Protective and susceptibility effects of hSKCa3 allelic variants on juvenile myoclonic epilepsy.
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Adolescent↗
Biomedical subjects
R Sridharan
Publications and source records attributed to R Sridharan.
Prevalence and pattern of epilepsy in India.
PURPOSE: To estimate the prevalence of epilepsy in India by meta-analysis of previously published and unpublished studies and to determine patterns of epilepsy by using community-based studies. METHODS: We attempted to identify as many previously published and unpublished studies as possible on the prevalence of epilepsy in India. The studies were assessed with regard to methods and definitions. The prevalence rates for rural and urban populations and for men and women were calculated with a 95% confidence interval (CI). The studies that provided details on age structure, age-specific rates, and patterns of epilepsy were chosen for meta-analysis. Both crude values and age-standardized prevalence rates were calculated after accounting for heterogeneity. RESULTS: Twenty studies were found involving a sample population of 598,910, among whom 3,207 had epilepsy. This resulted in a crude prevalence of 5.35/1,000. After a correction for heterogeneity due to interstudy variation, the overall prevalence per 1,000 (and its 95% CI) was 5.33 (4.25-6.41); with urban areas at 5.11 (3.49-6.73); rural areas, 5.47 (4.04-6.9); men, 5.88 (3.89-7.87); and women 5.51 (3.49-7.53). After correction for the variability in estimates of heterogeneity, age-standardized rates (from five studies) revealed that the prevalence rates per 1,000 (and the 95% CI), were as follows: overall, 5.59 (4.15-7.03); men, 6.05 (3.79-8.31); women, 5.18 (3.04-7.32); urban, 6.34 (3.43-9.25); rural, 4.94 (3.12-6.76). Urban men and women had a higher prevalence of epilepsy compared with rural ones, however the difference was not statistically significant. Age-specific prevalence rates were higher in the younger age group, with the onset of epilepsy reported mostly in the first three decades of the sample population's lives. The treatment gap (i.e., the percentage of those with epilepsy who were receiving no or inadequate treatment) was more than 70% in the rural areas. CONCLUSIONS: Based on the total projected population of India in the year 2001, the estimated number of people with epilepsy would be 5.5 million. Based on a single study on the incidence of epilepsy, the number of new cases of epilepsy each year would be close to half a million. Because rural population constitutes 74% of the Indian population, the number of people with epilepsy in rural areas will be approximately 4.1 million, three fourths of whom will not be getting any specific treatment as per the present standard.
Adolescent↗
Conformational analysis of A and B rings in 2-, 4-, and 6-bromosubstituted steroidal 4-en-3-ones by nuclear magnetic resonance.
The conformational preference of A and B rings in four differently functionalized bromosubstituted 4-en-3-one steroids is studied by concerted application of high-resolution one- and two-dimensional nuclear magnetic resonance (NMR) techniques, such as homonuclear and heteronuclear correlated spectroscopy, transient and steady-state nOe spectroscopy, temperature-dependent chemical chemical shift variation, and application of a modified Karplus equation. The steroids studied include 6 beta-bromocholest-4-en-3-one (3), 4,6 beta-dibromocholest-1,4-dien-3-one (2), 2 alpha,4,6 beta-tribromocholest-4-en-3-one (1), and (25R)-2 alpha,6 beta-dibromospirost-4-en-3-one (4). Steroids 1-4 were prepared by either acid-catalyzed or free-radical bromination from appropriate 4-en-3-one steroid. The study has yielded an insight into the factors responsible for conformational preferences of the A and B rings of these bromosubstituted steroids. Bromosubstitution at the 2 alpha position is responsible for the inversion of the A ring to inverted 1 beta,2 alpha-halfchair conformation. The electronic interaction between 4-bromine and carbonyl oxygen distorts the A-ring conformation further. Inversion of the A ring has a concomitant effect of distortion in the chair form of the B ring. Conformational preferences of A and B rings are not found to be influenced by transmission effect of a side chain or oxygenated ring system. Temperature-dependent NMR studies indicate the reduced conformational flexibility of the A ring for 2 alpha-bromosubstituted steroids. Complete assignment of the 13C and 1H resonances of two of the steroids studied (3 and 4) is presented.
