1/187. 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) |
2/187. 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) |
3/187. Dysgenesis of the internal carotid artery associated with transsphenoidal encephalocele: a neural crest syndrome?We describe two original cases of internal carotid artery dysgenesis associated with a malformative spectrum, which includes transsphenoidal encephalocele, optic nerve coloboma, hypopituitarism, and hypertelorism. Cephalic neural crest cells migrate to various regions in the head and neck where they contribute to the development of structures as diverse as the anterior skull base, the walls of the craniofacial arteries, the forebrain, and the face. Data suggest that the link between these rare malformations is abnormal neural crest development.- - - - - - - - - - ranking = 0.5keywords = brain (Clic here for more details about this article) |
4/187. New syndrome of growth and mental retardation, structural anomalies of the central nervous system, and first branchial arch, anophthalmia, heminasal a/hypoplasia, and atypical clefting: report on four Brazilian patients.We report on four unrelated Brazilian patients with growth and mental retardation, structural anomalies of the central nervous system (CNS), mainly callosal agenesis, prominent forehead, facial asymmetry, anophthalmia, heminasal a/hypoplasia, preauricular skin tags, structural anomalies of the external ears, and atypical clefting. This combination of anomalies is unique and, to our knowledge, is a previously undescribed syndrome of unknown etiology, although one of the patients was born to a consanguineous couple, suggesting the possibility of autosomal recessive inheritance. Clinical, genetic, and differential diagnosis aspects are discussed.- - - - - - - - - - ranking = 1.7693648536855keywords = central nervous system, nervous system (Clic here for more details about this article) |
5/187. Central brain herniation secondary to juvenile diabetic ketoacidosis.We present the CT, MR, and autopsy findings of central brain herniation in a 9-year-old boy undergoing treatment for diabetic ketoacidosis (DKA). Severe cerebral edema resulting in central brain herniation is an uncommon complication of the treatment of DKA but carries with it high morbidity and mortality. Radiologic imaging and autopsy findings in this case revealed striking infarctions of central brain structures.- - - - - - - - - - ranking = 3.5keywords = brain (Clic here for more details about this article) |
6/187. Intrasphenoidal encephalocele and spontaneous CSF rhinorrhoea.Intrasphenoidal encephalocele is a rare clinical entity. In the international literature only 16 cases have been reported up today, with female predominance. Clinically they manifest at middle and advanced ages (40-67 years), when spontaneous CSF rhinorrhoea or recurrent meningitis occurs. We present our case, a 46 years old female, who had CSF rhinorrhoea from the right vestibule for 10 months. The diagnosis was based on the history and the high-resolution brain and skull base CT-scanning in conjunction with opaque fluid injection in the subarachnoidal space through a lumbar puncture. She was successfully treated with an operation, through an endonasal trans-ethmoid microendoscopic approach, using the Draf and Stammberger technique. We discuss the pathogenesis of the intrasphenoidal encephalocele, the existence of small occult defects in the skull base, which cause, at the middle and advanced ages, CSF fistula with spontaneous CSF rhinorrhoea and/or recurrent meningitis. Finally we emphasize the advantages of the endonasal surgical approach for the treatment of this condition.- - - - - - - - - - ranking = 0.5keywords = brain (Clic here for more details about this article) |
7/187. 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) |
8/187. Neuroradiology of basal anterior fossa (transethmoidal) encephaloceles.Encephaloceles of the basal anterior fossa, though primarily congenital in origin, may be insidious in presentation and remain undetected until adulthood. Conventional plain films and hypocycloidal tomograms show a basal osseous defect with its margins bevelled toward the nasopharynx. Magnification angiography is the diagnostic study of choice and reveals herniation of the anterior inferior frontal artery through the osseous defect. pneumoencephalography, the classical technique of investigation, was found to be unrewarding in demonstrating the herniated sac or in determining the presence or absence of brain tissue within the sac; however, it is of value in demonstrating a ventricular communication with the encephalocele and evaluating ventricular dilatation.- - - - - - - - - - ranking = 0.5keywords = brain (Clic here for more details about this article) |
9/187. Therapeutic effectiveness of acetazolamide in hindbrain hernia headache.A 43-year-old man had been suffering from exertional headache for 10 years. Sagittal sections on magnetic resonance imaging showed a Chiari type 1 malformation and a cerebellar arachnoid cyst. This syndrome, named hindbrain hernia headache, disappeared with oral acetazolamide. This treatment should be tried in patients with hindbrain hernia headache prior to considering surgical decompression of the foramen magnum.- - - - - - - - - - ranking = 3keywords = brain (Clic here for more details about this article) |
10/187. Chiari malformation and sleep-disordered breathing: a review of diagnostic and management issues.Chiari Malformation (CM) encompasses several patterns of congenital or acquired cerebellar herniation through the foramen magnum. This may result in brain-stem compression that impacts control of breathing and is associated with obstructive and central apneas. A high clinical suspicion for sleep-disordered breathing is needed in the care of such patients after as well as before corrective surgery. To introduce a review of CM with a focus on the relevance to sleep medicine, we present a case of a 13-year-old female who was diagnosed with CM Type 1 in the course of an evaluation of symptomatic central sleep apnea. After initial improvement following surgery there was recurrence of brain-stem compression. The only clinical expression of which was polysomnographically evident recurrence of sleep apnea.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
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