Are epilepsy classifications based on epileptic syndromes and seizure types outdated?
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Epilepsy↗
Biomedical subjects
J Acharya
Publications and source records attributed to J Acharya.
The role of apolipoprotein E in cognitive decline after cardiac operation.
BACKGROUND: Recently, Tardiff and colleagues have suggested that the presence of the apolipoprotein E, epsilon4 allele was associated with increased likelihood of cognitive decline after coronary artery bypass grafting. The objective of the current study was to replicate this earlier work using an increased sample size. The increased sample also enabled an analysis by individual genotype in cognitive decline after coronary artery bypass grafting. METHODS: Apolipoprotein E genotyping was performed on 111 individuals undergoing coronary artery bypass grafting. Each participant underwent a battery of nine neuropsychological tests before operation and 4 to 7 weeks after operation. RESULTS: Cognitive decline, assessed by both continuous Z change scores and two categoric measures of cognitive deficit, was not significantly associated with either individual apolipoprotein E genotypes or categorization by the presence or absence of the epsilon4 allele. The examination of potential moderating factors did not alter this finding. CONCLUSIONS: This study suggests that the epsilon4 allele is not associated with cognitive decline in the weeks after coronary artery bypass grafting.
Apolipoproteins E↗
Antidotal efficacy of pyridinium oximes and cholineacetyltransferase inhibitors against organophosphorus intoxication in rodents.
In an attempt to develop effective antidote against organophosphorus intoxication, some new imidazole-pyridinium mono-oximes, long chain pyridinium mono-oximes and cholineacetyltransferase inhibitors were synthesised. These compounds were evaluated for their in vivo therapeutic protection and neuromuscular function studies in rodents. The results indicate that SPK-series oximes may be useful against sarin poisoning without any beneficial effect against VX (O-Ethyl S-2-NN-diisopropylaminoethyl methylphosphonofluoridate) intoxication. The cholineacetyltransferase (ChAT) inhibitors may not be of any help against any of the OP compounds studied in this study.
Animals↗
Symptomatology of epileptic seizures in the first three years of life.
PURPOSE: Few data are available concerning symptomatology of epileptic seizures in infants. METHODS: We reviewed 296 videotaped seizures from 76 patients aged 1-35 months (mean, 15.1 months) who underwent video-EEG monitoring at our institution from 1988 to 1998. Seizure symptomatology was first classified based on observable behavioral and motor manifestations and then correlated with ictal EEG. RESULTS: Four seizure types accounted for 81% of all seizures seen in this group: epileptic spasms (24%), clonic seizures (20%), tonic seizures (17%), and hypomotor seizures (20%; characterized by arrest or significant decrease of behavioral motor activity with indeterminate level of consciousness). The remaining seizures included small numbers of myoclonic, atonic, and versive seizures. All 12 focal motor seizures and all five versive seizures were associated with focal EEG seizure patterns, seen in the contralateral hemisphere in all but one patient with versive seizures. Generalized motor seizures (clinically generalized at onset) were accompanied either by focal (19 of 51; 37%) or generalized (32 of 51; 63%) EEG seizures. Hypomotor seizures also were associated with focal (14 of 20; 70%) or generalized (six of 20; 30%) EEG seizures. Four patients with generalized epileptic spasms had generalized EEG seizures in the setting of focal epilepsy based on neuroimaging, interictal EEG, and in two cases also on postresection seizure freedom. Seizure types not seen in this age group included auras, seizures with prominent automatisms (except in one case), and classic generalized tonic-clonic seizures. CONCLUSIONS: The repertoire of seizure manifestation in the first 3 years of life appears to be limited. In infants, focal motor seizures are reliably associated with focal EEG seizures in the contralateral hemisphere, whereas generalized motor and hypomotor clinical seizures may be either focal or generalized on EEG. Epileptic spasms may be seen in focal as well as generalized epilepsies. Video-EEG monitoring and neuroimaging may be critical for clarifying the focal or generalized nature of the epilepsy in infants.
