1.2.1

At the age of 3

The child was described as uncooperative and extremely agitated. She could not maintain her attention on a task for more than 30 seconds. She refused most of the games suggested to her, did not understand the instructions and threw the objects on the floor or banged them together or on the table. There was very significant utilization behaviour and an absence of spontaneous, constructive activity: BS liked to move around freely and indiscriminately grabbed anything within her reach (shoes, toys, furnishings, etc.), put them in her mouth and bit them, which required permanent surveillance. She was easily frustrated, screamed for, sometimes, long periods of time and threw objects. Her behaviour made the rehabilitation sessions difficult. She enjoyed contact with adults and often asked for a hug, which had a calming effect. In contrast, she had adversarial relationships with other children: she destroyed whatever they had built, intruded into their games and pulled objects out of their hands. She performed a number of aggressive gestures, such as hitting and biting. The severity of her behavioural disorders (irascibility, language replaced by strident and incessant screams, etc.), worsening attention disorders, agitation and night- and day-time sleep disorders) prompted the initiation of levomepromazine treatment. On the cognitive level, language was neither fluent nor informative, since it was limited to a few words (such as “again”, “dada” and “mama”) with phonological deformations. To convey the fact that she wanted an object, BS spontaneously pointed her finger at it while saying “that” or a word related to the desired activity . The understanding of simple commands was very variable, even in context: BS could indicate some parts of her body but the naming of objects or images was impossible. In Brunet–Lézine’s psychomotor development test, BS accomplished all of the 10-month items but none of the 30-month items. The development quotient (DQ) was 48.

On the neuromotor level, there was persistent right spastic hemiplegia mainly affecting the arm, which was not used – not even for support. The child was not toilet-trained.

In functional terms, BS could walk two or three steps on her own. She was dependent for all activities of daily living. She was capable of starting to eat unassisted but, after a few mouthfuls, stopped and threw her plate onto the floor. At home, she would often wake up during the night and would have trouble going back to sleep. The child was not able to attend school – not even part-time at the hospital’s special unit. The results of successive Brunet–Lézine tests evidenced a progressive developmental shortfall in the child’s abilities ( Figs. 1 and 2 ).

Change over time in performance in the Brunet-Lézine psychomotor development test. The overall development quotient (DQ) was evaluated three times between the ages of 15 months and 3 years; the results illustrate the extent of the cognitive damage and the progressive worsening in performance levels, compared with the expected ability as the child aged.

Change over time of the various subscores in the Brunet-Lézine psychomotor development test. The development quotients (DQs) in the domains of “postural behaviour”, “eye-hand coordination”, “verbal behaviour” and “social behaviour and independence” were evaluated three times between the ages of 15 months and 3 years. The results were subnormal in all cases and tended to worsen over time. The worsening was most severe for verbal behaviour, for which damage only becomes apparent when this ability is supposed to be functional.

1.2.2

At the age of 6 years and 8 months

Treatment with valproic acid and levomepromazine was ongoing. In behavioural terms, BS was calmer but still had sudden and violent gestures. She was not spontaneously interested by any type of activity and remained extremely slow, easily distracted and easily tired. Stereotypy and utilization behaviour were noted. The child continued to put objects into her mouth indiscriminately. Her degree of agitation required continuous surveillance and made impossible to perform a magnetic resonance imaging (MRI) scan other than under general anaesthesia.

In cognitive terms, the child’s performance level was below that of a 3-year-old, especially regarding orientation: BS could not say whether she was a girl or a boy and did not know how old she was. She could not explain where her home was situated and was unable to tell what point in the day it was. She did not distinguish right from left. Her language was very limited and consisted solely in associating up to three (many unintelligible) words. There were major comprehension disorders. She could obey a few isolated, simple commands but could not follow two simultaneous, simple instructions. She was not able to indicate cubes according to their colour, although she was able to sort them by colour. She could not count objects beyond two. BS could only copy horizontal lines and produce a few doodles. She could not complete a simple jigsaw puzzle and could only fit a few basic shapes together by trial and error. She was not able to imitate the construction of three cubes (a 3-year-old level activity). In the teddy bear cancellation test , BS made eight omissions out of 15, instead of the single omission usually expected at the age of 6. Her memory could not be tested.

In BS’s first attempt at the the Wechsler Preschool and Primary Scale of Intelligence test (WPPSI-III) , she scored the lowest possible score of 1 for all items. The overall intelligence quotient (IQ) was 40, the verbal IQ was 44 and the performance IQ was 45.

