Abstract: BACKGROUND AND PURPOSE: The detection of time-related maturational changes of the olfactory bulb (OB) on MR imaging may help early identification of patients with abnormal OB development and anatomic-based odor-cueing anomalies. MATERIALS AND METHODS: Two separate reviewers retrospectively analyzed coronal T2-weighted spin-echo MR images of the frontobasal region in 121 patients. There were 22 patients who underwent MR imaging examinations several times, accounting for a total of 156 studies. Age range was 1 day to 19.6 years. OBs were bilaterally identified in all cases and categorized according to their shape and signal intensity. RESULTS: Three different anatomic patterns were identified. In pattern 1 (median age, 15 days; age range, 1-168 days), the OBs were round to oval with a continuous external T2-hypointense rim and a prominent T2-hyperintense central area. In pattern 2 (median age, 287 days; age range, 4 days-22 months), the OBs were U shaped, with thinning and concave deformation of the superior layer. A hyperintense central area on T2-weighted images was still visible. In pattern 3 (median age, 5.2 years; age range, 107 days-19.6 years), the OBs were small, round, or J shaped with a more prominent lateral part. No difference in signal intensity between the central area and the peripheral layer was identified anymore. CONCLUSIONS: The OBs show time-related maturational changes on MR imaging. There is a progressive reorganization of the peripheral neuronal layers and signal intensity changes of the central area, which are completed at the end of the second year, paralleling cerebral maturational changes.
Abstract: SUMMARY: We present a case of Klippel-Feil syndrome and Sprengel deformity with a bovine aortic arch and an aberrant course of the left subclavian artery in a 14-year-old boy. CT and MR imaging of the neck and upper thorax demonstrated a cervical osseous segmentation anomaly, a left common carotid artery originating from the innominate artery, and a left subclavian artery coursing through the intraspinal space at the C6 through T1 level. Possible embryonic mechanisms and clinical significance of this variant are reviewed.
Abstract: BACKGROUND: Cortical signal intensity (SI) of the limbic system in adults is known to be higher than in neocortical structures, but time-related changes in SI during childhood have not been described. OBJECTIVE: To detect maturation-related SI changes within the limbic system using a fluid-attenuated inversion recovery (FLAIR) MR sequence. MATERIALS AND METHODS: Twenty children (10 boys, 10 girls; age 3.5-18 years, mean 11.2 years) with no neurological abnormality and normal MR imaging examination were retrospectively selected. On two coronal FLAIR slices, ten regions of interest (ROI) with a constant area of 10 mm2 were manually placed in the archeocortex (hippocampus), periarcheocortex (parahippocampal gyrus, subcallosal area, cingulate gyrus) and in the neocortex at the level of the superior frontal gyrus on both sides. RESULTS: Significant SI gradients were observed with a higher intensity in the archeocortex, intermediate intensity in the periarcheocortex and low intensity in the neocortex. Significant higher SI values in hippocampal and parahippocampal structures were detected in children up to 10 years of age. CONCLUSION: These differences mainly reflected differences in cortical structure and myelination state. Archeocortical structures especially showed significant age-related intensity progression suggesting ongoing organization and/or myelination until early adolescence.
Abstract: The correct assessment of the four most frequent infratentorial brain tumors in children (medulloblastoma, ependymoma, pilocytic astrocytoma and infiltrating glioma) has always been problematic. They are known to often resemble one another on conventional magnetic resonance (MR) imaging. We tested the hypothesis whether the combined strength of diffusion-weighted imaging (DWI) and proton MR spectroscopy (MRS) could help differentiate these tumors. Seventeen children with untreated posterior fossa tumors were investigated between January 2005 and January 2006 with conventional MR imaging and combined DWI and MR spectroscopy using a single-voxel technique at short and long echo time (TE) of 30 ms and 135 ms respectively. Apparent diffusion coefficient (ADC) values were retrieved after regions of interest were manually positioned within non necrotic tumor core. Water signal was quantified and metabolite signals were compared and analyzed using linear discriminant analysis. When a combination of ADC values and normalized metabolites was used, all tumors could be discriminated against one other. This could only be achieved when metabolites were normalized using water as an internal standard. They could not be discriminated when using metabolite ratios or ADC values alone, nor could they be differentiated using creatine (Cr) as an internal reference even in combination with ADC values. In conclusion, linear discriminant analysis and multiparametric combination of DWI and MRS, although not replacing histology, fully discriminates the four most frequent posterior fossa tumors in children, but metabolites have to be normalized using water and not Cr signal as an internal reference.
