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Results for “Dandy-Walker Syndrome”

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At least 19 recordsLinked to original sources
PubMed1979

Problems of diagnosis and treatment in the Dandy-Walker syndrome.

10 children with Dandy-Walker syndrome are presented to discuss their clinical assessment and therapy. The presence on physical examination of a large posterior fossa was the most reliable clinical finding and was diagnostic in 9 out of the 10 patients. The different diagnostic confirmatory studies are discussed. The most satisfactory treatment in this series was the combination of a shunt from the lateral ventricle and the posterior fossa to the peritoneum.

Cerebrospinal Fluid Shunts↗
PubMed1978

Dandy-Walker syndrome.

The problem of a patient with hydrocephalus, a posterior fossa 'cyst' and a cerebellar malformation is rather rare. Four cases of the syndrome are presented. The clinical manifestations, pathology, radiological investigations and management of the condition are briefly discussed.

Child↗
PubMed1977

[Diagnosis of increases in acute intracranial pressure in infancy and early childhood].

The results reported in this paper were obtained from 276 cases (babies and infants) with increases in intracranial pressure. In 88 cases the cause of disease was not clear, while 35 patients showed symptoms of an acute increase in intracranial pressure so that diagnosis was an urgent necessity. With subdural hygromata, clinical results obtained for acute rises of pressure usually differ greatly from those obtained for chronic increases of pressure. The diaphany of skull is usually negative. Fundal hemorrhages were observed in acute cases only. Critical cerebral pressures were most frequently observed in the case of tumors. The diagnosis is rather difficult. In the case of functional immaturity of the brain localized neurological symptoms are not usually observed. The cerebral pressure is generally counterbalanced for a long time so that an acute cerebral symptomatology is not produced until tumoral complications, shifts in cerebrospinal fluid passages, intracranial mass shifts, or tumoral hemorrhages occur. Unlike all other groups of diseases, symptoms of rises of intracranial pressure were very marked. Malformations of the posterior cranical fossa included acute occlusions in the case of Dandy-Walker syndromes and arachnoidal cysts. In the case of severe cerebral malformations, acute increases in intracranial pressure are sometimes erroneously concluded from an abnormal increase of the size of head accompanied by a complete lack of reactions on the part of children affected with them. Children with hydrocephalus caused by a disturbance of the circulation of liquor are now treated with pressure-controlled liquor drainage systems. Critical cerebral pressures may occur if the drainage system suddenly fails to operate properly.

Acute Disease↗
PubMed1979

[Pediatric metrizamide CT cisternography and CT ventriculography (author's transl)].

We have studied the diagnostic value of computed tomography with metrizamide CSF enhancement in 37 infants. According to the method of injection, we called CT cisternography, CT ventriculography and CT cystography. These methods were useful for the morphologic and dynamic evaluation of the CSF pathways. We used them especially for the evaluation of hydrocephalus, skull base tumor, infantile subdural hematoma and its allied diseases, or congenital cystic lesion such as Dandy-Walker syndrome. The side effects in children are much less than those in adults.

Adolescent↗
PubMed1975

Transitional forms of Arnold-Chiari and Dandy-Walker malformations.

The morphological findings in 12 cases of Arnold-Chiari and 3 cases of Dandy-Walker malformations are described and compared to those in 2 cases of congenital hydrocephalus of unknown origin, 1 case of isolated meningo-myelocele and 2 cases of normal newborn brains. Lesions common to both types of malformation indicate a same time-related embryogenetic defect of the roofplate of the rhombencephalon, that does not allow the inferior vermis and the choroid plexus to turn inward into the fourth ventricle.

Arnold-Chiari Malformation↗
PubMed1979

Teratogenetic periods for the principal malformations of the central nervous system.

The teratogenetic periods of the most important CNS malformations are defined and briefly discussed. The results are presented in a synoptic figure. The present study is based on the analysis of normal CNS development and on morphogenetic interpretations of the pertinent anomalies. The data compiled from the literature concerning normal development are presented in an analytical table and are also summarized in a synoptic figure.

Anencephaly↗
PubMed1976

Developmental defects of the cerebellum. A radiologic and anatomic investigation.

