Performance of the beta-glucan test for the diagnosis of invasive fusariosis and scedosporiosis: a meta-analysis.

The (1→3)-ß-D-glucan (BDG) is a component of the fungal cell wall that can be detected in serum and used as an adjunctive tool for the diagnosis of invasive mold infections (IMI) in patients with hematologic cancer or other immunosuppressive conditions. However, its use is limited by modest sensitivity/specificity, inability to differentiate between fungal pathogens, and lack of detection of mucormycosis. Data about BDG performance for other relevant IMI, such as invasive fusariosis (IF) and invasive scedosporiosis/lomentosporiosis (IS) are scarce. The objective of this study was to assess the sensitivity of BDG for the diagnosis of IF and IS through systematic literature review and meta-analysis. Immunosuppressed patients diagnosed with proven or probable IF and IS, with interpretable BDG data were eligible. A total of 73 IF and 27 IS cases were included. The sensitivity of BDG for IF and IS diagnosis was 76.7% and 81.5%, respectively. In comparison, the sensitivity of serum galactomannan for IF was 27%. Importantly, BDG positivity preceded the diagnosis by conventional methods (culture or histopathology) in 73% and 94% of IF and IS cases, respectively. Specificity was not assessed because of lacking data. In conclusion, BDG testing may be useful in patients with suspected IF or IS. Combining BDG and galactomannan testing may also help differentiating between the different types of IMI.

IF and IS are severe fungal infections for which diagnosis is often delayed. This meta-analysis shows that beta-glucan testing in serum had a sensitivity of about 80% for IF/IS and could detect the disease earlier compared to conventional diagnostic tests.

[Updated AWMF Guideline on the Diagnosis and Treatment of Langerhans cell Histiocytosis in Children and Adolescents]. / Aktualisierte AWMF Leitlinie zur Diagnostik und Therapie der Langerhanszell Histiozytose (LCH) im Kindes- und Jugendalter.

Langerhans cell Histiocytosis is a rare neoplastic disease, which occurs mainly in children and adolescents. The disease may affect any organ, and therefore, the clinical symptoms vary widely. Some patients have a spontaneous remission of the disease, whereas others experience a rapid and potentially lethal clinical course. The therapeutic approach depends on the extent of the disease, and reaches from a watch-and-wait strategy to chemotherapy with the standard drugs vinblastine and prednisone. The identification of mutations in the MAPK-pathway resulted in growing interest in targeted therapy using compounds such as the BRAF inhibitors. Chronic relapses and permanent sequelae are important problems of LCH and are the focus of current research.

Abusive head trauma: experience improves diagnosis.

PURPOSE: The diagnosis of abusive head trauma (AHT) is complex and neuroimaging plays a crucial role. Our goal was to determine whether non-neuroradiologists with standard neuroradiology knowledge perform as well as neuroradiologists with experience in pediatric neuroimaging in interpreting MRI in cases of presumptive AHT (pAHT). METHODS: Twenty children were retrospectively evaluated. Patients had been diagnosed with pAHT (6 patients), non-abusive head trauma-NAHT (5 patients), metabolic diseases (3 patients), and benign enlargement of the subarachnoid spaces (BESS) (6 patients). The MRI was assessed blindly, i.e., no clinical history was given to the 3 non-neuroradiologists and 3 neuroradiologists from 2 different institutions. RESULTS: Blindly, neuroradiologists demonstrated higher levels of sensitivity and positive predictive value in the diagnosis of pAHT (89%) than non-neuroradiologists (50%). Neuroradiologists chose correctly pAHT as the most probable diagnosis 16 out of 18 times; in contrast, non-neuroradiologists only chose 9 out of 18 times. In our series, the foremost important misdiagnosis for pAHT was NAHT (neuroradiologists twice and non-neuroradiologists 5 times). Only victims of motor vehicle accidents were blindly misdiagnosed as pAHT. No usual household NAHT was not misdiagnosed as pAHT. Neuroradiologists correctly ruled out pAHT in all cases of metabolic diseases and BESS. CONCLUSION: MRI in cases of suspected AHT should be evaluated by neuroradiologists with experience in pediatric neuroimaging. Neuroradiologists looked beyond the subdural hemorrhage (SDH) and were more precise in the assessment of pAHT and its differential diagnosis than non-neuroradiologists were. It seems that non-neuroradiologists mainly assess whether or not a pAHT is present depending on the presence or absence of SDH.