Abstract: PURPOSE: To assess the combined value of diffusion-weighted imaging (DWI) and proton magnetic resonance spectroscopy (1H-MRS) in differentiating medulloblastoma, ependymoma, pilocytic astrocytoma, and infiltrating glioma in a pediatric population. MATERIALS AND METHODS: A total of 17 children with untreated posterior fossa tumors (seven medulloblastoma, four infiltrating glioma, two ependymoma, and four pilocytic astrocytoma), were investigated with conventional MRI, DWI, and MRS using a single-voxel technique. Within the nonnecrotic tumor core, apparent diffusion coefficient (ADC) values using a standardized region of interest (ROI) were retrieved. Quantification of water signal and analysis of metabolite signals from MRS measurements in the same tumorous area were reviewed using multivariant linear discriminant analysis. RESULTS: Combination of ADC values and metabolites, which were normalized using water as an internal standard, allowed discrimination between the four tumor groups with a likelihood below 1 x 10(-9). Positive predictive value was 1 in all cases. Tumors could not be discriminated when using metabolite ratios or ADC values alone, nor could they be differentiated using creatine (Cr) as an internal reference even in combination with ADC values. CONCLUSION: Linear discriminant analysis using DWI and MRS using water as internal reference, fully discriminates the four most frequent posterior fossa tumors in children.
Abstract: Diffusion-weighted imaging (DWI) provides information about tissue maturation not seen on conventional magnetic resonance imaging. The aim of this study is to analyze the evolution over time of the apparent diffusion coefficient (ADC) of normal fetal brain in utero. DWI was performed on 78 fetuses, ranging from 23 to 37 gestational weeks (GW). All children showed at follow-up a normal neurological evaluation. ADC values were obtained in the deep white matter (DWM) of the centrum semiovale, the frontal, parietal, occipital and temporal lobe, in the cerebellar hemisphere, the brainstem, the basal ganglia (BG) and the thalamus. Mean ADC values in supratentorial DWM areas (1.68 +/- 0.05 mm(2)/s) were higher compared with the cerebellar hemisphere (1.25 +/- 0.06 mm(2)/s) and lowest in the pons (1.11 +/- 0.05 mm(2)/s). Thalamus and BG showed intermediate values (1.25 +/- 0.04 mm(2)/s). Brainstem, cerebellar hemisphere and thalamus showed a linear negative correlation with gestational age. Supratentorial areas revealed an increase in ADC values, followed by a decrease after the 30th GW. This study provides a normative data set that allows insights in the normal fetal brain maturation in utero, which has not yet been observed in previous studies on premature babies.
Abstract: A multidiffusion-tensor model (MDT) is presented containing two anisotropic and one isotropic diffusion tensors. This approach has the ability to detect areas of fiber crossings and resolve the direction of crossing fibers. The mean diffusivity and the ratio of the tensor compartments were merged to one independent parameter by fitting MDT to the diffusion-weighted intensities of a two-point data acquisition scheme. By an F-test between the errors of the standard single diffusion tensor and the more complex MDT, fiber crossings were detected and the more accurate model was chosen voxel by voxel. The performance of crossing detection was compared with the spherical harmonics approach in simulations as well as in vivo. Similar results were found in both methods. The MDT model, however, did not only detect crossings but also yielded the single fiber directions. The FACT algorithm and a probabilistic connectivity algorithm were extended to support the MDT model. For example, a mean angular error smaller than 10 degrees was found for the MDT model in a simulated fiber crossing with an SNR of 80. By tracking the corticospinal tract the MDT-based tracks reached a significantly greater area of the gyrus precentralis.
