one knows could derive from the results of performed actions. Naturally, perceptions and
emotions experienced by actually carrying out the designed action will become, from the neurophysiological point of
view, positive or negative reinforcements. Memory represents a retrospective facilitation, whereas desires represent an
anticipatory one.
Besides the complex of internal states, there are also external factors present in the environment (clues, suggestions,
possibilities and above all behavior models) which together constitute the affordances described by Gibson8 (1966).
External, or contextual, factors combine with internal ones to generate motivation.
What is the "primum movens" of motivation? In the Gospel according to John, it is written "In the beginning there was
the Word ... With this term the apostle indicates knowledge, a value that man cannot however possess a priori. In fact,
the gospel goes on to say that the Word (knowledge) was with God.
On the contrary, Goethe (1808), German playwright, makes his Faust (the devil) say: "In the beginning there was the
Act", from which derives the experience that will become the foundation of acquired knowledge. Sachs 9 (1985), a
famous neurologist, downsized the idea of action (a movement cognitively organized to achieve a goal) by stating that
"In the beginning there was the Instinct". Instinct is an innate genetically transmitted impulse, able to activate definite
behaviors, effective towards the sought-after result, but inevitably fixed and stereotyped. It is a form of knowledge, not
individual, but resulting from the evolution of our species, in the vision conveyed by Darwin (1859). Buber (1926), a
German psychiatrist, affirms that: "In the beginning there was the Relationship", that is, the individual's need to feel
part of the social environment (e.g. the development of language as an expression of an acquired function). Berthoz 10
(2003), a French neurophysiologist, traces a remarkable synthesis of these different positions stating that: "The act is
not movement, the act is the intention to interact with the world or with oneself as part of the world" and adds: "The act
is always the pursuit of an objective, it is always supported by an intention ". So, if the motivation supports the action,
whose purpose is to satisfy needs and desires by providing pleasure, the first question we should ask is whether CP
children can autonomously meet their needs, realize their desires and above all experience the same pleasure in moving
as their typically-developing peers.
2) Motivation supports Motor Imagery (MI), understood as the ability to mentally represent a movement without
physically executing it11. MI is the dynamic state during which the subject simulates a certain action. Libet 12 (2004), an
American neuropsychologist, investigating the ways in which simple motor decisions are made, observed that brain
signals indicating a decisional act could be detected several hundred milliseconds before a conscious awareness of it.
Since then, several authors have demonstrated MI compromise, also when task dependent, in CP children13. A recent
study conducted by our group on a sample of hemiplegic children vs. healthy controls using the paradigm of mental
chronometry in preferred hand grasping, transporting and releasing tasks demonstrated that the ability to use explicit MI
in CP children is primitively altered14.
3) Motivational and ideational components follow action design based on the use of cognitive maps that guide
execution; then a gesture for a purpose, in a given context, at a specific moment. If this design is compromised, we have
the ideational apraxia which, as De Renzi and Faglioni (1992) have taught us, concerns the ability to formulate or evoke
the action project as a whole (the individual does not know what to do). In hemiplegic children, ideational apraxia
naturally also involves the unaffected hand. This was evidenced by Surkar et al15 (2018) which, by proposing an action
projection and planning, showed longer observation times and increased latency compared to typically-developing
controls.
In CP children, design errors involve elements of CNS, intended as the organizing structure of movement (position and
gesture), ranging from the repertoire of remaining motor modules to the most complex synergies, through reflexes,
reactions and primary motor patterns, secondary automatisms, etc. Muscular and postural tone also achieve errors at this
level. Thus, according to Graham et al16... persistent or poorly inhibited 'primitive' reflexes, abnormal organization of
movement and posture, hyperactive reflexes and abnormal muscle tone, including spasticity ... can justify phenotypic
variability of CP forms.
In this regard, Milani Comparetti17 (1980) sustained that: "Emphasis on reflexes and muscle tone has been given undue
privilege in investigation of the Central Nervous System (CNS) as "hardware". Neurological examination based on
assessment of the integrity of the "machinery" which is capable of producing a motor program. This type of
investigation may be useful for lesion diagnosis and possible neurosurgical intervention. A different investigation is
necessary for a functional assessment of the "software" of motor programming, which takes place in the CNS and
reveals itself in the quality of the produced movements. The program of movement, which is one of the functions of the
CNS and consists of elaboration of the command, which the executive mechanism will then carry out. Functional
integrity of movement patterns implies freedom of operational choice”. Furthermore, concerning the integrity of the
motor patterns in CP: "Cerebral palsy is principally a disorder in building patterned structures of movement with the
result that there is a limitation of freedom of choice of movement and posture. In other words, because of a structurally
disordered nervous system, normal flexibility and plasticity of the system are impaired. Motor performance becomes
functionally impaired and stereotyped".
If presence of pathological patterns is the connotative element of CP, it is not clear why this diagnostic entity would
also include ataxia and disequilibrium, which are clinical forms characterized by an altered integration in time and
space of otherwise normal movement patterns.
Weak motivation, MI impairment and consequently altered action design constitute the first type errors committed by
CP children.
Planning
Projection is followed by executive planning, which is praxia, the sequential organization of action. This includes the
way to initiate, guide and complete the action both from a temporal and spatial point of view. De Renzi and Faglioni
(1992) speak of ideomotor apraxia when the subject "does not know how to do". In neurological science, apraxia
(dyspraxia for children) is a disorder of voluntary movement that cannot be attributed to paralysis (inability to perform
movements), sensory or sensorial disturbances, cerebellar disorders or mental deficits. In CP, therefore, one cannot
legitimately speak about dyspraxia, unless it is considered a disorder "that is masked" by paralysis, as Sabbadini18
(1995) has wisely highlighted. "The dyspraxic child has a reduced ability to represent himself, the object on which to
act, the whole action and the composing sequences; has difficulty in ordering and coordinating the relative elementary
movements in view of a purpose (programming), to start the relative program, to foresee (in anticipatory sense) a
certain result, to control each sequence and the whole activity in the course of the action (feedback), to verify the
obtained result as corresponding to the expected and desired one.”
In executive planning, the CP child can, in fact, make second type errors.
Action realization follows executive planning and includes two phases:

