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Background: Existing volumetric measurements of plexiform neurofibromas (PNs) are time consuming and error prone, as they require delineation of PN boundaries, a procedure that is not practical in the typical clinical setting. The aim of this study is to assess the Plexiform Neurofibroma Instant Segmentation Tool (PNist), a novel semi-automated segmentation program that we developed for PN delineation in a clinical context. PNist was designed to greatly simplify volumetric assessment of PNs through use of an intuitive user interface while providing objectively consistent results with minimal interobserver and intraobserver variabilities in reasonable time. Materials and methods: PNs were measured in 30 magnetic resonance imaging (MRI) scans from 12 patients with neurofibromatosis 1. Volumetric measurements were performed using PNist and compared to a standard semi-automated volumetric method (Analyze 9.0). Results: High correlation was detected between PNist and the semi-automated method ( R = 0.996), with a mean volume overlap error of 9.54 % and low intraobserver and interobserver variabilities. The segmentation time required for PNist was 60 % of the time required for Analyze 9.0 (360 versus 900 s, respectively). PNist was also reliable when assessing changes in tumor size over time, compared to the existing commercial method. Conclusions: Our study suggests that the new PNist method is accurate, intuitive, and less time consuming for PN segmentation compared to existing commercial volumetric methods. The workflow is simple and user-friendly, making it an important clinical tool to be used by radiologists, neurologists and neurosurgeons on a daily basis, helping them deal with the complex task of evaluating PN burden and progression. [ABSTRACT FROM AUTHOR]

Objective:To compare echogenicity detected using cranial ultrasound (cUS) and diffuse excessive high signal intensity (DEHSI) detected using magnetic resonance imaging (MRI) by identical region-based scoring criteria in preterm infants. To explore the association between these white matter (WM) signal changes with early neurobehavior.Study Design:Forty-nine pre-selected premature infants with only echogenicity on a first routine cUS1 underwent MRI and a repeated cUS2 at term equivalent age. Echogenicity and DEHSI were graded in various brain areas and diffusivity values were calculated. Neurobehavior was assessed using the Rapid Neonatal Neurobehavioral Assessment Procedure.Result:WM signal changes were significantly higher on cUS1 than cUS2; and higher in MRI than cUS2 in posterior regions. Infants with DEHSI demonstrated reduced tissue integrity. Imaging findings were not correlated with early neurobehavior.Conclusion:Echogenicity and DEHSI likely represent the same phenomenon. Reduction of over-interpretation of WM signal changes may help define criteria for the judicious use of imaging in routine follow-up of premature infants. [ABSTRACT FROM AUTHOR]

Background: Acute disseminated encephalomyelitis (ADEM) is a demyelinating disorder of the central nervous system (CNS). Differentiating ADEM from other inflammatory disorders, such as multiple sclerosis, is not always conclusive using conventional MRI.Objective: To evaluate longitudinal magnetic resonance spectroscopy (MRS) changes that distinguish ADEM from other inflammatory disorders.Materials and Methods: MRI/MRS scans were performed in seven patients with ADEM during the acute and chronic phases of the disease.Results: Partial recovery was detected between the acute and chronic phases in choline/creatine ratio. Major elevation of lipids and reduction in myo-inositol/creatine ratio was detected in all patients during the acute phase, followed by a reduction in lipids peak and elevation above normal in myo-inositol/creatine ratio during the chronic phase.Conclusion: Consistent and unique MRS changes in metabolite ratios between the acute and chronic presentations of the disease were found. To the best of our knowledge, these patterns have not been described in other inflammatory disorders and might assist in the early diagnosis of ADEM. [ABSTRACT FROM AUTHOR]

Cochlear dysplasia is a rare congenital anomaly. However, early detection of this anomaly can prevent serious consequences. We describe three cases of cochlear dysplasia that presented with recurrent meningitis and cerebro-spinal fluid (CSF) leak in children in which early diagnosis prevented further complications. [ABSTRACT FROM AUTHOR]

The article presents a case study of an 11-year-old boy who underwent magnetic resonance imaging (MRI) because of bilateral elevation of his optic nerve being suspected as papilledema. The patient has been treated with acetazolamide. Any pathological findings are absent in the MR scan performed three months prior to the lumbar puncture. It adds that follow-up MR scan has revealed the restored position of the patient's cerebellar tonsils and renormalization of his posterior fossa morphology.

Background: Tuberous sclerosis complex (TSC) is a genetic disorder caused by inactivating mutations in the TSC1 or TSC2 genes and characterized by slow-growing tumors in multiple organs. Of the affected individuals, 10% display subependymal giant cell astrocytomas (SEGAs), which can lead to substantial neurological morbidity. The TSC1/TSC2 protein complex is a negative regulator of the mTOR pathway. Hence, mutations in these genes in preclinical models are associated with increased mTOR pathway activation and heightened sensitivity to mTOR inhibitors. We hereby report our experience with RAD001 (Everolimus) therapy, a novel mTOR inhibitor, in inducing a dramatic regression of SEGAs. Methods: A patient with TSC and SEGAs was treated with 10 mg/day oral RAD001. MRIs and neuro-ophtalmological exams were performed before and at regular intervals following the initiation of therapy. Results: The lesions exhibited significant regression in several tumor locations and stabilization in others, accompanied with an improvement of his visual status. Treatment was well tolerated for 11 months but was than discontinued due to hypertension and elevated CPK, without evidence for rhabdomyolysis. Yet, during 9 months following the interruption of therapy, SEGAs remained unchanged. Conclusions: Oral RAD001 demonstrated preliminary encouraging results as treatment of astrocytomas associated with TSC. These preliminary results were recently supported by the Novartis announcement of the phase II study of RAD001 for SEGAs, which was not published yet. According to their statement, 75% of the patients showed reduction of SEGAs' volume following treatment with RAD001. Based on these results, RAD001 may be an alternative to surgery in selected patients with TSC and SEGAs. [ABSTRACT FROM AUTHOR]

Acute abdomen is caused by a wide variety of etiologies, many of which require surgical intervention. Two boys were hospitalized for acute abdominal pain and low-grade fever. Physical examination revealed epigastric and right upper abdominal fullness, and laboratory studies showed elevated erythrocyte sedimentation rates, with normal leukocyte counts and coagulation profiles. Abdominal ultrasound and computerized tomography revealed pseudotumor in both cases. Symptomatic treatment and cefazolin were administered, and pain and fever subsided after 6 to 10 days. Pseudotumor due to omental infarction can present as acute abdomen in children. A high index of suspicion and the use of both ultrasonography and computerized tomography will help avoid unnecessary surgical intervention in such cases. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 32-year-old male patient presented with progressive sensory-motor disturbances of the lower limbs. The patient has a history of lung sarcoidosis and was found with multifocal pial lesions with a large intramedullary lesion after magnetic resonance imaging (MRI). The clinical implications of postoperative hematomyelia are discussed.

A correction to the article "Tumor burden evaluation in NF1 patients with plexiform neurofibromas in daily clinical practice" that was published in the March 2015 issue is presented.