Cases reported "Glioma"

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1/12. Globular glioma of the tectum.

    Brainstem gliomas are a heterogeneous group of tumors whose prognosis and treatment depend not only on the histologic features but also on the location within the brainstem. magnetic resonance imaging allows the recognition of a distinct type of brainstem glioma of the tectal region of the midbrain, leading to aqueductal compression and hydrocephalus. The radiologic appearance of these tumors is usually rather uniform, with a characteristic nonenhancing thickening of the tectal plate. Because of its protracted course, no further treatment is necessary beyond cerebrospinal fluid diversion and close clinicoradiologic follow-up. The authors report two children with tectal plate gliomas of unusual but strikingly similar appearance. They present a clinical picture suggestive of intracranial hypertension without localizing signs. Magnetic resonance images reveal hydrocephalus related to the presence of perfectly circular lesions, hypointense on T1 and hyperintense on T2, which could be mistaken for parasitic cysts or represent dilated rostral portions of the sylvian aqueduct. After the cerebrospinal fluid diversion procedures, no further treatment was given, with one of the patients being monitored for 10 years and the other for 8 months, without tumor progression. These patients demonstrate that tectal gliomas, despite sharing a good prognosis, may have various patterns of growth, leading to unusual radiologic appearances that may pose diagnostic difficulties.
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2/12. An unusual periaqueductal glioma: A short report.

    A rare case of periaqueductal glioma with an unusual radiological picture is presented. A forty-five year old male presented with progressive bilateral third and eighth nerve paresis. magnetic resonance imaging (MRI) revealed a diffuse periaqueductal tumour with sparing of aqueduct and no hydrocephalus. MRI is a useful adjunct in such a situation for contemplating appropriate modality of treatment.
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ranking = 3.5
keywords = aqueduct
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3/12. Fetal aqueductal glioneuronal hamartoma: a clinicopathological and physiopathological study of three cases.

    Fetal hydrocephalus due to aqueductal stenosis is classified into two main groups: congenital (X-linked, atresia, septa and forking) and acquired (post-infectious or post-hemorrhagic, gliosis and tumors). MATERIAL AND methods: We report three fetal cases presenting with severe hydrocephalus, two of which being apparently sporadic, and the third possibly inherited. On macroscopic examination, no associated malformations were identified, either craniofacial dysmorphy, or visceral abnormalities. Neuropathological study revealed massive hydrocephalus caused by narrowing of the Aqueduct of Sylvius. Histological examination evidenced a nodular, well-demarcated mass producing into the aqueductal lumen, and containing numerous immature proliferating glioneuronal cells. Immunohistochemical analyses did not suggest a developmental abnormality of the subcommissural organ but rather a hamartomatous malformative process. RESULTS: hamartoma of the posterior fossa has been rarely reported. Post-natal cases have been described in the cerebello-pontine angle or in the quadrigeminal plate, and have always been diagnosed as pilocytic or low-grade astrocytomas. In our cases, the lesions could be related to so-called pencil glioma, sometimes associated with type 1 neurofibromatosis and, to our knowledge, have never been described prior to birth. The occurrence during fetal life and the progressive maturation of the nodules are more likely in favor of a hamartomatous process. CONCLUSION: Even though they could sporadically occur, an accurate genetic counseling should be required in order to ensure that there is no familial history of Recklinghausen disease, and to provide a more precise evaluation of recurrence risk.
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ranking = 3
keywords = aqueduct
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4/12. Symptomatic subependymoma--a case report.

    Subependymoma is a rare, slow-growing, benign noninvasive tumor of the central nervous system that may be located in the fourth ventricle, the septum pellucidum, the third and the lateral ventricles, the aqueduct, and the proximal spinal cord. Symptoms, if any, usually result either from direct compression of the brain stem or from acute hydrocephalus due to occlusion of the foramen of Monro or aqueduct of Sylvius. In this report, we describe a case of subependymoma of the lateral ventricle with headache in a young female patient. This is the first reported case subependymoma in korea that was documented along with Magnetic resonance image.
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keywords = aqueduct
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5/12. Optic glioma with progressive occlusion of the aqueduct of Sylvius in monozygotic twins with neurofibromatosis.

    Monozygotic twins sisters with optic glioma "in mirror image" (one with involvement of the left optic nerve and the other with the right optic nerve) and hydrocephalus secondary to progressive stenosis of the aqueduct have been found in a series of 128 cases below 14 years of age with neurofibromatosis. The optic glioma was diagnosed in each of the twins at 2 years of age. In one twin the tumor involved only the optic nerve but in the other the glioma affected the optic nerve and spread to the homolateral zone of the optic chiasm. First symptoms of hydrocephalus appeared at 8 years and 11 years of age respectively but ventriculo-peritoneal shunting procedures were performed to relieve intracranial hypertension at 11 years and 15 years of age respectively. At 2 years of age both twins had pneumoencephalography which demonstrated normal air passage through the aqueduct and cerebral ventricles of normal size and morphology. Posterior studies with CT-scan demonstrated progressive obstruction of the aqueduct with very slow progression of the hydrocephalus in each twin, although it was not observed simultaneously. The increased intracranial pressure was tolerated for many years in each twin without obvious symptoms which could be attributed to the slow progression of the aqueduct obstruction.
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ranking = 4
keywords = aqueduct
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6/12. A case of nasal glioma and neurofibromatosis. A new association.

