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Query: UMLS:C0264733 (ventricular dilatation)
 2,163 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Concerning the clinical signs and symptoms, we noticed skin white macula in 87%, adenoma sebaceum in 53%, mental retardation in 75%, retinal phacoma in 54% and seizure in 92%. Numbers of nodules ranged from zero to 11, and showed no correlation with aging. Half of the cases showed slight ventricular dilatation, one quarter showed moderate dilatation and the remainder were normal. There was no definite correspondence between size of the ventricle and number of nodules. There were relatively many cases with normal mental state in the group with normal ventricles. In adult cases a large ventricle does not always mean poor prognosis in mental state. Almost all of the cases with cortical atrophy had mental retardation. In the group with moderate dilatation infantile spasms were the most frequent type of seizure. On the contrary, grand mal was most frequent in the normal group. As for the position of nodules there was no difference between the normal and dilated groups. Nodules were predominantly seen in the lateral aspect of the body, trigone of the lateral ventricle and adjacent to the foramen of Monro. Asymmetrical lateral ventricles were noticed in 18%. Incidence of laterality increased as dilatation proceeded. It was larger on the left side in 9 out of 11 cases. We have no reasonable explanation of this tendency. There was one case with a tumor which was verified by enhanced CT. There were five cases with brain anomalies.
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PMID:Computed tomography in tuberous sclerosis--with special reference to relation between clinical manifestations and CT findings. 31 69

Tuberous sclerosis (Bourneville-Pringle phacomatosis) has been known to be associated with cardiac rhabdomyoma, but apparently never previously with primary pericardial mesothelioma. We present an autopsy case of this condition in a 59-year-old man, who had been diagnosed as having tuberous sclerosis in view of the presence of facial sebaceous adenoma, mental retardation, intracranial calcification, cerebral ventricular dilatation and renal tumor. During the clinical course, characterized by heart failure due to cardiac tamponade, cardiac sarcoma was diagnosed by imaging techniques. Autopsy revealed biphasic-type primary pericardial mesothelioma. As to the tuberous sclerosis, atypical giant cells in the tubers of the cerebral cortex and the lateral ventricular wall were found, which were considered to be derived from neurons rather than glial cells on the basis of staining with Bodian, Holzer, and antibodies against NSE, GFA and S-100 protein. In old tubers protruding into the lateral ventricles, fibrous glias were present with dense calcospherite deposits, coinciding with the CT findings. The renal tumors were angiomyolipomas, which were present bilaterally and showed partially infiltrative growth, but seemed to have a benign nature because of the lack of metastasis and atypism of the leiomyocytes.
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PMID:An autopsy case of tuberous sclerosis associated with primary pericardial mesothelioma. 248 38

The role of MR in evaluating tuberous sclerosis is reviewed in 15 patients. These studies were compared with CT scans, which were available in 14 patients. Four characteristic findings were noted on the MR images obtained. Subependymal nodules projecting into the lateral ventricles were seen in 12 of 15 patients on T1-weighted images. This was the most specific finding. Distortion of the normal cortical architecture was seen in 10 of 11 patients in whom T1-weighted images were obtained using a 256 X 256 matrix. These foci corresponded to multiple cortical areas of increased signal on T2-weighted images. Dilated ventricles were seen in five patients. In one patient, a known astrocytoma showed increased signal on the T2-weighted images, allowing differentiation from a benign subependymal nodule. MR depicted the cortical hamartomas more completely than did CT. The MR scans were abnormal in all cases, and a diagnosis could be confidently made in all 11 cases scanned using a 256 X 256 matrix. Our preliminary experience suggests that MR will at least equal and probably exceed CT, both for sensitivity and specificity, given the use of a 256 X 256 matrix.
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PMID:MR imaging of intracranial tuberous sclerosis. 349 66

The findings on computerized axial tomography of the brain in three patients with tuberous sclerosis are reported. Numerous intracranial calcified nodules varying in size from a few millimeters to one centimeter, located preferentially below the ependyma of the lateral ventricles and determining filling defects in its lumen were found in all cases. Other calcified lesions were found in a subcortical location. One patient presented areas of low density throughout the hemispheric white matter, and another showed ventricular dilatation of the ventricles and basal cisternas. Computerized axial tomography is useful in the evaluation of patients with tuberous sclerosis because of its early demonstration of the characteristic intracranial lesions and detection of complications such as calcification of basal and cerebellar ganglia, hydrocephalus, and development of gliomas. Low density areas may correspond to foci of demyelinization. Furthermore, computerized axial tomography allows the demonstration of cerebral tuberosities in asymptomatic relatives, thus offsetting the inconveniences of pneumoencephalography.
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PMID:[Computerized cerebral tomography in tuberous sclerosis. Study of 3 patients]. 720 79