A classification of cerebellar malformations is proposed, based on more than fifty cases with varying degrees of abnormality, where comparative anatomic and radiologic analysis was carried out. The anatomic information was collected at surgery or autopsy and the radiologic evidence was derived from encephalography or ventriculography and vertebral angiography. The malformations were classified in four groups in order of gravity, with regard to the morphologic and etiologic features: (1) total or sub-total agenesia of the cerebellar structures (vermis and hemispheres); (2) almost complete agenesia of the vermis associated with the Dandy-Walker malformation; (3) almost complete agenesia of the vermis without the Dandy-Walker malformation; (4) partial defects of the vermis, which could be separated into four subdivisions.

Arachnoid↗
PubMed1979

Relation of age and cerebral ventricle size to central canal in man. Morphological analysis.

The central canal of the spinal cord in man with and without hydrocephalus was studied histologically. The lumen was patent in most patients in the first two decades of life. Cells lining the canal in the prenatal and newborn state and in the first decade of life were predominantly pseudostratified ciliated epithelium. In the second decade, the epithelium became simple columnar or cuboidal. The central canal closed in most cases after the age of 20 years, secondary to proliferation of ependymal cells and astrocytes. Mechanisms whereby the number of glial cells increase are considered. The canal was closed in all adults with normal ventricular size, and in 94% of persons with various degrees of hydrocephalus. In the remaining 6% of cases with hydrocephalus, the lining of the canal resembled that seen in the first two decades, and could have acted as a pathway of cerebrospinal fluid (CSF) absorption. Three cases of severe hydrocephalus in the first two decades of life were encountered; the central canal was patent in one, and occluded in two. Based on these data, the canal was not a significant pathway of CSF absorption in most instances of hydrocephalus and in persons with dilated ventricles who were older than 20 years of age.

Adolescent↗
PubMed1976

The abnormal brain scan: specificity of descriptive parameters.

When a brain scan is abnormal, one can often describe the abnormality in terms of its location, shape, sharpness, brightness, and other descriptive parameters. Certain types of abnormality have been linked to certain specific diagnoses: for example, a crescent-shaped lesion would suggest subdural hematoma to many people, a wedge-shaped lesion would suggest cerebral vascular accident, etc. Some features thought to be characteristic of certain diseases are actually quite nonspecific. For example, the "doughnut" sign--at first believed to be characteristic of brain abscess--has also been found in primary and metastatic tumors, CVA, and subdural hematoma. The "crescent" sign that was at first thought to be specific for subdural hematoma occurs also in meningitis, scalp or skull trauma, meningioma en plaque, etc. Some features of abnormalities are highly specific for certain diseases; e.g., wedge- or flame-shaped lesions are rarely seen with disorders other than CVA, and lesions in the midline or in the posterior fossa are almost invariably tumors. This article reviews the features of abnormalities on brain scans that in the literature have been reportedly associated with specific types of disease and explores the strength and validity of the associations.

Adenoma, Chromophobe↗
PubMed1979

Cerebellar malformations: some pathogenetic considerations.

1) Destructive processes are responsible for most cases of cerebellar microgyria of the trabecular pattern. Erosion and subsequent fusion of the folia produce the disorganized pattern in which the various cellular elements retain their noraml relationship and are capable of normal maturation. Intrauterine infection is responsible for most cases; the evidence is conclusive in some cases, presumptive in others. 2) Faulty genetic coding, as illustrated by the trisomies, may lead to formation of heterotopias. The primitive cells aggregating around the dentate nucleus should be interpreted as matrix cells and not as cells of the external granular layer. Cortical heterotopias with attempted internal organisation also occur; their origin is obscure. The unusual, possibly unique, transposition of the internal granular and Purkinje cell layers observed in one case may be ascribed to faulty formation of the Bergmann glia by analogy with the weaver mouse. 3) It is impossible at present to disentangle the role of genetic and environmental factors in the pathogenesis of the hysraphic malformations. It is possible, however, that defective fusion of the intraventricular cerebellar primordium plays a part in the development of the Dandy-Walker malformation, of midine cerebellar clefts in some cases of occipital encephalocele, and of extra-axial ependymal cysts of the posterior fossa.

Brain Diseases↗