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Biomedical subjects

W S Ball

Publications and source records attributed to W S Ball.

At least 55 records · Page 3Linked to original sources

Familial erythrophagocytic lymphohistiocytosis: neuroradiologic evaluation with pathologic correlation.

PURPOSE: To report neuroradiologic findings in eight patients with familial erythrophagocytic lymphohistiocytosis (FEL), a rare, fatal autosomal recessive disease of infancy. MATERIALS AND METHODS: All patients underwent neuroradiologic magnetic resonance (MR) imaging and computed tomography before and after treatment. MR imaging-guided proton MR spectroscopy was performed in four. RESULTS: Seizures, focal neurologic deficits, and gait abnormalities were the most common neurologic manifestations. Brain involvement on MR images was progressive and consisted of parenchymal atrophy, diffuse abnormal signal intensity in the white matter on T2-weighted images, focal hyperintense lesions in both the white and gray matter, delayed myelination, and parenchymal calcifications. MR spectroscopy showed lower ratios of N-acetyl aspartate to total creatine and higher ratios of choline-containing compounds to total creatine, suggestive of decreased neuronal density and gliosis. CONCLUSION: Brain MR imaging and MR spectroscopy allow assessment of the severity of FEL and may aid in monitoring the response to treatment.

Brain↗

Meningioangiomatosis of the brain stem. Case report.

The case is reported of meningioangiomatosis of the brain stem in a 3 1/2-year-old girl who suffered from vomiting, left facial weakness, difficulty in swallowing, and ataxia. This is believed to be the first reported case of meningioangiomatosis in the brain stem. Computerized tomography showed an intensely enhancing hyperdense mass in the left restiform body. Magnetic resonance imaging revealed that the lesion was isointense to gray matter on the T1-weighted image and hypointense on the T2-weighted image, with a surrounding zone of high T2 signal and intense enhancement. Angiography was normal. Surgical exploration demonstrated an intramedullary firm mass that was partially resected. Histologically, the mass consisted of a low-grade lesion of meningeal origin with spindle cells in a whorling pattern that were occasionally focused around small vessels. On 2-year follow-up imaging, the lesion remains unchanged in size. Certain particularities of this lesion are discussed in the context of the literature.

Brain Stem↗

Congenital malformations causing skull base changes.

Recent advances in imaging have enabled both the clinician and radiologist to better understand the therapeutic implications of congenital anomalies involving the skull base. Critical to this understanding is a full knowledge of normal embryologic development, anatomic detail, and clinical manifestations in the wide variety of congenital malformations involving the skull base. This review focuses on individual malformations in terms of their pathophysiology and their imaging findings of critical importance.

Bone Diseases, Developmental↗

Cerebrovascular occlusive disease in childhood.

It is clear that for many disorders involving the CNS in children, cerebrovascular occlusion with stroke or strokelike episodes may be an important part of their pathophysiology. In contrast to adults, no single entity is responsible for the majority of strokes in children. The incidence of childhood cerebrovascular occlusive disease is likely much higher than is recorded in the literature if one takes into account conditions in which vascular occlusion plays a significant but secondary role. In some disorders, the occlusive process is often produced by the therapy used to treat the underlying primary disorder (e.g., radiation injury) and often accounts for the greatest morbidity and mortality to the patient. Imaging plays an important role in the detection and follow-up of cerebrovascular occlusive disease in children. A better understanding by the radiologist of the full range of conditions that may be complicated by stroke is important if we are to play a role in early diagnosis and to help guide specific therapy aimed at preventing a recurrence of the event. Imaging has become complex; however, a combination of anatomic and functional imaging is essential in the appropriate workup and management of all children with cerebrovascular occlusive disease.

Adolescent↗

Cystic malformations of the posterior fossa: differential diagnosis clarified through embryologic analysis.

Cystic or cyst-like malformations of the posterior fossa represent a spectrum of disorders, including the Dandy-Walker malformation, vermian-cerebellar hypoplasia, mega cisterna magna, and arachnoid cyst. Differentiation of these lesions may be difficult with routine cross-sectional imaging; however, an accurate diagnosis is essential for proper treatment planning and genetic counseling. Dandy-Walker malformation is easily diagnosed on the basis of the classic triad: complete or partial agenesis of the vermis, cystic dilatation of the fourth ventricle, and enlarged posterior fossa. Vermian-cerebellar hypoplasia is a general classification that describes congenital malformations with a normal-sized posterior fossa, varying degrees of vermian and cerebellar hypoplasia, and a prominent retrocerebellar cerebrospinal fluid space that communicates freely with a normal or dilated fourth ventricle. Mega cisterna magna can be asymmetric and can manifest apparent mass effect, simulating the appearance of an arachnoid cyst; therefore, ventriculography or cisternography may be needed to demonstrate communication of the cystic mass with the subarachnoid space. A careful review of the embryologic development is essential in understanding these malformations and in making a more accurate radiologic diagnosis.

Abnormalities, Multiple↗

Evaluation of the craniocervical junction in Down syndrome: correlation of measurements obtained with radiography and MR imaging.

