Author Correction: Postzygotic inactivating mutations of RHOA cause a mosaic neuroectodermal syndrome.

An amendment to this paper has been published and can be accessed via a link at the top of the paper.

Extracranial versus intracranial hydro-hemodynamics during aging: a PC-MRI pilot cross-sectional study.

BACKGROUND: Both aging and changes in blood flow velocity between the extracranial (intraspinal) and intracranial regions of cerebral vessels have an impact on brain hydro-hemodynamics. Arterial and venous cerebral blood flows interact with cerebrospinal fluid (CSF) in the both the cranial and spinal systems. Studies suggest that increased blood and CSF flow pulsatility plays an important role in certain neurological diseases. Here, we investigated the changes in blood-CSF flow pulsatility in the cranial and spinal systems with age as well as the impact of the intracranial compartment on flow patterns. METHOD: Phase-contrast magnetic resonance imaging (PC-MRI) was performed in 16 young and 19 elderly healthy volunteers to measure the flows of CSF and blood. CSF stroke volume (SV), blood SV, and arterial and venous pulsatility indexes (PIs) were assessed at intra- and extracranial levels in both samples. Correlations between ventricular and spinal CSF flow, and between blood and CSF flow during aging were also assessed. RESULTS: There was a significant decrease in arterial cerebral blood flow and intracranial venous cerebral blood flow with aging. We also found a significant increase of intracranial blood SV, spinal CSF SV and arterial/venous pulsatility indexes with aging. In regard to intracranial compartment impact, arterial and venous PIs decreased significantly at intracranial level in elderly volunteers, while young adults exhibited decrease in venous PI only. Intracranial venous PI was paradoxically lower than extracranial venous PI, regardless of age. In both sample groups, spinal CSF SV and aqueductal CSF SV were positively correlated, and so were extracranial blood and spinal CSF SVs. CONCLUSION: The study demonstrates that aging changes blood flow but preserves blood and CSF interactions. We also showed that many parameters related to blood and CSF flows differ between young and elderly adults.

Postzygotic inactivating mutations of RHOA cause a mosaic neuroectodermal syndrome.

Hypopigmentation along Blaschko's lines is a hallmark of a poorly defined group of mosaic syndromes whose genetic causes are unknown. Here we show that postzygotic inactivating mutations of RHOA cause a neuroectodermal syndrome combining linear hypopigmentation, alopecia, apparently asymptomatic leukoencephalopathy, and facial, ocular, dental and acral anomalies. Our findings pave the way toward elucidating the etiology of pigmentary mosaicism and highlight the role of RHOA in human development and disease.

Author Correction: Postzygotic inactivating mutations of RHOA cause a mosaic neuroectodermal syndrome.

An amendment to this paper has been published and can be accessed via a link at the top of the paper.

Cerebrospinal Fluid and Cerebral Blood Flows in Idiopathic Intracranial Hypertension.

OBJECTIVES: Cerebrospinal fluid (CSF) and blood flows have a strong relationship during a cardiac cycle. Idiopathic intracranial hypertension (IIH) is a pathology that seems to present hemodynamic and hydrodynamic disturbance. The aim of this study was to establish CSF and blood interaction in IIH. MATERIAL AND METHODS: We retrospectively studied cerebral hydrodynamic and hemodynamic flows by phase-contrast MRI (PCMRI) in 13 IIH subjects (according Dandy's criteria) and 16 controls. We analyzed arterial peak flow, pulsatility index, and resistive index in arterial and venous compartments (PFart, PIart, RIart, PFvein, PIvein, RIvein) and measured arteriovenous and CSF peak flow and stroke volume (PFav, SVVASC, PFCSF, SVCSF). RESULTS: We found no significant difference between IIH and control groups in arterial and venous parameters. Arteriovenous flow analysis showed higher PFav and SVVASC in the IIH group than in the control group (respectively 369 ± 27 mL/min and 286 ± 47 mL/min, p = 0.02; and 1085 ± 265 µL/cardiac cycle and 801 ± 226 µL/cardiac cycle, p = 0.007). PFCSF and SVCSF were higher in the IIH group than in the control group (respectively 206 ± 50 mL/min and 126.6 ± 24.8 mL/min, p = 0.04; and 570 ± 190 µL/cardiac cycle and 430 ± 100 µL/cardiac cycle, p = 0.0007). CONCLUSION: Although no significant change was found in arterial and venous flows, we showed that a small phase shift of venous outflow might cause an increase in the arteriovenous pulsatility and an increasing brain expansion during the cardiac cycle. This arteriovenous flow increase would result in an increase of CSF flushing through the foramen magnum and an increased ICP.

