The Perplexity Surrounding Chiari Malformations - Are We Any Wiser Now?

Chiari malformations are a diverse group of abnormalities of the brain, craniovertebral junction, and the spine. Chiari 0, I, and 1.5 malformations, likely a spectrum of the same malformation with increasing severity, are due to the inadequacy of the para-axial mesoderm, which leads to insufficient development of occipital somites. Chiari II malformation is possibly due to nonclosure of the caudal end of the neuropore, with similar pathogenesis in the rostral end, which causes a Chiari III malformation. There have been significant developments in the understanding of this complex entity owing to insights into the pathogenesis and advancements in imaging modalities and neurosurgical techniques. This article aims to review the different types and pathophysiology of the Chiari malformations, along with a description of the various associated abnormalities. We also highlight the role of ante- and postnatal imaging, with a focus on the newer techniques in the presurgical evaluation, with a brief mention of the surgical procedures and the associated postsurgical complications.

Brain MRI of multiple system atrophy of cerebellar type: a prospective study with implications for diagnosis criteria.

AIM: The second consensus statement for the diagnosis of multiple system atrophy type cerebellar (MSA-C) includes pons and middle cerebellar peduncle (MCP) atrophy as MRI features. However, other MRI abnormalities such as MCP hyperintensity, hot cross bun sign (HCB), putaminal hypointensity and hyperintense putaminal rim have been described. OBJECTIVES: To evaluate, in patients with sporadic late-onset cerebellar ataxia (SLOCA), the discriminative value of several MRI features for the diagnosis of MSA-C, to follow their evolution during the course of MSA-C, and to search for correlations between these MRI features and clinical signs. METHODS: Consecutive patients referred for SLOCA underwent comprehensive clinical evaluation and laboratory investigations, brain MRI, DaTscan and a 1-year follow-up. RESULTS: Among 80 patients, 26 had MSA-C, 22 another diagnosis, and 32 no diagnosis at the end of the follow-up. At baseline, MCP hyperintensity and HCB were more frequent in patients finally diagnosed with MSA-C than in other patients with SLOCA (p < 0.0001), and had the highest specificity (98.5%) and positive predictive value (91.7%) for the diagnosis of MSA-C, compared to all other MRI signs. The most relevant MRI sequence regarding HCB sign was the T2-proton density (DP) weighted. All MRI features were more frequent with disease duration. No correlation was found between any MRI feature and neither clinical data, nor dopaminergic neuronal loss (p = 0.5008), except between vermis atrophy and UPDRSIII score. CONCLUSION: MCP hyperintensity and HCB sign should be added into the list of additional features of possible MSA-C. MRI signal abnormalities suggestive of MSA-C should be searched for in suitable sequence.

Advanced magnetic resonance imaging (MRI) techniques of the spine and spinal cord in children and adults.

In this article, we illustrate the main advanced magnetic resonance imaging (MRI) techniques used for imaging of the spine and spinal cord in children and adults. This work focuses on daily clinical practice and aims to address the most common questions and needs of radiologists. We will also provide tips to solve common problems with which we were confronted. The main clinical indications for each MR technique, possible pitfalls and the challenges faced in spine imaging because of anatomical and physical constraints will be discussed. The major advanced MRI techniques dealt with in this article are CSF, (cerebrosopinal fluid) flow, diffusion, diffusion tensor imaging (DTI), MRA, dynamic contrast-enhanced T1-weighted perfusion, MR angiography, susceptibility-weighted imaging (SWI), functional imaging (fMRI) and spectroscopy. TEACHING POINTS: • DWI is essential to diagnose cord ischaemia in the acute stage. • MRA is useful to guide surgical planning or endovascular embolisation of AVMs. • Three Tesla is superior to 1.5 T for spine MR angiography and spectroscopy. • Advanced sequences should only be used together with conventional morphological sequences.

Subclinical haemorrhage in non-functional adenomas.

