Definition of infantile cerebral palsy: a long history

In 1964, Bax published the first definition of Infantile Cerebral Palsy: “chronic disorder of movement and posture caused by a non-progressive lesion of a premature brain”.

However, the way to reach this definition was long and difficult.

Historically, the discovery of this pathology goes back to an English orthopedic surgeon, Sir John William Little, who in 1892 observed and classified a series of congenital skeletal deformities of different types in children who showed signs of central nervous system disorders (treated in the “Idiot Hospital”, a terrible term with which children with difficulty were called) and were alike in that they had experienced difficulties at the time of delivery. Moreover, Dr. Little realized that the deformities in premature infants (i.e. born before the 37th week of pregnancy), who mainly showed lower-limb deficiencies, were different from those born at term, who showed deficiencies at all four extremities. Dr Little did not limit himself to classifying these small patients, but he also worked to alleviate their suffering by trying to surgically sever the tendons of the most contracted muscles, to improve the deformities that afflicted these children. The major difficulties he encountered in his work, however, mainly concerned one aspect, namely the distinction of these patients from those, until then much more common, affected by poliomyelitis(in fact the anti-polio, which today protects all children vaccinated against this terrible disease, still did not exist in those years). Moreover, as almost always happens to those who are pioneers in science, at first he was opposed, but over time other studies confirmed the hypotheses of Dr. Little who, as often happens in medicine, gave the name to what today is known as spastic diplegia (“Little’s disease”). However, only a century later, in 1962 it was discovered the brain injury that caused spastic diplegia, or “periventricular leukomalacia”, whose incidence will be significantly reduced from decade to decade, thanks to the progress of treatments and the increasingly specific activities of the Neonatal Intensive Care Units. Today, thanks to the sophisticated diagnostic imaging techniques, which are more and more precise and clear, we are able to associate a type of clinical manifestation with a single specific lesion, such as spastic tetraparesis as the result of perinatal asphyxia, hemiplegia as a result of a perinatal arterial stroke, spastic diplegia as a result of periventricular leukomalacia; for obvious technical reasons, Dr. Little could not distinguish the lesions that afflict his patients in a very precise way, so it was natural that the term “Infantile Cerebral Palsy” became a label affixed to all those children showing neurological pictures that were characterized by the involvement of the central nervous system, with apparently progressive and precociously arising motor difficulties.
In those years, the scientific debate on Neurology and on what would later become Neuroscience, was very prosperous: even a rather famous man wanted to expressed an opinion on this problem: his name was Sigmund Freud. In fact, trying to study the tissue damage being present in the brain and related to precise neurological manifestations, he was the first to realize that the definition of ICP given by Little was incomplete. In 1957, a club named the “Little Club” was founded, composed of scientists who wanted to situate this problem with greater precision. It is precisely in this context that Bax will publish the definition we have read in the first lines. 

Is that all here?

Of course not. Over the years, many scientists have also debunked on the effectiveness of the definition introduced by Bax. For this reason, in 2004 in Bethesda (USA), during a workshop, the definition that is nowadays referenced and which recites: “Infantile Cerebral Paralysis is a group of permanent disorders of movement and posture that causes a limitation of motor function, attributed to non-progressive brain damage incurred during fetal or neonatal life. Motor disorder is sometimes accompanied by other disorders, such as sensitivity, cognitive and communication disorders, epilepsy and secondary musculoskeletal problems “. What is important to stress in all these definitions is that non-progressivity refers to the mechanism of damage: the cause of damage is not progressive, while the manifestations and disturbances that this can cause must be related to the growth and development of a complex system like the brain and the body of a growing baby. In fact, a brain damage to a structure does not only imply the malfunction of that specific point of the brain, but also a future impossibility of connecting that area with others, links that can develop at a functional level during the growth. The series of disorders that may affect these patients is very varied and sometimes complex.
According to the latter definition, therefore, ICP is configured as a set of “developmental” disorders that primarily affect motor functions.

Who is affected by Infantile Cerebral Palsy?