Should isolated fetal ventriculomegaly measured below 12 mm be viewed as a variant of the norm? Results of a 5-year experience in a prenatal referral center.

BACKGROUND: Fetal ventriculomegaly (VM) is defined as lateral ventricles measured above 10 mm. Some authors believe VM <12 mm are variants of the norm and need not be addressed for referral ultrasound. METHODS: A retrospective continuous cohort study of 127 confirmed fetal VM was divided into three groups after initial referral sonographic assessment: isolated VM <12 mm (group A), isolated VM ≥12 mm (group B), and VM associated with other malformations (group C). We reviewed obstetric outcome and neonate evolution after 1 month with the aim of defining a pertinent prenatal workup. RESULTS: We reported fetal infections in all groups (p = .24) and chromosomal abnormalities only in group C (p = .41). Fetal magnetic resonance imaging (MRI) found initially undiagnosed brain abnormalities in groups B and C (12.5 and 14.1%, p < .05). Ratios of healthy children after 1 month stemming, respectively, from groups A, B, and C were 66.7, 62.5, and 20.2% (p < .05). CONCLUSIONS: Our results are in favor of a systematic referral ultrasound for every fetal VM, regardless of size, as soon as definition criterion is met. Additional paraclinical assessment (maternal serologic status for toxoplasmosis and cytomegalovirus, amniocentesis, fetal cerebral MRI) should be discussed depending on the situation.

Prenatal diagnosis of femoral facial syndrome: Three case reports and literature review.

Facial femoral syndrome (FFS) is a rare congenital abnormality, also known as femoral hypoplasia-unusual facies syndrome, characterized by variable degrees of femoral hypoplasia, associated with specific facial features. Other organ malformations are sometimes present. Most cases are sporadic, but rare family observations suggest genetic origin. However, no chromosomal or genetic abnormalities have ever been incriminated. We conducted a comprehensive literature review and added three new unreported observations. Through these 92 cases, authors aimed to determine sonographic signs that should direct towards diagnosis, and discuss potential genetic etiology. Diagnosis was suspected prenatally in 27.2% of cases, and maternal diabetes was found in 42.4% of patients. When fetal karyotype was available, it was normal in 97.1% of cases, but genomic variations of unknown significance were discovered in all three cases in which array comparative genomic hybridization (CGH) techniques were applied. Femoral affection defining FFS was hypoplasia in 78.3% of cases, agenesis in 12%, and both in 9.8%. Affection was bilateral in 84.8% of cases. Retrognathia was present in 65.2% of cases, cleft lip and/or palate in 63%, and other organ malformations in 53.3%. Intellectual development was normal in 79.2% of cases. Better prenatal recognition of this pathology, notably frequently associated malformations, should lead to a more precise estimation of functional prognosis. It seems likely that today's tendency to systematically employ array-CGH and exome/genome sequencing methods to investigate malformative sequences will allow the identification of a causal genetic abnormality in the near future.