- preparation
- execution
Automatic planning processes, such as anticipatory postural adjustments (APA), configuration of sensory receptors and
all those pathological ones referring to 'first motoneuron syndrome', located downstream from motor pattern
configuration, belong to the preparation phase. These include deregulation of reticular formation, accentuation of
stretching reflexes, alteration of reciprocal inhibition agonists - antagonists, liberation of pathological reactions such as
the sign of Babinski, extension of reflexogenic area for stretching reflexes, among others.
Instead, execution relates directly to the characteristics of locomotor apparatus.
Realization
The classic model of CP interpretation considers the locomotor apparatus as the “designated victim” of errors
committed by CNS, with well-known consequences represented by development of "secondary" deformities, currently
underlined by Graham et al16 which state that: Motor impairments with poor motor repertoire, hypertonia, progressive
muscle changes related to neuronal, nutritional and mechanical factors, lead to musculoskeletal deformities. This view
is only partially acceptable, while the idea that physiotherapy (in this case intended as movement administration) has to
"prevent secondary deformities" is completely unacceptable. In fact, CNS lesions produce two separate effects: on one
hand an alteration of development of functions (standing, walking, manipulating, speaking, etc.), and on the other one a
structure metaplasia of locomotor system (nerves, muscles, tendons, joints, bones). In the past, we investigated the role
of CNS in producing pathological reactions and reflexes, altering muscular and postural tone, modifying posture and
movement (position and gesture) patterns. Until relatively recently, not enough has been done to explore the role of
CNS in guiding maturation and differentiation of peripheral tissues and structures. It has not yet been possible to draw a
linear relationship between lesion (nature, size, location and onset) and form of altered functions (what is identified
under the term "paralysis"). It seems much easier to find a relationship between CNS injury and structural alterations of
locomotor apparatus. For example, the degree of heterometry of lower limbs in a hemiplegic child correlates to the
extent of loss of nervous substance of the contralateral hemisphere and not to the developed locomotor pattern.
In the near future, advanced emerging functional neuroimaging techniques will probably be able to establish a direct
and linear relationship between lesion (nature, measure, type and timing) and form/evolution of palsy19.
In a CP child, all the tissues of locomotor apparatus undergo alterations: among these, the muscle is the most widely
studied tissue: "It is clear that skeletal muscles from CP patients are altered secondly by the neurological lesion20 ".
Indeed, functional and structural alterations, both macroscopic and microscopic, are paradoxically contradictory:
Functional alterations
- denervation-reinnervation and myopathic type degenerative phenomena21 with altered activation of alpha motor
neuron, impaired reciprocal inhibition of agonists / antagonists with successive release capacity, contractile power
reduction26, active muscle failure22.
- altered rheological and metabolic26 muscle proprieties, including resistance to elongation23, deformability module24,
reduced tendon stiffness25
Macroscopic alterations:
- length and size muscle reduction30 compared to bone19
- increased fibrous component27 in correlation with spasticity28
Microscopic alterations:
- fiber type disproportion21 and reduced diameter 29
- reduced addition of sarcomere series19, 26, 31 , presence of fetal fibers 2C32, diminished number of satellite cells with
reduced repair / regeneration capacity33
- modified structure of Titina19, 24 and Myosin immaturity21
- increased amount of the extracellular matrix34.
Decrease in satellite cells can explain reduced fiber diameter, different growth and increased muscle reactivity33. It
should however be noted that, even if CP patients have immature and altered muscle fibers, they do not suffer from
muscle tear injuries, despite the great tension expressed during spasms and the presence of muscle co-contraction, but
rather they suffer from joint injuries. The balance between resistance of joint-structures and adjacent muscle tension is
in fact altered by CP24. Fragility of joint capsule can be a primitive problem related to the type of present musculature
and dominant pattern movement. Joint structures succumb in relation to palsy somatic distribution: the hip especially in
tetraplegia, the knee in diplegia and the foot in hemiplegia. Tendons and ligaments can develop stretch marks (sub-
