1/12. Rudimentary meningocele: remnant of a neural tube defect?BACKGROUND: Rudimentary meningocele, a malformation in which meningothelial elements are present in the skin and subcutaneous tissue, has been described in the past under a variety of different terms and has also been referred to as cutaneous meningioma. There has been debate as to whether rudimentary meningocele is an atretic form of meningocele or results from growth of meningeal cells displaced along cutaneous nerves OBJECTIVE: We reviewed the clinical, histological, and immunohistochemical characteristics of rudimentary meningocele in an attempt to assess the most likely pathologic mechanism for it. DESIGN: Retrospective study. SETTING: University hospitals. patients: Thirteen children with rudimentary meningocele. MAIN OUTCOME MEASURES: medical records were reviewed and histopathologic examination as well as immunohistochemistry studies were performed for each case. A panel of immunoperoxidase reagents (EMA, CD31, CD34, CD57, S-100, and CAM 5.2) was used to assess lineage and to confirm the meningothelial nature of these lesions. RESULTS: Recent evidence indicating a multisite closure of the neural tube in humans suggests that classic meningocele and rudimentary meningocele are on a continuous spectrum. CONCLUSION: Rudimentary meningocele seems to be a remnant of a neural tube defect in which abnormal attachment of the developing neural tube to skin (comparable to that in classic meningocele) could explain the presence of ectopic meningeal tissue. In the majority of cases, no underlying bony defect or communication to the meninges could be detected. However, in light of the probable pathogenesis, imaging studies to exclude any communication to the central nervous system should precede any invasive evaluation or intervention.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
2/12. valproic acid embryopathy: report of two siblings with further expansion of the phenotypic abnormalities and a review of the literature.Fetal Valproate syndrome (FVS) results from prenatal exposure to valproic acid (VPA). It is characterized by a distinctive facial appearance, a cluster of minor and major anomalies, and central nervous system dysfunction. In this study, two siblings who were exposed to monotherapy with VPA are described with documentation of long-term follow up. Both children had craniofacial findings, multiple systemic and orthopedic abnormalities, an overgrowth pattern, and developmental deficits. The literature from 1978-2000 is reviewed. A total of 69 cases that were solely exposed to VPA with adequate phenotypic description were identified. The clinical manifestations of FVS encompass a wide spectrum of abnormalities including consistent facial phenotype, multiple systemic and orthopedic involvement, central nervous system dysfunction, and altered physical growth. The facial appearance is characterized by a small broad nose, small ears, flat philtrum, a long upper lip with shallow philtrum, and micro/retrognathia. In this review, 62% of the patients had musculoskeletal abnormalities, 30% had minor skin defects, 26% had cardiovascular abnormalities, 22% had genital abnormalities, and 16% had pulmonary abnormalities. Less frequently encountered abnormalities included brain, eye, kidney, and hearing defects. neural tube defects were seen in 3% of the sample. Twelve percent of affected children died in infancy and 29% of surviving patients had developmental deficits/mental retardation. Although 15% of patients had growth retardation, an overgrowth pattern was seen in 9%. The data from this comprehensive review especially the developmental outcome should be added to the teratogenic risk, that arises in association with the use of VPA during pregnancy.- - - - - - - - - - ranking = 2keywords = central nervous system, nervous system (Clic here for more details about this article) |
3/12. A case of prenatally diagnosed fetal neurenteric cyst.The combination of a thoracic cystic mass with vertebral anomalies on prenatal ultrasound suggests a neurenteric cyst. The outcome of such cysts mostly depends on the extent of the displacement and functional impairment of the adjacent organs and of the associated central nervous system defects. We present a case of a neurenteric cyst diagnosed on prenatal ultrasound at 34 weeks of gestation which was treated successfully in the early neonatal period.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
4/12. In utero MR imaging and management of foetal hydrocephalus and NTDs in the third trimester.Although ultrasound (US) remains the gold standard in foetal imaging, in utero magnetic resonance (MR) imaging of the foetal nervous system may be useful in the late stages of pregnancy. The authors performed MR scanning in pregnant women in whose foetuses US examination had shown hydrocephalus and neural tube defects (NTDs). These cases are definitely diagnosed as hopeless at such a late stage. In this report, three pregnant patients are presented to illustrate the morphological imaging features, and their outcome is discussed. This report is an example of how radiological investigations can be most valuable, when US and MR imaging are used as complementary in imaging of the foetuses.- - - - - - - - - - ranking = 0.23994997820317keywords = nervous system (Clic here for more details about this article) |
