Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In 34 patients with hypertelorism aged on the average 12.6 years, 24 underwent intracranial surgery (combined intra- and extracranial approach), 2 (moderate) U-osteotomy (subcranial approach), and 8 (mild) canthoplasties. Improved results were obtained in the patients with various types of hypertelorism. The complications were reviewed. Of the 24 patients undergone intracranial surgery, one died, 4 had cerebrospinal fluid leakage, and 4 had keratitis. No seizure, cerebral edema, meningitis, blindness, and ptosis were observed in these patients. Average blood loss was 72% of blood volume. Average duration of surgery was 7.5 hours. The causes of hypertelorism such as craniofacial cleft, craniosynostosis, frontoethmoidal meningoencephalocele, frontonasal fibrous dysplasia, and trauma were also discussed. Satisfactory appearance was seen in most of the patients.
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PMID:[Surgical correction of 34 patients with hypertelorism]. 147 4

A series of 80 children with congenital malformations of the central nervous system treated at the Neurosurgery Unit of the Jos University Teaching Hospital (JUTH) in northern Nigeria from January 19844 to December 1985 is discussed. 5 children with hydrocephalus and 3 with myelomeningocele were not treated surgically and were excluded. These children accounted for 60% of all pediatric neurosurgery patients. They were initially seen from the age of 7 hours to 2 years. There were 47 (59%) with myelomeningocele, all but one, lumbosacral. Many of these either presented with rupture and infection or developed infectious postoperative complications. The postoperative mortality rate was 24%. There were 15 children, (19%) with hydrocephalus, 10 of whom were successfully implanted with ventriculo-peritoneal shunts; 3 children died. 12 children (15%) had encephalocele. 3 children, presenting at 8, 11, and 13 months, had craniosynostosis, 2 in the sagittal and 1 in the coronal plane. These patients have improved in terms of reduced seizure rates. 3 Arnold-Chiari malformations were found, one of whom survived surgery. Tools for assessing intellectual and neuromuscular deficits are needed for this population to objectify follow-up.
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PMID:Congenital malformations of the central nervous system at the Jos University Teaching Hospital, Jos Plateau State of Nigeria. 163 44

The influence of two stressogenic conditions, restraint at 4 degrees C for 30 min (cold-restraint stress; CRS) or swimming at 20 degrees C for 3 min (swim stress; SS), on nociception and on convulsions triggered by different agents was assessed in mice. In saline-pretreated mice CRS and SS caused analgesia (hot-plate test, 56 degrees C), delayed the onset of convulsions induced by pentylenetetrazol (PTZ, 100 mg/kg, IP) and aggravated convulsions elicited by maximal transcorneal electroshock (150 mA pulses at 60 Hz for 0.2 s). Pretreatment with naloxone (10 mg/kg, SC, 30 min prior to testing), which did not affect the responsiveness of nonstressed mice to the hot plate or to the convulsant treatments, attenuated the development of analgesia following CRS, but not SS, and further prolonged the latency to onset of PTZ-induced convulsions in both stressed groups. Thus the extent to which CRS and SS can each delay the onset of PTZ-triggered convulsion appears to be limited by activation of a proconvulsant opioid system. In contrast, naloxone pretreatment did not modify the effects of CRS or SS on the severity of electroshock-induced seizures. In conclusion, CRS and SS can each, simultaneously, exert anticonvulsant and proconvulsant influences on responsiveness to PTZ and electroshock, respectively. Also, both forms of stress can activate an opioid system modulating the onset of PTZ-induced seizures, which is distinct from that controlling nociception. These findings, together with those of other stress, convulsions and opioid systems, which depends on the characteristics of the stressogenic condition, species, convulsant agent and parameter considered.
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PMID:Effects of cold-restraint and swim stress on convulsions induced by pentylenetetrazol and electroshock: influence of naloxone pretreatment. 180 34