AIM: The prevalence and risk factors of subclinical haemorrhage on non-functional adenomas (NFA) remain controversial. The primary aim of our study was to assess the incidence of subclinical haemorrhage (SH) and the secondary objectives were to evaluate the risk factors of SH and the impact of SH on pituitary function at diagnosis. PATIENTS AND METHODS: This retrospective transversal analysis included 95 patients between January 2012 and December 2014. The patients included in this series were all adults (>18 years of age), who presented a non-functional adenoma confirmed by an endocrinological evaluation and on dedicated MRI pituitary imaging. Sixty-four patients were eligible for this study. Subclinical haemorrhage was defined by the presence of haemorrhage within pituitary adenoma confirmed by pituitary MRI with no clinical symptoms. A senior neuroradiologist blinded to the diagnosis reviewed all MRI. The population was prospectively divided into two groups based on MRI results (SH group vs. group control) to determine risk factors. RESULTS: SH was diagnosed in 22 patients (34.38%). No risk factors (age, sex, tumor size, chronic hypertension, diabetes mellitus, malignant disease, the use of anticoagulation or antithrombotic medication or Cabergoline treatment) were involved as regards the SH. At the diagnosis, pituitary deficiency was statistically significantly more frequent in the SH group (45.45%) than in the control group (19.04%) (P=0.04). CONCLUSION: The SH within NFA was observed in 34.38% of cases without an association of risk factors.

T1- or T2-weighted magnetic resonance imaging: what is the best choice to evaluate atrophy of the hippocampus?

BACKGROUND AND PURPOSE: Magnetic resonance imaging is part of the diagnostic criteria for Alzheimer's disease (AD) through the evaluation of hippocampal atrophy. The objective of this study was to evaluate which sequence of T1-weighted (T1WI) and T2-weighted (T2WI) imaging allowed the best visual evaluation of hippocampal atrophy. METHODS: Visual qualitative ratings of the hippocampus of 100 patients with mild cognitive impairment (MCI) and 50 patients with AD were made independently by four operators according to the medial temporal lobe atrophy score based either on T1WI or T2WI. These two evaluations were compared in terms of interobserver reproducibility, concordance with a quantitative volumetric measure, discrimination power between AD and MCI groups, and correlation with several neuropsychological tests. RESULTS: The medial temporal lobe atrophy score evaluated on either T1WI or T2WI exhibited similar interobserver variability and accordance with quantitative volumetric evaluation. However, the visual evaluation on T2WI seemed to provide better discrimination power between AD and MCI groups for both left (T1WI, P = 0.0001; T2WI, P = 7.072 × 10-5 ) and right (T1WI, P = 0.008; T2WI, P = 0.001) hippocampus, and a higher overall correlation with neuropsychological tests. CONCLUSIONS: The present study suggests that T2WI provides a more adequate visual rating of hippocampal atrophy.

A discriminative feature selection approach for shape analysis: Application to fetal brain cortical folding.

The development of post-processing reconstruction techniques has opened new possibilities for the study of in-utero fetal brain MRI data. Recent cortical surface analysis have led to the computation of quantitative maps characterizing brain folding of the developing brain. In this paper, we describe a novel feature selection-based approach that is used to extract the most discriminative and sparse set of features of a given dataset. The proposed method is used to sparsely characterize cortical folding patterns of an in-utero fetal MR dataset, labeled with heterogeneous gestational age ranging from 26 weeks to 34 weeks. The proposed algorithm is validated on a synthetic dataset with both linear and non-linear dynamics, supporting its ability to capture deformation patterns across the dataset within only a few features. Results on the fetal brain dataset show that the temporal process of cortical folding related to brain maturation can be characterized by a very small set of points, located in anatomical regions changing across time. Quantitative measurements of growth against time are extracted from the set selected features to compare multiple brain regions (e.g. lobes and hemispheres) during the considered period of gestation.

Spinal cord ischemia: practical imaging tips, pearls, and pitfalls.

Ischemia of the spinal cord is a rare entity with a poor prognosis. Brain ischemia is no longer a diagnostic challenge; on the contrary, ischemia of the spinal cord remains difficult, particularly in children. In this article, we illustrate the principal causes in children and adults, clinical presentation, different techniques for the diagnosis by MR imaging (diffusion, spinal MR angiography, and 1.5 versus 3T), pathophysiology, and differential diagnosis. We will discuss current knowledge, perspectives, and pitfalls.

Current and future imaging of the peripheral nervous system.