The role of PC-MRI in neurodegenerative diseases / O papel da RM com contraste nas doenças neurodegenerativas

Introduction: Neurodegenerative diseases, a major public health problem, could have a vascular origin. Phase-contrast magnetic resonance imaging (PC-MRI) enables reliable, non-invasive, and rapid measurements of cerebrospinal fluid (CSF) and blood flows, and evaluation of the mechanical coupling between cerebral blood and CSF flows throughout the cardiac cycle (CC). Objectives: Our purpose was to evaluate the potential of PC-MRI to the study of cerebral blood and CSF flows in patients with neurodegenerative diseases such as Alzheimer's disease (AD), Mild cognitive impairment with amnesic disorders (MCIa) and Vascular Dementia (VD). Methods: The elderly population consisted of 20 AD (age: 80 ± 5 years); 12 AD patients with vascular cerebral lesions (ADvasc) (age: 81 ± 5 years), 10 MCIa patients (age: 80 ± 7 years), and 8 VD patients (age: 78 ± 7 years) were identified. They underwent the same PC-MRI protocol and were compared to 13 age-matched Healthy Elderly (HE) (age: 71± 9 years). Arterial blood pressure was analyzed to detect patients with hypertension. Results: Significantly higher cerebral blood and CSF flows were observed in HE when compared to VD, AD and ADvasc, (p<0.05), but not MCIa patients who yielded the highest cerebral arterial and venous blood flows and stroke volumes compared to the other patients, (p<0.05). The highest oscillations of CSF were also detected in MCIa patients (p<0.05). Conclusion: Our preliminary data suggests an increase in cerebral arterial blood and CSF flows in MCIa. PC-MRI provides a new hydrodynamic view, which may help evaluate a potential role of cardiovascular alterations in neurodegenerative diseases.

Introdução: As doenças neurodegenerativas, um grande problema de saúde pública, podem ser de origem vascular. A ressonância magnética (RM) com contraste de fase permite medições confiáveis, não invasivas e rápidas do líquido cefalorraquidiano (LCR) e dos fluxos sanguíneos para avaliação do acoplamento mecânico de circulação cerebral e fluxo de LCR ao longo do ciclo cardíaco (CC). Objetivos: Nosso objetivo foi avaliar o potencial da RM com contraste de fase para o estudo do fluxo sanguíneo no cérebro e LCR em pacientes com doenças neurodegenerativas, como doença de Alzheimer (DA), comprometimento cognitivo leve (CCL) com distúrbios amnésicos e demência vascular (DV). Métodos: A população foi composta por 20 sujeitos idosos com DA (idade: 80 ± 5 anos); foram identificados 12 pacientes com lesões cerebrais vasculares (idade: 81 ± 5 anos), 10 pacientes com CCL e distúrbios amnésicos (idade: 80 ± 7 anos) e 8 com DV (idade: 78 ± 7 anos). Eles foram submetidos ao mesmo protocolo de RM com contraste de fase e comparados a 13 idosos saudáveis (idade: 71 ± 9 anos). A pressão arterial foi aferida para detectar pacientes com hipertensão arterial. Resultados: Observaram-se fluxos sanguíneos cerebrais e CSF significativamente maiores em pacientes com DV, DA e com lesões cerebrais (p<0,05), mas não nos pacientes com CCL, que tiveram os maiores fluxos arteriais e venosos cerebrais em relação aos demais pacientes (p<0,05). Maiores oscilações de LCR também foram detectadas em pacientes com CCL (p<0,05). Conclusão: Nossos dados preliminares sugerem um aumento no fluxo arterial cerebral e nos fluxos de LCR na CCL. A RM com contraste e fase fornece uma nova visão hidrodinâmica, o que pode ajudar a avaliar o possível papel das alterações cardiovasculares em doenças neurodegenerativas.

Effects of Methylphenidate on Default-Mode Network/Task-Positive Network Synchronization in Children With ADHD.

OBJECTIVE: A failure of the anti-phase synchronization between default-mode (DMN) and task-positive networks (TPN) may be involved in a main manifestation of ADHD: moment-to-moment variability. The study investigated whereby methylphenidate may improve TPN/DMN synchronization in ADHD. METHOD: Eleven drug-naive ADHD children and 11 typically developing (TD) children performed a flanker task during functional magnetic resonance imaging. The ADHD group was scanned without and 1 month later with methylphenidate. The signal was analyzed by independent component analysis. RESULTS: The TD group showed anti-phase DMN/TPN synchronization. The unmedicated ADHD group showed synchronous activity in the posterior DMN only, which was positively correlated with response time variability for the flanker task. Methylphenidate initiated a partial anti-phase TPN/DMN synchronization, reduced variability, and abolished the variability/DMN correlation. CONCLUSION: Although results should be interpreted cautiously because the sample size is small, they suggest that a failure of the TPN/DMN synchronization could be involved in the moment-to-moment variability in ADHD. Methylphenidate initiated TPN/DMN synchronization, which in turn appeared to reduce variability.