Pediatric CNS imaging and long-term effects of irradiation in pediatric oncology patients.

BACKGROUND: The aim of this study was to evaluate post-irradiation changes in the central nervous system (CNS) detected using magnetic resonance (MR) imaging. METHODS: Magnetic resonance images of 15 children with CNS tumors treated through whole-brain irradiation over 10 years were reviewed retrospectively. Variables such as age at the time of irradiation, total radiation dose, treatment length, and time interval between irradiation and MR changes, were evaluated. RESULTS: All patients included in the study had imaging abnormalities of the CNS. Eight patients (53%) developed CNS abnormalities within a short period of time - only a few months after irradiation (mean 4.8 months). Seven patients (47%) developed CNS abnormalities within a long time interval after treatment (mean 4.6 years). In almost all patients, a T2 increase in supra- and infratentorial white matter was observed. Follow-up examinations showed nine patients (60%) with cerebellar atrophy. CONCLUSIONS: In this sample of pediatric patients who underwent whole-brain irradiation, the time receiving irradiation was not related to the severity of the MR changes. A correlation between the age of the child or the length of the radiotherapy and the extent of the changes could not be confirmed. However, we observed a trend towards stronger brain parenchymal degeneration with cystic changes in the younger age group of children in our sample. Older children who received irradiation seem to be more susceptible to vascular dysplasia with cavernous hemangiomas and microbleeding.

The role of magnetic resonance imaging in the diagnosis of central nervous system involvement in children with acute lymphoblastic leukemia.

PURPOSE: Acute lymphoblastic leukemia (ALL) is the most frequent malignancy in childhood. As central nervous system (CNS) involvement requires an intensified CNS-targeted therapy, timely diagnosis is essential. The aim of this retrospective analysis was to evaluate whether cranial magnetic resonance imaging (MRI) examinations findings correlate with cerebrospinal fluid (CSF) analysis on CNS involvement and whether MRI examinations reveal incidental findings with a clinical consequence. METHODS: All pediatric patients with ALL at our institution between 1998 and 2016 were identified. Patients were divided into two groups: de novo and relapsed ALL. Both groups were analyzed separately for the presence of CNS involvement. Incidental findings were also evaluated. RESULTS: Two hundred fifteen patients with de novo ALL and 31 with relapsed ALL were identified. In the de novo group, no patient was diagnosed CNS positive based on MRI results alone. In relapsed patients, only one patient had a positive MRI with negative CSF results and no neurological symptoms, thus was classified CNS positive solely on the basis of the MRI. In both groups, no patient showed an incidental finding that required therapy. CONCLUSION: In our study, MRI examinations do not improve the detection of CNS involvement compared with CSF analysis alone. In addition, the analysis of incidental findings does not add value to the performance of an MRI examination performed prior to treatment. Overall, MRI prior to treatment in pediatric patients with ALL is not necessary.

European Society of Paediatric Radiology 2019 strategic research agenda: improving imaging for tomorrow's children.

The European Society of Paediatric Radiology (ESPR) research committee was established to initiate, drive forward and foster excellence in paediatric imaging, paediatric image-guided intervention and radiation protection research, by facilitating more evidence-based standards, protocols and multi-institutional collaborations. The ESPR Strategic Research Agenda outlines our current research approach, highlighting several areas of paediatric imaging where the society can help guide current and future research, and emphasizing those areas where early research ("seed") funding may need to be allocated by this and other societies as precursors to larger grant applications. The key aims are to evaluate normal variation in order to be able to confidently diagnose disease states, develop robust image-based classification systems to aid diagnosis and treatment monitoring, and help develop evidence-based clinical guidelines using current literature and experience to identify knowledge gaps. For this reason, the development of evidence-based imaging pipelines, broken down step-by-step to include diagnosis, classification and clinical effectiveness, should be the end goal for each disease entity for each affected child. Here, we outline the 2019 ESPR Strategic Research Agenda along three points in the clinical imaging pipeline: clinical referral, disease diagnosis and evolution, and clinical therapeutic evaluation and effectiveness. Through multicentre trials, using existing high-level experience and expertise, and nurturing the next generation of researchers, we will be able to achieve these aims.

