J K Curé

Medical University of South Carolina, Charleston, SC, United States

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Publications (25)144.38 Total impact

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    ABSTRACT: The epidermal nevus syndrome (ENS) is an unusual neurocutaneous disorder consisting of the combination of an epidermal nevus and a central nervous system (CNS), ophlhalmological, and/or skeletal abnormality. The study reports four new patients with ENS. Each had a confirmatory biopsy of the epidermal nevus, abnormal neurological examination findings, and documented CNS anatomical studies by imaging or autopsy. The paper also reviews the literature in English to determine neurological abnormalities found in skin-biopsy-proven cases of ENS. Hemi-atrophy, hemimegalencephaly, migrational abnormalities and vascular anomalies were found to be the most frequent intracranial abnormalities associated with ENS. Seizures and/or disabling moderate to severe developmental delays were present in a majority of patients. Seizure onset during the neonatal period or early infancy was associated with major hemispheric malformations. Neuroectodermal-derived ocular lesions were often bilateral. No consistent relation between laterality of the nevus and laterality of CNS abnormalities was found, supporting the gene mosaicism theory of pathogenesis.RÉSUMÉAnomalies neurologic/tie de développement et crises dans un syndrome de naevtis épidermique Le syndrome du naevus épidermique (ENS) est un trouble neurocutané inhabituel associant un naevus épidermique et des anomalies du système nerveux central (SNC), ophtalmologiques et/ou du squelette. Ľarticle rapport quatre nouveaux cas ďENS. Pour chacun, il y a une confirmation biopsique, des donnees anormales àľexamen neurologique et des études anatomiqucs bien documented sur le SNC par imageries et autopsie. Ľarticle fait également un relevé de la littérature dc langue anglaise sur les anomalies neurologiques trouvées en cas ďENS prouvé par biopsie. Les plus fréquentes anomalies intracrâniennes trouvées associées àľENS étaient ľhémiatrophie, ľhémimégalencéphalie, les anomalies de migration et les anomalies vasculaires. Des crises comitiales et/ou un retard de développement de modéréà sévère existaient chez la majorité des patients. Un début de crises durant la périodc néonatale ou la première enfance était associéà des malformations hémisphériques majeures. Les lésions oculaircs ďorigine neuro-ectodermique étaient souvent bilatérales. Aucune corrélation entre la latérulifi du naevus et la latéralité des anomalies du SNC n'a été trouvée, ce qui favorise la thèse pathogénique ďune mosaïque génétique.ZUSAMMENFASSUNGEntwicklungsbedingte neurale Miβbildungen und Anfälle beim epidermalen Nävus-Syndrom Das epidermale Nävus-Syndrom (ENS) ist einc ungewöhnliche neurocutane Erkrankung, die aus einer Kombination von epidermalem Nävus mit Mißbildungcn des Zentralnervensystems (ZNS), ophthalmologischcn Veränderungen und/oder Skelettanomalien besteht. Diese Studie berichtet über vier neue Patienten mit ENS. Bei alien war eine Biopsie des Nävus durchgeführt, waren abnorme neurologische Untersuchungsbefunde erhoben und anatomische ZNS Untersuchungen durch bildgebende Verfahren oder Autopsie dokumentiert worden. Die Arbeit enthält außerdem einen öberblick über die cnglische Literatur im Hinblick auf neurologische Anomalien bei bioptisch gesicherten Fällen mit ENS. Hemiatrophie, Hemimegalenzephalie, Migrationsanomalien und Gefaßveranderungen waren die häuflgsten intrakraniellen Mißbildungcn beim ENS. Krampfanfälle und/oder einschränkende mäßige bis schwere Entwicklungsverzögerungen waren bei den meisten Patienten vorhanden. Wenn die Anfälle in der Neonatalperiode oder im frühen Kindesaltcr begannen, konnten erhebliche cerebrate Mißbildungen nachgewiesen werden. Neuroektodermal bedingte Augenläsionen traten häufig bilateral auf. Es fand sich keine Relation zwischen der Seite des Nävus und der Seite