[A 6-week-old infant with failure to thrive: insidious presentation of group B streptococcal ventriculitis]. / Stagnation pondérale chez un nourrisson de six semaines : présentation insidieuse d'une ventriculite à streptocoque du groupe B.

Failure to thrive is frequently seen in breastfed infants. The most common diagnosis is insufficiency of breast milk in an otherwise healthy child. However, several differential diagnoses need to be considered. Progressive feeding difficulties and failure to thrive can be the first manifestation of group B streptococcal ventriculitis. This rare disease does not present with acute symptoms of sepsis or meningitis but evolves insidiously with no fever. Diagnosis is therefore often delayed and made only when intracranial hypertension develops. Cerebrospinal fluid (CSF) culture confirming the group B streptococcal infection and cerebral imaging are the necessary investigations for diagnosis. To our knowledge, only 10 cases have been previously reported.

Cerebellar lesions: is there a lateralisation effect on memory deficits?

BACKGROUND: Until recently, neurosurgeons eagerly removed cerebellar lesions without consideration of future cognitive impairment that might be caused by the resection. In children, transient cerebellar mutism after resection has lead to a diminished use of midline approaches and vermis transection, as well as reduced retraction of the cerebellar hemispheres. The role of the cerebellum in higher cognitive functions beyond coordination and motor control has recently attracted significant interest in the scientific community, and might change the neurosurgical approach to these lesions. The aim of this study was to investigate the specific effects of cerebellar lesions on memory, and to assess a possible lateralisation effect. METHODS: We studied 16 patients diagnosed with a cerebellar lesion, from January 1997 to April 2005, in the "Centre Hospitalier Universitaire Vaudois (CHUV)", Lausanne, Switzerland. Different neuropsychological tests assessing short term and anterograde memory, verbal and visuo-spatial modalities were performed pre-operatively. RESULTS: Severe memory deficits in at least one modality were identified in a majority (81%) of patients with cerebellar lesions. Only 1 patient (6%) had no memory deficit. In our series lateralisation of the lesion did not lead to a significant difference in verbal or visuo-spatial memory deficits. FINDINGS: These findings are consistent with findings in the literature concerning memory deficits in isolated cerebellar lesions. These can be explained by anatomical pathways. However, the cross-lateralisation theory cannot be demonstrated in our series. The high percentage of patients with a cerebellar lesion who demonstrate memory deficits should lead us to assess memory in all patients with cerebellar lesions.

[Treatment of posterior positional plagiocephaly]. / Traitement des plagiocéphalies postérieures d'origine positionnelle.

In 1992, the American academy of paediatrics has recommended that infants be placed on their backs to sleep, because prone sleeping has been correlated with sudden infant death syndrome. Following this article, medical paediatric community has documented an exponential increase in the diagnosis of posterior cranial deformities, which were considered as the consequence of unrelieved pressure onto the occiput during infant sleep. These last 15 years, management of posterior positional plagiocephaly has evolved but is still not standardized; it varies according to local specificities, and medical or parental preferences. Treatment of deformational plagiocephaly includes preventive counseling, repositioning adjustments and exercises, physiotherapy, osteopathy, treatment by dynamic cranial orthosis. On extremely rare occasions, corrective surgery is proposed. This article aims at reviewing the epidemiologic, diagnostic, and various therapeutic options of posterior positional plagiocephaly.

[Phacomatosis and genetically determined tumors: the transition from childhood to adulthood]. / Phacomatoses et tumeurs génétiquement déterminées: la transition enfant-adulte.

Phacomatoses, or neurocutaneous disorders, are a group of congenital and hereditary diseases characterized by developmental lesions of the neuroectoderm, leading to pathologies affecting the skin and the central nervous system. There is a wide range of pathologies affecting individuals at different moments of life. The genetics is variable: while neurofibromatosis 1 and 2, tuberous sclerosis and von Hippel-Lindau disease are all inherited as autosomal dominant traits, Sturge-Weber syndrome is sporadic. Other neurocutaneous disorders can be inherited as autosomal recessive traits (i.e., ataxia-telangiectasia), X-linked (i.e., incontinentia pigmenti) or explained by mosaicism (i.e., hypomelanosis of Ito, McCune-Albright syndrome). In this review, we discuss the major types of neurocutaneous disorders most frequently encountered by the neurosurgeon and followed beyond childhood. They include neurofibromatosis types 1 and 2, tuberous sclerosis, Sturge-Weber syndrome and von Hippel-Lindau disease. In each case, a review of the literature, including diagnosis, genetics and treatment will be presented. The lifespan of the disease with the implications for neurosurgeons will be emphasized. A review of cases, including both pediatric and adult patients, seen in neurosurgical practices in the Lille, France and Lausanne, Switzerland hospitals between 1961 and 2007 is presented to illustrate the pathologies seen in different age-groups. Because the genes mutated in most phacomatoses are involved in development and are activated following a timed schedule, the phenotype of these diseases evolves with age. The implication of the neurosurgeon varies depending on the patient's age and pathology. While neurosurgeons tend to see pediatric patients affected with neurofibromatosis type 1, tuberous sclerosis and Sturge-Weber syndrome, there will be a majority of adult patients with von Hippel-Lindau disease or neurofibromatosis type 2.