Age Factors↗
A new epileptic seizure classification based exclusively on ictal semiology.
Historically, seizure semiology was the main feature in the differential diagnosis of epileptic syndromes. With the development of clinical EEG, the definition of electroclinical complexes became an essential tool to define epileptic syndromes, particularly focal epileptic syndromes. Modern advances in diagnostic technology, particularly in neuroimaging and molecular biology, now permit better definitions of epileptic syndromes. At the same time detailed studies showed that there does not necessarily exist a one-to-one relationship between epileptic seizures or electroclinical complexes and epileptic syndromes. These developments call for the reintroduction of an epileptic seizure classification based exclusively on clinical semiology, similar to the seizure classifications which were used by neurologists before the introduction of the modern diagnostic methods. This classification of epileptic seizures should always be complemented by an epileptic syndrome classification based on all the available clinical information (clinical history, neurological exam, ictal semiology, EEG, anatomical and functional neuroimaging, etc.). Such an approach is more consistent with mainstream clinical neurology and would avoid the current confusion between the classification of epileptic seizures (which in the International Seizure Classification is actually a classification of electroclinical complexes) and the classification of epileptic syndromes.
Electroencephalography↗
Erythrocytosis due to a mutation in the erythropoietin receptor gene.
Familial erythrocytosis, associated with high haemoglobin levels and low serum erythropoietin (Epo), has been shown to co-segregate with a sequence repeat polymorphism at the 5' region of the erythropoietin receptor (EpoR) in a large Finnish family. We have investigated the cause of erythrocytosis in an English boy. Sequencing of the cytoplasmic region of the EpoR detected a de novo transition mutation of G to A at nucleotide 6002. This mutation resulted in the formation of a stop codon at amino acid 439 with the loss of 70 amino acids from the carboxy terminus. The mutation (G6002A) has arisen independently in a Finnish family and de novo in this English boy. Patients with unexplained erythrocytosis and low serum Epo levels should be investigated for EpoR mutations.
Adolescent↗
Semiological seizure classification.
We propose an epileptic seizure classification based exclusively on ictal semiology. In this semiological seizure classification (SSC), seizures are classified as follows: a. Auras are ictal manifestations having sensory, psychosensory, and experiential symptoms. b. Autonomic seizures are seizures in which the main ictal manifestations are objectively documented autonomic alterations. c. "Dialeptic" seizures have as their main ictal manifestations an alteration of consciousness that is independent of ictal EEG manifestations. The new term "dialeptic" seizure has been coined to differentiate this concept from absence seizures (dialeptic seizures with a generalized ictal EEG) and complex partial seizures (dialeptic seizures with a focal ictal EEG). d. Motor seizures are characterized mainly by motor symptoms and are subclassified as simple or complex. Simple motor seizures are characterized by simple, unnatural movements that can be elicited by electrical stimulation of the primary and supplementary motor area (myoclonic, tonic, clonic and tonic-clonic, versive). Complex motor seizures are characterized by complex motor movements that resemble natural movements but that occur in an inappropriate setting ("automatisms"). e. Special seizures include seizures characterized by "negative" features (atonic, astatic, hypomotor, akinetic, and aphasic seizures). The SSC identifies in detail the somatotopic distribution of the ictal semiology as well as the seizure evolution. The advantages of a pure SSC, as opposed to the current classification of the International League Against Epilepsy (ILAE), which is actually a classification of electroclinical syndromes, are discussed.
Electroencephalography↗
Implications for the use of X-chromosome inactivation patterns and their relevance to the myeloproliferative disorders.
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Adult↗
Olfactory epileptic auras.
