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Background: T2-weighted fast spin-echo imaging (T2-W FSE) is frequently degraded by motion in pediatric patients. MR imaging with periodically rotated overlapping parallel lines with enhanced reconstruction (PROPELLER) employs alternate sampling of k-space to achieve motion reduction., Objective: To compare T2-W PROPELLER FSE (T2-W PROP) with conventional T2-W FSE for: (1) image quality; (2) presence of artefacts; and (3) ability to detect lesions., Materials and Methods: Ninety-five pediatric patients undergoing brain MRI (1.5 T) were evaluated with T2-W FSE and T2-W PROP. Three independent radiologists rated T2-W FSE and T2-W PROP, assessing image quality, presence of artefacts, and diagnostic confidence. Chi-square analysis and Wilcoxon signed rank test were used to assess the radiologists' responses., Results: Compared with T2-W FSE, T2-W PROP demonstrated better image quality and reduced motion artefacts, with the greatest benefit in children younger than 6 months. Although detection rates were comparable for the two sequences, blood products were more conspicuous on T2-W FSE. Diagnostic confidence was higher using T2-W PROP in children younger than 6 months. Average inter-rater agreement was 87%., Conclusion: T2-W PROP showed reduced motion artefacts and improved diagnostic confidence in children younger than 6 months. Thus, use of T2-W PROP rather than T2-W FSE should be considered in routine imaging of this age group, with caution required in identifying blood products.

Background: The direct quantitative correlation between thickness of the corpus callosum and volume of cerebral white matter in children with cerebral palsy and developmental delay has not been demonstrated., Objective: This study was conducted to quantitatively correlate the thickness of the corpus callosum with the volume of cerebral white matter in children with cerebral palsy and developmental delay., Material and Methods: A clinical database of 70 children with cerebral palsy and developmental delay was established with children between the ages of 1 and 5 years. These children also demonstrated abnormal periventricular T2 hyperintensities associated with and without ventriculomegaly. Mid-sagittal T1-weighted images were used to measure the thickness (genu, mid-body, and splenium) and length of the corpus callosum. Volumes of interest were digitized based on gray-scale densities to define the hemispheric cerebral white matter on axial T2-weighted and FLAIR images. The thickness of the mid-body of the corpus callosum was correlated with cerebral white matter volume. Subgroup analysis was also performed to examine the relationship of this correlation with both gestational age and neuromotor outcome. Statistical analysis was performed using analysis of variance and Pearson correlation coefficients., Results: There was a positive correlation between the thickness of the mid-body of the corpus callosum and the volume of cerebral white matter across all children studied (R=0.665, P=0.0001). This correlation was not dependent on gestational age. The thickness of the mid-body of the corpus callosum was decreased in the spastic diplegia group compared to the two other groups (hypotonia and developmental delay only; P<0.0001). Within each neuromotor subgroup, there was a positive correlation between thickness of the mid-body of the corpus callosum and volume of the cerebral white matter., Conclusion: The thickness of the mid-body of the corpus callosum positively correlates with volume of cerebral white matter in children with cerebral palsy and developmental delay, regardless of gestational age or neuromotor outcome. Assessment of the thickness of the corpus callosum might help in estimating the extent of the loss of volume of cerebral white matter in children with a broad spectrum of periventricular white matter injury.

Background: The filamin-1 (FLN-1) gene is responsible for periventricular nodular heterotopia (PNH), which is an X-linked dominant neuronal migration disorder., Objective: To review the clinical and imaging findings in a series of patients with documented filamin-1 mutations., Materials and Methods: A retrospective review of the medical records and MR studies of a series of patients with PNH and confirmed FLN-1 mutations was done. There were 16 female patients (age range: .67-71 years; mean = 28.6) with filamin-1 gene mutations., Results: In six of the patients the same mutation was inherited in four generations in one pedigree. In a second pedigree, a distinct mutation was found in two patients in two generations. In a third pedigree, a third mutation was found in four patients in two generations. The remaining four patients had sporadic de novo mutations that were not present in the parents. Ten patients had seizures, and all patients had normal intelligence. In all 16 patients MR demonstrated bilateral near-continuous PNH. There were no consistent radiographic or clinical differences between patients carrying different mutations., Conclusion: Patients with confirmed FLN-1 gene mutations are usually female and have a distinctive MR pattern of PNH. Other female patients with this same MR pattern probably harbor FLN-1 mutations and risk transmission to their progeny. This information is important for genetic counseling.

