1/47. Idiopathic temporal encephalocele: report of two cases.BACKGROUND: Idiopathic brain herniation into the middle ear is a rare condition that represents diagnostic and therapeutic challenges. OBJECTIVE: The authors present here two new cases of idiopathic brain herniation with special clinical presentation and emphasis on radiographic studies, particularly computed tomographic scan and magnetic resonance imaging, which allowed the authors to detect the malformation. RESULTS: The two patients underwent surgical treatment with infratemporal approach and recovered perfectly.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/47. Spontaneous closure of bony defect in a frontoethmoidal encephalomeningocele patient.The frontoethmoidal encephalomeningocele (FEEM) is a congenital herniation of meninges and brain tissue through the skull bony defect at the foramen cecum. The size of the defect may vary from a few millimeters to many. Those patients with a small defect may not always require a risky operation during childhood. We report on an infant whose bony defect has closed spontaneously with definite clinical evidence. It is proved that the skull defect and brain herniation are able to heal naturally, and this affirms an existence of the abortive subtype of FEE. Conservative treatment may be considered in those with a small bony defect, and surgery can be considered later when it is required.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
3/47. A case in the spectrum of the oculo-encephalo-hepato-renal syndrome.An 18-year-old male is presented with unprecedented central nervous system findings (cerebral dysplasia and sacral meningocele) possibly in the spectrum of the oculo-encephalo-hepato-renal syndrome. He had severe mental retardation, triplegia, epilepsy, retinitis pigmentosa, and chronic renal failure. magnetic resonance imaging demonstrated cerebral dysplasia (left dominant abnormal gyri, hypoplastic white matter, basal ganglia, and thalamus, and absence of the septum pellucidum) and the hypoplastic cerebellum and brainstem. A sacral meningocele was observed first at 16 years of age. His renal function gradually worsened after 11 years of age. His liver function was normal. The previously reported 72 cases with the oculo-encephalo-hepato-renal syndrome are reviewed.- - - - - - - - - - ranking = 2.2274335963182keywords = central nervous system, brain, nervous system (Clic here for more details about this article) |
4/47. Spheno-pharyngeal meningocele and cleft palate. Case report with 12-year follow-up.A rare congenital anomaly consisting of a spheno-pharyngeal meningocele and cleft palate is presented. The repair of the meningocele was accomplished through an intra-oral transpalatal approach. A primary pharyngeal flap was used to accomplish the repair of the cleft palate. Fortunately, there were no important anomalies of the central nervous system. Twelve years later, the patient functions normally and speaks well. The only remaining symptom of midline craniofacial dysmorphia is a mild hypertelorism.- - - - - - - - - - ranking = 1.7274335963182keywords = central nervous system, nervous system (Clic here for more details about this article) |
5/47. Congenital unilateral buphthalmos in walker-warburg syndrome: a clinicopathological study.BACKGROUND AND PURPOSE: walker-warburg syndrome is a congenital autosomal recessive oculocerebral disorder characterised by hydrocephalus, brain agyria, microphthalmos and retinal dysplasia with or without meningoencephalocele. We describe an unusual finding of congenital unilateral glaucoma and buphthalmos in one eye and microphthalmos in the fellow eye of two neonates with Walker-Warburg syndrome. patients: Two neonates with walker-warburg syndrome and unusual findings of buphthalmos in one eye and a microphthalmic fellow eye are presented. RESULTS: Histological examination of the buphthalmic eyes revealed the presence of mesenchymal tissue in the anterior angle covered by endothelium. No anterior chamber angle was identified in the microphthalmic fellow eye and the iris was adherent to the corneal periphery. CONCLUSIONS: Congenital buphthalmos may also appear in walker-warburg syndrome. The buphthalmos may result from later embryonal ocular developmental arrest than that of the microphthalmic eye.- - - - - - - - - - ranking = 0.5keywords = brain (Clic here for more details about this article) |
6/47. Rudimentary meningocele presenting with an annular alopecia.Rudimentary meningocele (RM) is a relatively rare developmental anomaly in which meningotherial elements are found in the skin. We present a case presenting with an annular alopecia over a rudimentary meningocele. Due to the occasional presence of connection or relationship to the central nervous system in RM, knowledge of the various clinical appearances including annular alopecia is clinically important.- - - - - - - - - - ranking = 1.7274335963182keywords = central nervous system, nervous system (Clic here for more details about this article) |
7/47. Basal meningoencephalocele, anomaly of optic disc and panhypopituitarism in association with moyamoya disease.Basal meningoencephalocele is frequently associated with midfacial anomaly, optic disc anomaly, brain anomaly, cerebrospinal fluid rhinorrhea, chiasma syndrome, and endocrinologic disturbance. The combination of basal meningoencephalocele and moyamoya disease is extremely rare. A 29-year-old man had basal meningoencephalocele (transsphenoidal type), anomaly of the optic disc (morning glory syndrome), panhypopituitarism and moyamoya disease. The patient was treated by hormone replacement, but surgical intervention was not required. Basal meningoencephalocele and moyamoya disease are a possible combination of the diseases.- - - - - - - - - - ranking = 0.5keywords = brain (Clic here for more details about this article) |
8/47. Bilateral defects of the tegmen tympani associated with brain and dural prolapse in a patient with pulsatile tinnitus.A pulsating ear drum is a rare otological finding and usually indicates a vascular pathology. We report the case of a woman who presented with pulsatile tinnitus following an upper respiratory tract infection. She was found to have bilateral pulsating tympanic membranes on otological examination, in association with bilateral dehiscence of the tegmen tympani and a spontaneous encephalocele on the right side and a meningocele on the left side, demonstrated on computerized tomography (CT) scan of her temporal bones.- - - - - - - - - - ranking = 2keywords = brain (Clic here for more details about this article) |
9/47. 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 = 1.7274335963182keywords = central nervous system, nervous system (Clic here for more details about this article) |
10/47. Intradiploic meningoencephalocele.We report on a 36-year-old patient who presented with coordinative problems in his right leg. The MRI study of his brain showed the extremely rare intradiploic meningoencephalocele which explained his symptoms. Most cephaloceles are inborn developmental disturbances and present with symptoms of different severity depending on the degree of associated malformations in early childhood. They usually demand immediate treatment. In the patient presented here the cephalocele belongs to the rare type of intradiploic meningoencephalocele. This is a variant of the intradiploic cerebrospinal fluid cyst and of traumatic instead of developmental origin.- - - - - - - - - - ranking = 0.5keywords = brain (Clic here for more details about this article) |
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