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

Clinical and necropsy findings in 11 patients with sickle cell anemia (SS) indicate that intracranial hemorrhage (IH) is a delayed sequela of the same vasculopathy that causes cerebral infarction during childhood. Evidence of prior cerebral infarction during childhood included hemiparesis, seizures, an episode of coma, or mental retardation. Computerized tomography (CT) scans showed cerebral infarcts with lucent areas and dilated ventricles or cerebral atrophy. CT or magnetic resonance imaging (MRI) scans after the intracranial hemorrhage demonstrated intraventricular or intracerebral hemorrhages. Angiography or autopsy in seven patients showed widespread vascular occlusion and narrowing of arterial vessels. Moyamoya with internal carotid artery occlusion was identified in two cases. At the time of the IH, three patients were being treated with prophylactic transfusion regimens. We hypothesize that the central nervous system vasculopathy progresses over time and that arterial narrowing in both large and small vessels secondary to endothelial hyperplasia is followed by neovascularization and hemorrhage. Recognition of this pattern of delayed intracranial hemorrhage following cerebral infarction should encourage more intensive evaluation aimed at developing rational interventional therapy prior to a terminal intracranial hemorrhage.
 ...
PMID:Delayed intracranial hemorrhage following cerebral infarction in sickle cell anemia. 213 21

A retrospective study of 464 patients with delayed epilepsy was carried out. There were 311 males and 153 females with their ages ranging between 20 and 87 years. The seizures were Generalized in 298, and Partial in 139. Neurological examination showed abnormalities in 189, and normal finding in 275. The EEG studies and CT scans revealed abnormalities in 196 (80.6%) and 283 (61%) respectively. The CT scan findings were compatible with cerebrovascular diseases in 16.4%, with brain tumors in 12.9%, with craniocerebral trauma in 8.4%, and with diffuse brain atrophy in 7.4%. The CT scans would show a higher frequency of abnormalities if there were local neurological signs with the EEG findings suggesting, partial seizures and focal lesions of the brain. It was considered that a CT scan would be mandatory in the evaluation of patients with delayed epilepsy, even though the EEG and clinical examination were normal.
 ...
PMID:[Delayed epileptic seizures in adults]. 228 83

As pediatric cardiac surgical techniques have improved in recent years, mortality rates have dropped and attention has turned to residual morbidity, especially neurologic sequelae. Although the majority of children undergoing open-heart surgery for correction of congenital heart defects apparently emerge with no adverse consequences, a small percentage suffer permanent neurologic injury (seizures, motor disorders). Another small and not well-defined population may be left with disorders of higher cortical function, such as mental retardation or learning disabilities. A survey of six major pediatric cardiac surgery units in North America was undertaken in 1988-1989 to ascertain current approaches to the detection and management of neurologic sequelae of pediatric open-heart surgery. All units reported seeing a small but definite incidence of postoperative neurologic symptoms, including alterations of consciousness, seizures, and localized abnormalities such as hemiparesis or delayed choreoathetoid syndromes. Postoperative neuroimaging procedures have shown a disturbing incidence of hypoxic-ischemic encephalopathy, unsuspected cerebral atrophy, and subdural hematomas. Pathogenesis may include factors related to preoperative brain anomalies and/or hypoxic insults, altered cerebral blood flow and metabolism during hypothermic cardiopulmonary bypass with or without total circulatory arrest, embolization, and low cardiac output states postoperatively. Further studies are needed to examine the mechanisms of injury and to develop techniques to minimize the occurrence of these sequelae, as they may be associated with life-long neurologic disability and reduced quality of life.
 ...
PMID:Neurologic sequelae of open-heart surgery in children. An 'irritating question'. 230 48

The pigmentary type of orthochromatic leukodystrophy (OLD) is a rare disorder in adults; only one questionable childhood case has been observed. We report the sporadic case of a male aged 26 years with early onset and protracted course. He presented retarded motor development from birth with ataxic gait and, at age 13 years, developed progressive mental and neurologic deterioration with tetraparesis, ataxia and seizures and died in a disabled, mute state. Repeated CT scans showed progressive diffuse cerebral atrophy and low density of the hemispheric white matter. Autopsy revealed OLD with pigmented macrophages and glial cells, ultrastructurally showing storage of lipofuscin and ceroid with multilamellar bodies or finger-print profiles. Abnormal cytoplasmic inclusions in reduced oligodendroglial cells suggest demyelination due to a primary defect of oligodendroglia in this rare disorder.
 ...
PMID:Pigmentary type of orthochromatic leukodystrophy with early onset and protracted course. 231 58

Computed tomography (CT) of the brain was performed on 44 women with eclampsia, 31 during pregnancy and 13 postpartum. All CT scans were done within 24 hours of eclampsia, including 12 within 1 hour of the convulsions and eight before the repetition of additional seizures, 2 minutes to 14 hours later. Control scans were performed on 15 hypertensive pregnant patients and on ten normotensive pregnant women after fetal death or just after delivery. Among the women with eclampsia, 26 CT scans were considered normal with no evidence of cerebral edema, three displayed signs of cerebral hemorrhage or thrombosis, six showed areas of focalized hypodensity located in the cortical lobes and the subcortical white matter, and nine were classified as cerebral atrophy with enlarged cerebral ventricles. We conclude that radiologic evidence of diffuse brain edema is probably not a common feature in eclampsia.
 ...
PMID:Computed tomography of the brain in eclampsia. 234 46