BACKGROUND: Olfactory epileptic auras are rare, constituting about 0.9% of all auras, and are typically described as unpleasant. They have usually been associated with tumors, but in some recent studies they have not. METHODS: We identified 13 patients (7 male, 6 female) with olfactory epileptic auras from 1423 patients with partial epilepsy evaluated for intractable seizures between 1991 and 1996. All had routine EEGs and MRI. Twelve underwent prolonged video-EEG monitoring. RESULTS: Olfactory sensations were of various types but unpleasant in only seven. Auras evolved to complex partial seizures (automotor, hypermotor, or dialeptic seizures) in 12 patients, with further evolution to generalized tonic-clonic seizures in three and aphasic seizures in one patient. The EEG focus was localized to the mesial temporal region in all. Ten patients had a mesial temporal tumor; in one patient, it extended to the superior temporal gyrus, and in another, the frontal lobe. The tumor involved only the amygdala in two patients and both amygdala and hippocampus in six; none had hippocampal involvement alone. Surgery was performed in nine patients. All except one with partial tumor resection had a seizure-free outcome. This patient also became seizure-free after repeat surgery to remove residual tumor tissue in the amygdala. CONCLUSIONS: Olfactory auras are not necessarily unpleasant. The amygdala is the most likely symptomatogenic zone of olfactory auras. Tumors are the commonest etiology; mesial temporal sclerosis is a relatively rare cause.
Adolescent↗
Identification of latent myeloproliferative disease in patients with Budd-Chiari syndrome using X-chromosome inactivation patterns and in vitro erythroid colony formation.
Some patients with an early or latent myeloproliferative disorder (MPD) present with Budd-Chiari syndrome (BCS, hepatic vein thrombosis). Cell culture analysis of erythroid progenitors (BFU-E) can be used to discriminate primary from secondary MPD and examination of X-chromosome inactivation (in females) can be used to demonstrate clonality in neoplastic tissues. The present study used these techniques to examine whether a group of 7 female patients who presented with BCS had evidence to support a diagnosis of MPD. Unilateral X-inactivation and therefore clonality can be studied in females heterozygous for X-linked restriction fragment length polymorphisms (RFLP) by differences in methylation between active and inactive chromosomes. Probes for two polymorphic loci, phosphoglycerate kinase (PGK, at Xq13.3 [BstX1 RFLP]) and M27 beta (an anonymous locus DXS255 at Xp11.22 [Pst1 RFLP]) were used to study methylation patterns. All 7 patients were heterozygous using M27 beta and 2/7 were also heterozygous using the PGK probe. Polyclonal patterns of X-inactivation in granulocytes were demonstrated in 3/7, a skewed/monoclonal pattern in 1/7 and aberrant patterns in 3/7 using M27 beta. Two patients who had aberrant patterns of X inactivation with M27 beta demonstrated a skewed/monoclonal pattern with PGK. The results of BFU-E growth patterns and clonality were entirely concordant in 5/6 patients. Thus X-chromosome inactivation patterns, in conjunction with erythroid colony studies, can be used to assist in the diagnosis of an underlying MPD in BCS.
Biomarkers↗
Zinc improves the filterability of sickle erythrocytes at intermediate oxygen partial pressures.
1. The deformability of erythrocytes from patients with sickle-cell anaemia was measured with a St George's blood filtrometer at a range of oxygen partial pressures and at four levels of zinc loading. 2. When incubated in buffered saline containing zinc and the chelator ethyl maltol, erythrocytes rapidly accumulated zinc and thus their oxygen affinity was increased. 3. Neither the oxygen partial pressure nor zinc loading affected the filtration of normal erythrocytes. 4. Deoxygenation of sickle erythrocytes greatly impaired filtration, although the initial filtration rate declined sharply at different oxygen partial pressures (between 70 and 35 mmHg) in different patients. 5. Low levels of zinc (0.03 +/- 0.003 mol of zinc/mol of haemoglobin tetramer) were without effect on sickle cells, but at zinc/haemoglobin ratios of 0.6:1 and above, the sharp fall in filtration rate occurred at oxygen partial pressures 8-25 mmHg below the oxygen partial pressure that impaired filtration of untreated cells. 6. Hence, the deformability of sickle erythrocytes in vitro can be improved by increasing the intracellular content of zinc to 20-fold above normal. Further studies are now required to examine the stability of zinc in erythrocytes, the effects of high intracellular zinc concentrations on erythrocyte viability, and the toxicity of zinc released from zinc-laden cells.