Abstract: Developmental nasal midline masses in children are rare lesions. Neuroimaging is essential to characterise these lesions, to determine the exact location of the lesion and most importantly to exclude a possible intracranial extension or connection. Our objective was to evaluate CT and MRI in the diagnosis of developmental nasal midline masses. Eleven patients (mean age 4.5 years) with nasal midline masses were examined by CT and MRI. Neuroimaging was evaluated for (a) lesion location/size, (b) indirect (bifid or deformed crista galli, widened foramen caecum, defect of the cribriform plate) and direct (identification of intracranially located lesion components or signal alterations) imaging signs of intracranial extension, (c) secondary complications and (d) associated malformations. Surgical and histological findings served as gold standard. Nasal dermoid sinus cysts were diagnosed in 9 patients. One patient was diagnosed with an meningocele and another patient with a nasal glioma. Indirect CT and MRI signs correlated with the surgical results in 10 of 11 patients. Direct CT findings correlated with surgery in all patients, whereas the direct MRI signs correlated in 9 of 11 patients. In 2 patients MRI showed an intracranial signal alteration not seen on CT. Neuroimaging corrected the clinical diagnosis in 1 patient. One child presented with a meningitis. In none of the patients was an associated malformation diagnosed. Intracranial extension is equally well detected by CT and MRI using indirect imaging signs. Evaluating the direct imaging signs, MRI suspected intracranial components in 2 patients without a correlate on CT. This could represent an isolated intracranial component that got undetected on CT and surgery. In 9 patients CT and MRI matched the surgical findings. The MRI did not show any false-negative results. These results in combination with the multiplanar MRI capabilities, the different image contrasts that can be generated by MRI and the lack of radiation favour the use of MRI as primary imaging tool in these young patients in which the region of imaging is usually centred on the radiosensitive eye lenses.
Abstract: We investigated the isotropic diffusion coefficient (D') and fractional anisotropy (FA) in white matter (WM) during brain development, using an optimised diffusion-tensor imaging (DTI) method with whole brain coverage in a clinically acceptable time. We images 52 children with no evident neurological abnormality (30 boys, 22 girls aged 1 day-16 years) using high-angle DTI with optimised temporal gradient performance. D' and FA were calculated in 10 regions of interest in white matter. We saw that the age-related reduction in D' and increase in FA follow a mono- or biexponential model in white matter, probably depending on the compactness and myelination rate of the fibre tracts. In contrast to other areas, in which adult values were reached during the third year, there is a trend to continuous increase in FA in all deep white-matter areas, suggesting continuing maturation and organisation of deep tracts not detected on conventional MRI.
Abstract: We report three boys in whom a diagnosis of Lhermitte-Duclos disease (LDD) was assumed from characteristic neuroimaging findings. LDD was confirmed by an open biopsy in patient 1, while a biopsy in patient 2 was inconclusive. Histologic confirmation in patient 3 was deliberately not attempted. However, a follow-up observation of stable clinical and neuroimaging findings over 2, 5 and 11 years, respectively, support the diagnosis of LDD. Despite extensive expansion of the lesion with brainstem involvement, clinical signs in two boys were minimal, while one patient has cognitive impairment and a complex oculomotor disturbance. So far we found no evidence for an association with Cowden disease (CD). No germline PTEN mutations were detected in these children, but the amount of available biopsy tissue in patients 1 and 2 was insufficient for a complete genetic analysis of tumor tissue. In conclusion, LDD can usually be diagnosed by MRI. In view of the favourable natural history, a conservative "wait and see" strategy is justified, particularly if radical tumor resection is not possible. LDD is often not associated with CD and germline PTEN mutations seem not to be present in isolated LDD.
Abstract: Diffusion-weighted imaging (DWI) allows us to image the motion of tissue water. This has been used to demonstrate acute ischaemia. Diffusion imaging is also sensitive to water movement along neuronal tracts. Our objective was to map brain maturation in vivo using maps of apparent diffusion coefficient (ADC). We studied 22 children without neurological disease aged between 2 and 720 days. MRI was performed at 1.5 tesla. Multislice single-shot echoplanar DWI was performed at b 0 and 1000 s/mm(2). ADC maps were generated automatically and measurements were performed in the basal ganglia, frontal and temporal white matter and the pons. There was a decrease over time in water diffusion in the areas examined, most marked in the frontal (0.887-1.898 x 10(-3) mm(2)/s) and temporal (1.077-1.748 x 10(-3) mm(2)/s)lobes. There was little change, after an initial decrease, in the basal ganglia (0.690-1.336 x 10(-3) mm(2)/s). There was a difference in water diffusion between the anterior (0.687-1.581 x 10(-3) mm(2)/s) and posterior (0.533-1.393 x 10(-3) mm(2)/s) pons. These changes correlate well with those observed in progressive myelination: the increased water content probably reflects incomplete myelination and the decrease with time in water motion reflects the increase in myelinated brain.
