1.1

Clinical case

In 2009, at the L’Adapt – Rhône neuropsychological reeducation and social support center, we made the acquaintance of a 20-year-old woman (born on 09/30/1988) who had suffered severe craniofacial trauma at the age of 12 (on 04/07/2001). Her initial Glasgow score was 6 and she was in a coma for 57 days. On 07/12/2001, the patient underwent external ventricular derivation (EVD). On 07/20/2001, she underwent meningeal repair at the base of the skull and obstruction of the left orbital and nasion fractures. Derivation ablation occurred on 08/14/2001 and she was admitted to the reeducation center on 08/27/2001. In 2002, 18 months subsequent to the trauma, the deficiencies in the right-handed girl were summarized as follows: Satisfactory recovery from left-sided hemiparesis, mnesic troubles with approximately six months of post-traumatic amnesia, deficitary anterograde verbal and visual memory, executive function disorders, frontal dysfunction and blindness in the left eye. Numerous examinations carried out in a specialized medical and educational establishment led, in 2008, to a diagnosis of “ difficulties as regards inhibition (large-scale perseverations), verbal working memory and flexibility; temporal disorders” . MRI tests objectified major and diffuse sequels: bifrontal contusion with thin corpus callosum, left frontal porencephaly and ventricular dilatation. The patient had earned a French CAP diploma as all-around food service employee, but her internship failed on account of the above-mentioned perseverations and behavioral troubles. She was characterized as a “liar” and said to “grandstand”. During weekly observation periods at the center, the young woman regularly presented with frontal disorders such as impulsiveness and logorrhea; little by little, members of her assistance team began to report spontaneous confabulations. She could spontaneously recount, with no difference in terms of either context or emotion, two memories such as those presented below. Upon verification, only the first memory was found, one word excepted, to be authentic ! Her father, with whom she lived, pointed out that she “ so constantly wished to say something that it was necessary to verify what she actually did ”. For example, she had explained to him the stress entailed by her having to prepare a presentation of Venice and initiate a task of which nobody had ever spoken.

1.2

Neurophysiological examination results

The patient underwent a classical neuropsychological examination ( Table 1 ).

The main points are as follows. The intellectual quotient (IQ) was normal. The patient was slow in taking all the tests except for the Rey-Osterrieth Complex Figure (ROCF) copying exercise and the errand test, which she carried out hurriedly, in less than three out of the 15 minutes allotted. Her haste led to poor performance in a test meant to assess subjects as ecologically as possible. Moreover and even though she is fully used to cooking at home, she encountered particularly pronounced difficulties with the “chocolate cake” script . There were numerous errors of all kinds: addition, inversion, commentaries, substitution and omission. On the other hand, she had no problems with the classic executive tests other than her slowness in taking the fluency, Stroop and TMT tests; as for the Wisconsin Card Sorting Test (WCST), she rather astonishingly failed to persevere. Reading Span test results were poor. As for the Paced Auditory Serial Addition Test (PASAT), a demanding assessment of working memory in which series of figures are not only repeated, but also tallied, it had to be interrupted. Major difficulties were likewise observed with regard to the D2 timed test of selective attention, which consists in marking every single “d” with a dash in two adjacent lines. The particularly high number of omissions may be explained by inadequate working memory. In fact, the simple action of turning over a leaf of paper when shifting from one part of a test to the next caused her to forget the instructions. As for memory itself, she had trouble with tests of which the encoding cannot be controlled; on the other hand, her performances in recognition and serial-order recall improved. It should be noted that she did not produce intrusion or confabulations and that false recognition remained within bounds. More informally, she was asked to tell three out of a possible six fairy tales and she chose Little Red Riding Hood , Three Little Pigs and Cinderella . Her narratives were rich, full of details, tinged with humor and devoid of confabulation.

As regards biographical memory, given the patient’s youth and the fact that the trauma occurred when she was under 12 years of age, we decided to favor autobiographical fluency tests and an individualized questionnaire. Over the course of several sessions, she was able to reconstruct her family tree over three generations (24 individuals) in the correct chronological order and she could provide some information on each person. She accurately gave the names of those close to her and talked about personal events or milestones; in each case, she indicated the contents, the place, the time and the emotional climate. More precisely, as regards each event, she managed to specify the degree of periodicity in accordance with the Know/Remember/Guess (K/R/G) procedure and from the standpoint of actor and spectator alike ( Table 2 ). With her agreement and that of her father, we were able to assure ourselves that the information she had provided indeed corresponded to reality. As for the 20 confabulations produced spontaneously during six months of weekly meetings of which the contents were monitored by her family or outside professionals, we noted that on each occasion, the patient provided information about the details, the place, the time and the emotion connected to the episode, as was the case when she produced a true or authentic memory. Since the K/R/G procedure could not be used throughout the sessions (at times the patient deemed it too difficult and declined to apply it), we asked her to approach the memory from the standpoint of either actor or spectator. None of the confabulations generated the “actor” reply and the falsifications of the 20 true memories that in 70% of the cases generated a specific type of response (memory 1: actor answer; memory 2: spectator answer) were clearly distinguished.