Insights into cerebrospinal fluid and cerebral blood flows in infants and young children.

This study investigates the craniospinal flows of blood and cerebrospinal fluid using phase-contrast magnetic resonance imaging (MRI) on 23 control neonates and infants (5 d-68 mo old). Mean arterial cerebral blood flow increased with age of infant from 180 mL/min after birth to 1330 mL/min around 6 years of age. This corresponds to 51 mL/min/100 g and 95 mL/min/100 g, respectively. Cervical cerebrospinal fluid stroke volume increased from 38 × 10(-3) mL to 752 × 10(-3) mL per cardiac cycle. After arterial systolic blood inflow, we observed a delay of the venous outflow that was always preceded by cerebrospinal fluid flushing out through the spinal canal. These results highlighted the importance of compliance of the spinal compartment and the interaction of blood and cerebrospinal fluid dynamics. The capacity of the spinal compartment to receive intracranial cerebrospinal fluid in presence of fontanels was demonstrated. We provide reference values to understand the physiology of cerebrospinal fluid and cerebral blood.

Relationship between cerebrospinal fluid flow, ventricles morphology, and DTI properties in internal capsules: differences between Alzheimer's disease and normal-pressure hydrocephalus.

BACKGROUND: Normal-pressure hydrocephalus (NPH) and Alzheimer's disease (AD) have some similar clinical features and both involve white matter and cerebrospinal fluid (CSF) disorders. PURPOSE: To compare putative relationships between ventricular morphology, CSF flow, and white matter diffusion in AD and NPH. MATERIAL AND METHODS: Thirty patients (18 with AD and 12 with suspected NPH) were included in the study. All patients underwent a 3-Tesla MRI scan, which included phase-contrast MRI of the aqueduct (to assess the aqueductal CSF stroke volume) and a DTI session (to calculate the fractional anisotropy [FA] and apparent diffusion coefficient [ADC]) in the internal capsules). RESULTS: FA was correlated with ventricular volume in the suspected NPH population (P < 0.001; rs = 0.88), whereas the ADC was highly correlated with the aqueductal CSF stroke volume in AD (P < 0.001; rs = 0.79). CONCLUSION: Although AD and NPH both involve CSF disorders, the two diseases do not have the same impact on the internal capsules. The magnitude of the ADC is related to the aqueductal CSF stroke volume in AD, whereas FA is related to ventricular volume in NPH.

An innovative approach to investigate the dynamics of the cerebrospinal fluid in the prepontine cistern: A feasibility study using spatial saturation-prepared cine PC-MRI.

PURPOSES: Accurate measurements of the cerebrospinal fluid that flows through the prepontine cistern (PPC) are challenging due to artefacts originating from basilar artery blood flow. We aim to accurately quantify cerebrospinal fluid (CSF) flow and stroke volume in the PPC, which is essential before endoscopic third ventriculostomy. MATERIALS AND METHODS: We developed a new PC-MRI sequence prepared with Hadamard saturation bands to accurately quantify CSF flow in the PPC by suppressing the blood signal in the surrounding vessels. In total, 28 adult hydrocephalic patients (age 59 ± 20 years) were scanned using conventional PC-MRI and our developed sequence. CSF was separately extracted from the PPC and the foramen of Magendie, and flow (min and max) and stroke volume were quantified. RESULTS: Our modifications result in a complete deletion of signal from flowing blood, resulting in significantly reduced CSF stroke volume (Conv = 446 ± 113 mm(3), Dev = 390 ± 119 mm(3), p = 0.006) and flow, both minimum (Conv = -1630 ± 486 mm(3)/s, Dev = -1430 ± 406 mm(3)/s, p = 0.005) and maximum (Conv = 2384 ± 657 mm(3)/s, Dev = 1971 ± 62 mm(3)/s, p = 0.002) compared with the conventional sequence, whereas no change in the area of interest was noted (Conv = 236 ± 65 mm(2), Dev = 249 ± 75 mm(2), p = 0.21). CONCLUSIONS: Accurate and reproducible CSF flow and stroke volume measurements in the PPC can be achieved with sat-band prepared cine PC-MRI.