Abstract: BACKGROUND AND PURPOSE: Diffusion tensor imaging measures the proton diffusivity and preferential orientation of the diffusion tensor. X-linked adrenoleukodystrophy is a demyelinating disease for which therapy depends on the onset and extension of demyelination. We investigated the ability of diffusion tensor imaging to detect changes in the demyelinated lesions and in the normal appearing white matter. METHODS: Diffusion tensor imaging of three related boys with X-linked adrenoleukodystrophy and seven age-matched control participants was performed. Isotropic diffusion (D') and fractional anisotropy (FA) values were determined in 18 regions of interest in the white matter of both hemispheres. RESULTS: In all the demyelinated white matter areas, a pattern with increased D' and loss of FA was found. For example, mean D' was 1.772 x 10(-3)mm(2)/s in patient 2 with blindness and extensive demyelination of the occipital white matter and was 0.693 x 10(-3)mm(2)/s in control participants (P =.01). In the same region, mean FA was 0.103 (0.464 in control participants, P <.0001). Significant alterations of D' and FA were also observed in normal appearing white matter. For example, mean D' was 0.802 x 10(-3)mm(2)/s in the parietal white matter of patient 1 with no visible alterations on T2-weighted images (0.715 x 10(-3) mm(2)/s in control patients, P =.03), whereas mean FA was 0.320 (0.400 in control participants, P =.003). CONCLUSION: Elevated D' and loss of FA revealed by diffusion tensor imaging are consistent with severe demyelination in patients with X-linked adrenoleukodystrophy. Significant alterations of D' and FA in normal appearing white matter may indicate early demyelination in areas that are not yet visibly altered on conventional MR images. Further evaluation in a larger series of patients and long-term study are needed.
Abstract: We report a 12-year-old boy who presented with incomplete right ophthalmoplegia, exophthalmos and headache. Initial CT and MRI revealed a mass in the right cavernous sinus. During tumour work-up, CT identified additional tumour within the mediastinum. Biopsy of the mediastinal lesion identified Burkitt's lymphoma. We report on this case because radiologists and clinicians should be alerted to identify sites of primary Burkitt's lymphoma outside of the central nervous system if clinical symptoms indicate, or imaging shows, CNS lesions. Primary CNS involvement in Burkitt's lymphoma is rare.
Abstract: Our aim was to assess the value of a new fast contrast-enhanced MR venography (CE-MRV) sequence in the investigation of normal and diseased cerebral veins. Conventional time-of-flight (TOF) MRV is time consuming, with imaging for a single sequence taking many minutes. MRI was performed with a clinical 1.5-T scanner; conventional TOF MRV followed by CE-MRV was performed using a modified 3D first-pass MR angiography sequence. Ten control subjects without cerebral pathology were studied as well as ten patients with cerebral venous thrombosis for a total of 20 studies with both sequences. CE-MRV was able to provide a set of complete MRV images in a significantly shorter time than conventional MRV sequencing could. The field of view also provided greater coverage of the vessels of the head and neck. CE-MRV also provided more extensive small vein detail and provided a better demonstration of intraluminal defects, despite a slightly lower resolution. Both methods were equally suited for the demonstration of venous thrombosis and demonstrated all cases equally well; however, CE-MRV provided more detailed information by showing partially obstructed sinuses and by showing better the presence of cortical collateral venous drainage.
Abstract: Rhombencephalosynapsis is a rare congenital abnormality characterised by dorsal fusion of the cerebellar hemispheres, agenesis or hypogenesis of the vermis, fusion of dentate nuclei and superior cerebellar peduncles. We describe 9 children, aged 1.5 to 6 years, with rhombencephalosynapsis. Isolated rhombencephalosynapsis was found in 2 patients, hydrocephalus in 3 children and another 3 children had ventriculomegaly. Additional supratentorial abnormalities were documented in 5 patients. Clinical findings ranged from mild truncal ataxia and normal cognitive abilities to severe cerebral palsy and mental retardation. No correlation between clinical findings and magnetic resonance imaging could be established so far.
Abstract: We performed functional MRI experiments using a mute version of a gradient echo sequence on adult volunteers using either a simple visual stimulus (flicker goggles: 4 subjects) or an auditory stimulus (music: 4 subjects). Because the mute sequence delivers fewer images per unit time than a fast echo planar imaging (EPI) sequence, we explored our data using a parametric ANOVA test and a non-parametric Wilcoxon-Mann-Whitney test in addition to performing a cross-correlation analysis. All three methods were in close agreement regarding the location of the BOLD contrast signal change. We demonstrated that, using appropriate statistical analysis, functional MRI using an MR sequence that is acoustically inaudible to the subject is feasible. Furthermore compared with the "silent" event-related procedures involving an EPI protocol, our mGE protocol compares favourably with respect to experiment time and the BOLD signal.
