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)

Despite the fact that measles is severe and presents in very young Black children in Natal, South Africa, no case of subacute sclerosing panencephalitis was reported from this region prior to 1982. A retrospective study was therefore made over the six-year period 1982-1987 of 18 patients who presented to the King Edward VIII teaching hospital, Durban, with clinical and laboratory features of subacute sclerosing panencephalitis. The majority of patients (66 per cent) were between 8 and 12 years of age. The mean age of onset was 9.3 years, the youngest patient being four years nine months and the oldest 14 years. The male to female ratio was 1.25:1. A previous history of primary measles infection was obtained in 44.4 per cent of cases; 62.5 per cent occurred before the second birthday. The commonest mode of presentation was personality, intellectual and behaviour disorders (83 per cent) followed by myoclonic seizures (61 per cent) and choreiform movements (28 per cent). Measles antibody was present in the CSF in all cases. The EEG was abnormal in all recorded cases with pathognomonic periodic complexes being found in 56.2 per cent. Confirmation of the diagnosis was provided by brain biopsy in two cases and by necropsy in one case. The findings of this study suggest that subacute sclerosing panencephalitis may not be as uncommon in Black children as has hitherto been thought.
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PMID:Subacute sclerosing panencephalitis in black children--a review of 18 cases. 221 87

In 1988 a therapeutic drug monitoring programme for anticonvulsants was introduced in the paediatric neurology clinic at King Edward VIII Hospital, Durban. Although before this serum drug levels were routinely measured, the new approach involved the application of clinical pharmacokinetics to the results so that an individualised drug profile was available for use in conjunction with clinical evaluation at the patient's next visit. Case reports are presented to illustrate the value of using serum levels in this way. Of the 58 patients entered into the study in the course of 1 year, 32 (55%) benefited from the monitoring programme in that the pharmacokinetic report contributed to a dosage adjustment that resulted in a reduction in either seizure frequency or side-effects. The greatest value of the programme proved to be in rationalizing the use of phenytoin. Because of wide inter-patient variation and non-linear kinetics, it is difficult to establish an optimum maintenance dose for this drug. The individualised dose achieved in the present study using Bayesian forecasting resulted in improved seizure control in 10 of the 11 patients (91%) receiving phenytoin.
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PMID:Therapeutic drug monitoring in a paediatric epilepsy clinic. 234 49

Cephalic neural crest cells contribute to the formation of the external and middle ears, the supporting cells of the statoacoustic ganglion, other cranial nerve components, and the face. The anlage of otic sensory structures receive inductive stimuli from adjacent rhombencephalic tissue. The complex series of interactions that guide organogenesis of the outer, middle, and inner ear structures may explain why neurologic dysfunction is likely to be associated with malformations of the ear. We reviewed the records of 100 patients with complex ear anomalies with or without hearing loss. Mean age was 4.2 years (range 1 day-27 years). Malformations, either bilateral (70) or unilateral (30), involved the external ear (94), middle ear (16), and/or inner ear (12). Eighty-five patients had neurologic dysfunction. Cranial nerve dysfunction was found in 56 patients and involved nerves VIII (39 auditory and/or vestibular), VII (22), II (11), VI (8), V (4), III (3), X (3), XII (1), and IX (1). Sixty-four patients had evidence of central nervous system dysfunction such as mental deficiency/developmental delay (44), non-paretic gait disorders (17), hypotonia (16), microcephaly (13), seizures (8), motor deficits (8), autistic features (7), and radiographically confirmed intracranial abnormalities (5). Eleven of 19 children with hypoactive vestibules had delayed motor development or poor balance. Seventy-four patients had anomalies in other organ systems: 56 craniofacial, 28 osseous, 19 cardiac, 16 genito-urinary, 14 ocular, 11 gastrointestinal, and 7 cutaneous. Sixty-one patients had syndromic conditions, 32 of them branchial arch syndromes. The level of cognitive competence was not related to severity of craniofacial, ear, or cranial nerve abnormality. Children with ear malformations deserve neurologic and pediatric evaluations in addition to an otologic work-up.
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PMID:Neurologic findings in children with ear malformations. 362 8

The incidence of initial audiogenic seizures in mice from crosses between a susceptible and a resistant inbred strain is attributable to the effects of a single pair of alleles. The locus responsible for this behavioral variation is located in Linkage Group VIII of the mouse. Sensitization-dependent convulsions are influenced in whole or in part by alleles at an independent locus.
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PMID:Audiogenic seizure prone (asp): a gene affecting behavior in linkage group 8 of the mouse. 569 55

The case of a professional musician with a right temporal lobe epilepsy is presented. Whilst playing an organ concert (John Stanley's Voluntary VIII, Op. 5), he suffered a complex partial seizure. The recorded concert performance (with the seizure) was analysed and compared with other available exercise records and with the composition. The musical analysis of the seizure-induced variations reveals that at the beginning of the seizure, the left hand started to become unprecise in time and deviated from the score, whereas the right hand remained faultless at this time. With increasing duration of the seizure discharge, the dissociation of both hands from the score increased but the right hand compensated for the errors of the left hand in a musically meaningful way, i.e. with the aim to compensate for the seizure-induced errors of the left hand. The case illustrates untroubled musical judgement during epileptic activity in the right temporal lobe at the beginning of the seizure. Whereas the temporal formation of the performance was markedly impaired, the ability of improvisation-in the sense of a 'perfect musical solution' to errors of the left hand-remained intact.
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PMID:Untroubled musical judgement of a performing organist during early epileptic seizure of the right temporal lobe. 898 76