    A man of 25 with von Recklinghausen's neurofibromatosis (VR) developed nasal polyps. Subarachnoid haemorrhage occurred shortly after surgical excision and he died 9 weeks later. Post-mortem examination showed chronic hydrocephalus due to aqueduct stenosis. Histologically the polyps were a nasal glioma, a forme fruste of anterior encephalocoele not previously associated with VR, though other cranial defects are well recognized. Nasal glioma should be considered in the differential diagnosis of nasal lesions in VR.
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keywords = aqueduct
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7/12. Large basal ganglia cyst in site of previously radiated glioma. Case report.

    A 3 1/2-year-old boy developed a mass lesion in the right basal ganglia and midbrain, compatible with glioma. During radiation therapy, aqueduct obstruction developed, necessitating a ventriculoatrial (VA) shunt. The child improved and remained well for 6 years, when he developed recurrent symptoms. A computerized tomographic (CT) scan and ventriculogram revealed a large cyst arising from the region of the right basal ganglia, the site of the previous tumor. The VA shunt was converted to a cyst atrial shunt. Subsequently, the cyst decreased in size, but hydrocephalus recurred, as demonstrated by a second CT scan. A Y-tube shunt (one catheter in the cyst, one in the ventricle) has controlled symptoms and signs since that time.
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ranking = 0.5
keywords = aqueduct
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8/12. The pathophysiology of oral pharyngeal apraxia and mutism following posterior fossa tumor resection in children.

    mutism following posterior fossa tumor resection in pediatric patients has been previously recognized, although its pathophysiology remains unclear. A review of the available literature reveals 33 individuals with this condition, with only a few adults documented in the population. All of these patients had large midline posterior fossa tumors. To better understand the incidence and anatomical substrate of this syndrome, the authors reviewed a 7-year series of 110 children who underwent a posterior fossa tumor resection. During that time, nine (8.2%) of the 110 children exhibited mutism postoperatively. They ranged from 2.5 to 20 years of age (mean 8.1 years) and became mute within 12 to 48 hours of surgery. The period of mutism lasted from 1.5 to 12 weeks after onset: all children had difficulty coordinating their oral pharyngeal musculature as manifested by postoperative drooling and inability to swallow. Further analysis of these cases revealed that all children had splitting of the entire inferior vermis at surgery, as confirmed on postoperative magnetic resonance studies. Lower cranial nerve function was intact in all nine patients. Current concepts of cerebellar physiology emphasize the importance of the cerebellum in learning and language. The syndrome described resembles a loss of learned activities, or an apraxia, of the oral and pharyngeal musculature. To avoid the apraxia, therefore, the inferior vermis must be preserved. For large midline tumors that extend to the aqueduct, a combined approach through the fourth ventricle and a midvermis split may be used to avoid injuring the inferior vermis.
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ranking = 0.5
keywords = aqueduct
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9/12. Bobble-head doll syndrome and drop attacks in a child with a cystic choroid plexus papilloma of the third ventricle. Case report.

    The authors report an unusual case of a 2-year-old boy with a 3-month history of episodic rightward anterolateral head tilt and large-amplitude positional anteroposterior head bobbing reminiscent of bobble-head doll syndrome. This child experienced a sudden onset of drop attacks and then, within several hours, deep coma. The causative lesion was a contrast-enhancing, partially cystic third ventricular mass, which ultimately obstructed the aqueduct, producing profound obstructive hydrocephalus. An emergency ventriculostomy and endoscopic fenestration of the septum pellucidum was performed. Four days later, the tumor was completely resected by a transcallosal-transforaminal approach. The lesion was freely mobile within the third ventricle and contained a large cyst within its posterior pole; following drainage of the cyst, the lesion was easily delivered through the foramen of Monro. The histopathological diagnosis was choroid plexus papilloma. The child's neurological deficits, head tilt, and head bobbing resolved immediately after operation. To the best of the authors' knowledge, this represents the first well-documented report of bobble-head doll syndrome and drop attacks secondary to a choroid plexus papilloma. The highly mobile nature of the cystic lesion presumably led to its intermittent impaction within the foramen of Monro and/or proximal aqueduct; this produced the intermittent head tilt and bobble-head symptoms and, ultimately, resulted in acute obstruction of the aqueduct, causing the child's precipitous neurological decline.
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ranking = 1.5
keywords = aqueduct
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10/12. Neurofibromatosis in childhood: a review of 25 cases.

    The initial symptoms and signs of neurofibromatosis in 25 children are reported, and the literature is briefly reviewed. Two little known associations of neurofibromatosis are revealed. Four children showed hydrocephalus related to stricture of the aqueduct of Sylvius. Four other children presented with failure to thrive in infancy which was regarded in retrospect as a non-specific association of neurofibromatosis. It is emphasized that such manifestations may be the presenting features of neurofibromatosis in children.
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keywords = aqueduct
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