Cells, Cultured↗
Red cell lipid peroxidation and antioxidant enzymes in iron deficiency.
Whether iron deficient RBC in humans have a reduced, or an increased, susceptibility to lipid peroxidation was studied in the iron deficiency states of primary proliferative polycythaemia and iron deficiency anaemia and related to changes in the activities of iron-dependent and non-iron dependent antioxidant enzymes. Susceptibility of RBCs to lipid peroxidation was increased when expressed per g Hb. However, this was a result of the low RBC Hb giving an increased membrane lipid: Hb ratio in the incubations. Results were normal when expressed either per cell, or per ml, RBC. Glutathione reductase was normal. Increased RBC superoxide dismutase activity in iron deficiency may be explained by the younger RBC population and reductions in glutathione peroxidase and catalase activities by the microcytic hypochromic changes and the lack of availability of iron, respectively. There is no evidence of an increased susceptibility of RBC to lipid peroxidation in iron deficiency.
Aged↗
Pulse oximetry in sickle cell disease.
The place of pulse oximetry in monitoring arterial oxygen saturation in sickle cell disease has been evaluated. In four admissions of patients with sickle cell anaemia with varying degrees of arterial haemoglobin oxygen desaturation, pulse oximetry was compared with a simultaneous assessment of oxygen saturation by arterial blood gas measurement and oxygen dissociation curve (ODC) analysis. Close agreement was found between the oxygen saturation measured by pulse oximetry and that calculated from the measured arterial partial pressure of oxygen (PaO2) with reference to the patient's own ODC. Calculation of oxygen saturation by the blood gas analyser assuming a normal ODC was erroneous. Pulse oximetry is an accurate and effective non-invasive method for monitoring the arterial oxygen saturation in sickle cell disease.
Anemia, Sickle Cell↗
An improved acidified glycerol lysis test (AGLT) used to detect spherocytes in pregnancy.
The acidified glycerol lysis test (Zanella et al, 1980) had proved unreliable in our hands. We now report the test to be stable and reliable when conducted at 37 degrees C rather than at ambient laboratory temperature as used by earlier workers. With this modification, blood samples were tested from 167 pregnant subjects. Forty-eight were less than 26 weeks pregnant and gave normal AGLT values (T50 greater than 1800 s) while of 119 more than 26 weeks pregnant 57 (48%) gave shortened T50 values indicative of the presence of spherocytes.
Female↗
Red-cell metabolism in patients with iron deficiency.
18 components of metabolism were determined in the red cells of iron-deficient patients and data were expressed per 10(12) red cells to avoid the complicating effects of hypochromia and microcytosis. Glucose consumption, AMP and ATP, glycolytic intermediates except 2,3-bisphosphoglycerate (2,3-DPG) and phosphoenolpyruvate (PEP), red-cell Na+ and the net passive leakage of Na+ and K+ at 4 degrees C were all normal. Creatine, 6-phospho-D-gluconate: NADP oxidoreductase (6PGD) activity and fresh red-cell K+ were raised, suggestive of a young cell population. However, ATP: D-fructose-6-phosphate 1-phosphotransferase (PFK) activity and ADP were low. An elevated 2,3-DPG level was attributable to the anaemia present but the somewhat raised PEP level is unexplained. It is concluded that red cells in iron deficiency show some characteristics of a young cell population; in other respects they appear normal, but in containing a low PFK activity they are abnormal.
2,3-Diphosphoglycerate↗