Posterior positional plagiocephaly treated with cranial remodeling orthosis.

PRINCIPLES: Since the recommendation that infants sleep in the supine position, there has been an increase in cases of posterior positional plagiocephaly. Even though this condition is a purely cosmetic problem, if it is severe it may affect the child psychologically. Positioning may help in mild or moderate cases, but more active treatment may be necessary in severe cases. METHODS: A prospective study of 260 children treated by dynamic orthotic cranioplasty for posterior positional plagiocephaly was conducted in Lausanne from 1995 to 2001. Construction of these cranial remodelling helmets is decribed in detail. RESULTS: The treatment lasted 3 months on average, was effective, well tolerated, and had zero morbidity. The ideal period for initiating this therapy is between the ages of 4 and 6 months. CONCLUSION: The remodelling helmet is a convincing option which can be recommended in infants with posterior positional plagiocephaly whose skull deformity is not satisfactorily corrected by physiotherapy. It should always be used before surgery is considered for patients with recognised positional plagiocephaly in the first year of life.

(133)Xe SPECT cerebral blood flow study in a healthy population: determination of T-scores.

UNLABELLED: Dementia is becoming a major health problem as the population of the Northern Hemisphere ages. Early differential diagnosis between normal cognitive decline and dementia is particularly difficult. If psychometric evaluation can contribute to the diagnosis, quantitative cerebral functional imaging would play an important role. We therefore proposed, first, to constitute a normative dataset that could later be used to identify subjects at risk for neurodegenerative processes and, second, to describe the risk of abnormal global cerebral blood flow (gCBF) by defining categories based on the standardized cutoff scores of a young, healthy population (T-score). METHODS: Of a total of 203 healthy volunteers, 187 were included in the protocol, which included evaluation of medical history, neurologic and neuropsychologic status, and body composition; analysis of blood; and measurement of gCBF by means of (133)Xe SPECT. RESULTS: With ANOVA analysis using age and sex as between-subject factors and gCBF as a within-subject factor, a significantly higher gCBF was found in women than in men. In addition, a linear reduction as a function of age was observed for both sexes (-0.3%/y). T-score was determined for the 18- to 28-y-old age group, for whom gCBF was found to be 46.7 +/- 5.1 mL/min/100 g tissue in men and 49.0 +/- 5.0 mL/min/100 g tissue in women. The age-dependent decrease could thus be expressed in T-scores and, in the 29- to 38-y-old, 39- to 48-y-old, and >48-y-old age groups, averaged -0.63, -1.29, and -1.92, respectively, in men and -0.63, -0.83, and-2.40, respectively, in women. Cognitive performance, body composition, and blood analysis revealed the expected significant effects from sex and age. CONCLUSION: The large-scale reference database of gCBF measurements constituted from a healthy, well-controlled population enabled age and sex stratification, which showed significant differences between the sexes and a significant decline as a function of age. T-scores were determined and warrant further studies on the prospective identification of early dementia by (133)Xe SPECT in elderly individuals.

3D ultrasound system to investigate intraventricular hemorrhage in preterm neonates.