Quantitative T2, T2*, and T2' MR imaging in patients with ischemic leukoaraiosis might detect microstructural changes and cortical hypoxia.

INTRODUCTION: Quantitative MRI with T2, T2*, and T2' mapping has been shown to non-invasively depict microstructural changes (T2) and oxygenation status (T2* and T2') that are invisible on conventional MRI. Therefore, we aimed to assess whether T2 and T2' quantification detects cerebral (micro-)structural damage and chronic hypoxia in lesions and in normal appearing white matter (WM) and gray matter (GM) of patients with ischemic leukoaraiosis (IL). Measurements were complemented by the assessment of the cerebral blood flow (CBF) and the degree of GM and WM atrophy. METHODS: Eighteen patients with IL and 18 age-matched healthy controls were included. High-resolution, motion-corrected T2, T2*, and T2' mapping, CBF mapping (pulsed arterial spin labeling, PASL), and segmentation of GM and WM were used to depict specific changes in both groups. All parameters were compared between patients and healthy controls, using t testing. Values of p < 0.05 were accepted as statistically significant. RESULTS: Patients showed significantly increased T2 in lesions (p < 0.01) and in unaffected WM (p = 0.045) as well as significantly increased T2* in lesions (p = 0.003). A significant decrease of T2' was detected in patients in unaffected WM (p = 0.027), while no T2' changes were observed in GM (p = 0.13). Both unaffected WM and GM were significantly decreased in volume in the patient-group (p < 0.01). No differences of PASL-based CBF could be shown. CONCLUSION: Non-invasive quantitative MRI with T2, T2*, and T2' mapping might be used to detect subtle structural and metabolic changes in IL. Assessing the grade of microstructural damage and hypoxia might be helpful to monitor disease progression and to perform risk assessment.

Central nervous system imaging in childhood Langerhans cell histiocytosis - a reference center analysis.

BACKGROUND: The aim of our study was (1) to describe central nervous system (CNS) manifestations in children with Langerhans cell histiocytosis (LCH) based on images sent to a reference center and meeting minimum requirements and (2) to assess the inter-rater agreement of CNS-MRI results, which represents the overall reproducibility of this investigation. METHODS: We retrospectively reviewed brain MRI examinations in children with LCH, for which MRI minimum requirements were met. Abnormalities were rated by two experienced neuroradiologists, and the inter-rater agreement was assessed. RESULTS: Out of a total of 94 imaging studies, only 31 MRIs met the minimum criteria, which included T2w, FLAIR, T1w images before/after contrast in at least two different section planes, and thin post contrast sagittal slices T1w through the sella. The most common changes were osseous abnormalities, followed by solid enlargement of the pineal gland, thickened enhancing stalk and signal changes of the dentate nucleus. Whereas inter-rater agreement in assessing most of the CNS lesions was relatively high (κ > 0.61), the application of minimum criteria often did not allow to evaluate the posterior pituitary. CONCLUSIONS: The diversity of radiological protocols from different institutions leads to difficulties in the diagnosis of CNS abnormalities in children with LCH. Although the inter-rater agreement between neuroradiologists was high, not all the LCH manifestations could be completely ruled out when using the minimum criteria. Brain MRIs should therefore follow LCH guideline protocols and include T1 pre-gadolinium sagittal images, and be centrally reviewed in order to improve the comparison of clinical trials.

Odontoid infiltration and spinal compression in Farber Disease: reversal by haematopoietic stem cell transplantation.