5/12. Iniencephaly. A case report.Prenatal ultrasound diagnosed iniencephaly apertus at 21 weeks' gestation. In this rare central nervous system (CNS) malformation the brain and neck show the main pathologies. Retroflexion of the head with exaggerated cervicothoracic lordosis is always present, and CNS malformations in the form of anencephaly, spina bifida and encephalocele are often present. The ultrasonic diagnosis should be based on the finding of extreme dorsiflexion of the head accompanied by an abnormally short and deformed spine.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
6/12. Possible evidence for secondary degeneration of central nervous system in the pathogenesis of anencephaly and brain dysraphia. A study in young human fetuses.In an attempt to help elucidate pathogenetically those human cases exemplifying secondary degeneration of the neural tube causing brain dysraphia, macroscopic and histologic observations of two young human fetuses are described. A nine-week-old anencephalic fetus exhibited an absence of spinal cord (amyelia) with retention of neural crest derivatives (dorsal root ganglion cells and thusir processes, and sympathetic ganglia) implying the presence of a neural tube in early gestation. The second, ten-week-old exencephalic case exhibited restricted brain hemorrhage and necrosis of the telencephalon and brain stem amongst otherwise normal brain and spinal cord tissue. These two young fetal cases may represent examples of a previously normal neural tube which has undergone degeneration at a stage where neural crest has already undergone differentiation, and thus distinguishes them from cases of complete dysraphism which probably results from primary degeneration during neurulation.- - - - - - - - - - ranking = 4keywords = central nervous system, nervous system (Clic here for more details about this article) |
7/12. Far-lateral transcondylar approach: surgical technique and its application in neurenteric cysts of the cervicomedullary junction. Report of two cases.Neurenteric cysts are rare benign lesions of the central nervous system that are lined by endodermal cell-derived epithelium. Although they occur mostly in the spine, they can occur intracranially, most often in the posterior fossa. Neurenteric cysts that are located in the anterior cervicomedullary junction are even rarer and often require a skull base approach for adequate resection. The authors describe two cases of neurenteric cysts arising from the cervicomedullary junction that were resected via a far-lateral transcondylar approach. They discuss the surgical approach and operative nuances involved in removing these lesions, and review the clinical presentation of neurenteric cysts in this region as well as the neuroimaging characteristics, histopathological findings, and surgical management. Intraoperative videos are presented.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
8/12. Intracranial neurenteric cyst with recurrence and extensive craniospinal dissemination.Neurenteric cyst represents a rare cystic lesion of the central nervous system, and is generally thought to result from failure of separation of neuro-ectodermal and endodermal elements during week 3 of embryogenesis. Although recurrences have been reported, only one case of craniospinal dissemination has previously been reported. We describe a unique intracranial neurenteric cyst with recurrence and dissemination to the spinal cord.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
9/12. A rare neural tube defect: a report of eight cases in pakistan.Iniencephaly is a rare congenital malformation of central nervous system and axial skeleton. Eight cases were seen in the Department of obstetrics and Gynaecology, Jinnah Post-Graduate Medical Centre over a 12 month period constituting 0.1% of all deliveries conducted, the estimated frequency being 1: 850 deliveries. The purpose of this paper is to describe our cases and to report several considerations which might influence the antenatal diagnosis of this rare anomaly.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
10/12. Unusual cause of cerebellar abscess: occipital dermal sinus and dermoid cyst.Cerebellar abscess induced by a contiguous dermal sinus is a rare event. In a large series of acute posterior fossa abscedation, otogenic suppuration is the cause in 93% of the cases, while hematogenous infection is infrequent, probably because of the relatively weak bloodflow in this part of the central nervous system. The authors present an occipital dermal sinus and dermoid cyst revealed by a cerebellar abscess. The literature is reviewed briefly, treatment and prophylactic measures are discussed.- - - - - - - - - - ranking = 1keywords = central nervous system, nervous system (Clic here for more details about this article) |
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