Peripheral nervous system (PNS) imaging is usually carried out by ultrasound and MRI. Thanks to its wide availability and excellent spatial resolution, ultrasound is a mature investigation with clearly established indications, particularly in entrapment syndromes and tumors. MRI is generally a second-line examination, which provides decisive additional information thanks to its excellent contrast resolution and its multiplanar abilities. This review describes the current methods for imaging the PNS, concentrating on acquisition techniques, normal results and basic pathological semiology. Ongoing and future developments are described in order to underline the forthcoming changes in this very dynamic field of musculoskeletal radiology.

Probabilistic tractography using Q-ball imaging and particle filtering: application to adult and in-utero fetal brain studies.

By assuming that orientation information of brain white matter fibers can be inferred from Diffusion-Weighted Magnetic Resonance Imaging (DW-MRI) measurements, tractography algorithms provide an estimation of the brain connectivity in vivo. The two key ingredients of tractography are the diffusion model (tensor, high-order tensor, Q-ball, etc.) and the means to deal with uncertainty during the tracking process (deterministic vs probabilistic mathematical framework). In this paper, we investigate the use of an analytical Q-ball model for the diffusion data within a well-formalized particle filtering framework. The proposed method is validated and compared to other tracking algorithms on the MICCAI'09 contest Fiber Cup phantom. Tractographies of in vivo adult and fetal brain Diffusion-Weighted Images (DWIs) are also shown to illustrate the robustness of the algorithm.

Tips and traps in neurological imaging: imaging the perimedullary spaces.

The spinal canal is frequently a source of difficulties, traps and diagnostic errors. Pitfalls related to artifacts are resolved by using appropriate sequences. Good knowledge of the appearance of certain particular anatomical structures (the cauda equina roots, the radicular veins of the lumbar spine and conus medullaris, the dorsal root ganglion) and of frequent variants (fibrolipoma of the filum terminale, common root sheaths, root cysts) will avoid a good many errors. Dilatation of epidural veins in intracranial hypotension can simulate the contrast enhancement of a tumour. An increase in epidural fat can induce pathogenic stenosis of the dural sheath.

3T tractography of the median nerve: optimisation of acquisition parameters and normative diffusion values.

PURPOSE: To define the optimal acquisition parameters for diffusion tensor imaging of the median nerve at the wrist and to measure the diffusion values. PATIENTS AND METHODS: The wrists of 14 healthy volunteers were explored using 3T MRI with a dedicated wrist coil. Each examination consisted of six sequences, varying the number of directions (15-20-30) and the b values (500-800-1000-2000s/mm(2)). The tractography images were evaluated by two radiologists using a qualitative scale with six levels. The diffusion and anisotropy coefficients (ADC and FA) were measured and the values compared with the data in the literature. RESULTS: Median nerve tractography was successfully achieved in all sequences. The 20 direction sequence with b=1000s/mm(2) seemed to provide the best results for a reasonable acquisition time (5'38″). The diffusion values showed large interindividual variations. CONCLUSION: Optimisation of acquisitions parameters is important in peripheral nerve tractography, and may facilitate a routine use of the technique. The images obtained are easy to analyse but the diffusivity and anisotropy coefficients are too variable, however, to be used in practice.

Sentinel lesions in osteoarticular traumatology: signs to watch out for.

Sentinel lesions are lesions of the bone or soft tissue, visible in standard X-rays carried out within a traumatic context, indicating bone or more severe capsular ligament lesions not visible on these X-rays. A detailed review of the peripheral joints as well as the spine is carried out with an example of each type of lesion in a standard X-ray. Confrontation with the reference examination, CT or MRI, depending on the case, is then carried out.

Diffusion tensor imaging of normal-appearing white matter in neuromyelitis optica.