patellar, posterior tibial, peroneal) due to a variation in collagen components, with subsequent alteration in transmission
of forces35 according to CP clinical forms. Deficit of height growth is a consequence of reduced metaphyseal cartilage
activity due to a lack of or altered fetal remodeling and/or mutated instructions. Torsion phenomena of long bones are
not due to spasticity, but rather represent the direct outcome of the failure of intrauterine fetal remodeling 36 and
therefore depend more on primary brain injury rather than expressed motor pattern. The tibia may develop deformities
both in intratorsion and in extratorsion. Even the significant reduction in bone mineral density does not derive so much
from a true mineral loss, but rather from an altered growth rate of mineralized components, which are lower than those
of healthy peers37. According to the law of Julius Wolff (1872), bone structures are morphologically and quantitatively
rearranged in order to withstand imposed deforming forces. Connective tissue remodeling behaves analogously. These
transformations can therefore be considered as adaptive.
Given the effects of CNS post-lesional rearrangement and its plasticity, taking into account metaplasia phenomena
suffered by peripheral tissues, following altered trophic impulses coming from CNS, and given the “adaptive”
alterations induced by Wolff's law, amplified by somatic growth, we should now ask ourselves if “cerebral” palsy can
still be considered a "cerebral" problem and a “non-evolutionary” affliction.
In any case, we can think that the locomotor apparatus, initially seen as a “victim” of CNS errors, in reality over time
turns out to be a “partner in crime” and then eventually goes on to become a “promoter” of choices carried out by CNS.
In the construction of positions and gestures (movement), CNS must take into account the characteristics possessed by
locomotor apparatus that it has initially contributed to change. In CP, all alterations suffered by the locomotor apparatus
affect the realization of all possible actions. Now Gage’s statement (2004) "In its response the muscle is confused ...
especially bi-articular muscles have lost their normal function and are recruited for different tasks" is completely
comprehensible.
The most correct scheme for interpreting the relations between center and periphery could be as follows:
Figure 2 CNS and secondary deformities
Secondary deformities, which inevitably develop in CP, are not due to the lack of physiotherapy or to the fact that the
adopted physiotherapy method was not the correct one, or that the physiotherapist was not the right one, but are due to
the metaplasia phenomena suffered by all tissues of locomotor apparatus. In literature, we often read about “traditional”
or “usual” or “standard” physiotherapy when confronting new therapeutic proposals, however these terms have never
been clearly defined. This ambiguity masks the difficulty in declaring which re-education approach could be the most
efficient and suitable for CP. Most of the time, these terms include vastly different and inhomogeneous approaches,
frequently based on muscle stretching, strengthening, joint mobilization or at most muscle relaxation through passive
mobilization. This is revealed by changes in muscular strength and tension, with secondary lever arm alterations and
consequent modifications of the biomechanics of muscles, especially of bi-articular ones, and joints. The role of
adductors and medial hamstrings, which have always been considered responsible for the posterior hip dislocation, is
now also being questioned.
According to studies performed on biomechanical models 38 reproducing the characteristics of CP movement patterns
(flexed-adducted-intrarotated thighs with increased anteversion of femoral neck), many muscles forming part of the
group of intrarotators (long adductor, short adductor, pectineous, adductor portion of large adductor, semitendinous and
semimembranosus) would not only have an negligible intrarotatory moment, but when thighs are flexed, adducted and
intrarotated, could take on an extrarotatory action.
Figure 3 Miracle of Saint Cosmas and Damian. Unknown author - Landesmuseum Württemberg
If, following the example of the twins Cosmas and Damian, respectively the patron saints of Medicine and Pharmacy,
we wanted to transplant two completely normal lower limbs to a diplegic child, it would be impossible to think that this
child could use them properly, due to the “cerebral” nature of the palsy. The opposite thought however is less
immediate. If those paretic limbs of the diplegic child were transplanted to an otherwise healthy child (with a normal
CNS), he/she would not be able to move them normally, due to locomotor apparatus alterations. These constitute the