Measurement of the atlas-dens interval is the radiographic standard for identification of patients with Down syndrome at high risk for neurologic injury from spinal cord compression. In 17 pediatric patients with Down syndrome, measurements of the atlas-dens interval, distance between the clivus and posterior odontoid process, and width of the neural canal obtained with plain radiographs were compared to predict the width of the subarachnoid space on magnetic resonance (MR) images. In all patients, cervical radiography and craniocervical MR imaging were prospectively performed during lateral flexion and extension. The width of the subarachnoid space was measured with MR imaging. When the 95% confidence interval for correlation coefficients of regression was used, subarachnoid space width on MR images correlated with neural canal width on radiographs better than with either atlas-dens interval or clivus-posterior odontoid process distance (P = .05). Measurement of neural canal width is a better predictor of potential spinal cord compression than the atlas-dens interval or clivus-posterior odontoid process distance and should be emphasized in screening examinations performed with plain radiography.

Adolescent↗

Phase III multicenter clinical investigation to determine the safety and efficacy of gadoteridol in children suspected of having neurologic disease.

A phase III open-label clinical trial was conducted at 11 institutions to determine the safety and efficacy of gadoteridol in children suspected of having neurologic disease. One hundred three children were included in the safety analysis; 92 were evaluated for efficacy (76 intracranial and 16 spinal examinations). Three adverse events were reported in two children. All adverse events were considered minor and resolved spontaneously without treatment or sequelae. In a comparison of enhanced T1-weighted magnetic resonance images with unenhanced T1- and T2-weighted images, enhancement of disease was noted in 70% of the intracranial and 38% of the spinal examinations. Additional diagnostic information was reported in 82% of the postcontrast intracranial examinations and 62% of the spinal examinations. Use of this additional information contributed to a potential modification of patient diagnosis in 48% of intracranial and 20% of spinal cases with additional information. These results indicate excellent safety and efficacy for use of gadoteridol in children with suspected intracranial or spinal disease.

Adolescent↗

Cerebral perfusion in children: detection with dynamic contrast-enhanced T2*-weighted MR images.

Cerebral perfusion dynamics were assessed with dynamic contrast material-enhanced T2*-weighted magnetic resonance (MR) imaging in 33 subjects aged 3-20 years. Group A (n = 20) had sickle cell anemia without clinical evidence of cerebrovascular disease. Group B (n = 13) consisted of 12 patients with cerebrovascular disease and homozygous sickle cell anemia and one patient without that anemia. All subjects underwent conventional MR imaging and a dynamic study in which a spoiled gradient-echo pulse sequence was used to generate images (acquisition time, 2.5 seconds) during injection of a compact bolus of gadopentetate dimeglumine (0.1 mmol/kg). For qualitative analysis, the dynamic images were displayed in cine mode. Group A demonstrated symmetric sequential region patterns of loss of signal intensity within 10 seconds of injection. Group B exhibited signal loss asymmetries that corresponded to cerebrovascular lesions on conventional MR images. Quantitative analysis enabled estimation of hemodynamic parameters, including relative cerebral blood volume, relative cerebral blood flow, and mean transit time. This method of assessment of cerebral perfusion dynamics complements conventional MR imaging.

Adolescent↗

Childhood adrenoleukodystrophy: assessment with proton MR spectroscopy.

PURPOSE: Image-guided, single-voxel, localized proton magnetic resonance (MR) spectroscopy was performed to assess white matter in childhood adrenoleukodystrophy (ALD). MATERIALS AND METHODS: Eleven X-linked ALD subjects, seven with neurologic symptoms or white matter lesions at MR imaging and four asymptomatic patients, were compared with nine aged-matched, healthy volunteers. RESULTS: Compared with those from normal white matter, MR spectra from white matter lesions (n = 6) showed 65% reduction in the ratio of N-acetyl aspartate (NAA) to total creatine (tCr) (P < .01); 55% increase in the ratio of choline-containing compounds (Cho) to tCr (P < .02); substantial levels of tCr; 94% increase in the ratio of glutamate, glutamine, or inositol to tCr (P < .02); and lactate accumulation in four patients. Patients without brain lesions (n = 4) exhibited a 51% increase in Cho-to-tCr ratio (P < .01) and 11% nonsignificant increase in NAA-to-tCr ratio. CONCLUSION: Proton MR spectroscopy may prove a valuable technique for noninvasive diagnostic and prognostic assessment of ALD.

Adolescent↗

Efficacy of gadoteridol for contrast-enhanced magnetic resonance imaging in children.