OBJECTIVES: Neuromyelitis optica (NMO) is an inflammatory disease of the central nervous system characterized by severe attacks of optic neuritis and myelitis. Brain was classically, unlike in multiple sclerosis (MS), spared. Nevertheless recent studies showed that brain lesions can be seen with MRI. We studied the diffusion characteristics of normal-appearing white matter (NAWM) and abnormal white matter in NMO patients compared with NAWM in healthy subjects. PATIENTS AND METHODS: Diffusion tensor imaging (DTI) scans of the brain and spinal cord were obtained from 25 patients with NMO and 20 age- and gender-matched healthy subjects. Region of interest (ROI) analysis of the apparent diffusivity coefficient (ADC) and fractional anisotropy (FA) was performed in brain NAWM (optic radiations, corpus callosum [CC] and anterior and posterior limbs of the internal capsule [IC]) and in spinal cord NAWM and in lesions. RESULTS: ADC was increased and FA decreased in NMO patients in the posterior limb of the IC in the optic radiations and in spinal cord NAWM. FA was lower in spinal cord lesions. In contrast, there was no difference between the two groups in the anterior limb of the IC nor in the CC. CONCLUSION: These results suggest that DTI abnormalities are very severe in NMO spinal cord lesions. In our study, DTI abnormalities in NAWM were restricted to optic radiations and cortico-spinal tracts, suggesting secondary Wallerian degeneration. In contrast, NAWM outside these tracts (CC and anterior IC) remained normal suggesting that, unlike what is observed in MS, there is no infra-lesional abnormality in NMO.

Diffusion-weighted MRI, dynamic susceptibility contrast MRI and ultrasound perfusion quantification of denervated muscle in rabbits.

OBJECTIVE: The purpose of this study was to assess denervated muscle perfusion using dynamic susceptibility contrast MRI (DSCMRI) and contrast-enhanced ultrasound (CEUS), and to measure denervated muscle apparent diffusion coefficient (ADC) on b1000 diffusion-weighted MRI (DWMRI) at 3 T in order to clarify whether muscle denervation leads to an increase in the extracellular extravascular space, or an increase in blood flow-or both. MATERIALS AND METHODS: Axotomy of the right sciatic nerve of six white rabbits was performed at day 0. At day 9, hind limb muscles MRI and CEUS were performed to assess the consequences of denervation and both semimembranosus muscles of each rabbit were explanted for histological studies. Signal intensity on T2- and T1-weighted MRI, ADC on DWMRI, maximum signal drop (MSD) on DSCMRI and the area under the curve (AUC) on CEUS were measured over circular regions of interest (ROI), in both semimembranosus muscles. Non-parametric Wilcoxon matched-pairs tests were used to assess the mean differences between denervated and normal muscles. RESULTS: T2 fat-saturated (FS) MRI studies showed a strong signal in the right semimembranosus muscles compared with the left side, and gadolinium enhancement was observed on T1 FS MRI. Denervated muscles show a significant increase in ADC on DWMRI (p < 0.01) and a significant signal enhancement on DSCMR imaging (p < 0.05) and on first-pass CEUS (p < 0.05). CONCLUSION: The results of this study--based on perfusion- and diffusion-weighted images--suggest that, after denervation, both increased blood flow through muscle tissue and expansion of the extracellular water volume are present.

Scaphotrapezial ligament: normal arthro-CT and arthro-MRI appearance with anatomical and clinical correlation.

The purpose of our study was to demonstrate and describe the MR and arthro-CT anatomic appearance of the scaphotrapezial ligament and illustrate some of the pathologies involving this structure. This ligament consists of two slips that originate from the radiopalmar aspect of the scaphoid tuberosity and extend distally, forming a V shape. The ulnar fibers, which are just radial to the flexor carpi radialis sheath, inserted along the trapezial ridge. The radial fibers were found to be thinner and inserted at the radial aspect of the trapezium. Twelve fresh cadaver wrists were dissected, with close attention paid to the scaphotrapezio-trapezoidal (STT) joint. An osseoligamentous specimen was dissected with removal of all musculotendinous structures around the STT joint and was performed with high-resolution acquisition in a 128-MDCT scanner. Samples of the wrist area were collected from two fetal specimens. A retrospective study of 55 patients with wrist pain that were submitted to arthrography, arthro-CT, and arthro-MRI imaging was performed (10 patients on a 3-T superconducting magnet and 45 patients on a 1.5-T system). Another ten patients had high-resolution images on a 3-T superconducting magnet without arthrographic injection. MR arthrography and arthro-CT improved visualization and provided detailed information about the anatomy of the scaphotrapezial ligament. Knowledge of the appearance of this normal ligament on MRI allows accurate diagnosis of lesions and will aid when surgery is indicated or may have a role in avoiding unnecessary immobilization.

