Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P50583 (asymmetrical)
 12,197 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two cases of spontaneous cerebral ventriculostium are presented. The first case is that of a 3 year-old girl with a thumb-sized soft scalp tumor of the occipital region (dural hypertrophy) and hydroencephalodysplasia (Picaza). PVG revealed noncommunicating hydrocephalus with asymmetrical deformity of the lateral ventricle and agenesis of corpus callosum (Fig. 1). Ventriculoatrial shunt was performed. Three years passed under the useful life when she readmitted to our clinic complaining headache, nausea and vomiting. On the first hospital day she fell into respiratory arrest accompanied with coma after the tonic convulsion, and eventually, she died on the fourth hospital day. Postmortem examination revealed spontaneous cerebral ventriculostium which communicated with the posteromedial trigone of the left lateral ventricle (Fig. 3). Combined other malformations such as dysgenesis of the corpus callosum and only one anterior cerebral artery, etc. were found. The second case is that of a young adult, a 22 year-old male with rapidly progressing intracranial hypertension. PVG revealed marked dilatation of the lateral and the third ventricle, non-filling of the aqueduct and spontaneous cerebral ventriculostium which communicated with the posterior part of the third ventricle (Fig. 4). And insidiously he fell into akinetic mutism. After suboccipital exploratory craniotomy and ventriculo-peritoneal shunt akinetic mutism improved gradually, and he was discharged on foot after 7 months. PEG performed on June 8, 1973, showed no evidence of aqueduct obstruction and injected air passed from the fourth ventricle to the third one smoothly. He lives on now under a useful condition. These 2 cases are the first report on literatures in Japan, but presumably there must be many other cases. Since W. H. Sweet reported his own two cases of spontaneous cerebral ventriculostium on 1940, more than thirty cases have been published on literatures. However, there are found various expressions to describe the same condition (Table 1). We would like to propose that the most suitable expression is "ventriculostium" not only in deference to the originality of W. H. Sweet but also not to confuse this pathogenetic state with other similar conditions. The author's next interest is the chronological fact that from W. H. Sweet (1940) to A. Torkildsen (1948), all but one ostiums reported situated at the posteromedial trigone of the lateral ventricle, whereas after A. Torkildsen, they were found at the posterior part of the third ventricle in many cases. The reason is unknown. It would appear that three main conditions are necessary for the development of ventricluostium just beneath the tentorium. The first, there must be increased pressure within the lateral or the third ventricle. The second essential feature is the lack of any large space occupying lesion in the the infratentorial space. The third, there must be wider space between the tentorial incisura and the brain stem.
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PMID:[Spontaneous cerebral ventriculostium (author's transl)]. 94 70

This is a report of 22 cases of papillomas of the choroid plexus diagnosed and managed personally by the author. The angiographic diagnosis and extension of choroid plexus papillomas are described, as is the surgical technique for removing these tumors from the lateral, third, and fourth ventricles. Specific attention is given to using the angiogram as the study of choice upon which surgical technique is planned. The diagnosis of choroid plexus papilloma of the lateral ventricle is made by observing the presence of a hypertrophied anterior choroidal artery, a 'double tumor' sign with one tumor at the trigone and the other within the temporal or frontal horn, tumor stain at the trigone, and asymmetrical hydrocephalus, generally, with a shift away from the side of the larger ventricle. Posterior fossa papillomas cause symmetrical hydrocephalus and generally have tumor stain located within the midline, with the major feeding coming from either the superior cerebellar or the posteroinferior cerebellar arteries. Surgical removal of the tumor should entail an 'en bloc' resection following occlusion of the feeding and draining vessels. The tumor should be removed directly when it is in the lateral ventricle and following ventriculocerebral spinal fluid shunting when it is in the posterior fossa.
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PMID:Diagnosis and surgical treatment of choroid plexus papillomas. 108 Oct 32

A Danish family with seven males with hydrocephalus in three generations is described. Frequent findings in this x-linked recessive form of the disease are irregular cranium, asymmetrical face, thumb held across palm, spasticity of the legs and paradoxical plantar responses. The literature on this mode of inheritance is reviewed.
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PMID:Sex-linked hydrocephalus. 118 43

Electroroentgenography was used for the first time in neurological practice in 70 patients. Indications for its use were brain tumors, intracranial hematomas, epileptical seizures and a necessity to define inflammatory, traumatical changes and brain tumors. These studies permitted to depict hydrocephaly, basal and convexital arachnoiditis, asymmetrical ascites, local dilatation of the third ventricule, a dislocation and deformation of the ventricules in tumors and intracranial hematomas, sequalae of closed brain injuries. It was also proved that the use of electroroentgenography is leasible and has advantages in the evaluation of the CSF system.
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PMID:[Use of electroroentgenography in air contrast studies of the brain]. 121 Sep 37

Five patients were studied with the diagnosis of secondary hydrocephaly to neurocysticercosis. Valvular dysfunction was observed due to the obstruction of the ventricular catheter caused by cysticercus cysts. The Biomed System was used in four cases and the Hakim System in one. Valvular dysfunction was observed in patients within a period of 18 to 24 months after derivation, they also had a history of several valvular dysfunctions. The diagnosis was made upon extraction of the catheter where the cyst was found to be attached to the ventricular brush. Subsequent evolution has not been satisfactory. The reasons for this complication are of a hydrodynamic and pharmacological nature and are also due to the growth of the cyst. This complication is not often suspected, therefore we recommend that in cases of frequent valvular dysfunction and asymmetrical hydrocephaly, studies like iodine-tomography or magnetic resonance be carried out in order to rule out this factor.
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PMID:Valvular dysfunction due to the obstruction of the ventricular catheter caused by cysticercus cyst. 181 85

