Polaris, a protein involved in left-right axis patterning, localizes to basal bodies and cilia.
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Mutations in Tg737 cause a wide spectrum of phenotypes, including random left-right axis specification, polycystic kidney disease, liver and pancreatic defects, hydrocephalus, and skeletal patterning abnormalities. To further assess the biological function of Tg737 and its role in the mutant pathology, we identified the cell population expressing Tg737 and determined the subcellular localization of its protein product called Polaris. Tg737 expression is associated with cells possessing either motile or immotile cilia and sperm. Similarly, Polaris concentrated just below the apical membrane in the region of the basal bodies and within the cilia or flagellar axoneme. The data suggest that Polaris functions in a ciliogenic pathway or in cilia maintenance, a role supported by the loss of cilia on the ependymal cell layer in ventricles of Tg737(orpk) brains and by the lack of node cilia in Tg737(Delta2-3betaGal) mutants. (+info)
Increased cerebrospinal fluid concentrations of soluble Fas (CD95/Apo-1) in hydrocephalus.
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BACKGROUND AND AIMS: The ventricular enlargement observed in children with chronically raised intracranial pressure (ICP) causes a secondary loss of brain tissue. In animal studies of hydrocephalus, programmed cell death (apoptosis) has been found as a major mechanism of neuronal injury. One of the regulators of the apoptotic cell death programme is the receptor mediated Fas/Fas ligand interaction. METHODS: The apoptosis regulating cytokines soluble Fas (sFas) and soluble Fas ligand (sFasL) were studied in the cerebrospinal fluid (CSF) of 31 hydrocephalic children undergoing shunt surgery for symptomatic hydrocephalus and 18 controls. RESULTS: High concentrations of sFas were observed in children with hydrocephalus (median 252 ng/ml); in controls sFas was below the detection limit (0.5 ng/ml). sFasL was undetectable in all but one sample. CONCLUSION: High concentrations of sFas in the CSF of children with hydrocephalus suggest intrinsic sFas production, potentially antagonising pressure mediated Fas activation. (+info)
Outcome of prenatally diagnosed mild unilateral cerebral ventriculomegaly.
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The objective of this study was to determine the frequency of prenatally diagnosed unilateral cerebral ventriculomegaly and also to assess neonatal outcome in infants with this prenatal diagnosis. A computerized ultrasonography database identified fetuses with isolated and nonisolated unilateral cerebral ventriculomegaly from October 1994 to June 1999. The Denver II Developmental Screening Test was used to assess developmental skills. Unilateral cerebral ventriculomegaly was diagnosed in 15 of 21,172 (1 per 1,411) pregnancies. The width of the enlarged lateral ventricle ranged from 1.0 to 1.9 cm. In 10 (67%) of 15 cases unilateral cerebral ventriculomegaly was an isolated finding. Eight of the 14 infants who were born at 36 weeks' gestation or later had postnatal cranial imaging, and ventricular asymmetry was confirmed in 5 (63%). One infant with an arachnoid cyst and cerebral palsy died at 2 years of age. The remaining 11 infants in whom developmental milestones were assessed had age-appropriate skills. Unilateral fetal ventriculomegaly is usually an isolated finding and when isolated has little measurable effect on developmental outcome. (+info)
Postnatal outcome of fetuses with the prenatal diagnosis of asymmetric hydrocephalus.
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We sought to assess the sonographic findings and postnatal outcome in fetuses with the prenatal diagnosis of asymmetric hydrocephalus. The sonograms from cases of asymmetric hydrocephalus diagnosed prenatally at our institution were reviewed. Postnatal outcome was obtained from maternal, neonatal, and pediatric records. Fourteen fetuses at 17.3 to 38.9 weeks' gestational age on prenatal sonography had a maximum ventricular measurement of 10.2 to 48.8 mm, with the degree of asymmetry ranging from 2.2 to 27.3 mm. Thirteen of 14 had a normal-sized contralateral ventricle. Other fetal anomalies identified at sonography included Dandy-Walker malformation, intraventricular hemorrhage, porencephalic cyst, hydronephrosis, pleural effusion, and mild dilatation of a renal pelvis. Eleven fetuses had follow-up prenatal sonography. Among these, ventricular dilatation resolved in 5, remained the same in 3, increased in 2, and decreased in 1. Postnatal outcome was normal in 6 cases (43%) and abnormal in 8 (57%), including 2 cases of in utero intracranial hemorrhage, 2 with congenital syndromes, 1 with an imperforate foramen of Monro, 1 with tuberous sclerosis, 1 with developmental delays, and 1 with cerebral palsy. Asymmetric unilateral hydrocephalus appears to represent an entity different from bilateral hydrocephalus in that there is less risk of perinatal death, there are fewer associated anomalies, and the overall prognosis is better. Outcome may be normal, but fetuses with increasing unilateral ventriculomegaly and cases associated with other brain abnormalities tend to have a poor neurologic outcome. (+info)
Cerebrovascular adaptation in chronic hydrocephalus.