Lastly, the patient was asked to provide in writing 20 pieces of information pertaining to the self : I am (as in: I am my brother’s sister, interested in food, in fine shape, in blue jeans, too fat …), I have, I do. Each list was drawn up in less than three minutes! The responses were fitting and in line with her personality.

1.3

Discussion

The cognitive status of confabulating subjects remains quite variable. In the literature, the role played by the dysexecutive syndrome has been underlined repeatedly; confabulations are likely to be associated with imitation behaviors and perseverations , even though in some instances, this is not the case . That said, such exceptions are dated, the tests assessing frontal dysfunction are few and far between and the confabulations reported were essentially provoked. “Existence of a memory deficit is an important factor explaining the presence [the absence] of confabulation, but it is the gravity of the executive deficit that determines the gravity of the confabulation”: such is the cautious conclusion of Fisher et al . ( 1995) .

The patient whose history has been reported presents a classic clinical table of spontaneous confabulations, which are rarely found in the literature. One of the peculiarities of her case consists in its occurrence following severe brain injury during childhood, at a time when the self is still being developed. It is worth underlining how difficult it is to identify confabulations, especially when they do not entail phantasmatic elements . In this case, the family believed that the young woman so often “made things up” that what she said had to be verified and that if she did so, it was on account of her “quest for identity and need to exist”. And notwithstanding numerous assessments over the years along with follow-up at the reeducation center, her verbal emissions seem to have been subsumed in a frontal behavioral inventory associating perseverations and impulsiveness at an age where it is supposed that children frequently lie, especially when they are suffering. It was impossible for us to determine whether the above symptoms arose immediately following the trauma or as time elapsed. In the literature, possible connections between this type of symptomatology and the onset of confabulations have been pointed out, but a cause and effect relation does not necessarily exist. In our patient, the frontal behavioral inventory may have occulted confabulations to the point where they were erroneously considered to be lies, inventions and fantasies .

The brain damage is massive and the orbitofrontal region is among those involved. In this particular case, it is impossible to make any further affirmation and to draw even the slightest anatomoclinical conclusion.

While the patient was amnesiac, we noted that in the tests, even when asked to recount fairy tales, she did not commit intrusion errors, fantasize or engage in false recognition . This finding goes against a psychopathological hypothesis. The young woman showed no signs of suggestibility (Pick, 1905) . When she failed to perform, she did not set up defense mechanisms by giving false answers (Weinstein and Kahn, 1955) , nor did she resort to gap filling (Barbizet, 1963) . When mentioning events or proffering autobiographical information, the patient did not fantasize and was competent. Our finding is congruent with the data reported by Burgess and Shallice (1986) , who studied a highly particular perturbation of autobiographical memory. As for Conway (2005) , this author postulated that in autobiographical memory, the long-term self constitutes a personal knowledge structure allowing us to organize the memories we hold of our personal experiences. The conceptual self consequently brings together the attitudes, values and beliefs guaranteeing its own integrity and continuity. The young woman indeed appreciated the constitutive elements of her “self” when having to say I am, I have, I do . As for her inventions, which occur in a context of insufficient long-term memory, they do not appear to have literally filled a biographical gap or put an end to some identity disorder; moreover, they take place in a state of “clear consciousness” .

The confabulations arise hurriedly and without aforethought, indeed, the patient has had no time to think and it is possible to conclude that she can’t control herself (Stuss, 1978) or correct herself (Shapiro, 1981) . One may add that while the patient showed slow reaction time, she had no difficulty with the flexibility and the immediate attention tests. On the other hand, she had great difficulty taking the D2 timed test of selective attention, in which she was penalized for her most pronounced executive disorder, namely impulsiveness, whereas in spite of her slow reaction time, she adequately carried out the Stroop, TMT and Wisconsin tests. Moreover, impulsiveness led the patient to perform the errands test “too quickly” and the simple, scripted “chocolate cake” test was failed, as she made undue haste or had her attention captured by a multitude of stimulations.

One possible key component of confabulation reported long ago is the temporal factor (Van der Horst, 1932) . In our case, the patient does not present temporal disorientation, chronological incoherence in her biographical statements, or trouble pertaining to the conceptual “self”. She nonetheless associates slowness and hurriedness. Her working memory is clearly insufficient. Even though she successfully carried out the TEA attention assessment and Wisconsin tests, she failed to complete the PASAT. Her peculiar inability to deter the generation of fictions may reflect a problem of control as regards short-term memory or “working self”. The trouble arises in the process of reconstructing autobiographical memories and it encompasses a number of control processes oriented in terms of the subject’s goals, desires and beliefs . Direct access to memory is a rare occurrence and it is widely recognized that many purportedly direct reconstructions are largely illusory and essentially serves to safeguard the coherence of the “self” (Guyard & Piolino, 2006) . As regards our patient, whose confabulations are plausible and in no way fantasies, perhaps her plausibility control processes have lost their effectiveness, or perhaps she did not have — or did not take — the time to put them to work. And it is not impossible, especially when referring to authors of past studies that the confabulations compensate for a lack in working self and thereby ensure a form of psychic continuity that was gravely jeopardized by the fact that the brain injury took place at an early stage of the patient’s life.

