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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This report describes three children, each of whom developed an unusual malformation consisting of one or more intracerebral arteriovenous fistulas and a large intraparenchymal venous varix. Their clinical symptoms were similar to those produced by aneurysms of the vein of Galen: increasing head circumference, seizures, hemorrhage, and developmental delay. We treated each child with endovascular embolization and/or surgery and obtained complete closure of all fistulas without mortality.
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PMID:Intracerebral arteriovenous fistulas associated with intraparenchymal varix in childhood: case reports. 229 62

A patient with Proteus syndrome is reported. He had hemihypertrophy, bilateral hypertrophy of the third fingers, skin anomalies, and varicosities, as well as mental retardation, seizures resistant to anticonvulsant treatment, and a very severe course with death at the age of 2 years.
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PMID:Proteus syndrome: report of a case with severe brain impairment and fatal course. 235 6

Immunocytochemical techniques were used to study changes of GABA immunoreactivity in the rat hippocampal CA1 region during kindling epileptogenesis, gradually developing over a period of two weeks by daily electrical tetanization of Schaffer collaterals/commissural fibres. The number of GABA-immunoreactive somata per mm2 was quantified in CA1 region after 6 and 14 stimulus-induced afterdischarges and in fully kindled animals. The absolute values were compared with those obtained from controls and the relative difference between the side where the stimulations were applied and the contralateral side was determined. In comparison to controls the rats showed, after 6 afterdischarges at the ipsilateral, stimulated side, a slight increase in cell density of about 10%; after 14 afterdischarges, there was a significant increase of 38% together with an enhanced labelling density, while in fully kindled rats (34 afterdischarges) there was no significant difference with controls. At the contralateral side, we observed a different pattern of, respectively, a small decrease (17%), no significant difference, and a significant increase of 22% in fully kindled animals. This result is discussed in relation to observations that fully kindled rats, investigated 24 days after the last seizure of the acquisition phase, showed rather a significant decrease by 35% of cell density at the stimulated side and a regional loss of immunoreactivity of varicosities. At this point in time, no significant changes were found at the contralateral side. The complex time-course of GABA immunoreactivity during epileptogenesis consists of a transient increase in the early phase of kindling acquisition followed by a process of gradual reduction that leads to the long-term decrease of GABA immunoreactivity and high excitability.
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PMID:The development of changes in hippocampal GABA immunoreactivity in the rat kindling model of epilepsy: a light microscopic study with GABA antibodies. 343 73

Enlarged veins in two vascular malformations blocked the cerebrospinal fluid outflow pathways, causing hydrocephalus. Both patients presented not with the usual clinical picture (hemorrhage, seizure, etc.), but with signs of increased intracranial pressure. Computed tomography and angiography were necessary to understand the pathophysiology. An arteriovenous malformation was responsible for the first patient's headache, and a venous varix was the causative lesion in the second patient. Hydrocephalus caused by a venous varix has not been reported before.
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PMID:Ventricular obstruction secondary to vascular malformations. 686 43

Arteriovenous (AV) fistulas of cerebral and spinal arteries are characterized angiographically by an immediate AV transition without a capillary bed or "nidus" as occurs in AV malformations (AVM's). The clinical presentation, morphology, radiology, and treatment of 12 patients with cerebral AV fistulas and of 12 patients with spinal AV fistulas are reviewed. In the patients with cerebral lesions, headache and seizure disorders were the most common presentations followed by subarachnoid hemorrhage, cardiac failure, progressive neurological dysfunction, and incidental detection on prenatal ultrasound study. In patients with spinal AV fistulas, weakness and sensory disturbance in the lower extremities were the most frequent clinical presentations followed by back pain, disturbances of micturition, and grand mal seizure. The etiology of the symptom complex produced by AV fistulas in each of these locations differed, with venous hypertension being important in spinal cord lesions. Of the patients with cerebral lesions, nine had a single AV fistula, one had two fistulas, and two had multiple fistulas. An AVM was observed in five patients with fistulas (two large, three small). Nine patients exhibited extramedullary AV fistulas of the spine, of whom eight had a single fistula and one had three fistulas; three patients had intramedullary spinal AV fistulas. An arterial aneurysm was found in association with two fistulas, one cerebral and one spinal. Venous ectasias or varices, frequently exhibiting mural calcification, were observed to be prominent in all AV fistulas involving cerebral arteries and in two involving spinal arteries. The location and size of the venous complexes reflected the diameter of the fistula. In addition to conventional imaging techniques (cerebral angiography, computerized tomography, and magnetic resonance (MR) imaging), MR angiography was a helpful adjunct in the evaluation of fistulas. Treatment strategies employed for AV fistulas in both locations included open surgical and endovascular procedures, frequently used in combination. A satisfactory outcome was observed in all patients.
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PMID:Arteriovenous fistulas of the brain and the spinal cord. 827 Oct 12

Synapses of the hippocampal mossy fiber pathway exhibit several characteristic features, including a unique form of long-term potentiation that does not require activation of the N-methyl-D-aspartate receptor by glutamate, a complex postsynaptic architecture, and sprouting in response to seizures. However, these connections have proven difficult to study in hippocampal slices because of their relative paucity (<0.4%) compared to commissural-collateral synapses. To overcome this problem, we have developed a novel dissociated cell culture system in which we have enriched mossy fiber synapses by increasing the ratio of granule-to-pyramidal cells. As in vivo, mossy fiber connections are composed of large dynorphin A-positive varicosities contacting complex spines (but without a restricted localization). The elementary synaptic connections are glutamatergic, inhibited by dynorphin A, and exhibit N-methyl-D-aspartate-independent long-term potentiation. Thus, the simplicity and experimental accessibility of this enriched in vitro mossy fiber pathway provides a new perspective for studying nonassociative plasticity in the mammalian central nervous system.
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PMID:Reconstitution of the hippocampal mossy fiber and associational-commissural pathways in a novel dissociated cell culture system. 864 67