A 16 year old boy with epilepsy and learning difficulties is reported. At 3 years of age he was diagnosed with common acute lymphoblastic leukaemia, and received therapy according to the UK protocol, UKALL VIII. This included prophylactic CNS radiotherapy and chemotherapy. He did not develop CNS leukaemia, and complete remission was achieved. At age 7, he began to experience lethargy and learning difficulties, especially problems with hand-writing, concentration and memory. Furthermore, he began experiencing atypical absence seizures, which were provoked by concentration at times of tiredness. EEG showed bilateral non-specific abnormalities, with some epileptiform features. Over the following 9 years, several anti-epileptic drugs were prescribed. Although with the changes in therapy initial remissions have been achieved, the seizures have, each time, continued to relapse. At age 12, EEG was very abnormal, showing frequent generalized slow or sharp waves. At age 13, MRI revealed multiple discrete small high-intensity lesions in the subcortical white matter of both hemispheres. Problems with lethargy, concentration and memory persist and although multiple anti-epileptic drugs have been prescribed, seizures continue to occur almost daily.
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PMID:Leukoencephalopathy after CNS prophylaxis for acute lymphoblastic leukaemia. 966 10

The changes in the permeability of the blood-brain barrier (BBB) during bicuculline-induced seizures were investigated in ovariectomized female and orchidectomized male rats. The rats were anesthetized with diethyl ether. Evans blue, which was used as a BBB tracer, was injected into femoral vein 5 min before administering bicuculline to induce grandmal seizures. Ten groups of rats were studied: Group I: control female; Group II: control male; Group IIl: intact female + bicuculline; Group IV: intact male + bicuculline; Group V: ovariectomized female; Group VI: orchidectomized male; Group VII: ovariectomized female + bicuculline; Group VIII: orchidectomized male + bicuculline (1.2 mg/kg, i.v.); Group IX: ovariectomized female + estrogen + bicuculline; Group X: orchidectomized male + estrogen + bicuculline. Adult male and female rats were orchidectomized and ovariectomized 3 weeks before the experiments, or sham operated under general anesthesia. During bicucculline-induced seizures, the mean arterial blood pressure increased significantly in both intact and ovariectomized and orchidectomized rats. BBB lesions were present in 80 percent of intact female rats and 50 percent of ovariectomized rats after bicuculline-induced seizures. This difference between intact and ovariectomized rats was found to be significant (p < 0.01). There was no statistically significant change in the BBB permeability between intact and orchidectomized rats after convulsion. Generating seizures in both ovariectomized and orchidectomised rats, after administrating of estrogen, did not lead to any significant alteration in BBB permeability. Our results suggest that the extravasation of Evans blue albumin was most pronounced in the brain of intact female rats when compared to ovariectomized rats after bicuculline-induced seizures. After administrating estrogen, the decreased BBB permeability values of ovariectomised rats could not reach the values in intact rats.
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PMID:Influence of orchidectomy and ovariectomy on the blood-brain barrier permeability during bicuculline-induced seizures. 976 79

An increased incidence of cerebral thromboembolic events has been reported in young patients with inflammatory bowel disease (IBD). It has been suggested that a hypercoagulable state is associated with clinical activity of the disease, with elevation of factors V, VIII, fibrinogen and platelets and a lowering of anti-thrombin III. We present the case of a 35 y/o male with refractory Crohn's disease who complained of headaches, blurred vision and tonic-clonic seizures. The studies demonstrated an ischemic stroke of the left cerebral hemisphere, without vascular abnormalities. Elevation of factor VIII, platelets, and antithrombin III were found. The symptoms were relieved with medical treatment and the patient has continued in good health after resection of the diseased terminal ileum.
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PMID:Cerebral thrombosis associated with active Crohn's disease. 988 77

A series of 1,2-aminoalkanol derivatives were prepared and evaluated for anticonvulsant activity in the maximal electroshock seizure (MES) and subcutaneous pentylenetetrazole seizure threshold (scMet) assays and for neurotoxicity (TOX). Most interesting were the anticonvulsant results of S-(+)-2-amino-1-butanol derivative VIII, which displayed anti-MES activity with a protective index (TD50/ED50) of 4.55 corresponding with that for phenytoin, carbamazepine and valproate.
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PMID:Synthesis and anticonvulsant activity of 1,2-aminoalkanol derivatives. 1007 36

Astrocytic tumors, particularly gliosarcoma, may contain epithelial features in the form of trabecular, adenoid, papillary arrangement, and squamous metaplasia. A case of gliosarcoma with unusual epithelial feature is described. The patient was a 60-year-old male with frequent seizures. The mass was 4 cm and in the left frontal lobe. Trabecular or rarely adenoid arrangement of neoplastic astrocytes was present in the mucinous stroma, and there was a distinctive transition between the trabecular area and typical anaplastic astrocytoma. The tumor cells in the trabecular area showed positive immunostain for glial fibrillary acidic protein, but did not react with various kinds of cytokeratin. The sarcomatous area was undifferentiated and was not labeled by factor-VIII, desmin, and anti-smooth muscle actin. Occurrence and histogenesis of epithelial features in gliosarcoma are reviewed. The importance to recognize the existence of epithelial feature in malignant astrocytic tumor is emphasized.
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PMID:Gliosarcoma: a case with unusual epithelial feature. 1040 83


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