As per definition, this pathology affects the child’s age, and it results from different injuries in the brain. Distinguishing the large neonatal population in children born at the end  of pregnancy (i.e. after the 37th week of pregnancy) and those born before the end of pregnancy, we can say that the causes of damage in the first group are more often prenatal (genetic, infectious, malformative) and less often perinatal (before, during and shortly after birth), while in the second group, especially in extremely premature babies, perinatal factors and problems predominantly arise after birth but they depend on the immaturity of the central nervous system, vulnerable to many injuries and easily prone to develop complications, such as hemorrhages (in the cerebral ventricles, in the cerebellum), ischemia (venous infarction), infections and inflammation in many areas of the brain. 0Questa patologia colpisce, come da definizione, l’età infantile, ed è il risultato di lesioni di diverso tipo a livello cerebrale. Distinguendo la grande popolazione neonatale in bambini nati a termine di gravidanza (cioè dopo la 37° settimana di gravidanza) e quelli nati prima del termine della gravidanza, possiamo dire che le cause di danno nel primo gruppo sono più spesso prenatali (fattori genetici, infettivi, malformativi) e meno spesso perinatali (prima, durante e poco dopo la nascita), mentre nel secondo gruppo, soprattutto nei bimbi estremamente prematuri, agiscono fattori prevalentemente perinatali e problemi che insorgono dopo la nascita ma che dipendono dalla immaturità del sistema nervoso centrale, vulnerabile a tanti insulti e facilmente incline a sviluppare complicanze quali emorragie (nei ventricoli cerebrali, nel cervelletto), ischemie (infarti venosi), infezioni ed infiammazioni in molte aree dell’encefalo.

How frequent is it?

PCI is the most frequent cause of motor deficits in the pediatric age and affects approximately 2-3 children per 1,000 with different levels of severity and various problems.

How does it present itself and what does it involve in the newborn’s life?

As we have mentioned, the presentation and the symptomatology of this group of disorders is varied. There are three prevalent forms that can be distinguished, but very often they can overlap each other:
spastic syndromes: hemiplegic, diplegic and tetraplegic forms;
dyskinetic syndromes;
ataxic syndromes.

Spastic Syndromes These syndromes are characterized by rigidity and plegia (abolition of the voluntary movement). They are defined “hemi-plegias” if they involve lower and upper limb of the same side, “di-plegias” if they affect both the lower limbs (more rarely the upper ones) and “tetra -plegias” if they affect all 4 limbs.
The disorders usually occur a few months after birth: one notices that the child uses an arm less frequently than the other one, or has a strange motor attitude (for example, in the first attempts at prehension). The involvement of the lower limb is detected later. naturally there are also clinical pictures characterized by not complete loss of movement, instead by a reduced or lower fluidity: everything depends on the severity of the primary neurological damage and on the possibility of a rehabilitation intervention.

These syndromes are characterized by rigidity and plegia (abolition of the voluntary movement). They are defined “hemi-plegias” if they involve lower and upper limb of the same side, “di-plegias” if they affect both the lower limbs (more rarely the upper ones) and “tetra -plegias” if they affect all 4 limbs.
The disorders usually occur a few months after birth: one notices that the child uses an arm less frequently than the other one, or has a strange motor attitude (for example, in the first attempts at prehension). The involvement of the lower limb is detected later. naturally there are also clinical pictures characterized by not complete loss of movement, instead by a reduced or lower fluidity: everything depends on the severity of the primary neurological damage and on the possibility of a rehabilitation intervention.

Diskinetic syndromes
These children are unable to regulate muscle tone, organize and perform voluntary movements and maintain a posture. They may be subjected to involuntary movements carried out without any purpose, which prevent the child from carrying out a clean and finalistic voluntary movement. This syndrome is mainly due to the injury of the so-called “basal nuclei”. Intelligence is not compromised, but a severe motor involvement can limit their ability to adapt. For example, writing is difficult to be acquired by these children.

Ataxic Sindromes
This event happens much rarer than in the past and affects children with primary injuries of the cerebellum. The term “ataxia” derives from the Greek “taxia” which means “to order”, preceded by “a” which means “without”, thus signifying “disordered movement”, which in practice means incapacity in coordination and maintaining equilibrium, and carrying out imprecise movements.

How to treat it?

The treatment of this pathology is a multidisciplinary rehabilitation treatment because the initial damage, caused by various kinds of injuries, is unfortunately not repairable. Rehabilitation uses figures, such as the physiotherapist, the neurodevelopmental disorders therapist and the speech therapist who, led by the child neuropsychiatrist, will follow the child from the first months of life, to ensure a guided development with the aim of achieving the maximum functional capacity in every child. Il trattamento di questa patologia è un trattamento multidisciplinare riabilitativo, perché il danno iniziale, provocato da insulti di vario tipo, non è purtroppo riparabile. La riabilitazione si avvale di figure quali il fisioterapista, il neuropsicomotricista, il logopedista, che guidati dal neuropsichiatra infantile, seguiranno il bambino fin dai primi mesi di vita, per far in modo che lo sviluppo sia guidato, con l’obiettivo di raggiungere la massima capacità funzionale in ogni bambino.

Ilaria Giordano