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This study characterizes the regional changes in vascularity, which accompanies chronic progressive hydrocephalus. Fifteen dogs underwent surgical induction of hydrocephalus and were used for histologic studies. Animals were divided into 4 groups: surgical control, short term (< or = 5 weeks), intermediate term (8 weeks), and long term (10 to 12 weeks). Vessel diameter, density, and luminal area were calculated by imaging quantification after manual vessel identification in the cortical gray, white matter, and caudate nucleus. Capillary vessel diameter decreased 23.5% to 30.2% (P < 0.01) in the caudate, but then returned to normal at 12 weeks. Capillary vessel density decreased 53.5% (P < 0.05) in the cortical gray, but then increased to 234.8% (P < 0.01) over surgical controls at 12 weeks. There was no initial decrease in capillary density in the caudate; however, the long-term group capillary density was significantly greater (172.8% to 210.5%, P < 0.01) than surgical controls. Overall, there was a short-term decrease in lumen area, with recovery in the longer term. Glial fibrillary acidic protein (GFAP) immunohistochemistry demonstrated the pattern of GFAP staining and reactive astrocytes differed in the caudate compared with the occipital cortex. This data suggest that an increase in capillary density and diameter may be an adaptive process allowing maintenance of adequate cerebral perfusion and metabolic support in the hypoxic environment of chronic hydrocephalus. (+info)
Sonography and neuropathology of a congenital brain tumor: report of a rare incident.
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A rare case of a congenital brain tumor was diagnosed by sonography in a fetus at 33 weeks' gestation. The ultrasound examination showed a large area of both increased echogenicity and echolucency in one hemisphere suggestive of brain tumor or hemorrhage. The neuropathological examination revealed an undifferentiated glial tumor with large areas of necrosis. (+info)
Cytologic detection of Toxoplasma gondii tachyzoites in cerebrospinal fluid.
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We reviewed our case records to see how often Toxoplasma gondii organisms were identified by cytologic evaluation of cerebrospinal fluid (CSF). During a 12-year period, 6,090 CSF specimens were examined, and 2 cases (0.03%) showed tachyzoites. Both patients were immunocompromised. One patient underwent lumbar and ventricular taps, and the other underwent only ventricular tap. Organisms were identified in the ventricular specimens but not in the lumbar sample. Both patients were treated, and subsequent ventricular CSF samples were negative. Toxoplasma gondii can be identified by cytologic examination of CSF. Our results confirm prior observations that in patients with obstructive hydrocephalus, tachyzoites are more likely to be found in ventricular rather than lumbar specimens. (+info)
Hypertensive caudate hemorrhage prognostic predictor, outcome, and role of external ventricular drainage.
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BACKGROUND AND PURPOSE: The purpose of the present study was to analyze the outcome and outcome predictors of caudate hemorrhage and role of external ventricular drainage in acute hydrocephalus. METHODS: Clinical data from 36 consecutive patients with hypertensive caudate hemorrhage was used in the present study. Age, gender, volume of parenchymal hematoma, hematoma in the internal capsule, initial Glasgow Coma Scale (GCS), hydrocephalus, severity of intraventricular hemorrhage, and hemorrhagic dilatation of the fourth ventricle were analyzed for effect on outcome. Effect of external ventricle drainage for hydrocephalus was evaluated by comparing preoperative and postoperative GCS scores. RESULTS: By univariate analyses, poor outcome was associated with a poor initial GCS score (P=0.016), hydrocephalus (P<0.001), intraventricular hemorrhage severity (P<0.01), and hemorrhagic dilatation of the fourth ventricle (P=0.02). By multivariate analysis, stepwise logistic regression revealed that hydrocephalus was the only independent prognostic factor for poor outcome (P<0.001). Postoperative 48-hour GCS score was better than the preoperative score by use of paired-sample t test (P<0.001). CONCLUSIONS: Hydrocephalus is the most important predictor of poor outcome. External ventricular drainage response for hydrocephalus was good in the present study, whereas an early decision should be made regarding preoperative neurological condition. (+info)