We reviewed retrospectively a series of 100 inpatients with onset of epileptic seizure after the age of 60. All of them were investigated by EEG and 96 by CT scan. The most frequent cause of seizure was previous stroke, with 25 cerebral infarcts and 5 hemorrhages. Neoplastic lesions were present in 18 cases, with glioma (high grade), meningioma and metastases in the same proportion. Other etiologies included toxico-metabolic (18 cases), post-traumatic (9 cases), cerebral atrophy (4 cases) and miscellaneous (14 cases). The causes of seizure remained unknown in 7 patients, of whom 6 had focal signs in either clinical examination or EEG. Focal seizures (with or without secondary generalization) accounted for 65% of all cases and generalized seizures for 35%. The EEG was normal in 12 patients and abnormal in 88, with diffuse slowing in 55 patients and focal signs in 70 (some patients had both diffuse slowing and focal signs). Fourteen patients presented status epilepticus. Ten died during hospitalization. We conclude that epileptic seizure with onset after age 60 is nearly always symptomatic, and neuroradiologic investigations are necessary in the search for cerebral lesions. In our study, the prevalence of "idiopathic epilepsy" is lower than previously described.
 ...
PMID:[Initial epileptic crisis after the age of 60: etiology, clinical aspects and EEG]. 234 61

A 10-month-old girl with mild developmental delay became hemiplegic after seizures. Cranial CT scan and magnetic resonance imaging (MRI) revealed no lesions related to vascular diseases, but brain atrophy on the right side was remarkable. Digital subtraction angiography showed slightly decreased visualization of peripheral branches of the right medial cerebral artery. Propionic acidaemia was diagnosed on the basis of high plasma levels of propionic acid and its metabolites and the elevated urinary excretion of these acids. With therapy, the levels of these acids fell, and her left hemiplegia disappeared 3 months later.
 ...
PMID:Acute infantile hemiplegia in a patient with propionic acidaemia. 237 22

From January 1983 to April 1988, 40 cases of epilepsy were treated surgically. There were 15 cases of posttraumatic cicatrix, 5 cases of grade I astrocytoma, 9 cases of diffuse gliosis, 4 cases of vascular malformation, 1 case of calcification and cicatrix of temporal lobe, 2 cases of microglia malformation, 1 case of brain atrophy complicating ganglioneuroma, 2 cases of localized brain atrophy, 1 case of post-hematoma cicatrix. The end results were seizures subsided in 26, and reduction of the seriousness in 8. No mortality in this series. Follow-up study showed 85% of these patients with effectiveness. The indication of surgery, utilizing of electrocorticography, excision of epileptogenic focus are discussed.
 ...
PMID:[Surgical treatment of epilepsy with electrocorticography monitoring]. 251 53

An epileptoid attack induced by orthostatic hypotension seen in a 72-year-old man was reported. The patient had been suffering from progressive autonomic failure with parkinsonism for six years and he had severe orthostatic hypotension, syncope and generalized convulsion when he stood up. The convulsion sometimes associated with urinary incontinence, ceased immediately when he lay down although he remained drowsy for a while. Occasionally the seizure ceased spontaneously and he regained consciousness even while he was kept standing. On lying position his blood pressure was 167/88 mmHg and no abnormality was seen in electroencephalogram. When he was tilted up to 50 degrees his blood pressure fell to 70/46 mmHg, and he became unconscious followed by jaw twitching and generalized clonic seizure. Electroencephalogram during seizure showed sharp wave and rhythmic spikes. Other laboratory examination revealed diffuse and severe autonomic dysfunction and slight cerebral atrophy on brain CT scanning. He was treated with diphenylhydantoin 300 mg/day and the seizure responded partially. The reasons why the patient's seizure was thought to be epileptic rather than the convulsive syncope were as follows: the type of the seizure was similar to an epileptic generalized convulsion, the seizure and unconsciousness ceased spontaneously even during standing position, the seizure and impaired consciousness partially responded to diphenylhydantoin administration. The underlying pathophysiology of the seizure was thought to be transient cerebral hypoperfusion induced by orthostatic hypotension.
 ...
PMID:[Orthostatically induced epileptoid attack]. 262 24

Functional methionine synthase deficiency is generally characterized by homocystinuria and hypomethioninemia in the absence of methylmalonic aciduria. Patients are divided into two classes, cblE and cblG, on the basis of complementation analysis. Presentation has usually been in the first 2 years of life, but one patient came to medical attention at age 21 years with symptoms initially diagnosed as multiple sclerosis. Common findings among 11 patients (4 with cblE and 7 with cblG) have included megaloblastic anemia (all patients) and various neurological deficits including developmental retardation (10 patients), cerebral atrophy (8 patients), hypotonia (7 patients), EEG abnormalities (6 patients), and nystagmus (5 patients). Hypertonia, seizures, blindness, and ataxia were less frequent. All patients have responded to therapy with cobalamin with resolution of anemia and biochemical abnormalities; neurological deficits resolved more slowly and in some cases incompletely. Hydroxycobalamin has been more effective than cyanocobalamin. Fibroblasts from patients with cblE (5 patients) and cblG (6 patients) all showed decreased intracellular levels of methylcobalamin (MeCbl) and decreased incorporation of label from 5-methyltetrahydrofolate into macromolecules, suggesting decreased activity of the MeCbl-dependent enzyme methionine synthase. Methionine synthase specific activity in extracts of all cblE fibroblasts was normal or near-normal under standard reducing conditions; synthase specific activity in extracts of 5 cblG patients was low but was high in a 6th patient measured in another laboratory. Thus, there is heterogeneity among patients with functional methionine synthase deficiency both in clinical presentation and in the results of biochemical studies of cultured cells.
 ...
PMID:Functional methionine synthase deficiency (cblE and cblG): clinical and biochemical heterogeneity. 268 21


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>