On the neuromotor level, the motor symptoms had not changed but right dorsal scoliosis had appeared. In functional terms, BS was able to walk unaided but standing on one foot, jumping, crouching and running remained impossible. She was not independent with regard to even the simplest activities of daily living. She could only eat with a spoon. In the toilet, she had to be undressed, wiped and dressed again. Outdoors, it was necessary to hold her hand constantly and in shops, she had to be restrained by being placed in the shopping trolley.

1.2.3

Future care

In terms of future care, successive attempts to refer her to a nursery (even a specialist one) and then a primary school ended in failure (despite the best efforts and diligence of the host organisations), in view of BS’s behavioural disorders. The child was also refused by several care homes before finally being admitted (at the age of five) to a highly specialized educational unit.

1.2.4

Legal aspects

T he Neurosurgery Department had filed a legal report for SBS in view of the observed subdural haematoma and retinal haemorrhages having occurred at different time points. Following the initiation of proceedings by the public prosecutor, the examining magistrate charged the suspected perpetrator with “voluntary violence to an under-16 by a person holding authority and having led to total, temporary infirmity for more than a week”. The parents filed as plaintiffs claiming damages and applied to France’s Crime Victim Compensation Commission. The expert medical-legal opinion (given when the child was 2 years old) confirmed that “all the lesions were related to violent shaking, such as that performed in SBS, and no cause other than violent injury could be envisaged”. In the absence of prior anomalies, the development impairments were considered as being directly and unquestionably ascribable to the brain damage. Prior to legal consolidation (in France, the point after which no additional medical treatment can be deemed to improve the handicap and thus at which the state of incapacity is judged to be permanent; in children with severe head injuries, this does not occur before the age of 20), the courts granted compensation for loss of earnings after the mother had given up her job to look after BS and for covering the expenses resulting from the brain damage. The compensation also helped pay for several hours per day of third-party care.

1.3.1

In neurological terms

In neurological terms and in addition to persistent, invalidating, right side spastic hemiplegia (complicated by the subsequent appearance of scoliosis), BS continues to suffer from relatively severe epilepsy. In SBS, a motor deficit such as hemiplegia or quadriplegia appears to be both persistent and frequent (in 60% of cases in the series reported by Barlow et al. ). Epilepsy appears to be much more common after SBS than after accidental head injury and is often severe and drug-resistant .

1.3.2

Major behavioural disorders

The SBS victim presented major behavioural disorders, which constituted the prime factor in the child’s failure to be admitted to specialist care units. These behaviour disorders also constitute a major aspect of the sequelae found in the literature and were noted in 52% of cases by Barlow et al. . The latter researchers also reported on the high frequency of sleep disorders (24%), which often have a major impact on a family’s quality of life. Prasad et al. and Damasio have suggested that orbitofrontal brain damage early in life perturbs behavioural regulatory mechanisms in general and impairs initiative and adaptation to a social context (with difficulty learning social rules) in particular. In our first study and despite the patients’ young age, behaviour disorders in daily life were already being reported by a quarter of the parents.

1.3.3

With regard to the onset of the sequelae

Some of BS’s disorders only became severe and invalidating after a certain length of time. For example, the behavioural disorders started to cause major problems from the age of 3 onwards. This underlines the importance of the sequelae’s time to onset. Two studies have focused on this aspect of the condition. In 1995, Bonnier et al. reported on outcomes in 13 children with a 7-year follow-up (range: 5 to 13 years). A critical notion in this study was the variable time delay in expression of the symptoms: brain growth arrest after 4 months, epilepsy after 2 years and behavioural disorders appearing and causing problems only 3 to 6 years after the trauma. Only one child was still considered as being sequelae-free after 7 years of monitoring. To the best of our knowledge, this study was the first to emphasize the potential mid- and long-term appearance of more subtle disorders than those immediately seen in the shaken babies. Secondly, Barlow et al. studied outcomes in a series of 25 SBS victim. The initial population consisted of 55 children (six of whom died). With a mean monitoring period of 5 years and with almost half the subjects lost to follow-up, 68% of the survivors presented very diverse sequelae. Thirty-six percent had a severe disorder and were totally dependent. Motor deficits were found in 60% of cases, with visual disorders in 48% of cases, epilepsy in 20% of cases, language disorders in 64% of cases and behavioural disorders in 52% of cases.