We report on 4 cases of brain tumors associated with tuberous sclerosis. In each case the tumor was in the region of Monro's foramen and was associated with ventricular dilatation. Computer tomography scans, which were performed in 3 cases, revealed well-circumscribed high-density masses which were markedly enhanced by contrast medium. 3 patients underwent craniotomy. Total extirpation was performed in 2 patients. Both of them are doing well postoperatively. However, 1 patients whose tumor had been partially resected died of recurrence 6 years after surgery. The pathology of three tumors was different in each case: plump cell astrocytoma, hemangioma and astroblastoma.
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PMID:Brain tumors in tuberous sclerosis. Report of 4 cases. 726 91

Two hundred eighty six infants with Down syndrome have been studied. Infantile spasms have been identified in nine of them, in which background, EEG pattern and its evolution, modalities of treatment and its effects, neuroimaging and development course have been revised. None of these patients had either familiar or personal pathological antecedents. The pattern in the first EEG made was hypsarrhytmic in all cases except one which showed a multifocal paroxystical activity, with intermittent and bilateral bursts of spike-waves. The treatment first used was ACTH in four cases, valproate in three cases and phenobarbital in two cases (one of these associated with nitrazepam). The ACTH treatment was effective in seven infants, either was the first or the second choice. An infant in whom the first treatment with ACTH was not successful, responded to the association with valproate+clonazepam. One patient treated initially with phenobarbital+nitrazepam, having no response to different prescriptions, responded finally to the association of carbamacepin+vigabatrin. The first treatment with valproate or phenobarbital had no effect in all patients. The EEG pattern improved in all cases just after the treatment response. Normal tracing was found for a period of two months to three years. Cranial TC was performed to three infants showing one of them a discrete ventricular dilatation and periventricular calcifications that suggested tuberous sclerosis. It is important to point out that, although the good effect of therapy and EEG pattern normalization, the development is below what had been expected in children with Down syndrome. Behavioral problems have been found in seven (77.7%) of these children.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Infantile spasms in children with Down's syndrome]. 749 84

The article reports two cases of childhood autism in tuberous sclerosis (TS). Certain atypical features are highlighted. The probands did not show the common seizure types associated with either TS or autism. No ventricular dilatation, cerebral atrophy or temporal lobe involvement was evident. The high prevalence of childhood autism in TS probands with moderate to severe mental retardation has been emphasized.
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PMID:Childhood autism in tuberous sclerosis. 1077 14

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.
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PMID:Postnatal outcome of fetuses with the prenatal diagnosis of asymmetric hydrocephalus. 1127 May 20

Fetal brain MRI provides complementary information to ultrasonography exploration, the gold standard for antenatal evaluation. Certain specific correlations between the antenatal and postnatal findings merit discussion: cysts after increase in volume during the perinatal period; certain anomalies are at the limit of resolution (tuber of tuberous sclerosis, heterotopy); anatomic visibility of certain structures does not exclude function deficiency (optical chiasma in septo-optic dysplasia; ventricular dilatation is a frequent sign but can occur in very different entities.
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PMID:[Brain MRI: ante and postnatal correlations]. 1576 24

Fetal magnetic resonance imaging (MRI) is an adjunct to sonography (US), often necessary when cerebral abnormality is suspected. With use of fast sequences, such as T2 HASTE or SSFSE, gradient-echo T1- weighted images and diffusion-weighted imaging, it is possible to obtain images of fetal brain in three planes without mother's sedation. Diagnosing brain anomalies using MRI requires good knowledge of normal anatomy depending on gestational age: phases of neuronal migration, sulcation and gyration, myelination in particular. The main indications to perform MRI are as follows : ventricular dilatation, midline and posterior fossa abnormalities, microcephaly (in search for migrational disorders), cerebral location of tuberous sclerosis which is suspected when cardiac tumours are detected. MRI allows to confirm US diagnosis and to answer the question whether the abnormality is isolated or complex. This enables not only to establish the diagnosis but also the prognosis. This method plays an important role in the work of the interdisciplinary team managing the pregnancies with a suspicion of congenital anomalies. Prenatal MRI is a great progress in diagnosing brain anomalies and has become indispensable in modern perinatology in the last decades. The situation of mother and child after the anomaly had been detected requires discussion and care of the interdisciplinary team consisting of an obstetrician, neonatologist, radiologist, geneticist, pathologist, psychologist and paediatric neurologist.
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PMID:[What is the impact of fetal magnetic resonance imaging (MRI) on prenatal diagnosis of cerebral anomalies]. 2225 23


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