third type of CP errors.
Perceptual Anticipation
We can now consider the right-hand column of the ideomotor model (Fig 1). The starting point is the horizontal arrow
that from executive planning leads to perceptual anticipation, closing two separate circuits, the first one being feed-
forward anticipation, the second one being a retroactive analysis or feedback. In the second half of the nineteenth
century it became clear to neurophysiologists that they could not perform an analysis of the result of a completed action
if they were not able to anticipate its consequences. For this purpose, before a programmed and planned action can be
carried out, its results must be anticipated in perceptual terms. The horizontal arrow represents what neurophysiologists
call “collateral discharge”.
Hermann von Helmholtz (1866) supported the idea of the necessity to compare the output signal and corresponding
sensorial afference, aimed at verifying their coherence and thus giving stability to perception. Berthoz39 (1997) talks
about anticipatory configuration, a collateral copy of the action program. This perceptual anticipation is an internal
simulation of the results of the action before it could be executed. In essence, this is an anticipation of the consequences
of the action. "The essential part of the act is the anticipation and our brain is essentially a machine that anticipates this
by creating a series of probabilities, which simulate reality in the extremely brief period preceding the action39". The
resulting judgment (perceptual consent to the action) is based on a recognition process where the categorization of
sensation data coming from the anticipated reversal of the motor project requires a comparison with previously
memorized information. The collateral discharge is essential to precisely know if it is possible to perform that action
and successively control its execution step by step. The memory of past experiences must be recalled for the present
action or to foresee the consequences of a future action. "We can think that our brain builds a virtual world, a kind of
dream lived by its double, which allows a permanent simulation of the action and a choice of the best solution or an
inhibition of useless acts ... The probabilistic anticipation allows us to compare sensory data with the consequences of
past actions and predict the consequences of the ongoing action. This double check takes place at the thalamus level39".
If a negative result is foreseen, or judged intolerable, the perceptual consensus (conscious or unconscious) is denied and
the programmed action is not performed. The mechanism allows wrong ideas, as Karl Popper sustains, “to die in place
of us".
The modular theory of mind proposed by Fodor40 (1983), an American cognitive philosopher and scientist, is able to
explain why an alteration of interpretive frames used by CNS can lead to erroneous anticipations of the effects of
actions that the individual is actually able to perform (thus misleading consequences or results). To better understand
what these interpretative frames are, we could think about an elementary arithmetic operation: the sum of 11 + 11. The
obvious answer is 22, but if we misalign the figures, the result could be 121 or even 1,111. Figure alignment represents
the interpretive frame. For this reason, when teaching math in primary school, teachers require their students to use
graph paper. This condition means that "in many psychiatric or neurological diseases, also in those affecting CP
children, manipulation of these different reference systems is compromised, and this can produce apparently motor
symptoms39 ". The perceptual disturbance of cerebral palsy can be explained by the modular theory of Fodor because, if
the central access was not bound to wrong interpretive frames, the patient could quickly realize the mistake in
interpreting the information and provide to correct it, exploiting cognitive abilities.
Action Consent
The similarity to panic disturbances leads us to think that perceptual disorders of cerebral palsy could be sustained by an
altered activity of amygdala. According to Rizzolatti, it is possible that due to altered nerve connections between
sensory areas and amygdala, or between these and other CNS regions, individuals affected by perceptual disorders may
have a distorted emotional map: an archive of misinformation that induces the amygdala to make non-appropriate
decisions and produces no calibrated reactions respect to stimuli41. Disorders of this kind lead to fourth-type errors.
Information Collection and Comparison
Once perceptual consent is obtained, the previously projected and planned action can be performed. Its execution makes