der ZNS Mißbildungen, was für die Gen Mosaik Theorie zur Pathogenese spricht.RESUMENAnomalías en el neuro-desarrollo y convulsiones en el síndrome de Nevus epidérmicoE sindromes de nevus epidérmico (SNE) es una rara alteración neuro-cutánea que consisten en la combinación de un nevus epidérmico y una anomalía del SNC, oftalmológica y/o esquclética. El presente estudio aporta cuatro nuevos pacientes con SNE. Todos tenían una biopsia conftrmatoria del nevus epidérmico. hallazgos anómalos en el examen neurológico y estudios anatómicos de su SNC con imágencs o autopsia.La comunicación también revisa la literalura inglcsa para dcterminar ias anomalías neurológicas en casos de SNE con biopsia cutánea positiva. Se halló que las anomalías intracraneales más frccuentes asociadas al SNE eran la hemiatrofia, la hemimegaloencefalia, las anomalías migracionales y las anomalías vasculares. En la mayoría de los pacientes había también convulsiones y/o retrasos moderados o graves en el desarrollo discapacitantes. El inicio de las convulsiones en el periodo neonatal o precozmente durante la lactancia iba asociado a malformaciones hemisfericas importantes. Las lesiones oculares derivadas de la situación neurocctodérmica a menudo eran bilaterales. No se halló ninguna relación consislente entre la lateralidad del nevus y la de las anomalías del SNC, lo que apoya la teoría patogenética de un mosaicismo genético.
    Developmental Medicine & Child Neurology 11/2008; 38(8):716 - 723. DOI:10.1111/j.1469-8749.1996.tb12141.x · 2.68 Impact Factor
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    ABSTRACT: Sturge-Weber syndrome is characterized by a facial port-wine nevus, leptomeningeal angiomatosis, and glaucoma; it is commonly complicated by epilepsy and hemiparesis. We present a patient with a head and neck port-wine nevus, glaucoma, abnormalities of the intracranial deep veins, and untreated communicating hydrocephalus. The patient lacks any radiologic or clinical evidence of cerebral leptomeningeal angiomatosis. Considering that intracranial venous anomalies also are likely compatible with the embryologic explanation of Sturge-Weber syndrome, this child can serve as an unusual example of Sturge-Weber syndrome type II.
    Journal of Child Neurology 03/2006; 21(2):155-7. DOI:10.1177/08830738060210020801 · 1.67 Impact Factor
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    ABSTRACT: We compared magnetic resonance imaging (MRI), magnetic resonance angiography, and transcranial Doppler ultrasonography as predictors of specific neurocognitive functions in children with sickle cell disease. Participants were 27 children with sickle cell anemia (hemoglobin SS) who were participants in the Stroke Prevention Trial in Sickle Cell Anemia (STOP) and had no documented history of stroke. Children's MRIs were classified as normal or silent infarct, and their magnetic resonance angiograms were classified as normal or abnormal. The highest time-averaged mean flow velocity on transcranial Doppler ultrasonographic examination of the major cerebral arteries was analyzed. Age and hematocrit also were analyzed as predictor variables. The battery of neurocognitive tests included measures of intellectual functioning, academic achievement, attention, memory, visual-motor integration, and executive functions. MRI, magnetic resonance angiography, transcranial Doppler ultrasonography, age, and hematocrit were analyzed as predictors of participants' performance on the various measures of neurocognitive functioning. Age and hematocrit were robust predictors of a number of global and specific neurocognitive functions. When age and hematocrit were controlled, transcranial Doppler ultrasonography was a significantly unique predictor of verbal memory. We found an association between low hemoglobin and neurocognitive impairment. We also found that abnormalities on transcranial Doppler ultrasonography can herald subtle neurocognitive deficits. (J Child Neurol 2006;21:37-44).