Farber disease (FD) is a lysosomal storage disorder caused by accumulation of ceramide in various organs and tissues, most notably the central nervous system, subcutaneous tissues and respiratory tract. We report a girl who developed major destructive bone involvement, which affected the odontoid process and produced spinal compression at 9 years of age. Bone involvement was proven histologically but resolved, as assessed by serial MRI scanning, following matched unrelated donor haematopoietic stem cell transplantation. This transplant resulted in only partial donor chimerism (less than 10 % donor cells in peripheral blood), yet this was sufficient to almost normalize acid ceramidase levels in leukocytes and to produce dramatic improvements in subcutaneous nodules and joint mobility as well as the beneficial effect on the involved bone. Unfortunately, the transplant was rejected after 2 years but the patient was rescued from an aplastic state by successful haploidentical peripheral blood stem cell transplantation and remained a full donor chimera without recurrence of the bone involvement and with steadily improving mobility at the age of 17 years. We describe an FD patient who presented with severe destruction of the odontoid by inflammatory tissue which was reversed after long-term control achieved by allogeneic hematopoietic stem cell transplantation. After extensive literature search, we believe that this is the first report of bony involvement in Farber disease.

Focally Enlarged Perivascular Spaces in Pediatric and Adolescent Patients with Polymicrogyria-an MRI Study.

PURPOSE: Polymicrogyria (PMG) is a cortical malformation frequently associated with epilepsy. Our aim was to investigate the frequency and conspicuity of enlarged perivascular spaces (EPVS) underneath dysplastic cortex as a potentially underrecognized feature of PMG in pediatric and adolescent patients undergoing clinical magnetic resonance imaging (MRI). METHODS: We analyzed data from 28 pediatric and adolescent patients with PMG and a matched control group, ranging in age from 2 days to 21 years, who underwent MRI at 1.5T or 3T. T2-weighted MR images were examined for the presence of EPVS underneath the dysplastic cortex. The quantity of EPVS was graded from 0 to 4 (0: none, 1: < 10, 2: 11-20, 3: 21-40, 4: > 40 EPVS). We then compared the presence and quantity of EPVS to the matched controls in terms of total EPVS scores, and EPVS scores underneath the dysplastsic cortex depending on the age groups, the localization of PMG, and the MRI field strength. RESULTS: In 23/28 (82%) PMG patients, EPVS spatially related to the dysplastic cortex were identified. EPVS scores were significantly higher in PMG patients compared to controls, independent from age or PMG location. No significant differences were observed in EPVS scores in patients examined at 1.5T compared to those examined at 3T. CONCLUSION: EPVS underneath the dysplastic cortex were identified in 82% of patients. EPVS may serve as an important clue for PMG and a marker for cortical malformation.

Characterization of MRI White Matter Signal Abnormalities in the Pediatric Population.

(1) Background and Purpose: The aim of this study was to retrospectively characterize WMSAs in an unselected patient cohort at a large pediatric neuroimaging facility, in order to learn more about the spectrum of the underlying disorders encountered in everyday clinical practice. (2) Materials and Methods: Radiology reports of 5166 consecutive patients with standard brain MRI (2006-2018) were searched for predefined keywords describing WMSAs. A neuroradiology specialist enrolled patients with WMSAs following a structured approach. Imaging characteristics, etiology (autoimmune disorders, non-genetic hypoxic and ischemic insults, traumatic white matter injuries, no final diagnosis due to insufficient clinical information, "non-specific" WMSAs, infectious white matter damage, leukodystrophies, toxic white matter injuries, inborn errors of metabolism, and white matter damage caused by tumor infiltration/cancer-like disease), and age/gender distribution were evaluated. (3) Results: Overall, WMSAs were found in 3.4% of pediatric patients scanned at our and referring hospitals within the ten-year study period. The majority were found in the supratentorial region only (87%) and were non-enhancing (78% of CE-MRI). WMSAs caused by autoimmune disorders formed the largest group (23%), followed by "non-specific" WMSAs (18%), as well as non-genetic hypoxic and ischemic insults (17%). The majority were therefore acquired as opposed to inherited. Etiology-based classification of WMSAs was affected by age but not by gender. In 17% of the study population, a definite diagnosis could not be established due to insufficient clinical information (mostly external radiology consults). (4) Conclusions: An "integrated diagnosis" that combines baseline demographics, including patient age as an important factor, clinical characteristics, and additional diagnostic workup with imaging patterns can be made in the majority of cases.

