Search

New challenges in rehabilitation for children with neurological diseases directly depend on advances made in medical research and on the quality of the environment. This is relevant to motor function as a whole, to new therapeutic avenues in spasticity, to global approaches in the evaluation of cognitive and learning disabilities, as well as curative perspectives in neuromuscular disease. Networking with the family and other actors in the environmental field is essential to achieve a better social integration. A true collaboration between physicians and pediatricians is necessary to work toward more progress.

Although unilateral spatial neglect is well documented in adults after a cerebral damage, the description in children is rare and no motor neglect has yet been reported. We report four children age range from three to thirteen years old, in patients in a rehabilitation department. A cancellation task "the Teddy bear test" was used to assess spatial neglect in children. The children were presented a page containing line drawings of usual objects, including fifteen Teddy bears. The children were asked to cross out each Teddy bear. These four children omitted five to eleven teddy bears located on the left space, whereas children of the controlled group never omitted more than one Teddy bear. The four children also exhibited neglect in drawings, and during daily life activities. Moreover, one of them had a motor neglect with no more defects of strength and an under-utilization of his left side. Spatial unilateral neglect and motor neglect should be systematically investigated in children after a cerebral damage, and should be considered during rehabilitation.

Purpose: To assess the impact of emergency management on mortality and morbidity of acute rupture of cerebral arteriovenous malformations resulting in deep coma in children, and the factors predicting outcome. Methods: Retrospective chart review of 20 children with a Glasgow Coma Scale ≤ 8 with acute hemorrhagic stroke from a cerebral arteriovenous malformation rupture was conducted. Protocol included: early resuscitation with tracheal intubation and ventilation after induction of anesthesia with sufentanil, and benzodiazepine, and mannitol 20% or hypertonic saline 7.5% infusion for life-threatening brain herniation. Radiological exploration was limited to contrast-enhanced CT scan preceding immediate surgical decompression. Postoperatively, children were deeply sedated and intracranial pressure monitoring allowed titration with osmotherapy, vasopressors, hyperventilation or barbiturate coma to control cerebral perfusion pressure. Analysis used stratification of the type of hemorrhage (supra or infra tentorial), location (intraparenchymal and subarachnoid, intraparenchymal and intraventricular or intraventricular alone) and relationship between presentation, evolution with resuscitation, type of cerebral lesion, and outcome. Results: Patients had a severe initial presentation (median Glasgow Coma Scale five), eight had unilateral and eight bilateral third nerve palsy. Compressive hematoma in supratentorial localisation represented 75% of the cases. Global mortality was 40%. Persistence of mydriasis after resuscitation increased mortality to 75%. Massive intraventricular flooding was associated with increased mortality. Good functional outcome was achieved in survivors. Conclusion: Acute rupture of an AVM can result in rapidly progressing coma. Emergency management with early resuscitation, minimal radiological exploration before rapid surgical decompression results in a mortality rate of 40%, but a good functional outcome can be expected in the survivors.

We report the case of a traumatic Foix-Chavany-Marie syndrome (SFMC) which is the cortico-subcortical type of suprabulbar palsy. A 10-year-old boy was brain injured in a traffic accident in August 1996. He was found comatous (initial GCS = 6) without any focal neurological deficit. The hemodynamic situation was stable even though he presented two wounds of the scalp and a hemoperitoneum that required intensive perfusions. The initial CT scan elicited a frontal fracture, ischemo hemorrhagic lesions of the right frontopolar and anterior temporal cortex. On the second day, he developed on the left side a subdural collection and a extradural hematoma which was surgically withdrawn. The comatous state ended on the ninth day. On examination, The child was awake and alert, able to understand spoken and written language but unable to speak. There was masticatory diplegia: the mouth was half open, the patient was drooling, chewing was impossible. The most striking feature was the automatic voluntary dissociation which might be observed on laughing, crying and yawning. The patient was unable to initiate swallowing but reflex swallowing was preserved once food was placed into the pharynx. The child had a deficit of voluntary control of muscles supplied by nerves V, VI, IX, X, XI. These clinical features are the hallmarks of SFMC. The first case was reported in 1837 by Magnus. The syndrome was described by Foix Chavany et Marie in 1926, and called SFMC by Weller (1993). His literature review of 62 SFMC allowed the differentiation of five clinical types: the classical and most common form associated with cerebrovascular disease, a subacute form caused by central nervous system infections, a developmental form, a reversible form in children with epilepsy and a rare type associated with neurodegenerative disorders. Bilateral opercular lesions was confirmed in 31 of 41 patients who had CT or MRI performed, and by necropsy in 7 of 10 patients. As previously reported, the outcome was poor for this boy who recovered very limited orofacial motor abilities. The medical functional readaptation was long et tedious and took in consideration the fact that the speech disturbance was anarthria and not an aphasic or an apraxic one and the age of onset of this acute acquired syndrome.

Introduction Aucun cas de narcolepsie post-traumatique n’est decrit chez l’enfant. Nous rapportons un cas chez une enfant de 9 ans avec un recul de 5 ans (2201516). Observation En 2001, apres une chute avec choc cervical posterieur suivie de cervicalgies, sans perte de connaissance, se developpa une narcolepsie. On observait 5 a 10 acces d’endormissement diurnes, sans cataplexie, associes a un mauvais sommeil nocturne. Le diagnostic fut authentifie apres 9 mois par un enregistrement du sommeil objectivant une latence moyenne d’endormissement effondre associee a des endormissements en sommeil paradoxal. Le taux d’hypocretine etait effondre. La narcolepsie etait severe comme l’attestait le score de 18 sur 21 a l’echelle de somnolence d’Epworth (anormal si > 10). La somnolence diurne majeure retentit sur l’autonomie et les apprentissages scolaires malgre des capacites intellectuelles preservees. L’adolescente devait etre stimulee anormalement afin d’essayer de limiter les acces de sommeil. Apres 5 ans de recul la narcolepsie etait resistante au traitement par modafinil et methylphenidate. Il existait une desinsertion scolaire et sociale ainsi qu’une prise de poids de plus de 50 kilogrammes. Discussion L’haplotype HLA, DR15, DQB110602, present chez 90 p. 100 des narcolepsies idiopathiques, 47 p. 100 des symptomatiques et 20 p. 100 des controles n’est ni necessaire ni suffisant au declenchement d’une narcolepsie. Sont en faveur d’une cataplexie symptomatique le jeune âge, l’absence de cataplexie avec taux d’hyposecretine effondre, enfin la chronologie des faits. Aucun des 20 cas colliges de narcolepsie symptomatique de l’enfant n’est post-traumatique. Conclusion L’origine traumatique semble averee. La narcolepsie chez l’enfant a des consequences deleteres propres. Elle entrave, par les phases d’endormissement, les apprentissages et l’autonomisation.

Resume Les difficultes comportementales de l'enfant apres traumatisme craniocerebral ne semblent pas avoir de caractere specifique, a l'exception de comportement de desinhibition lors de traumatisme severe. Les troubles sont souvent intriques: lenteur, defaut d'initiative, impulsivite, irritabilite, anosognosie, etc. Les difficultes methodologiques d'appreciation et d'interpretation des troubles du comportement sont majorees par l'existence de dysfonctionnements sociofamiliaux preexistants. Une filiere de soins s'est mise en place en Ile-de-France.