Cases reported "Hydrocephalus"

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1/460. neurofibrillary tangles in the dementia of "normal pressure" hydrocephalus.

    Routine neuropathological examination could not explain the dramatic improvement exhibited by one patient with "normal pressure" hydrocephalus after shunting. The improved patient contrasted remarkably with the unchanged condition of four others also shunted successfully. The five brains were analysed by quantitative morphometry to determine the degree of neurofibrillary tangle formation in mesial temporal neurons. The density of tangle-bearing nerve cells in the four unimproved cases was markedly greater than in age-matched control brains from nineteen normal subjects, and fell in the same range as that of eight dements with neuropathologically confirmed Alzheimer's disease. The density of the one who recovered was within normal limits. The duration of dementia before shunting, and the total duration of dementia in these five patients rank in the same order as their degree of neurofibrillary formation. Furthermore, a positive linear correlation exists between the Tangle Indices and the total duration of dementia. The data suggest that early diagnosis may improve the chances of reversing the dementia of normal pressure hydrocephalus before histological alterations prove too severe.
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2/460. Complete remission of a diffuse pontine glioma.

    A patient is described in whom a large diffuse glioma of the pons extending into the midbrain was diagnosed at the age of 2 years. biopsy showed a fibrillary astrocytoma. After shunting of a hydrocephalus, the clinical symptoms abated without conventional therapy. Repeated MRI studies showed a continuous decrease of the tumour which was no longer visible when the patient was 6.6 years old. In reviews on spontaneous remissions of oncologic disorders we were unable to find a case of a biologically benign brain stem tumour. There is one isolated report on a similar case, though without histologic documentation.
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3/460. craniopharyngioma of the pineal region.

    Craniopharyngiomas generally develop either in the suprasellar region or in both suprasellar and intrasellar regions. We report on a nontypical location of the craniopharyngioma in the pineal region. An 8-year-old boy was admitted to the department of pediatric neurosurgery in a grave condition. An MRI scan of the brain was performed after the neurological examination and revealed a large neoplasm situated in the posterior part of the III ventricle and in the pineal region, measuring 8.5x6.5x5 cm. The size of the tumor and its location meant it was occluding three ventricles, with subsequent hydrocephaly. Total removal of the tumor was carried out. Microscopic investigation of the tissue removed showed the typical structure characteristic for craniopharyngioma of the adamantinomatous type. Results of a consultation 6 months after the operation revealed that the patient was feeling well, attending school regularly and had finished the first semester with excellent results. On control MRI scan no tumor was found.
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4/460. Venous subarachnoid hemorrhage after inferior petrosal sinus sampling for adrenocorticotropic hormone.

    Neurologic complications associated with inferior petrosal sinus sampling for adrenocorticotropic hormone in the diagnosis of cushing syndrome are rare. Previously reported complications include brain stem infarction and pontine hemorrhage. We report a case of venous subarachnoid hemorrhage with subsequent acute obstructive hydrocephalus occurring during inferior petrosal sinus sampling for cushing syndrome.
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5/460. The origin of hypsarrhythmia and tonic spasms in West syndrome: evidence from a case of porencephaly and hydrocephalus with focal hypsarrhythmia.

    We report on a 3-year-old girl with West syndrome and with focal hypsarrhythmia. The left hemisphere of the patient was virtually completely defective and continuous hypsarrhythmia was only seen in the residual right frontal cortex, where an interictal single photon emission computed tomography (SPECT) showed hyperperfusion. Despite a focal epileptic pattern, the tonic spasms were quite symmetrical. In our patient, spasms might not require the sensorimotor cortex, but the brainstem containing the descending pathways that control spinal reflexes and other infratentorial structures seem to be essential for the occurrence of spasms. This is in accordance with the result of an ictal SPECT that showed hyperperfusion of the brainstem and cerebellum. These findings suggest that hypsarrhythmia originates from cortical lesions, while subcortical structures may be primarily responsible for the tonic spasms in this patient.
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6/460. December 1998--16 year old female with headaches, lethargy and a sellar/suprasellar mass.

