1/35. 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 = 1keywords = nervous system (Clic here for more details about this article) |
2/35. ganglioglioma causing acute uncal herniation.Gangliogliomas are rare but mostly benign tumors of the central nervous system. Early surgical resection is recommended to prevent seizures and further growth. Therefore, an uncal herniation caused by a ganglioglioma is a very rare event. We report such a case, where a patient with an initially small lesion was lost to follow-up.- - - - - - - - - - ranking = 0.2keywords = nervous system (Clic here for more details about this article) |
3/35. Apparent encephalocele in twin fetus papyraceus with twin-reversal arterial perfusion.An apparently iniencephalic or exencephalic monoamniotic monochorionic female twin fetus, delivered as a fetus papyraceus at 28 weeks of gestation, had severe anomalies of the central nervous system and spine, including occipital encephalocele with a defect of the occipital bone. The encephalocele contained a spherical mass of autolyzed brain tissue without identifiable cerebellum or vermis. The cervical canal was widely patent dorsally, there were severe bony anomalies, including agenesis and fusion of vertebrae in the cervicothoracic spine. The upper limbs were absent. All organs were hypoplastic and autolyzed. The hypoplastic heart had three chambers. The placenta was monochorionic and monoamniotic; barium injection showed a twin-reversal arterial perfusion and entanglement of the umbilical cords.- - - - - - - - - - ranking = 0.2keywords = nervous system (Clic here for more details about this article) |
4/35. central nervous system consequences of an unusual body disposal strategy: case report and brief experimental investigation.The body of a 73-year-old man was dismembered by his female companion for the purpose of covert disposal. The method employed included skillful separation of body parts with hacksaw and knife, piecemeal disposal of fragments, and prolonged boiling of the decapitated head. The latter treatment resulted in marked shrinkage of cranial dura mater, separation of dura mater from skull, and extrusion of brain fragments into the resultant enlarged epidural space through a dural defect due to the disproportionately greater shrinkage of dura mater compared to brain parenchyma. This resulted in curd-like brain fragments filling an enlarged epidural space and overlying a shrunken, leathery dura mater. The cranial dura mater, still adherent to the skull base, resembled a "shrunken brain" in contour but contained only the remnants of brain tissue not already extruded through the dural defect. This unusual thermal artifact is rarely illustrated or mentioned in forensic literature. The development of this postmortem artifact likely requires the presence of a specific combination of conditions which must be, but apparently rarely are, simultaneously present.- - - - - - - - - - ranking = 0.8keywords = nervous system (Clic here for more details about this article) |
5/35. 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 = 0.2keywords = nervous system (Clic here for more details about this article) |
6/35. Severe complications after intrathecal methotrexate (MTX) for treatment of primary central nervous system lymphoma (PCNSL).Primary central nervous system lymphoma (PCNSL) is a rare and highly malignant tumor with increasing incidence. survival has improved with the use of nonsurgical treatment modalities, of which methotrexate (MTX)-based intrathecal chemotherapy has been an important option. Here, we describe devastating complications in three patients with diffuse large B-cell lymphomas. After intrathecal MTX therapy two patients died secondary to fulminant brain edema. A third patient developed severe dementia, gait disturbance and urinary incontinence due to leukencephalopathy. Since bulky disease was present in all three patients, CSF flow may have been impaired. This might have exposed adjacent tissue to prolonged toxic drug concentrations. Regarding the severe complications seen in these patients, it is useful to consider high-dose intravenous MTX treatment only in periventricular PCNSL.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
7/35. Anterior encephalocele with subcutaneous right facial nodule.Encephaloceles consist of heterotopic brain tissue that remains connected to the central nervous system. As such, these lesions can occur anywhere along the midline of the head, neck, and back. The clinical findings associated with an encephalocele are often cutaneous, prompting consultation with a dermatologist. Although abnormalities of the skin overlying the spinal cord are readily recognized by our specialty as markers for dysraphism, head and neck lesions may present a diagnostic challenge. We describe a case of an anterior encephalocele to increase awareness of this disorder and to emphasize the clinical findings that will assist with diagnosis. Our case is of particular interest because of the parasagittal location of the facial nodules and minimal actual midline involvement.- - - - - - - - - - ranking = 0.2keywords = nervous system (Clic here for more details about this article) |
8/35. Intraoperative management of an infant with a nasofrontal encephalocele.1. An encephalocele is a congenital malformation in which the structures of the central nervous system, in communication with cerebrospinal fluid pathways, herniate through a cranial defect. 2. classification of encephaloceles depends on their anatomical location within the cranium, and the prognosis and treatment largely depend on the site of such defects. 3. Although the operation is delayed until the infant gains strength, early correction (within the first month) minimizes facial deformity, prevents further damage to brain tissue herniating through the defect, and increases the chance of normal binocular vision.- - - - - - - - - - ranking = 0.2keywords = nervous system (Clic here for more details about this article) |
9/35. prenatal diagnosis of cri du chat syndrome with encephalocele.A 19-year-old primigravida was found to have an encephalocele on screening ultrasound study. amniocentesis indicated cri du chat syndrome, 5p-. Although cri du chat syndrome has been noted in association with central nervous system malformations, encephalocele is a rare finding in this syndrome.- - - - - - - - - - ranking = 0.2keywords = nervous system (Clic here for more details about this article) |
10/35. Central brain herniation secondary to fulminant acute disseminated encephalomyelitis: implications for neurosurgical management. Case report.Acute disseminated encephalomyelitis (ADEM), also known as postinfectious encephalomyelitis, is an immunologically mediated demyelinating disorder affecting the central nervous system that typically occurs after infection or vaccination. The prognosis of ADEM is generally favorable. In a small subset of patients with ADEM, however, fulminant cerebral edema requiring neurosurgical intervention will develop. Few recommendations are available to help the neurosurgeon in dealing with such cases. In this report, the authors present the case of a patient with ADEM in whom central brain herniation developed secondary to medically intractable cerebral edema. The authors review the salient features of the disease and suggest a role for neurosurgeons in cases of fulminant ADEM.- - - - - - - - - - ranking = 0.2keywords = nervous system (Clic here for more details about this article) |
| | Next -> |