it possible to receive the expected information. Processes of collecting and interpreting basic information, comparing
and integrating it into perceptions, recognizing and storing them in representations, constructing memories and
procedural maps, etc., are all part of CNS activities. In CP, also these processes are altered because, as Henri Poincaré
(1905) claimed, none of the senses would be functional without the contribution of movement, or in other words there
cannot be perception if there is no action9. Three different mechanisms contribute to alterations of collected
information:
- Receptors, especially neuromuscular spindles, that represent the most sophisticated sensory organ, can present
primitive alterations that compromise functionality42. The motor plate may be also altered43. Intramuscular
mechanoreceptors may be reduced in number44. Deficits of tactile sensitivity and disturbances of
proprioception can compromise manipulative capacity45.
- Due to severe lack of sensory integration, CP children cannot literally trust their collected sensory
information46, nor adequately organize it for the purposes of motor coordination 47. Correspondence between
sensory disturbances and degradation of motor performance is justifiable47. In addition to compromising
manipulative and reaching capacity48, "sensorimotor" deficits affect locomotion, navigation and spatial
orientation in walking function49. Visuo-kinesthetic dissociation45 creates particular difficulties in overcoming
obstacles, even inexistent ones, such as the threshold separating two rooms or a colored pattern of floor tiles.
- If movement is primitively distorted and the action imprecisely programmed, perception will be consequently
influenced. In order to gather reliable information, specific and specialized movements are required. The
opposite statement is however equally true: to produce precise movements, reliable information is necessary.
- Especially in cases of hemiplegia, ambiguous information is centrally removed by CNS, as happens with a
cross-eyed child in gaze palsy. Reducing contribution of information limits cortical representation of involved
segments50 with consequent minor motor use and the beginning of a dangerous vicious circle.
- The effects produced by failure of respecting critical periods for activity-dependent and use-dependent
plasticity, and restricted sensorimotor experience (less neural stimulation owing to fewer or less complex
movements) and poor perceptual development experiences16 should be added to the previous elements. We
must however carefully reflect on these statements, since other diseases with a similar severe motor
impairment such as Spinal Muscle Atrophy do not present the same disorders.
We can consider that these factors lead to fifth-level errors.
The information however collected after carrying out the action, must be compared with what had been imagined in
terms of perceptual anticipation. Here the sense of agency intervenes, i.e. awareness of being the author of one's own
actions or experiencing oneself as the agent51.
This is “a central feature of the different phenomenal experience constituting self-consciousness”52. Within the sense of
agency, the feeling of agency can be differentiated from the judgment of being the author of the considered action
(judgment of agency). The first level is linked to basic sensomotor processes, the second one to higher order procedures
that control awareness of having performed the action53. We can describe this hypothetical "sixth sense" as the ability to
diachronically control action performance through integration of visual and kinesthetic information. Proprioception is
indispensable for the sense of ourselves (coenesthesia), since it is only thanks to this that we perceive our body as ours,
as our property, as truly ours (Sherrington, 1940). Without proprioception, the body becomes blind and deaf to itself
and ceases to be the owner of itself, to feel itself as itself 9.
In CP children, a poor integration between intention of movement and visual and proprioceptive information, related to
the performed action, can represent an important functional disorder.
Figure 4 Action comparator
The theory of the action comparator of Blakemore54, confirms the validity of the ideomotor model, from which we
started, and completes it by adding the judgment of having been or not the actor of the perceived action. Following this
model, the sense of agency refers to the feed-forward loop, based on the collateral discharge, namely the perceptual
anticipation of the projected and planned action, which will be then compared to the actual collected sensory
information (feedback loop).
Figure 5 Alien hand experiment
In CP children, the difficulties that take place at this level has recently been demonstrated in the "alien hand"