    Journal of Child Neurology 02/2006; 21(1):37-44. DOI:10.1177/08830738060210010701 · 1.67 Impact Factor
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    ABSTRACT: The stroke prevention study in sickle cell disease (STOP) demonstrated a 90% reduction in stroke risk with transfusion among patients with time-averaged mean cerebral blood velocity (TAMV) of 200 cm/s or more as measured by transcranial Doppler (TCD). In STOP, 232 brain magnetic resonance angiograms (MRAs) were performed on 100 patients, 47 in the transfusion arm and 53 in the standard care arm. Baseline MRA findings were interpreted as normal in 75 patients and as indicating mild stenosis in 4 patients and severe stenosis in 21 patients. Among 35 patients who underwent magnetic resonance angiography within 30 days of random assignment, the TAMV was significantly higher in 7 patients with severe stenosis compared with 28 patients with normal MRA findings or mild stenosis (276.7 +/- 34 vs 215 +/- 15.6 cm/s; P<.001). In the standard care arm, 4 of 13 patients with abnormal MRA findings had strokes compared with 5 of 40 patients with normal MRA findings (P=.03). In this arm, TAMV became normal (less than 170 cm/s) or conditional (170-199 cm/s) in 26 of 38 patients with normal or mildly abnormal baseline MRA but remained abnormal in 8 of 10 patients with severely abnormal baseline MRA. These results suggest that TCD often detects flow abnormalities indicative of stroke risk before MRA lesions become evident. Furthermore, patients with abnormal MRA findings and higher TCD velocities are at higher risk for stroke, and their cerebral TAMVs are unlikely to decrease without transfusion.
    Blood 04/2004; 103(7):2822-6. DOI:10.1182/blood-2003-06-1972 · 9.78 Impact Factor
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    ABSTRACT: We conducted a retrospective study to determine whether the presence of moyamoya collaterals influenced the risk of recurrence of cerebrovascular events (CVEs: stroke or transient ischemic attack) in patients with sickle cell disease placed on chronic transfusions after a stroke. Forty-three patients with homozygous sickle cell anemia (HbSS) and 1 with HbSO(Arab) (16 females, 28 males) who had suffered strokes while under the age of 18 were studied. All patients had been on transfusions aimed at maintaining the sickle hemoglobin (HbS) level below 30%. They were followed for a mean of 6.6 years (2.2 to 20.4 years). The presence of collaterals was diagnosed based on either magnetic resonance angiography or conventional angiography. Eighteen (41%) of the 44 patients suffered recurrent CVEs. Nineteen (43%) (6 females, 13 males) patients had moyamoya collaterals. Eleven (58%) of these 19 experienced 21 total recurrent CVEs, including 4 strokes in 4 patients (21%). In comparison, 7 (28%) of 25 patients without moyamoya collaterals experienced 9 recurrent CVEs (P <.05) with only 1 recurrent stroke (4%). Moyamoya patients were also more likely to have 2 recurrent CVEs (42% vs 8%, P <.05) as well as poorer neuropsychological testing results. A proportional hazards regression analysis indicated that patients with moyamoya were more than twice as likely to incur a subsequent CVE (hazard ratio, 2.40; 95% confidence interval, 0.85, 6.75). We conclude that up to 41% of patients with sickle cell disease experience recurrent CVEs after an initial stroke despite chronic transfusions and that the risk of recurrence is significantly higher for those who have moyamoya collaterals.
    Blood 05/2002; 99(9):3144-50. DOI:10.1182/blood.V99.9.3144 · 9.78 Impact Factor
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    ABSTRACT: The pathophysiological factors of neurogenic or sympathetically mediated essential hypertension are unknown. Neurons close to the surface of the ventrolateral medulla (specifically, in the retro-olivary sulcus [ROS]) are integrally involved in the control of blood pressure by means of efferent connections to presympathetic neurons in the spinal cord. It is hypothesized that vascular contact with the ROS is pathogenically involved in neurogenically mediated hypertension. We evaluated that theory in 20 subjects with uncomplicated stage 1 to stage 2 essential hypertension (EHTN) (18 of whom completed the study). The baseline supine plasma norepinephrine level served as an index of central sympathetic outflow. The response of blood pressure to clonidine was used as a surrogate marker for neurogenically mediated hypertension. We also examined the relationship between those markers and evidence of anatomic abnormalities in the area of the ROS that was provided by magnetic resonance imaging. A vessel contacted the left ROS in 5 of the 18 subjects. Those 5 subjects had higher plasma norepinephrine concentrations than did the 13 subjects without this vascular contact (358+/-46 versus 76+/-43 pg/mL, P<0.001). These 5 subjects also exhibited a significant depressor response to clonidine that tended to be greater than that seen in the 13 subjects without vascular contact (-20.6+/-3.2 versus -13.6+/-9 mm Hg). Both race and baseline mean blood pressure had only an independent effect on the depressor response to clonidine. The findings are consistent with the theory that vascular contact with the left ROS may contribute to neurogenically mediated "essential" hypertension in some patients.