Effect of surgery on periventricular white matter in normal pressure hydrocephalus patients: comparison of two methods of DTI analysis.

BACKGROUND: Diffusion tensor imaging (DTI) is a useful tool for assessing changes that occur in microstructures. We have developed a novel method for region of interest (ROI) delineation in the assessment of DTI parameters in patients with normal pressure hydrocephalus (NPH). PURPOSE: To compare the standard method and our novel method in an evaluation of the impact of surgery on periventricular white matter in patients with NPH. MATERIAL AND METHODS: Ten patients with NPH underwent 3T magnetic resonance imaging (MRI; including 12-direction DTI sequences) before and after surgery. We recorded diffusion parameters (λi, the fractional anisotropy [FA], the apparent diffusion coefficient, and Dr) in the internal capsule (IC) and the body of the corpus callosum (BCC). Using the standard delineation technique, regions of interest (ROIs) were positioned according to anatomical and functional considerations and then filled with several sub-ROIs. The ROIs delineated with our novel technique (extracted as the six sub-ROIs with the lowest standard deviation for the FA) were arranged in two rows (medial and lateral), from the ventricle to the brain surface. RESULTS: The within-ROI homogeneity was higher with the novel method than with the conventional method (P<10(-4)). When the conventional delineation method was applied to the IC data, only λ2 was found to be significantly greater after surgery; in contrast, application of our novel method evidenced a significant decrease in FA and λ1 and a significant increase in λ2 (P<0.05). Both before and after surgery, the FA in the medial row of ROIs was greater than the FA in the lateral row (P<0.01). In the BCC, only λ2 and Dr varied significantly (when evaluated with the novel method). CONCLUSION: Our results show that use of a novel method of DTI data analysis may be more sensitive to local changes induced by surgical procedures. Furthermore, this novel method was able to detect the transmantle pressure gradient related to the regional stress distribution.

Temporal resolvability analysis of macroscopic morphological development in neonatal cerebral magnetic resonance images.

OBJECTIVES: Reliable gradation of neonatal brain development is important for clinical investigation of neurological disorders. A prerequisite for such quantification of development is knowledge about temporal resolvability. METHODS: We hypothesized 2-week interval as the temporal resolvability of age-related templates to study macroscopic morphological brain development in the early weeks after birth. Therefore, we constructed two templates for the gestational age (GA) ranges of 39 to 40 and 41 to 42 weeks using T1-weighted magnetic resonance (MR) images. Then, we compared the spatial variation of anatomical landmarks and the average and the maximal length of spatial deformation in 30 subjects normalized to the two templates along x, y, and z directions. RESULTS: Multivariate analysis of variance (MANOVA) revealed significant difference between spatial variations of the above macroscopic features in the two age ranges. Furthermore, quantitative analysis of feature scattering yielded the same result even in features for which the null hypothesis was not rejected by MANOVA. Moreover, the same procedure was reiterated on two sets of subjects with the closer age range of 1 week (40 and 41 week's GA) and no significant difference could be detected. CONCLUSIONS: The results strengthen the hypothesis that 2-week is the temporal resolvability of age-related templates for macroscopic morphological studies of the developing brain in the early weeks after birth.

Measurement of choroid plexus perfusion using dynamic susceptibility MR imaging: capillary permeability and age-related changes.