Twenty-three cases of craniosynostosis were studied with regard to age at operation, symptoms, operative methods, and long-term results. Long-term results of 20 cases that were followed up for more than 1 year after surgery showed that mental retardation remained in 8 cases; a slight improvement in mental function was observed in only 2 cases. Of the 8 patients with mental retardation, 7 were operated on more than 6 months after birth, 7 showed striking digital impression on skull X-ray films and 5 were suffering from oxycephaly. Of the 8 patients with mental retardation, 5 had suffered from perinatal asphyxia or had seizures during delivery and subsequent past history. From these observations, factors affecting prognosis are: (1) age at operation; (2) the degree and duration of increased intracranial pressure; (3) the extent and degree of suture closures; (4) perinatal asphyxia and seizure disorders.
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PMID:Functional prognosis of surgical treatment of craniosynostosis. 398 42

An experimental and clinical study was undertaken to determine whether Zenker's solution without glacial acetic acid (modified Zenker's solution) applied to the external surface of the dura mater can penetrate to injure the underlying cortex. Adult cats and dogs, the dura of which had an average thickness of 0.2 and 0.25 mm, respectively, were given intravenous Evans blue dye as an indicator of blood-brain barrier breakdown. This was followed by the application of modified Zenker's solution to the dura in a manner identical to the clinical practice. When the animals were killed 1 or 2 hours later, the cortex beneath the dura painted with modified Zenker's solution was stained massively. The thickness of human dura was measured; it varied from 0.22 to 0.28 mm in infants of 3 months or less, 0.27 to 0.42mm in infants 4 months to 12 months of age, and 0.32 to 0.45 mm in children to 1 to 18 years of age. Modified Zenker's solution applied to fresh dura for 3 minutes at autopsy gave visual evidence 1 hour later of penetration to the cortex beneath. In operative procedures for craniosynostosis, 69 patients were treated with modified Zenker's solution; of these, 7 developed seizures in the immediate postoperative period. No seizures occurred among the 39 patients on whom modified Zenker's solution was not used. It is concluded that modified Zenker's solution applied to the dura can damage the cortex beneath.
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PMID:Cortical damage from Zenker's solution applied to the dura mater. 720 76

Zenker's solution is a tissue fixative containing mercuric chloride, potassium bichromate, sodium sulfate, and glacial acetic acid. In 1956, Anderson and Johnson reported its use in clinical neurosurgery. They applied the solution to the exposed dura after craniectomy. Delayed bone formation was thought to be due to the suppression of the osteoblastic activity of the outer layer of the dura. The fixative has since become a well-accepted adjuvant to the treatment of craniosynostosis. In 1972, Pawl and Sugar reported postoperative seizures in 6 of 34 patients treated with this solution. They assumed that the fixative penetrated the dura and irritated or damaged the cortex. To clarify the effect of Zenker's solution on the underlying brain, we performed bilateral parasagittal craniectomies in a group of kittens and adult cats. Zenker's solution was applied to one side and the other side served as a control. The animals were killed after periods varying from 24 hours to 2 months. We then examined the cortex under the craniectomies. There was immediate breakdown of the blood-brain barrier, as evidenced by the penetration of intravenous Evans blue. In the postoperative period investigated, an inflammatory response in the underlying brain with thickening of the arachnoid occurred. The results and implications of these experiments are presented. (Neurosurgery, 6: 45--48, 1980)
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PMID:Effect of the dural application of Zenker's solution on the feline brain. 735

Early surgery for isolated craniosynostosis is designed to improve morphology, to prevent functional disturbances, and equally important, to enhance the psychosocial development of the child. As the first of a two-part series, 104 patients with isolated craniofacial synostosis were retrospectively analyzed. Diagnoses included bilateral coronal (10), unilateral coronal (57), metopic (29), and sagittal synostosis (8). All patients underwent primary fronto-orbital advancement-calvarial vault remodeling procedures at less than 18 months of age (mean 8.1 months). Thirteen percent of patients (14) required a secondary cranial vault operation (mean age 22.6 months) to address residual deficits in craniofacial form. Perioperative complications were minimal (5.0 percent), and there was no mortality. Average length of postoperative follow-up was 46.0 months. By the classification of Whitaker et al., which assesses surgical results, 87.5 percent of patients were considered to have at least satisfactory craniofacial form (category I-II) at latest evaluation. Overall rates of hydrocephalus, shunt placement, and seizures (3.8, 1.0, and 2.9 percent, respectively) were low. Among the isolated craniosynostoses, unilateral coronal synostosis/plagiocephaly poses the most complex problems, including vertical orbital dystopia, nasal tip deviation, and residual craniofacial asymmetry; there is also a wide spectrum of findings and growth patterns in this subgroup.
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PMID:Twenty-year experience with early surgery for craniosynostosis: I. Isolated craniofacial synostosis--results and unsolved problems. 762