    Hypertension 08/2000; 36(1):78-82. DOI:10.1161/01.HYP.36.1.78 · 7.63 Impact Factor
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    ABSTRACT: X-linked adrenoleukodystrophy (X-ALD) is a progressive demyelinating disorder whose neurological signs and symptoms can manifest in childhood as cerebral ALD or in adulthood in the form of a progressive myelopathy (AMN). The consistent metabolic abnormality in all forms of X-ALD is an inherited defect in the peroxisomal beta-oxidation of very long chain (VLC) fatty acids (>C(22:0)) which may in turn lead to a neuroinflammatory process associated with demyelination of the cerebral white matter. The current treatment for X-ALD with Lorenzo's oil aims to lower the excessive quantities of VLC fatty acids that accumulate in the patients' plasma and tissues, but does not directly address the inflammatory process in X-ALD. We have previously demonstrated that lovastatin and other 3-HMG-CoA reductase inhibitors are capable of normalizing VLC fatty acid levels in primary skin fibroblasts derived from X-ALD patients. Lovastatin can block the induction of inducible nitric oxide synthase and proinflammatory cytokines in astrocytes, microglia, and macrophages in vitro. In a preliminary report, we demonstrated that lovastatin therapy can normalize VLC fatty acids in the plasma of patients with X-ALD. Here we report our clinical and biochemical observations on 12 patients with X-ALD who were treated with lovastatin for up to 12 months. Our results show that the high plasma levels of hexacosanoic acid (C(26:0)) showed a decline from pretreatment values within 1 to 3 months of starting therapy with 40 mg of lovastatin per day and stabilized at various levels during a period of observation up to 12 months. The percentage decline from pretreatment values varied and did not correlate with the type of ALD gene mutation (point mutation versus gene deletion). In 6 patients, in whom red cell membrane fatty acid composition was studied, a mean correction of 50% of the excess C(26:0) was observed after 6 months of therapy suggesting sustained benefit. In a few patients who discontinued lovastatin therapy plasma C(26:0) levels reverted to pretreatment values suggesting a cause and effect relationship between these events. Two patients dropped out of the study claiming no clinical benefit, 1 was withdrawn due to adverse effects, and an adult patient with cerebral involvement died during the study. A 10-year-old boy with severe cerebral involvement showed worsening of his neurological status. All patients with AMN remained neurologically stable or showed modest subjective improvement. All patients who did not have Addison's disease at the time of enrollment maintained normal adrenal function throughout the study. The implications of our findings for developing an effective therapy for X-ALD are discussed.
    Molecular Genetics and Metabolism 04/2000; 69(4):312-22. DOI:10.1006/mgme.2000.2977 · 2.83 Impact Factor
  • M. T. Smith, J. K. Curé, K. R. Holden
    Journal of Neuropathology and Experimental Neurology 05/1998; 57(5). DOI:10.1097/00005072-199805000-00133 · 4.37 Impact Factor
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    P Van Tassel, J K Curé, K R Holden
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    ABSTRACT: To investigate the presence of small cystlike structures in the cerebral hemispheric white matter on MR images of patients with tuberous sclerosis. The MR images of 18 consecutive patients with tuberous sclerosis were reviewed retrospectively. Eight of the 18 patients were found to have cystlike structures in the cerebral white matter. The signal intensity of these lesions was isointense with cerebrospinal fluid on T1-, proton density-, and T2-weighted images. Four patients were imaged with a fluid-attenuated inversion recovery sequence, which in each case also showed fluid-type signal in these areas. Three of the patients had CT for correlation, and these scans supported the diagnosis of cystic lesions. Cysts ranged in number from one to 12 per patient and were usually smaller than 1 cm. The most common location was adjacent to the occipital horn or trigone of the lateral ventricle (six of eight patients). Less frequent sites were near the frontal horns, in the corpus callosum, and in the deep white matter near the body of the lateral ventricle. Cysts in five patients were either immediately adjacent to a cortical tuber or in the center of a white matter dysplastic lesion. A cyst in one patient had septa, and none of the cysts enhanced. Cystlike structures in the cerebral hemispheric white matter were seen on the MR images of 44% of 18 patients with tuberous sclerosis. Whether these findings represent cystic degeneration of dysplastic tissue or are unrelated to the disease process of tuberous sclerosis is unknown. More than one pathogenesis may exist.