INTRODUCTION: The cerebrospinal fluid (CSF) plays a major role in the physiology of the central nervous system. The continuous turnover of CSF is mainly attributed to the highly vascularized choroid plexus (CP) located in the cerebral ventricles which represent a complex interface between blood and CSF. We propose a method for evaluating CP functionality in vivo using perfusion MR imaging and establish the age-related changes of associated parameters. METHODS: Fifteen patients with small intracranial tumors were retrospectively studied. MR Imaging was performed on a 3T MR Scanner. Gradient-echo echo planar images were acquired after bolus injection of gadolinium-based contrast agent (CA). The software developed used the combined T1- and T2-effects. The decomposition of the relaxivity signals enables the calculation of the CP capillary permeability (K2). The relative cerebral blood volume (rCBV), mean transit time (MTT), and signal slope decrease (SSD) were also calculated. RESULTS: The mean permeability K2 of the extracted CP was 0.033+/-0.18 s(-1). K2 and SSD significantly decreased with subject's age whereas MTT significantly increased with subject's age. No significant correlation was found for age-related changes in rCBV and rCBF. CONCLUSION: The decrease in CP permeability is in line with the age-related changes in CSF secretion observed in animals. The MTT increase indicates significant structural changes corroborated by microscopy studies in animals or humans. Overall, DSC MR-perfusion enables an in vivo evaluation of the hemodynamic state of CP. Clinical applications such as neurodegenerative diseases could be considered thanks to specific functional studies of CP.

Estimation of the lateral ventricles volumes from a 2D image and its relationship with cerebrospinal fluid flow.

PURPOSE: This work suggests a fast estimation method of the lateral ventricles volume from a 2D image and then determines if this volume is correlated with the cerebrospinal fluid flow at the aqueductal and cerebral levels in neurodegenerative diseases. MATERIALS AND METHODS: Forty-five elderly patients suffering from Alzheimer's disease (19), normal pressure hydrocephalus (13), and vascular dementia (13) were involved and underwent anatomical and phase contrast MRI scans. Lateral ventricles and stroke volumes were assessed on anatomical and phase contrast scans, respectively. A common reference plane was used to calculate the lateral ventricles' area on 2D images. RESULTS: The largest volumes were observed in hydrocephalus patients. The linear regression between volumes and areas was computed, and a strong positive correlation was detected (R² = 0.9). A derived equation was determined to represent the volumes for any given area. On the other hand, no significant correlations were detected between ventricles and stroke volumes (R² ≤ 0.15). CONCLUSION: Lateral ventricles volumes are significantly proportional to the 2D reference section area and could be used for patients' follow-up even if 3D images are unavailable. The cerebrospinal fluid fluctuations in brain disorders may depend on many physiological parameters other than the ventricular morphology.

Cerebrospinal fluid and blood flow in mild cognitive impairment and Alzheimer's disease: a differential diagnosis from idiopathic normal pressure hydrocephalus.

BACKGROUND: Phase-contrast magnetic resonance imaging (PC-MRI) enables quantification of cerebrospinal fluid (CSF) flow and total cerebral blood (tCBF) flow and may be of value for the etiological diagnosis of neurodegenerative diseases. This investigation aimed to study CSF flow and intracerebral vascular flow in patients with Alzheimer's disease (AD) and patients with amnesic mild cognitive impairment (a-MCI) and to compare the results with patients with idiopathic normal pressure hydrocephalus (NPH) and with healthy elderly volunteers (HEV). METHODS: Ten a-MCI and 9 mild AD patients were identified in a comprehensive neurological and neuropsychological assessment. They underwent brain MRI; PC-MRI pulse sequence was performed with the following parameters: two views per segment; flip angle: 25° for vascular flow and 20° for CSF flow; field-of-view (FOV): 14 × 14 mm²; matrix: 256 × 128; slice thickness: 5 mm; with one excitation for exams on the 3 T machine, and 2 excitations for the 1.5 T machine exams. Velocity (encoding) sensitization was set to 80 cm/s for the vessels at the cervical level, 10 or 20 cm/s for the aqueduct and 5 cm/s for the cervical subarachnoid space (SAS). Dynamic flow images were analyzed with in-house processing software. The patients' results were compared with those obtained for HEVs (n = 12), and for NPH patients (n = 13), using multivariate analysis. RESULTS: Arterial tCBF and the calculated pulsatility index were significantly greater in a-MCI patients than in HEVs. In contrast, vascular parameters were lower in NPH patients. Cervical CSF flow analysis yielded similar values for all four populations. Aqueductal CSF stroke volumes (in µl per cardiac cycle) were similar in HEVs (34 ± 17) and AD patients (39 ± 18). In contrast, the aqueductal CSF was hyperdynamic in a-MCI patients (73 ± 33) and even more so in NPH patients (167 ± 89). CONCLUSION: Our preliminary data show that a-MCI patients present with high systolic arterial peak flows, which are associated with higher mean total cerebral arterial flows. Aqueductal CSF oscillations are within normal range in AD and higher than normal in NPH. This study provides an original dynamic vision of cerebral neurodegenerative diseases, consistent with the vascular theory for AD, and supporting primary flow disturbances different from those observed in NPH.