Abstract: The diagnosis of cerebral venous thrombosis is often difficult both clinically and radiologically and until now there is no method available to predict if brain lesions, detected clinically and using conventional brain imaging methods, may lead to full recovery, as expected in vasogenic edema or ischemic infarcts and even a hematoma. New fast neuroimaging techniques such as diffusion-weighted imaging (DWI) are sensitive to different reasons of changes in local tissular water concentration thus giving further insight into the pathophysiological mechanism as well as prognosis of cerebral venous thrombosis. We report the cases of 18 consecutive patients with a diagnosis of cerebral venous thrombosis based on clinical and imaging criteria. All patients underwent magnetic resonance imaging (MRI) of the brain, which comprised isotropic diffusion-weighted MR. Diffusion-weighted MRI showed positive findings in 17/18 cases. In 7 cases the clot could be directly visualized as an area of hyperintensity in the affected vein on DWI. In 7 cases DWI showed areas of signal loss corresponding to hematomas. In 6 cases DWI showed changes in signal intensity that were more subtle. In 4 cases of superficial venous thrombosis, there were areas of decreased ADC values (0.65-0.79 x 10(-3) mm(2)/s) whereas in 2 cases of deep venous thrombosis, increased DWI intensities could be found that corresponded to both an increase and a decrease in ADC, corresponding to a coexistence of cytotoxic and vasogenic edemas. Diffusion-weighted MRI can demonstrate directly the presence of an intravenous clot in a select number of patients. It can also demonstrate early ischemic changes, and can differentiate conventional T2-weighted MR areas of cytotoxic from vasogenic edema.
Abstract: N-acetylaspartate (NAA) contributes to the most prominent signal in proton magnetic resonance spectroscopy (1H-MRS) of the adult human brain. We report the absence of NAA in the brain of a 3-year-old child with neurodevelopmental retardation and moderately delayed myelination. Since normal concentration of NAA in body fluids is hardly detectable, 1H-MRS is a noninvasive technique for identifying neurometabolic diseases with absent NAA. This report puts NAA as a neuronal marker to question.
Abstract: The oculocerebrorenal syndrome of Lowe (OCRL) is an X-linked disorder characterized by major abnormalities of eyes, nervous system, and kidneys. We report two patients with typical intracranial lesions on MRI. The proton spectroscopy study of the periventricular white matter showed a moderate elevation of the signal at 3.56 ppm in the patient with cystic lesions. This resonance is usually assigned to myo-inositol and interpreted as a glial marker. In our patient it could also represent a true accumulation inside the cysts of phosphatidylinositol 4,5-biphosphate which is not degraded in patients with Lowe syndrome.
Abstract: We report a 20-year-old woman who suffered headaches before presenting with a state of fluctuating vigilance. MRI showed diffuse high signal in the basal ganglia bilaterally on diffusion- and T2-weighted images, which had areas of both low and high apparent diffusion coefficient, presumed to correspond to cytotoxic and vasogenic oedema. MR venography showed no flow in the deep cerebral veins or straight sinus. Heparin was given, with clinical recovery. On follow-up MRI, the appearances became normal.
Abstract: Diffusion-weighted MRI (DWI) is becoming important for assessment of acute stroke. Until recently single-shot DWI required expensive technology such as echoplanar imaging (EPI) available only at some research sites. A new medium-field (1.0 T) short-bore MR imager has been developed with which DWI data sets can be acquired. We prospectively studied 169 patients on this 1.0 T commercial system. After conventional imaging, DWI was performed with a single-shot multi-slice sequence with b values 0 an 900 s/mm2, and with the gradients switched in three directions. The apparent diffusion coefficients were calculated with online calculation software. There were 50 patients with totally normal MRI, and 17 had strokes, these strokes were detected as areas of high signal on the images at a maximal b value. There was a drop in the ADC in ischaemic regions: in sub-acute infarcts, the values were between 0.41 and 0.531 x 10(-3) mm2/s. In old infarcts the ADC was 1.15 x 10(-3) mm2/s. Cerebrospinal fluid (CSF) gave low signal whereas areas in the brain had more intermediate intensities (CSF: 3.00; deep white matter: 0.75, cortical grey matter: 0.80, basal ganglia (thalamus): 0.70 and cerebellar white matter: 0.65 x 10(-3) mm2/s. Anisotropy was detected as areas of restricted diffusion along the tracts. These preliminary data show that DWI can be acquired successfully on a medium-field short-bore system. This should allow the technique to be implemented at more sites, therefore facilitating the diagnosis of acute stroke and rendering early intervention feasible.