RATIONALE AND OBJECTIVES: This study assesses the efficacy of gadoteridol for contrast-enhanced magnetic resonance imaging (MRI) in children. METHODS: Patients were examined by MRI before and after receiving 0.10 mmol/kg gadoteridol. Blinded and unblinded readers analyzed brain and spine MRI studies from a multicenter clinical trial involving 101 patients at 11 sites. Ninety-two cases (76 brain, 16 spine) were evaluated by unblinded investigators, and 91 cases (76 brain, 15 spine) were evaluated by three neuroradiologists unaffiliated with any investigational site and blinded to clinical information. RESULTS: Unblinded readers noted enhancement of brain pathology in 70% of cases versus 50% to 67% among blinded readers. Unblinded readers determined that additional diagnostic information was available after contrast in 82% of brain studies (average, 64% for blinded readers) and would have changed patient diagnoses in 48% of these studies (average, 46% for blinded readers). In spine cases, enhancement of pathology was noted in 38% (unblinded) and 33% to 40% (blinded). Additional diagnostic information was available after contrast in 63% of spine studies (unblinded), or an average of 58% (blinded), and patient diagnoses would have changed in 20% (unblinded), or an average of 59% (blinded). CONCLUSIONS: Gadoteridol is suitable for enhanced MRI detection, localization, and characterization of central nervous system pathology in children.

Adolescent↗

Balloon dilatation of esophageal strictures in children.

Traditionally, the treatment of esophageal strictures in children has been done with mercury bougies in a prograde fashion or with Tucker dilators passed along a guide wire in a retrograde manner. An alternative approach has used a balloon dilatation catheter passed over a guide wire previously placed under fluoroscopic guidance. Over the past 5 years, this technique has been used in 43 procedures for both discrete and diffuse strictures. The interventions were performed in a total of 15 patients ranging in age from 8 months to 21 years. In all cases, there was improvement of the preceding symptoms following dilatation. This report details the experience with this technique at Children's Hospital Medical Center, Cincinnati, Ohio, emphasizing the indications, contraindications, and potential complications associated with balloon dilatation of esophageal strictures in children.

Adolescent↗

Cerebral infarctions due to CNS infection with Enterobacter sakazakii.

Recent reports have implicated Enterobacter sakazakii, a gram-negative enteric bacillus, in neonatal sepsis and meningitis. Cases of severe central nervous system involvement, including ventriculitis, brain abscess, infarction, and cyst formation, have been described. We present serial head CT findings in a case of neonatal E. sakazakii meningitis complicated by a ring enhancing cerebral infarction which mimicked abscess formation. In meningitis secondary to this agent, a recognized pattern of cerebral hypodensity with or without cystic degeneration late in the course of the infection is likely to represent cerebral infarction rather than an abscess especially if there is a lack of culture evidence of a bacterial infection.

Brain↗

Color Doppler US imaging during pediatric neurosurgical and neuroradiologic procedures.

Experience with color Doppler imaging (CDI) during 16 pediatric intraoperative and one percutaneous neuro-radiologic procedures was reviewed to assess whether CDI increased the success rate or decreased the procedure time, thus contributing substantially to procedure performance. Intraoperative CDI was used to rapidly identify abnormal vessels or displacement of normal vessels and correlated with preoperative studies. In six cases (four vascular malformations, one mycotic aneurysm, and one hamartoma), surgical resection was altered on the basis of flow information obtained. In one case of percutaneous embolization of a Galenic malformation, CDI provided information contributing to the cessation of the procedure. In six cases (debulking of three gliomas, resection of one vascular malformation, and two biopsies of nonneoplastic conditions), information was added but did not alter the surgical approach. In the remaining four cases (three cerebrospinal fluid drainage procedures, one posterior fossa decompression), no additional information was obtained. Consultation among the ultrasound staff, neuroradiologists, and neurosurgeons before the operative procedure maximized the usefulness of CDI, thus aiding in the success of surgery.

Adolescent↗

Bilateral congenital ocular cysts.

This report presents an unusual case of bilateral congenital ocular cysts. These are in the spectrum of congenital cystic eye and microphthalmos with cysts. Diagnostic evaluation, including orbital B-scan ultrasonography, computed tomography and magnetic resonance imaging is described. Pathologic evaluation of the orbital cysts was consistent with congenital cystic eye. An associated microphthalmic eye was not detected with serial sectioning of both specimens. Examination of the orbit at exploration as well as pre- and post-operative MRI scans failed to demonstrate the presence of a microphthalmic eye in either orbit. Failure to detect a microphthalmic eye clinically, by diagnostic imaging studies, or serial microscopic sections of the cysts supports the diagnosis of congenital cystic eye. This child has associated mild facial clefting (median cleft lip and cleft palate) and basal cephalocele. Histopathologically documented cases of ocular abnormalities associated with basal encephalocele have not been reported. To the authors' knowledge, MRI findings of congenital ocular cysts have been reported once previously in a case of microphthalmos with cyst, however, histopathologic correlation has not been reported previously.

Cysts↗

Imaging of the brain in children.

As proclaimed by the National Institutes of Health, the 1990s have been designated the "decade of the brain." Within this decade, we can expect to see an emergence of functional imaging in addition to the traditional role of anatomic neuroimaging to separate normal from pathologic anatomy. Technical advances continue to redefine the upper limits of resolution for both anatomic and functional imaging and provide us with a greater understanding of how disease processes affect normal brain growth and development. For example, whereas improvements in anatomic resolution will provide greater insight into understanding congenital malformations of the brain, through functional imaging, we will better understand the effect of disease on normal brain growth and development.

Brain↗