Brainstem acoustic evoked potentials (BAEPs) were measured in 14 children with different type of posterior fossa tumours several times during the clinical course, in order to assess the value of this simple and non-invasive method in the diagnosis and follow-up of posterior fossa tumours in childhood. Eight children had midline medulloblastoma, three children had lateral astrocytoma, three had intrinsic brainstem glioma. Different BAEP patterns could be detected in different tumour's type: bilateral symmetrical or slightly asymmetrical I-V. IPL prolongation in midline medulloblastomas, unilateral or markedly asymmetrical I.-V. IPL prolongation or wave V. depression on the contralateral side in lateral astrocytomas, and severely distorted asymmetrical waveform in intrinsic brainstem gliomas. The BAEPs were abnormal earlier than CT scan in a case of craniospinal astrocytoma. BAEPs were useful in the follow-up: the effect of the preoperative chemotherapy or the progression of the inoperable tumours could be as well documented by this method, as by the CT scan. BAEPs proved effective in the assessment of postoperative neurological complications: bilateral symmetrical IPL prolongation and wave V. depression with clinical signs of increased intracranial pressure occurred in a case of postoperative occlusive hydrocephalus, unilateral IPL prolongation occurred during irradiation or chemotherapy after medulloblastoma removal as signs of cerebral oedema.
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PMID:Value of brainstem acoustic evoked potentials in posterior fossa tumours in childhood. 186 81

Five patients were studied who suffered secondary hydrocephalus due to neurocysticercosis. Shunt dysfunction was due to the obstruction of the ventricular catheter caused by the own cysticercus cysts. The Biomed system was used in four cases and the Hakim system in one. Valvular dysfunction was observed in patients within a period of 18 to 24 months after surgery, they had a history of several valvular dysfunctions. The diagnosis was made upon of extracting the catheter where the cyst was found to be attached to the ventricular brush. Subsequent evolution has not been satisfactory. The reasons for this complication are of a hydrodynamic and pharmacological nature and are also due to the growth of the cyst. This complication is not often suspected, therefore we recommend that in case of frequent valvular dysfunctions and asymmetrical hydrocephalus, studies like iodinetomography or magnetic resonance imaging be carried out in order to discard this possibility.
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PMID:Obstruction of the ventricular catheter of a CSF shunt system due to the own cyst of Taenia solium. 210 12

Because transcranial Doppler ultrasound is a blind procedure, it is not possible to routinely correct for insonation angle errors, which are presumed to be small. In anatomically normal brains this is a valid assumption; however, in some patients with distorted vascular anatomy (as in hydrocephalus) a small insonation angle cannot be assumed, and measurements of flow velocity may be misleadingly low. The orientation of the middle cerebral arteries was studied on magnetic resonance images of 17 control patients and three patients with hydrocephalus, and estimates of insonation angle errors in velocity measurements were made. When asymmetrical vessel distortion is present, relative flow to each hemisphere may not be accurately reflected in the measured velocities. Under these circumstances, the resistivity index may be a more reliable hemodynamic parameter.
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PMID:Impact of vessel distortion on transcranial Doppler velocity measurements: correlation with magnetic resonance imaging. 220 91

We report a family with an 18p trisomic mother and two 18p tetrasomic daughters. The mother is phenotypically normal and healthy, but with an unusual type of trisomy 18p: 47,XX,del(18)(pter----p11.21),+i(18p) de novo. The older sister has microcephaly, mental retardation, an asymmetrical and peculiar face with low set ears, pinched up nose, high arched palate, small mouth, micrognathia, tapering fingers, asymmetrical length of legs, and an asthenic body. The younger sister was stillborn with extensive defects of the skull, congenital hydrocephalus, severe facial anomalies, and lumbosacral meningocele. Both daughters have inherited one normal chromosome 18 and an isochromosome 18p from their mother, and one normal chromosome 18 from their father. Although one quite similar family has been reported, to the best of our knowledge there have been no reports of families in which two daughters with tetrasomy 18p syndrome have been born to a mother with trisomy 18p with isochromosomes.
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PMID:Sibs with tetrasomy 18p born to a mother with trisomy 18p. 265 71

A 9-year old country boy developed blepharitis with inflammation of the face and, 1 month later, eosinophilic meningitis with paralysis of 3 limbs and of an abducent nerve. Nuclear magnetic resonance imaging of the central nervous system disclosed a lesional signal beneath the floor of the 4th ventricle, which was compatible with the presence of a larva of fly. Treatment with thiabendazole was tried, and the clinical signs regressed. Six months later, an asymmetrical hydrocephalus due to obstruction of Monroe's foramen by an inflammatory granuloma was discovered. Human hypodermyasis, due to migration in tissues of larvae of flies, is not rare in cattle-breeding areas. Neuromeningeal disorders are observed in 12% of the cases, consisting of eosinophilic meningitis sometimes associated with neurological deficit or seizures. Such complications as intracerebral haematoma or meningeal haemorrhage may occur during the usually benign course of the disease.
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PMID:[Neuromeningeal hypodermyiasis complicated by hydrocephaly. Value of nuclear magnetic resonance imaging]. 295 74


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