Hippocampal dysgenesis and variable neuropsychiatric phenotypes in patients with Bardet-Biedl syndrome underline complex CNS impact of primary cilia.

The Bardet-Biedl syndrome (BBS) is a rare ciliopathy clinically defined by the association of retinitis pigmentosa, polydactyly, obesity, kidney disease and cognitive impairment. The cognitive functioning, behavioral phenotype, prevalence of psychiatric diseases and memory performances of a cohort of 34 patients with BBS were evaluated and a systemic brain magnetic resonance imaging (MRI) was performed. The patients' cognitive functioning was of marked variable efficiency ranging from normal to disabling performances. Neuropsychological disorders such as slow thought process, attention difficulties and obsessive-compulsive traits were observed. Our main finding was hippocampal dysgenesis, diagnosed by MRI, found in 42.31% of the patients in this cohort. Moreover, we show that BBS proteins are expressed in the human hippocampus and in the human brain in the normal subject. Recent literature in the murine model shows that hippocampal neurogenesis, in particular in the adult mouse, requires an intact primary cilia. These results encourage us to further investigate the possible role of BBS proteins in the hippocampus and related central nervous system structures.

Glioblastoma multiforme presenting with ischemic stroke: case report and review of the literature.

A 58-year-old woman presented with acute onset of global aphasia. Imaging studies revealed a left frontotemporal enhancing tumor and ischemic stroke in the territory of the middle cerebral artery. The patient was operated on, and the diagnosis of glioblastoma multiforme was confirmed. At the time of surgery, several branches of the left middle cerebral artery were found embedded in the tumor. One branch, which was infiltrated by tumor and completely occluded, was resected to achieve complete resection. Postoperatively, the stroke area within the middle cerebral artery territory increased, together with worsening of the patient's clinical status, thus requiring urgent decompressive craniectomy. Thereafter, the patient gradually improved, and received radiation therapy and chemotherapy with no recurrence after 24 months of follow-up. To our knowledge, glioblastomas presenting with ischemic stroke are rare, and such patients should be considered to be at high surgical risk.

On super-resolution for fetal brain MRI.

Super-resolution techniques provide a route to studying fine scale anatomical detail using multiple lower resolution acquisitions. In particular, techniques that do not depend on regular sampling can be used in medical imaging situations where imaging time and resolution are limited by subject motion. We investigate in this work the use of a super-resolution technique for anisotropic fetal brain MR data reconstruction without modifying the data acquisition protocol. The approach, which consists of iterative motion correction and high resolution image estimation, is compared with a previously used scattered data interpolation-based reconstruction method. To optimize acquisition time, an evaluation of the influence of the number of input images and image noise is also performed. Evaluation on simulated MR images and real data show significant improvements in performance provided by the super-resolution approach.

[The spinal canal: from imaging anatomy to diagnosis]. / Espaces rachidiens intracanalaires: de l'anatomie radiologique au diagnostic étiologique.

The spinal canal is divided into epidural, subdural and subarachnoid spaces. Intraspinal processes should be correctly placed into their space of origin. MRI is the best imaging modality to achieve this task. Accurate determination of the space of origin routinely requires the acquisition of two different pulse sequences, typically T1W and T2W images, in two orthogonal planes, usually axial and sagittal. Simple imaging features can assist in determining the site of origin: changes to the epidural fat, compression or widening of subarachnoid spaces. The epidural space, bordered medially by dura, contains fat and vascular structures. The subdural space is a virtual space in between the dura and arachnoid membrane. The subarachnoid space is home to the CSF, spinal cord and nerve rootlets. An epidural process replaces the epidural fat, displaces the dura and narrows the subarachnoid space. A subarachnoid process widens the subarachnoid space and spares the epidural fat. Epidural processes usually are infectious or tumoral, either primary or secondary to spinal involvement. Subarachnoid processes include primary tumors, leptomeningeal metastases, arachnoiditis and hemorrhage. Nerve sheath tumors and meningiomas are the most frequent intradural extramedullary tumors.