    American Journal of Neuroradiology 09/1997; 18(7):1367-73. · 3.68 Impact Factor
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    ABSTRACT: The epidermal nevus syndrome (ENS) is an unusual neurocutaneous disorder consisting of the combination of an epidermal nevus and a central nervous system (CNS), ophthalmological, and/or skeletal abnormality. The study reports four new patients with ENS. Each had a confirmatory biopsy of the epidermal nevus, abnormal neurological examination findings, and documented CNS anatomical studies by imaging or autopsy. The paper also reviews the literature in English to determine neurological abnormalities found in skin-biopsy-proven cases of ENS. Hemi-atrophy, hemimegalencephaly, migrational abnormalities and vascular anomalies were found to be the most frequent intracranial abnormalities associated with ENS. Seizures and/or disabling moderate to severe developmental delays were present in a majority of patients. Seizure onset during the neonatal period or early infancy was associated with major hemispheric malformations. Neuroectodermal-derived ocular lesions were often bilateral. No consistent relation between laterality of the nevus and laterality of CNS abnormalities was found, supporting the gene mosaicism theory of pathogenesis.
    Developmental Medicine & Child Neurology 09/1996; 38(8):716-23. · 3.29 Impact Factor
  • J K Curé, L L Key, L Shankar, A J Gross
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    ABSTRACT: To study the association between petrous carotid canal (PCC) and internal carotid artery (ICA) stenoses in patients with malignant osteopetrosis. Mean and minimum PCC diameters obtained from cranial computed tomographic (CT) scans in 20 patients were compared with similar measurements in 52 control subjects. ICA caliber, evaluated with magnetic resonance (MR) arteriography, was correlated with age and PCC dimensions. There was a statistically significant difference between patient and control PCC diameters. There was a strong positive correlation between age and PCC diameter in the control subjects, but only a weakly positive correlation in the patients. One or both ICAs were stenotic on all patient MR arteriograms. MR angiographic stenosis grade correlated positively with age but not with PCC diameters. PCC and ICA stenoses occur frequently in patients with malignant osteopetrosis. Bony overgrowth or a "persistent fetal state" may produce the PPC stenoses. The findings do not support progressive PCC narrowing in these patients.
    Radiology 06/1996; 199(2):415-21. DOI:10.1148/radiology.199.2.8668787 · 6.21 Impact Factor
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    ABSTRACT: We report the MR imaging findings in two cases of nasolabial cysts. Demonstration of their extraosseous location with cross-sectional imaging should prevent confusion with maxillary cysts and obviate unwarranted dental or maxillary surgery.
    American Journal of Neuroradiology 04/1996; 17(3):585-8. · 3.68 Impact Factor
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    ABSTRACT: A boy with sickle cell anemia underwent bone marrow transplantation (BMT). He was normal on neurological examination, but had radiologic evidence of an old left frontal lobe infarct, multiple cerebral vascular stenoses and moyamoya collaterals. After BMT he developed seizures with extension of the infarct and subarachnoid hemorrhage. One year later angiography revealed worsening stenosis of the M1 segments of both middle cerebral arteries. At that time an increase in von Willebrand's factor with decreased large molecular weight multimers (LvWF) was observed. We speculate that LvWF dependent, shear-induced platelet aggregation, together with endothelial damage may have contributed to the development of neurologic complications in this patient.