    A 16 year female with a history of developmental delay and shunted hydrocephalus presented with two months of progressive headaches, lethargy and visual disturbances. An MRI of the brain revealed a sellar and suprasellar cystic mass which was absent on a previous MRI six years earlier. The pre-operative clinical diagnosis was pituitary adenoma vs. craniopharyngioma. Histologically, the fibrous wall of the ciliated epithelial-lined cyst was thickened by non-caseating granulomatous inflammation, hemorrhage, hemosiderin, and cholesterol clefts, consistent with cyst rupture. Rathke's cleft cysts are uncommon symptomatic lesions in young people, and must be distinguished from craniopharyngioma.
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7/460. Fetal hydrocephalus secondary to intraventricular hemorrhage diagnosed by ultrasonography and in utero fast magnetic resonance imaging. A case report.

    Although fetal hydrocephalus is commonly detected by prenatal ultrasonographic examination, posthemorrhagic hydrocephalus has rarely been observed in the fetus. We report a case of hydrocephalus secondary to intraventricular hemorrhage (IVH) diagnosed by in utero magnetic resonance imaging (MRI) at 37 1 weeks of gestation. ultrasonography revealed enlargement of the bilateral ventricles and an irregular mass measuring 20 x 12 x 10 mm in the right lateral ventricle. T1-weighted images with two-dimensional fast low-angle shot (2D-FLASH) and T2-weighted images with half-Fourier single-shot turbo spin echo (HASTE) demonstrated that an old hemorrhagic clot existed in the right lateral ventricle of the fetus. hydrocephalus secondary to IVH was confirmed by postnatal MRI and ventriculoscopy. Fast MRI is especially useful for prenatal diagnosis of fetal brain abnormalities because it minimizes the artifact of fetal movement.
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8/460. Endoscopic-guided proximal catheter placement in treatment of posterior fossa cysts.

    PURPOSE: Treatment of posterior fossa cysts by cystoperitoneal shunting may be complicated by a malpositioned proximal catheter located within the brainstem or cerebellum causing acute shunt malfunction or neurological deficits. We propose that proximal catheter placement from a posterior fossa approach aided by a malleable endoscope may prevent malposition and its complications. methods: We present 4 procedures we performed on 3 patients with posterior fossa cysts using a posterior fossa approach. In each case, the proximal catheter was molded along with a malleable endoscope to place the catheter parallel to the long axis of the fourth ventricle. Direct visualization during catheter placement insured an intracavitary position. RESULTS: Ultimately, the procedure was successful in all 3 patients as judged by intracavitary catheter position and decrease in cyst size on postoperative imaging. In 1 patient, revision using the same technique was required based upon suboptimal catheter position within one of numerous cystic compartments within the posterior fossa. There were no complications related to direct or indirect brainstem injury. CONCLUSIONS: Many posterior fossa cysts can be treated effectively and safely via a posterior fossa approach with the aid of a malleable endoscope. Direct visualization facilitates intracavitary catheter placement and orientation of the catheter in the long axis of the cyst, thereby decreasing the risk of injury to surrounding structures.
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9/460. 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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10/460. Bilateral sensorineural deafness, partial agenesis of the corpus callosum, and arachnoid cysts in two sisters.

    We describe two sisters (ages 10 and 3 years, respectively) with a normal development and a combination of congenital sensorineural hearing loss, partial agenesis of the corpus callosum, arachnoid cyst, and hydrocephalus. Neither girl has distinctive physical anomalies. In the oldest girl, there was a hearing loss of 80 dB bilaterally, and the most severe loss on audiogram was seen at 2,000-4,000 Hz. In the youngest girl, there was a hearing loss of 100 dB bilaterally. Above 2,000 Hz no neural reactions were seen. Cerebral magnetic resonance imaging in one girl and computed tomography in the other showed a partial agenesis of the corpus callosum and a cyst in the pineal region, causing an aqueduct stenosis by compression and consequent hydrocephalus. The parents have normal hearing, and brain magnetic resonance imaging showed no abnormalities. They are nonconsanguineous but from the same small village. This is the first report of a combination of congenital sensorineural hearing loss, partial agenesis of the corpus callosum, and an arachnoid cyst. The pattern of inheritance is probably autosomal recessive.
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