Adult-type and Pediatric-type Diffuse Gliomas : What the Neuroradiologist Should Know.

The classification of diffuse gliomas into the adult type and the pediatric type is the new basis for the diagnosis and clinical evaluation. The knowledge for the neuroradiologist should not remain limited to radiological aspects but should be based additionally on the current edition of the World Health Organization (WHO) classification of tumors of the central nervous system (CNS). This classification defines the 11 entities of diffuse gliomas, which are included in the 3 large groups of adult-type diffuse gliomas, pediatric-type diffuse low-grade gliomas, and pediatric-type diffuse high-grade gliomas. This article provides a detailed overview of important molecular, morphological, and clinical aspects for all 11 entities, such as typical genetic alterations, age distribution, variability of the tumor localization, variability of histopathological and radiological findings within each entity, as well as currently available statistical information on prognosis and outcome. Important differential diagnoses are also discussed.

Detectability of multiple sclerosis lesions with 3T MRI: a comparison of proton density-weighted and FLAIR sequences.

OBJECTIVE: Due to the high signal intensity of cerebrospinal fluid (CSF), proton density-weighted (PD-w) images with long repeat times (TR) may be less able to detect periventricular lesions in patients with multiple sclerosis (MS). However, we have found good detectability of MS lesions with PD-w using long TR at 3 Tesla (3 T). For this reason, the aim of this study was to prospectively investigate the detectability of MS lesions at 3 T in PD-w compared with fluid-attenuated inversion recovery (FLAIR) sequences. PATIENTS AND METHODS: A total of 11 MS patients were examined by a 3T magnetic resonance (MR) scanner, and their MS lesions were prospectively analyzed on PD-w and FLAIR images by two evaluators; detectability was rated by a three-point scoring system. The Wilcoxon signed-rank test was used for comparisons, and the level of significance was P<0.05. RESULTS: Significantly more lesions were detectable on PD-w images (P<0.001 for both evaluators). In particular, PD-w was superior to FLAIR for the detection of periventricular (P=0.001 and P=0.013 for each evaluator respectively) and infratentorial (P<0.001 for both evaluators) lesions. CONCLUSION: This was the first study to compare FLAIR and PD-w with long TR at 3 T; it revealed that PD-w is superior for detecting infratentorial and even periventricular MS lesions, despite the higher signal intensity of CSF. This might be due to the high spin density of MS lesions, thus distinguishing them from the surrounding brain tissue. For this reason, double-echo T2-weighted sequences at 3T are recommended to improve the detectability of MS lesions.

[Severe courses of meningitis and encephalitis in children and adolescents]. / Schwer verlaufende Meningitis und Enzephalitis bei Kindern und Jugendlichen.

Bacterial meningitis and viral encephalitis are infectious diseases of the central nervous system (CNS), mostly with severe sequelae up to a fatal outcome. Despite progress in prevention by vaccination, the global impact of bacterial meningitis is enormous. Before the coronavirus disease 2019 (COVID-19) pandemic, the incidence of viral encephalitis in childhood was increasing also due to the growing incidence of emerging pathogens, such as enterovirus (EV)-A71 and West Nile virus in temperate climates as well as the wider use of immunosuppressive treatment and stem cell transplantation in childhood. The following article summarizes the data on the frequency and clinical signs of infectious CNS diseases and presents the current treatment recommendations.

Rare brain biopsy findings in a first ADEM-like event of pediatric MS: histopathologic, neuroradiologic and clinical features.