Unlabelled: BACKGROUND. Cervicomedullary astrocytomas are a unique subset of brainstem tumors in children because they have a good prognosis when compared to the pontine subset of brainstem gliomas. Objective. To review the clinical and imaging findings in a series of children with cervicomedullary astrocytomas as to diagnosis and management., Materials and Methods: A retrospective review of eleven children (six females, five males, age range: 10 days-18 years; mean = 7 years) with cervicomedullary tumors was done including the clinical presentation, imaging studies (MR: eleven, CT and MR: four), surgical findings, pathological results, and follow-up clinical and imaging findings (range: 0.2-11 years; mean = 5.2 years)., Results: Symptoms and signs were delayed and protracted, often occurring over months to years (mean = 2.3 years, range 0.5-7 years). The tumors expanded the dorsal medulla and involved the upper cervical spinal cord (mean maximum tumor diameter = 4.4 cm). Only three patients had hydrocephalus. In three of four cases the tumor was not seen on CT. On MR, the majority of the tumors were T1 hypointense and T2 hyperintense. Treatment consisted of surgery only in six patients, surgery and radiation therapy in four, and surgery, chemotherapy, and radiation in one. There was recurrent local disease in four patients and on follow-up metastatic disease in the brain in one. On follow-up the majority of the patients are alive and stable (mean = 5.2 years, range 0.2-11 years). There has been one death. The majority of tumors were pilocytic astrocytomas., Conclusion: Cervicomedullary tumors are a unique subset of brainstem gliomas in childhood that present with a long duration of symptoms and a greater long-term survival than pontine gliomas.

Purpose. To determine the incidence of neurologic complications of cerebral angiography in children with moyamoya syndrome (MMS) as compared to children without MMS. Materials and methods. One-hundred-ninety consecutive cerebral angiograms obtained in 152 children were evaluated. Sixty of these angiograms were obtained in 40 children with MMS. Patients underwent neurologic evaluation prior to and after the procedure. For this study, a neurologic complication was defined as any new focal neurologic deficit or alteration in mental status occurring during the procedure or within the ensuing 24 hours. Results. There were 2 neurologic complications within 24 hours of angiography, one in the MMS group and one in the non-MMS group. One patient with MMS became mute following angiography. The symptom resolved within 12 hours. One patient without MMS being examined postoperatively for residual arteriovenous malformation developed intracranial hemorrhage requiring reexploration 12 hours after the angiogram. Using a two-tail Fisher's exact test, there was no significant statistical difference in the ischemic (P = 0.3) or hemorrhagic (P = 1.0) complication rates between the group of patients with MMS and the non-MMS groups. Conclusion. The risk of a neurologic complication from cerebral angiography in children with MMS is low and not statistically different from the risk in children with other cerebrovascular disorders.

Background: Childhood moyamoya disease is a rare progressive cerebrovascular disease., Objective: To evaluate cerebral hemodynamics using dynamic Gd-DTPA-enhanced imaging in children with moyamoya disease., Materials and Methods: Eight children (2-11 years of age) with the clinical and angiographic findings typical of moyamoya disease, before and/or after surgical intervention (pial synangiosis), underwent conventional MR imaging (MRI) and hemodynamic MR imaging (HMRI). HMRI used a spoiled gradient-echo with low flip angle (10 deg) and long TE (TR/TE = 24/15 ms) to minimize T 1 effects and emphasize T 2* weighting. Raw and calculated hemodynamic images were reviewed. Three-dimensional time-of-flight MR angiography (MRA) and perfusion brain single photon emission computed tomography (SPECT) were also performed., Results: Abnormal hemodynamic maps resulting from vascular stenosis or occlusion and basal collaterals were observed in six patient studies. HMRI depicted perfusion dynamics of affected cerebrovascular territories, detected cortical perfusion deficits, and complemented conventional MRI and MRA. HMRI findings were consistent with those of catheter angiography and perfusion SPECT., Conclusion: Our preliminary experience suggests that HMRI may be of value in the preoperative and postoperative evaluation of surgical interventions in moyamoya disease.

A 16-month-old boy presented with left exophthalmos. He was found to have an enhancing intraconal soft-tissue mass. The differential diagnosis of the mass is discussed. The lesion was proven to be a malignant ectomesenchymoma, a very unusual tumor.

A five-and-a-half-year-old boy with a history of craniopharyngioma presented with an enlarging scalp mass. The clinical history, CT images, histological findings and relevant discussion are presented.

Twenty-six cases of developmental retrocerebellar cyst (RCC) formation are studied with respect to determining the usefulness and anatomic relevance of separate terms currently in use, including Dandy-Walker complex, Dandy-Walker malformation, Dandy-Walker variant, mega-cisterna magna (MCM), and Blake's pouch cyst. An anatomic and embryological continuum between Dandy-Walker complex and Blake's pouch cyst is proposed. A method for the useful assessment of RCC is outlined. The patency or closure of the aqueduct is crucial to the evaluation and management of hydrocephalus associated with RCC formation.