    Bone Marrow Transplantation 04/1996; 17(3):405-7. · 3.47 Impact Factor
  • Journal of Child Neurology 10/1995; 10(5):411-3. DOI:10.1177/088307389501000516 · 1.67 Impact Factor
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    Radiographics 10/1995; 15(5):1231-4. DOI:10.1148/radiographics.15.5.7501862 · 2.73 Impact Factor
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    J K Curé, K R Holden, P Van Tassel
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    ABSTRACT: Progressive cerebral sinovenous occlusion in a neonate with Sturge-Weber syndrome was documented by using two-dimensional time-of-flight MR venography. There was no evidence of intraluminal thrombus on routine spin-echo images obtained either before or after the onset of seizures, despite MR venographic evidence in both studies of venous abnormalities.
    American Journal of Neuroradiology 09/1995; 16(7):1539-42. · 3.68 Impact Factor
  • Pamela Van Tassel, Joel K Curé
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    ABSTRACT: This article reviews the gamut of cystic lesions, unrelated to neoplastic disease, that are found in the brain, subarachnoid space, and ventricles. The utility of MRI in diagnosing these entities is shown.
    Seminars in Ultrasound CT and MRI 07/1995; 16(3):186-211. DOI:10.1016/0887-2171(95)90018-7 · 1.08 Impact Factor
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    ABSTRACT: Congenital osteopetrosis is a rare osteosclerotic bone disease characterized by both a defect in osteoclastic function and reduced generation of superoxide by leukocytes. The disease is frequently fatal during the first decade of life. A six-month trial of therapy with recombinant human interferon gamma-1b in eight patients with osteopetrosis provided evidence of benefit, prompting this study of more prolonged therapy. We studied 14 patients with severe osteopetrosis treated with subcutaneous injections of recombinant human interferon gamma-1b (1.5 micrograms per kilogram of body weight per dose) three times per week for at least 6 months; 11 patients were treated for 18 months. We assessed the effect of therapy by evaluating the patients' clinical status, measuring blood counts and biochemical markers of bone turnover, and performing bone marrow imaging and bone biopsies. After 6 months of therapy, all 14 patients had decreases in trabecular-bone area (determined by histomorphometric analysis of bone-biopsy specimens) and increases in bone marrow space (determined by marrow imaging), and the improvement was sustained in the 11 patients treated for 18 months. The mean (+SD) hemoglobin concentration increased from 7.5 +/- 2.9 to 10.5 +/- 0.3 g per deciliter (P = 0.05), and superoxide generation by granulocyte-macrophage colonies increased (P < 0.001) after 18 months of therapy. In six patients for whom pretreatment data were available, there was a 96 percent decrease in the frequency of infections requiring antibiotic therapy during interferon treatment. There were no side effects necessitating the discontinuation of therapy. Long-term therapy with interferon gamma in patients with osteopetrosis increases bone resorption and hematopoiesis and improves leukocyte function.
    New England Journal of Medicine 07/1995; 332(24):1594-9. DOI:10.1056/NEJM199506153322402 · 54.42 Impact Factor
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    ABSTRACT: The clinical, MR imaging, surgical, and histologic findings in two cases of cystic aberrant cervical thymus were reviewed. Aberrant cervical thymic tissue was sharply circumscribed and extended deep to the posterior pharyngeal wall at the level of the piriform sinus in both cases. Both lesions had cyst contents that were of increased signal intensity on T1-weighted images. Solid components displayed MR signal characteristics identical to normal thymus.
    American Journal of Neuroradiology 06/1995; 16(5):1124-7. · 3.68 Impact Factor
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    ABSTRACT: The normal and variant anatomy of the cerebral veins and dural venous sinuses is poorly understood by many radiologists. Beginning with a discussion of cerebral venous anatomy, this review illustrates clinically pertinent anatomy of the cerebral sinovenous system. Various methods of imaging cerebral veins and dural venous sinuses are described. Techniques and pitfalls of MR venography are emphasized.
    Seminars in Ultrasound CT and MRI 01/1995; 15(6):499-519. DOI:10.1016/S0887-2171(05)80019-8 · 1.08 Impact Factor