As the second of a two-part series, 76 patients with pansynostosis and craniofacial synostosis syndromes were retrospectively analyzed. Diagnoses included pansynostosis (7), craniofrontonasal dysplasia (8), and Apert (24), Crouzon (15), and Pfeiffer (15) syndromes. All patients underwent primary fronto-orbital advancement-calvarial vault remodeling procedures at less than 18 months of age (mean 6.1 months). Twenty-eight patients (36.8 percent) required a secondary cranial vault operation (mean age 28.4 months). Additionally, a major tertiary procedure was necessary in 5 patients to deal with persistent unacceptable craniofacial form. To address the associated finding of midface hypoplasia, 64.8 percent (n = 35) of patients underwent Le Fort III midface advancement or had that procedure recommended for them. The remainder were awaiting appropriate age for this reconstruction. The more extensive pathologic involvement of the pansynostosis and craniofacial syndrome group is illustrated. As compared with the isolated craniofacial synostosis group previously reported, the incidence of major secondary procedures (36.8 versus 13.5 percent), perioperative complications (11.3 versus 5.0 percent), follow-up complications (44.7 versus 7.7 percent), hydrocephalus (42.1 versus 3.9 percent), shunt placement (22.4 versus 1.0 percent), and seizures (11.8 versus 2.9 percent) was significantly increased. Complex problems including those of increased intracranial pressure, airway obstruction, and recurrent turricephaly or cranial vault maldevelopment are repeatedly encountered. In addition, that early fronto-orbital advancement-cranial vault remodeling failed to promote midface development and hypoplasia of this region is almost a consistent finding in the craniofacial syndromic group. The average length of postoperative follow-up was 6 years. According to the classification of Whitaker et al., which assesses surgical results, 73.7 percent of patients were considered to have at least satisfactory craniofacial form (category I-II) at latest evaluation. An algorithmic approach to the treatment of all patients with craniosynostosis is presented utilizing early surgical intervention as the key element.
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PMID:Twenty-year experience with early surgery for craniosynostosis: II. The craniofacial synostosis syndromes and pansynostosis--results and unsolved problems. 762 1

Hennekam syndrome is a rare autosomal recessive syndrome which was described for the first time in 1989. Here, we present a girl with intestinal lymphangiectasia, severe lymphedema of limbs, seizures, mild mental retardation, and facial anomalies consistent with the diagnosis of Hennekam syndrome. In addition, she had an ectopic kidney and craniosynostosis of the coronal suture, 2 manifestations not previously reported in this syndrome. While the molecular basis of Hennekam syndrome remains, as yet, unknown, this report illustrates its variable clinical expression.
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PMID:Craniosynostosis and kidney malformation in a case of Hennekam syndrome. 764 2

We report a family with a newly recognized form of autosomal dominant craniosynostosis. The disorder has high penetrance and variable expression with respect to sutural involvement and cranial abnormalities, ranging from fronto-orbital recession to clover-leaf skull deformity. Associated problems included headache, poor vision, and seizures; intelligence, however, is normal. Assignment of a well-described syndromic designation, e.g., Crouzon, Pfeiffer, Saethre-Chotzen, or Jackson-Weiss, is precluded based upon the absence of characteristic changes, i.e., midfacial hypoplasia, orbital hypertelorism, blepharoptosis, hand anomalies, or foot anomalies. The large size of this family and high penetrance of the disorder suggests that this may be an excellent candidate for positional cloning of a locus important in craniofacial development.
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PMID:Newly recognized autosomal dominant disorder with craniosynostosis. 835 19


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