Design and construction of a brain phantom to simulate neonatal MR images.

This paper presents the design and construction of a 3D digital neonatal neurocranial phantom and its application for the simulation of brain magnetic resonance (MR) images. Commonly used digital brain phantoms (e.g. BrainWeb) are based on the adult brain. With the growing interest in computer-aided methods for neonatal MR image processing, there is a growing demand a digital phantom and brain MR image simulator especially for the neonatal brains. This is due to the pronounced differences between adult and neonatal brains not only in terms of size but also, more importantly, in terms of geometrical proportions and the need to subdivide white matter into two different tissue types in neonates. Therefore the neonatal brain phantom created in the here presented work consists of 9 different tissue types: skin, fat, muscle, skull, dura mater, gray matter, myelinated white matter, nonmyelinated white matter and cerebrospinal fluid. Each voxel has a vector consisting of 9 components, one for each of these nine tissue types. This digital phantom can be used to map simulated magnetic resonance signal intensities resulting in simulated MR images of the newborns head. These images with controlled degradation of the image data present a representative, reproducible data set ideal for development and evaluation of neonatal MRI analysis methods, e.g. segmentation and registration algorithms.

[Contribution of phase-contrast MRI to the management of patients with normal pressure hydrocephalus: Can it predict response to shunting?]. / Apport de l'IRM de flux dans les hydrocéphalies à pression normale de l'adulte : intérêt prédictif dans les indications chirurgicales.

The diagnosis and management of patients with idiopathic normal-pressure hydrocephalus (NPH) remain somewhat controversial and there is no clear guideline for assessing the post-shunt outcome. The objective of this study was to investigate whether cerebrospinal fluid (CSF) flow dynamics is linked to post-shunt improvement. Fourteen NPH patients (nine males and five females; mean age, 68 years) investigated by magnetic resonance imaging (MRI) before surgical diversion of CSF were retrospectively reviewed. Phase-contrast sequences were added to the morphological clinical protocol for quantification of CSF oscillations, which were recorded at the level of the cerebral aqueduct and the C2 and C3 subarachnoid spaces (SAS). The phase-contrast images were analysed with custom-designed dedicated flow segmentation software. The oscillations measured in this hydrocephalus population were compared to a previously studied healthy population. A difference of at least two standard deviations was used to define a hyperdynamic or hypodynamic state of CSF flow. The cervical CSF flow of the hydrocephalus patients was not significantly different from those of the volunteer population. Of the 14 hydrocephalus patients, 12 had a good response to the shunt. Of these, 10 presented an increased ventricular CSF flow, one a low ventricular CSF flow, and the last one had a normal ventricular CSF flow. Phase-contrast MRI can help develop guidelines for surgical management of NPH. The shunt responders appear to be the patients with hyperdynamic ventricular CSF flow and normal cervical CSF flow.

Cognitive forms of multiple sclerosis: report of a dementia case.

Cognitive impairment is now well documented in patients with multiple sclerosis (MS), and series of MS patients with predominant cognitive problems have been published recently. We report the observation of a female patient with severe cognitive presentation at the onset of MS, with dramatically demented evolution, and show MRI examination results. We discuss the published reports of primary cognitive types of MS.