[Foix-Chavany-Marie syndrome in a child caused by a head trauma]. / Syndrome de Foix-Chavany-Marie d'origine traumatique chez l'enfant.

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 On the second day, he developed on the left side a subdural collection and a extradural hematoma which was surgically withdrawn.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.

[Syndrome of delayed maturation of the sucking-swallowing reflex]. / Syndrome de retard de maturation de la succion-déglutition.

BACKGROUND: Neonates with neurological diseases often have difficulty in sucking or swallowing. This report describes such difficulties in a group of infants with normal development, suggesting late maturation of sucking and swallowing. POPULATION AND METHODS: Seven infants (four girls, three boys) had suffered from aspiration since their first day of life (four cases) with severe asphyxia (two cases), late repeated episodes of airway obstruction by accumulated secretions (two cases), and weak isolated sucking and swallowing (one case). The lack of sucking and swallowing or difficulty with them were the main manifestations in these newborns: the face lacked expression and the tongue movements were abnormal with, in two cases, hyper-extension of the neck. A second phase during the first months of life was marked by episodes of bradycardia and/or drowsiness. A third phase during the first years of life was characterized by repeated episodes of respiratory infections, recurrent stridor and accumulation of saliva. These manifestations required prolonged hospitalization (mean: 2.5 months), nasogastric feeding, and in two cases, surgery. The investigations during the acute phases included a cineradiographic study of swallowing, laryngoscopy, monitoring of the distal esophagus pH, a barium swallow, polygraphic recording during sleep and MR imaging of the brain stem. The difficulties in sucking or swallowing disappeared before the age of 6 months (two cases), between 6 and 12 months (one case), between 1 and 5 years (four cases) without any sequelae or speech disorders. CONCLUSION: These transient sucking or swallowing difficulties suggest late maturation of praxis. They required specialized prolonged education after careful, essentially clinical, investigation.

[Neurogenic bladder in children with acute transverse myelopathy]. / Troubles sphinctériens de la myélopathie aiguë transverse de l'enfant. Aspects diagnostiques, pronostiques, rééducatifs.

BACKGROUND: Neurogenic sphincter dysfunction may result from acute transverse myelopathy. The aim of this paper is to study the course of such a dysfunction and to propose management techniques. PATIENTS AND METHODS: The files of 21 children admitted at the mean age of 8 years 5 months (2 to 14 years, 8 months) for acute transverse myelopathy were retrospectively studied. RESULTS: Bladder sphincter dysfunction occurred in the first days of disease in 85% of these patients. Abnormal perception of micturition was one of the most constant and specific symptoms. Anorectal function was also impaired. Complete regressive course was noted in 38% of patients, minor sequellae in 39% and major sequellae beyond 6 months of course in 23%. None upper tract deterioration was noted after 3 years of course. Factors of favorable prognosis were early motor function recovery (especially recommencement of walking before 20 days) and early management of bladder dysfunction (inability to void had better prognosis than urinary incontinence). CONCLUSION: Early systematic bladder drainage in case of inability to void might be essential for improved prognosis.

[Recent progress in pediatric neurological rehabilitation]. / Quoi de neuf en rééducation neurologique infantile?

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.

The association between developmental handicaps and traumatic brain injury during pregnancy: an issue that deserves more systematic evaluation.

AIMS: Trauma during pregnancy is commonly viewed as benign for the foetus when the delivery occurs normally. This study revisits that point of view. METHOD: We included eighteen patients having a neurological handicap with an anamnesis of an accident during pregnancy and a follow-up sufficient to determine a definite outcome. RESULTS: Pregnancy outcome and observed management. Foetal abnormalities were detected in six cases between the first and the thirteenth day after the trauma. Emergency delivery or rapid birth after signs of foetal distress occurred in five cases. One baby died soon after birth. One-third of cases were not submitted to any investigation. VARIOUS NEUROLOGICAL HANDICAPS WERE RECORDED: Congenital microcephaly (three patients), congenital hydrocephalus (three), Infantile cerebral hemiplegy (six), quadriplegy with severe encephalopathy (four), diplegy (one), clumsiness with cerebellar atrophy (one), Moebius syndrome (one), mental retardation with autistic features (two), learning disability (one) auditory agnosia (one). Cerebral imaging showed macroscopic abnormalities in fourteen patients, evoking various pathogenetic hypotheses. CONCLUSION: The association between maternal trauma and foetal brain lesions lacks sufficient investigation in many cases. Prospective studies are needed to clarify both medical and legal issues. Guidelines are proposed for obstetrical and paediatric management after significant maternal trauma.