Pediatric MS tends to present more often with an acute onset and a polysymptomatic form of the disease, possibly with encephalopathy and large tumefactive lesions similar to those observed in some cases of acute disseminated encephalomyelitis (ADEM), which makes it more difficult to differentiate between an explosive and severe onset of MS vs. ADEM. An ADEM-like first demyelinating event can be the first attack of pediatric MS, but international consensus definitions require two or more non-ADEM demyelinating events for diagnosis of MS. In our patient KIDMUS MRI criteria for MS (Mikaeloff et al. J Pediatr 144:246-252, 2004a; Mikaeloff et al. Brain 127:1942-1947, 2004b) were negative at first attack, but Barkhof criteria for lesion dissemination in space in adults (Barkhof et al. 120:2059-2069, 1997), Callen modified MS-criteria and Callen MS-ADEM criteria for children (Callen et al. Neurology 72:961-967, 2009a; Callen et al. Neurology 72:968-973, 2009b) were positive suggesting pediatric MS. As the clinical course was devastating with non-responsiveness upon high-dose immune modulatory therapy and due to the absence of an alternative diagnosis other than demyelinating disease brain biopsy was performed. Brain biopsy studies or autopsy case reports of fulminant pediatric MS patients are extremely rare. Histopathology revealed an inflammatory demyelinating CNS process with confluent demyelination, indicating the likelihood of a relapsing disease course compatible with an acute to subacute demyelinating inflammatory disease. This pattern was corresponding to the early active multiple sclerosis subtype I of Lucchinetti et al. (Ann Neurol 47(6):707-717, 2000).

Magnetic Resonance Imaging as the Primary Imaging Modality in Children Presenting with Inflammatory Nontraumatic Atlantoaxial Rotatory Subluxation.

Inflammatory nontraumatic atlantoaxial rotatory subluxation (AAS) in children is an often-missed diagnosis, especially in the early stages of disease. Abscess formation and spinal cord compression are serious risks that call for immediate surgical attention. Neither radiographs nor non-enhanced computed tomography (CT) images sufficiently indicate inflammatory processes. Magnetic resonance imaging (MRI) allows a thorough evaluation of paraspinal soft tissues, joints, and ligaments. In addition, it can show evidence of vertebral distraction and spinal cord compression. After conducting a scoping review of the literature, along with scientific and practical considerations, we outlined a standardized pediatric MRI protocol for suspected inflammatory nontraumatic AAS. We recommend contrast-enhanced MRI as the primary diagnostic imaging modality in children with signs of torticollis in combination with nasopharyngeal inflammatory or ear nose and throat (ENT) surgical history.

Central Nervous System Mold Infections in Children with Hematological Malignancies: Advances in Diagnosis and Treatment.

The incidence of invasive mold disease (IMD) has significantly increased over the last decades, and IMD of the central nervous system (CNS) is a particularly severe form of this infection. Solid data on the incidence of CNS IMD in the pediatric setting are lacking, in which Aspergillus spp. is the most prevalent pathogen, followed by mucorales. CNS IMD is difficult to diagnose, and although imaging tools such as magnetic resonance imaging have considerably improved, these techniques are still unspecific. As microscopy and culture have a low sensitivity, non-culture-based assays such as the detection of fungal antigens (e.g., galactomannan or beta-D-glucan) or the detection of fungal nucleic acids by molecular assays need to be validated in children with suspected CNS IMD. New and potent antifungal compounds helped to improve outcome of CNS IMD, but not all agents are approved for children and a pediatric dosage has not been established. Therefore, studies have to rapidly evaluate dosage, safety and efficacy of antifungal compounds in the pediatric setting. This review will summarize the current knowledge on diagnostic tools and on the management of CNS IMD with a focus on pediatric patients.

Extracerebellar MRI-lesions in ataxia telangiectasia go along with deficiency of the GH/IGF-1 axis, markedly reduced body weight, high ataxia scores and advanced age.