Intraventricular hemorrhage (IVH) is a common disorder among preterm neonates that is routinely diagnosed and monitored by 2D cranial ultrasound (US). The cerebral ventricles of patients with IVH often have a period of ventricular dilation (ventriculomegaly). This initial increase in ventricle size can either spontaneously resolve, which often shows clinically as a period of stabilization in ventricle size and eventual decline back towards a more normal size, or progressive ventricular dilation that does not stabilize and which may require interventional therapy to reduce symptoms relating to increased intracranial pressure. To improve the characterization of ventricle dilation, we developed a 3D US imaging system that can be used with a conventional clinical US scanner to image the ventricular system of preterm neonates at risk of ventriculomegaly. A motorized transducer housing was designed specifically for hand-held use inside an incubator using a transducer commonly used for cranial 2D US scans. This system was validated using geometric phantoms, US/MRI compatible ventricle volume phantoms, and patient images to determine 3D reconstruction accuracy and inter- and intra-observer volume estimation variability. 3D US geometric reconstruction was found to be accurate with an error of <0.2%. Measured volumes of a US/MRI compatible ventricle-like phantom were within 5% of gold standard water displacement measurements. Intra-class correlation for the three observers was 0.97, showing very high agreement between observers. The coefficient of variation was between 1.8-6.3% for repeated segmentations of the same patient. The minimum detectable difference was calculated to be 0.63 cm(3) for a single observer. Results from ANOVA for three observers segmenting three patients of IVH grade II did not show any significant differences (p > 0.05) for the measured ventricle volumes between observers. This 3D US system can reliably produce 3D US images of the neonatal ventricular system. There is the potential to use this system to monitor the progression of ventriculomegaly over time in patients with IVH.

[Orbitotemporal facial involvement in type 1 neurofibromatosis (NF1)]. / L'atteinte orbito-temporo-faciale dans la neurofibromatose de type 1 (NF1).

Plexiform neurofibromas of the orbit, sometimes extending to the temporal region and the face, are considered to be a rare but devastating and disfiguring complication of neurofibromatosis type 1. The first symptoms appear in infancy and the involvement of the orbit and the face is present in nearly all children after the age of 5. The disease is unilateral in most cases but can exceptionally involve both sides of the face. Progressive deformation of the orbital frame due to the expanding plexiform neurofibroma and buphthalmos occurs in a large proportion of cases. The associated sphenoidal dysplasia, which is thought to be, according to the most recent hypothesis, genetically determined, will inescapably increase the burden to the orbital content, cause pulsating proptosis and will endanger noble structures, finally resulting in loss of vision. Using the Jackson classification, the authors report their personal series of 22 cases (19 operated). Until now, there has been no effective medical treatment for plexiform neurofibroma and surgery remains the standard care for these patients. Controversies remain about the timing of the first operation and today most multidisciplinary teams involving plastic, maxillofacial, ophthalmologic, and neurosurgeons favor early intervention to try to minimize the secondary deformation of the orbital and facial skeleton. A number of cases of plexiform neurofibromas are illustrated within the three Jackson groups and treatment results of the rare elephantiasis neuromatosa cases are presented. Special techniques such as preoperative embolization of heavily vascularized plexiform neurofibroma are also discussed.

The role of posterior fossa decompression in acute cerebellitis.

BACKGROUND: We present two cases of children who were diagnosed with cerebellitis with acute cerebellar swelling. This rare pathology is potentially fatal, and no clear treatment guidelines are described in the literature. DISCUSSION: Considering our experience, we discuss the different therapeutic strategies and propose aggressive surgical measures consisting of external ventricular drainage and posterior fossa decompression in case of failure of early response to medical treatment to limit secondary cerebellar and brainstem lesions.

Negative neurofunctional effects of frequency, depth and environment in recreational scuba diving: the Geneva "memory dive" study.

OBJECTIVES: To explore relationships between scuba diving activity, brain, and behaviour, and more specifically between global cerebral blood flow (CBF) or cognitive performance and total, annual, or last 6 months' frequencies, for standard dives or dives performed below 40 m, in cold water or warm sea geographical environments. METHODS: A prospective cohort study was used to examine divers from diving clubs around Lac Léman and Geneva University Hospital. The subjects were 215 healthy recreational divers (diving with self-contained underwater breathing apparatus). Main outcome measures were: measurement of global CBF by (133)Xe SPECT (single photon emission computed tomography); psychometric and neuropsychological tests to assess perceptual-motor abilities, spatial discrimination, attentional resources, executive functioning, and memory; evaluation of scuba diving activity by questionnaire focusing on number and maximum depth of dives and geographical site of the diving activity (cold water v warm water); and body composition analyses (BMI). RESULTS: (1) A negative influence of depth of dives on CBF and its combined effect with BMI and age was found. (2) A specific diving environment (more than 80% of dives in lakes) had a negative effect on CBF. (3) Depth and number of dives had a negative influence on cognitive performance (speed, flexibility and inhibition processing in attentional tasks). (4) A negative effect of a specific diving environment on cognitive performance (flexibility and inhibition components) was found. CONCLUSIONS: Scuba diving may have long-term negative neurofunctional effects when performed in extreme conditions, namely cold water, with more than 100 dives per year, and maximal depth below 40 m.