Overall, as illustrated by our case report, the few long-term SBS follow-up studies generally describe a poor long-term prognosis in 30% of the apparently unaffected children, given that this figure falls over time due to learning problems and the appearance of more demanding environmental requirements, notably at the usual age of school entry. This unfavourable prognosis contrasts with what is still too often erroneously presented as the “Kennard Principle” . This principle was established following work in 1936 by Margaret Kennard who, in order to study motricity in the monkey, created lesions in the motor and pre-motor areas in animals of different ages. She noted that young monkeys recovered more fully and more rapidly than adult monkeys. Subsequently, other authors have extrapolated from the monkey to humans and from pre-motor lesions to all types of lesions – giving rise to a “Kennard Principle” that was not at all formulated by Margaret Kennard! Independently of the experimental work, many rigorous, prospective, controlled studies have since shown in a reproducible manner that this “Kennard Principle” does not hold in cases of widespread lesions (particularly those with frontal predominance and particularly in the young child). In fact, the cognitive sequelae are more severe in the child than in the adult, since the damage affects vulnerable, rapidly developing functions and thus learning abilities. Moreover, the earlier the damage occurs, the more severe the sequelae, since age is one of the major prognostic factors in head injury in the child .

Nevertheless, these false beliefs remain well anchored in the minds of physicians and lawyers: in 1996, Webb et al. set out to study the impact of the “Kennard Principle” on physicians and paramedical professionals. He asked them to comment on the extent of recovery in four fictitious case studies of brain-damaged adults and children in which only the patient’s age differed. In all cases and regardless of their medical speciality or the time since their medical training, the medical professionals predicted a better prognosis when the patient was under 10. Webb et al. concluded that the “Kennard Principle” was still strongly anchored in people’s minds. Likewise, in 2003, Johnson et al. questioned magistrates on the value of expert medical-legal opinions expertises according to whether or not they announced a better prognosis in the child. He found the same result; all the magistrates wrongly thought that brain damage suffered earlier in life had a better prognosis. It is thus important to remain cautious when announcing a prognosis following brain damage in the child – above all when the damage is widespread, as in SBS. In addition to the immediately apparent problems, subtle but nevertheless damaging disorders (such as attention, concentration and memory disorders) may subsequently appear. Moreover, these disorders are exacerbated by the growing demands placed on the child as he/she grows up. It is not possible to predict the outcome with certainty. Extremely long follow-up by a multidisciplinary team is essential and must enable care provision to change as function of the child’s altering condition. The child’s learning ability (and notably cognitive status) must be assessed on a regular basis, in order to determine the time course of these changes.

1.3.4

Better knowledge of the severity of the prognosis in SBS should help reinforce preventive measures

This can be very effective when they are performed systematically with parents in the maternity ward immediately after the child’s birth . Understanding the harmful effect of shaking helps the adult to avoid committing the act and to find a solution other than shaking to stop the baby from crying. Furthermore, reducing the consequences of shaking involves very proactive therapeutic management in the intensive care unit. Lastly, it is important to prevent recurrence. Indeed, one key point is to have diagnosed SBS at the outset . This diagnosis can be broken down into four steps :observation of the existence of intracranial lesions, which must be considered even when confronted with symptoms that are not specific for neurological damage, such as vomiting;a diagnosis of shaking;determination of the shaking’s context;identification of the perpetrator.

The physician is solely responsible for the first two steps. A diagnosis of shaking is necessary and sufficient for a diagnosis of SBS and must involve the application of a rigorous approach. If shaking cannot be unambiguously diagnosed, a diagnosis of SBS must not be made.

Lastly, in order to protect the child and aid the adult, the diagnosis must be reported . In France, the confidentiality of the physician–patient relationship no longer holds in cases of abuse and cruelty to under-16s. In such an event, France’s code of professional medical conduct obliges the physician “to notify the legal, medical or administrative authorities” (which do not include the local protection maternelle et infantile midwifery and social services) “except under particular circumstances which he/she shall judge in all conscience”. What, then, might justify not reporting such an incident? The eventuality that the exasperated adult only shook the child once, perhaps? But would the physician have the adopted same attitude with an adult or elderly victim? Fear of an overly severe response from the police or judicial system? But is this risk serious enough to prevent reporting? Fear that the adult might be imprisoned? In fact, the penal response to such an incident features a wide range of potential solutions other than imprisonment, including the obligation to seek medical or psychiatric treatment. Fear of worsening the relationship with the family or those within the family? But surely shaking is more harmful for a family than a report of shaking?