Recognition of the existence of confabulations has entailed three major clinical repercussions. Firstly, the neurological symptom could be convincingly explained to the patient and her family and the latter would no longer experience guilt over having considered the former as “a liar”. Secondly, the finding facilitated the work of the expert as he elaborated his report on the sequels of the accident, which obviously could not be denied. Even though the patient’s cognitive capacities had to an appreciable extent been conserved and in spite of her having earned the French CAP diploma, the behavioral aspect of a severe dysexecutive syndrome remained present and negatively affected her family and social life. Lastly, thought was given to the means of reining in the confabulations, of which the patient recognized the peculiar nature, without aspiring to put an end to them. She understood that as a step in that direction, limiting her impulsiveness would be necessary but not altogether sufficient. It was consequently suggested to her that she waited before speaking and that she refrained from speaking if the memory was something known from a spectator’s standpoint rather than a way to relive something she remembered as an actor. At present, our data do not allow us to reach a conclusion.

Disclosure of interest

The authors declare that they have no conflicts of interest concerning this article.

2.1

Cas clinique

Nous avons rencontré une jeune femme de 20 ans (née le 30 septembre 1988) qui a été victime d’un traumatisme crânio-facial sévère, le 7 avril 2001 à l’âge de 12 ans, dans le Centre spécialisé de rééducation neuropsychologique et d’accompagnement social de L’Adapt-Rhône en 2009. Le score de Glasgow initial était à 6 et le coma dura 57 jours. La patiente subit une dérivation ventriculaire externe le 12 juillet 2001, une réparation méningée de la base et l’obstruction de fractures orbitaires gauches et du nasion le 20 juillet 2001 puis une ablation de la dérivation le 14 août 2001 et fut admis dans un Centre de rééducation le 27 août 2001. Il fut noté en 2002, à 18 mois du traumatisme, en termes de déficiences, chez une enfant droitière une hémiparésie gauche ayant bien récupéré, des troubles mnésiques avec une amnésie posttraumatique de six mois environ, une mémoire antérograde déficitaire sur le plan verbal et visuel, des troubles des fonctions exécutives, un dysfonctionnement frontal, une cécité de l’œil gauche. Les nombreux bilans réalisés dans une institution spécialisée médicoéducative concluaient en 2008 à « des difficultés d’inhibition (persévérations importantes), de mémoire de travail verbale et de flexibilité et des désordres temporels ». L’IRM objectivait des séquelles majeures et diffuses : contusion bifrontale avec aspect aminci du corps calleux, cavité porencéphalique frontale gauche et dilatation ventriculaire. Elle avait réussi un CAP d’agent polyvalent de restauration, mais elle était en échec en stage en raison de persévérations et de troubles de comportement. Elle était qualifiée de « menteuse » et il était rapporté qu’elle « faisait l’intéressante ». La jeune femme se présentait au quotidien, lorsqu’elle fut observée de façon hebdomadaire, dans le centre des éléments frontaux de type impulsivité et logorrhée et peu à peu l’équipe fut conduite à rapporter des confabulations spontanées. Elle pouvait raconter spontanément sans aucune différence de contexte ou d’émotion deux souvenirs tels que ceux présentés dans l’encadré. Seul le premier souvenir (au mot près) s’avérait après vérification, être authentique ! Vivant avec son père, celui-ci témoignait qu’elle « voulait toujours répondre quelque chose au point qu’il fallait vérifier ce qu’elle entreprenait ». Elle avait pu ainsi lui expliquer le stress que représentait un exposé sur Venise qu’elle devait préparer et démarrer un travail dont nul n’avait jamais parlé.

Souvenir 1

Hier, j’ai été au concert de Mylène Farmer avec mon père et ma sœur… On était tout devant. Mon père, il avait acheté les places les plus chères. Elle est super ! Elle ne fait pas son âge, elle fait encore le grand écart… et elle est hyper sexy. Sur la scène, il y avait ses soutiens gorge et j’ai vu sa culotte jaune, pas chouette…

Souvenir 2

Hier soir, mon petit copain, il est venu dîner, il est chiant, il est comme tous les mecs, tu lui donnes cela et il veut cela. Cela m’a énervée, j’avais fait un super gueuleton, des pâtes avec une super sauce. On s’est engueulé : vous savez comme cela fait avec moi et bien il s’est cassé et j’ai tout bouffé, cela va pas m’arranger !

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