Ataxia telangiectasia (AT) is a rare autosomal recessive disorder characterized by progressive ataxia, neurodegeneration, immunodeficiency, and cancer predisposition. Pathoanatomical studies reported a degeneration of cerebellar Purkinje cells as the striking feature of the disease. Although recent studies suggested the involvement of extracerebellar structures such as the brainstem and basal ganglia, this has rarely been studied in human AT. Thus, we performed a detailed cliniconeuroradiological investigation of 11 AT patients, aged 8 to 26 years by collecting clinical neurological data, ataxia scores, growth status, body mass index (BMI), growth hormone (GH), and insulin-like-growth factor 1 (IGF-1) and correlated them to extracerebellar neuroimaging findings in human AT. Neuroimaging was done by cranial and spine magnetic resonance imaging (MRI) with T1- and T2-weighted spin-echo and fluid attenuated inversion recovery sequences. We compared clinical and neuroradiological findings of six patients with IGF-1 levels and BMI below the third percentile to five patients with normal IGF-1 serum levels and BMI above the third percentile. Three of the six first mentioned patients older than 20 years and two patients older than 12 years showed noticeable high Klockgether ataxia scores above 25 points. Three of these patients presented with marked hyperintense lesions in the cerebral white matter of T2-weighted MR images. Interestingly, all six patients suffered from marked spinal atrophy. Two of the patients presented with severe extra-pyramidal symptoms, but only one patient showed associated MRI abnormalities of the basal ganglia. MRI in patients with normal IGF-1 levels showed the expected cerebellar lesions in four patients, whereas spinal atrophy was found only in two patients. There was no affection of the cerebral white matter or basal ganglia in this group. We conclude that central cerebral white matter affection, spinal atrophy, and extrapyramidal symptoms are more often present in patients with pronounced deficiency of the GH/IGF-1 axis accompanied by markedly reduced body weight and high ataxia scores. This may point to a major role of IGF-1 and nutritional status in neuroprotective signaling.

Spectroscopy of untreated pilocytic astrocytomas: do children and adults share some metabolic features in addition to their morphologic similarities?

OBJECTIVE: Pilocytic astrocytomas may show heterogeneous histopathological and imaging features which are commonly attributed to malignant gliomas. Using magnetic resonance (MR) spectroscopy, we assessed if pilocytic astrocytomas show increased choline (tCho), classically related to proliferation and malignancy of gliomas. METHODS: Sixteen patients (five adults, age 20-55 years and 11 children, age 6 months-15 years) with histologically proven pilocytic astrocytomas were evaluated retrospectively. MR spectroscopy was performed prior to surgery or biopsy in all patients. Intensities of tCho and total creatine (tCr) signals were normalised to the respective signal intensity of contralateral brain tissue and statistically evaluated for group differences between adults and children. RESULTS: The tCho levels covered a wide range with a trend towards elevated values, especially in the adult group. tCho levels ranged from 0.78 to 2.92 in the paediatric group (mean 1.15) and from 1.15 to 3.03 in the adult group (mean 1.35). Diminished or normal tCr values were observed in all patients but one. CONCLUSIONS: The well-known positive correlation between increase of tCho and the grade of gliomas seems to be violated by WHO grade I pilocytic astrocytomas showing a wide range of tCho values with an even marked increase in some cases. No significant differences have been identified in the MR spectroscopy metabolite profiles between paediatric and adult pilocytic astrocytomas.

Leptomeningeal metastases in pediatrics: magnetic resonance image manifestations and correlation with cerebral spinal fluid cytology.

BACKGROUND: Detection of leptomeningeal metastases is fundamental to a complete evaluation of central nervous system (CNS) or non-CNS tumor with suspected involvement of the neuroaxis. Our purpose was to assess the appearances of different magnetic resonance (MR) sequences in the diagnosis of leptomeningeal metastases and correlate those positive findings with the cerebral spinal fluid (CSF) cytology results. METHODS: The authors reviewed the medical records and MR image manifestations of leptomeningeal metastases from 18 children who had positive MR findings and retrospectively correlated them with CSF cytologic results. There was a uniform MR protocol and the patients were examined with the same sequences. RESULTS: The abnormalities included pial-arachnoid disease (n = 16), disease coating the nerves (n = 12), hydrocephalus (n = 3) and subependymal metastases (n = 2). Enhanced T1 images were better than unenhanced fluid attenuated inversion recovery (FLAIR) and T2 to delineate cranial and spinal leptomeningeal metastases. In our sample, seven out of 18 cases were cytologically negative on a single lumbar puncture. CONCLUSIONS: Contrast-enhanced MR imaging can be invaluable, detecting the false-negative lumbar punctures. FLAIR and diffusion images can be helpful in diagnosing leptomeningeal metastases of non-enhancing primary tumors. Prognosis was more related to the primary tumor type than to the leptomeningeal enhancement MR pattern.