1.3.5

Notifying an incident to the legal authorities also enables initiation of the compensation process

This is fundamental in the event of subsequent sequelae (which are impossible to accurately forecast in the initial phase). In France, whatever the context and the perpetrator, shaking constitutes an offence (i.e. a voluntarily or involuntarily harmful act committed by a third person) and the victim becomes eligible for compensation. This can be obtained as damages before a penal or civil court or by petitioning the Crime Victim Compensation Commission until up to 3 years after the victim comes of age (i.e. at the age of 21, in France), even if the perpetrator has not been identified. Nevertheless, a causal relationship with the offense constituted by the shaking must have been confirmed in a medical-legal appraisal. The lack of acknowledgement of the offense deprives the child of the right to any subsequent compensation – compensation that is a major factor in reducing handicap because it enables the payment of ongoing costs, such as medical care that is not reimbursed by the health insurance system (occupational therapy, psychomotricity, etc.) or the cost of third-party care. This attention must be provided as soon as the child’s need for care becomes related to its brain damage rather than its age and must not be skimped on when the parents themselves assume this role.

At the time of legal consolidation of the injuries (i.e. not before the age of 20, in France ), this procedure also enables payment of costs in adult life and particularly third-party care if the individual is not independent.

If one of the parents is potentially the perpetrator of the shaking, this situation may lead to conflict between the child’s interests and those of the parent(s); it is then necessary to request the designation of a legal professional as an ad hoc administrator for defending the child.

In conclusion, this detailed clinical case report illustrates the immediate and delayed consequences of shaking-induced brain damage in a shaken baby and underlines the importance of long-term follow-up. It also illustrates the value of diagnosing and reporting the shaking in order to protect the child and maintain its rights in general and its right to compensation in particular.

2.2.1

À l’âge de trois ans

L’enfant était décrite comme peu coopérante, extrêmement agitée. Elle ne pouvait maintenir son attention sur une tâche plus de 30 secondes. Elle refusait la plupart des jeux proposés, ne comprenait pas les consignes, jetait les objets par terre ou les tapait les uns sur les autres ou sur la table. Il existait un comportement d’utilisation très important sans aucune activité construite spontanée : BS aimait se déplacer librement et attrapait sans discernement tout ce qui se trouvait sur son passage (chaussures, jeux, meubles) pour le porter à la bouche et le mordre, ce qui nécessitait une surveillance permanente. Elle était intolérante à la frustration, poussant des cris stridents parfois ininterrompus et jetant les objets autour d’elle. Son comportement rendait les séances de rééducation difficiles. Elle appréciait le contact de l’adulte et réclamait d’être prise dans les bras, ce qui la calmait. En revanche, elle avait des relations conflictuelles avec les autres enfants, car elle détruisait leurs constructions, était intrusive dans leurs jeux et leur arrachait les objets des mains. Elle avait des gestes agressifs comme taper, mordre. L’importance des troubles du comportement (irascibilité, langage remplacé par des cris stridents et incessants, accroissement des troubles attentionnels, de l’agitation, troubles du sommeil nocturne et diurne) avait conduit à instaurer un traitement par levomépromazine. Sur le plan cognitif, le langage était non fluent, non informatif, limité à quelques mots comme « encore, papa, maman », avec des déformations phonologiques. La dénomination d’objets ou d’images était totalement impossible. Pour faire comprendre son désir d’un objet, elle recourrait spontanément au pointage du doigt associé au mot « ça » ou à un mot en lien avec l’activité désirée . La compréhension d’ordres simples était très inconstante, même en contexte : BS pouvait désigner sur elle-même certaines parties du corps mais la désignation d’objets ou d’images était impossible. À l’examen du développement psychomoteur de Brunet–Lézine, tous les items de dix mois étaient réussis et aucun item de 30 mois. Le quotient de développement (QD) était de 48.

Sur le plan neuromoteur, il persistait une hémiplégie droite spastique, prédominant au membre supérieur droit. Le membre supérieur n’était pas utilisé, même comme membre d’appoint. L’enfant n’avait pas acquis la propreté.

Sur le plan fonctionnel, l’enfant pouvait faire deux ou trois pas seule. Elle était dépendante pour toutes les activités de la vie quotidienne. Elle pouvait commencer à manger seule, mais après quelques bouchées, s’arrêtait ou jetait son assiette par terre. Au domicile, il lui arrivait encore souvent de se réveiller la nuit, et se rendormait difficilement. L’enfant n’était pas scolarisable, même à l’école adaptée de l’hôpital à temps très partiel. Les résultats aux passations successives de l’examen du développement psychomoteur de Brunet-Lézine objectivaient le décalage progressif des compétences de l’enfant ( Fig. 1 et 2 ).

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