We present the morphology and the laminar distribution of the serotonin (5-hydroxytryptamine, 5-HT) innervation of the cerebral cortex of patients who underwent cortical resection for partial seizures. The limits of the resections were established by stereoelectroencephalography. The 5-HT innervation was mapped by using an antiserum anti-5-HT. Two patients had cryptogenic epilepsies and two others had seizures related to focal cortical dysplasia. 5-HT immunoreactive axons were morphologically heterogeneous and projected diffusely to the cerebral cortex with regional-specific densities. Two types of terminal axon were demonstrated. Type I had large and spherical (intensely immunoreactive) varicosities and was distributed sparsely with a characteristic predominance in the molecular layer. Type II had fine and pleiomorphic varicosities (granular or fusiform) and was distributed through all cortical layers. The distribution of the 5-HT innervation varied according to the different architectonic areas investigated. The granular cortical areas characterized by a highly developed layer IV (primary somatosensory, primary visual and prefrontal cortices) had the highest density of 5-HT-ir fibers distributed from layer I to layer V. The agranular primary motor cortex had the lowest density with fibers preferentially seen in layers I, IIIa and V-VI. The orbital cortex with a poorly defined layer IV had an intermediate density with a laminar repartition predominant in the supragranular layers. In patients with cryptogenic epilepsies, the brain epileptogenic tissue was histologically normal as well as the serotonergic innervation. In contrast, in patients with focal cortical dysplasia, the dysplastic epileptogenic tissue was characterized by a serotonergic hyperinnervation. In agreement with previous data in primates, we give morphological evidence for two morphologically distinct serotonergic subsystems and for regional specific densities in the human cerebral cortex. Moreover, we previously reported an altered pattern of the catecholaminergic innervation in the same dysplasia areas. All these results provide evidence that this development epileptogenic lesion involves several sets of neurons which may contribute to epileptogenic activity.
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PMID:The serotonergic innervation of the cerebral cortex in man and its changes in focal cortical dysplasia. 888 67

Two cases of moyamoya disease observed in two children are reported. The two cases recall the frequency of ischemic strokes, transient ischemic attacks, and seizures revealing the disease. Diagnosis is made by cerebral arteriography showing carotid stenosis and the dense deep arterial collateral as seen in these two cases. Magnetic resonance imaging makes it possible to identify this vascular disease as it reveals vascular varicosities in the basal ganglia, as in case 1. Strokes can sometimes be prevented by constructing an anastomosis between the superficial temporal artery and the middle cerebral artery, as demonstrated in case 1. Thus, the diagnosis of moyamoya disease has been improved by magnetic resonance imaging and there is now a surgical treatment to prevent ischemic stroke.
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PMID:Moyamoya disease in children. A review of the clinical and radiological features and current treatment. 945 75

Homozygous tottering (tg/tg) and compound heterozygous tottering/leaner (tg/tg(la)) mutant mice exhibit juvenile onset of three abnormal neurological phenotypes: (i) petit mal-like epilepsy; (ii) ataxia; and (iii) an intermittent myoclonus-like movement disorder. Homozygous leaner mice (tg(la)/tg(la)) exhibit early onset of ataxia (postnatal days 10-12), and also exhibit the myoclonus-like movement disorder and evidence of absence seizure activity; the myoclonus-like disorder is most evident in the first month of life, then diminishes in severity and frequency. The ultrastructure of the cerebellar molecular layer was examined in adult (six to eight months) and juvenile (20-25 days) mice of all three mutant genotypes. In mice of all three genotypes and both ages, Purkinje cell dendritic spines were observed to make multiple contacts with individual parallel fiber varicosities in all sections analysed. These multiple synaptic units were observed in both anterior and posterior vermis and hemispheres of the cerebellum, and ranged from two to nine spines/parallel fiber varicosity. Occasionally, one of the postsynaptic spines belonged to an ectopic spine emerging from the proximal region of a Purkinje cell dendrite. This increase in the multiple synaptic index of some parallel fiber varicosities was observed in juvenile tottering mice before the onset of the symptoms of the neurological disorders. This is highly suggestive that the onset of the neurological phenotype is not a primary cause of multiple Purkinje cell dendritic spines synapsing with single parallel fiber varicosities in these mice, but on the contrary, that it could be the cause of the ataxic symptoms.
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PMID:An ultrastructural study of granule cell/Purkinje cell synapses in tottering (tg/tg), leaner (tg(la)/tg(la)) and compound heterozygous tottering/leaner (tg/tg(la)) mice. 1021 73

A case of Sturge-Weber syndrome (SWS) with gastrointestinal hemorrhage is presented. SWS is a neurocutaneous disorder characterized by cutaneous facial angioma leptomeningeal angioma with seizures and other neurologic complications. Associated anomalies beyond the encephalofacial territory are very rare. The patient presented repeated bleeds from extensive gastric varices of the fundus secondary to a splenic venous malformation. This is the first report on this association to our knowledge.
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PMID:Sturge-Weber syndrome: a rare cause of gastrointestinal hemorrhage. 1063 20


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