experiment, carried out by Nielsen (1963). In this experiment the impairment of the ability to recognize if the observed
movement was performed by one's hand or by an "alien" hand was documented.
A computer vertically projects six icons, the lower one representing the starting point and one of the upper five the target to be reached by tracing a
line with an electronic pen on a tablet. This tablet, invisible to the subject, records the line trajectories and therefore hand movements. The researcher
can interfere by deviating from five to 20 degrees the actual line drawn by the CP child. One out of five trials is "made up" without the patient's
awareness. This test reveals that CP subjects perform less linear trajectories and take more time to complete the trial. In addition, they can only
recognize researcher interferences greater than 15 - 20 degrees. They attribute errors of less than 15 degrees to themselves. This research was
conducted on hemiplegic CP children who used their preferred hand rather than their affected one.
Figure 6 sense of agency
In the experiment of joining two points by observing lines drawn on a screen, without seeing the hand, Ritterband-
Rosenbaum et al55 (2011) have observed that, while typically developing (TD) controls regulate their movements based
on anticipatory mechanisms (feed-forward), CP children base their strategies on retroactive processes (feedback), in
which movements are progressively re-adjusted, mainly based on visual information 54.
Today we can easily explore the disorders related to the sense of agency through the use of video games equipped with
three-axis wireless accelerometers able to react in real time to movements performed by patient within three-
dimensional space with absolute precision. These devices, easily found on the market, also record rotations and
inclinations through a digital gyroscope.
The CP child, observing the screen, undergoes a "third person" experience thanks to the presence of an avatar (virtual
image of himself) that can be customized as desired.
The videogame allows for exploration of the quality of motor control during the action performed by the child. By
taking control of the avatar, without patient awareness, the researcher can assess to what extent the child is able to
perceive differences between his/her own voluntary movements and those performed by the avatar when guided by the
researcher.
Disturbances in the “sense of agency” constitute the sixth type of error of CP children.
CONCLUSIONS
Can we still define Cerebral Palsy as principally a disturbance of posture and movement? If we, as a minimum, talk
about altered motor control, we could include both the capacity to collect information and produce relative motor
adjustments (concerning position and gesture). In alternative, we could accept the French expression “Infirmité motrice
origine cérébral” but with the intention to only emphasize the idea of CP as a systemic pathology originating from a
brain lesion, nevertheless still ignoring sensation, cognition, communication, perception, and / or behavior
components4.
In any case, we should keep in mind that in CP different types of errors can contribute to creating the cumulative
clinical picture and the continuous reciprocal interaction between CNS and locomotor apparatus.
Considering “cerebral palsy” as an “umbrella term”, that covers different and inhomogeneous conditions, does not help
us find the common connections that could link the different elements that compose this disease. The ideomotor model
tries to solve this problem.
How should we take these different error types into account when selecting therapeutic proposals?
Are we completely aware of how much there is still to discover and learn about cerebral palsy?
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From Movement To Action A New Framework For Cerebral Palsy

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European Journal of Physical and Rehabilitation Medicine

EDIZIONI MINERVA MEDICA

From movement to action: a new framework for cerebral

European Journal of Physical and Rehabilitation Medicine 2019 Sep 24

Article type: Special Article

© 2019 EDIZIONI MINERVA MEDICA

Article first published online: September 24, 2019

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EDIZIONI MINERVA MEDICA

From movement to action: a new framework for cerebral palsy

Fig. 1 Ideomotor model

THE IDEOMOTOR MODEL

Action realization follows executive planning and includes two phases:

Figure 2 CNS and secondary deformities

Information Collection and Comparison

Figure 4 Action comparator

Figure 5 Alien hand experiment

Figure 6 sense of agency

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