Bromides↗
Risk factors for ischemic stroke: a case control analysis.
Case control analysis of 204 patients with acute ischemic stroke revealed the matched pair odds ratio (and 95% confidence limits) for hypertension, ECG abnormality, heart disease of any type, diabetes, smoking and alcohol intake to be 3.95 (2.5, 6.2), 2.1 (1.4, 3.1), 2.1 (1.4, 3.2), 1.7 (1.1, 2.6), 1.8 (1.1, 2.8) and 1.5 (0.86, 2.6), respectively. Except alcohol intake, the other factors were statistically significant. Hemoglobin, packed cell volume (hematocrit), serum cholesterol, triglycerides and low-density lipoprotein cholesterol levels were not found to be significant. High-density lipoprotein (HDL) cholesterol and uric acid were significantly lower and the ratio of total cholesterol to HDL cholesterol (TC/HDL) was higher among stroke patients. The risk was considerably higher when there was any combination of hypertension, heart disease and HDL cholesterol level lower than 45 mg/dl. Logistic regression revealed hypertension, heart disease of any type, lower HDL cholesterol and uric acid and higher ratio of TC/HDL to be significant factors.
Adult↗
Clinical and epidemiological study of Bell's palsy in Benghazi, Libya.
An intensive search for 2 years for patients with Bell's palsy, conducted through the polyclinics, university hospitals and physiotherapy centres in Benghazi, revealed a total of 242 cases. The average annual incidence per 100,000 population was 23.35 and the age-adjusted incidence 35.72. The incidence increased with age up to the sixth decade. Seasonal clustering was noted in the months of December and January. Hypertension and diabetes mellitus were associated in 4.1% and 7% of patients, respectively. Only four patients presented during pregnancy. Recurrent facial paralysis was encountered in 5.4% of patients and was characterized by male preponderance and a tendency to recur more frequently on the same side as the initial paralysis. Familial incidence and bilateral involvement were rare features. Twelve per cent of patients who were followed up had moderate to severe residual weakness.
Adolescent↗
Epidemiology of Parkinson's disease in Benghazi, North-East Libya.
An epidemiological study of Parkinsonism was carried out in Benghazi situated in North-Eastern Libya. On the prevalence day, January 1, 1985, a total of 163 patients with Parkinson's disease were found living in the investigated area, which had a population of 518,745. The crude prevalence rate was 31.4 per 100,000 population and the incidence rate was 4.5 per 100,000 population per year. The average age at death was 71.2 years and the mean duration of the illness was 6.7 years. Our findings suggest that the risk of Parkinson's disease observed in the present study is intermediate to that reported among the white and black races and comparable with the yellow race and the Sardinians.
Age Factors↗
Stroke in the young: incidence and pattern in Benghazi, Libya.
Out of 329 cases of stroke registered in Benghazi between November 1, 1983 and October 30, 1984, 63 patients comprising 32 males and 31 females belonged to the 15-40-year age group (stroke in the young). The annual age-specific crude incidence of stroke in the young was 39.3 and 40.3 per 100,000 for men and women, respectively. The age-adjusted incidence rate for both sexes for the age-specific (15-40 years) population was 47 per 100,000. The sex-dependent difference in the incidence was not statistically significant. These results emphasize that the high incidence of stroke in the young in Benghazi is not a reflection of the age distribution of the population. Hypertension, diabetes mellitus, heart disease and hypercholesterolemia were important components of high stroke profile in the young. One or more risk factors were identified in 78% of total young stroke victims and in all the patients under the age of 30. Eight young stroke subjects died during the study period.
Adolescent↗
Incidence and pattern of cerebrovascular diseases in Benghazi, Libya.
During a 12 month study period, a total of 329 incident cases of stroke occurred in Benghazi, Libya. This provided an unadjusted annual incidence rate for stroke of 63 per 100,000 population; the corresponding rates for the male and female were 69 and 58 per 100,000 respectively. The sex dependent difference in the incidence was not statistically significant. Cerebral infarction was the commonest type of stroke accounting for 80.9%. The incidence rates increased with age in all categories of stroke. Hypertension and ischaemic heart disease were common risk factors among the male patients, while diabetes and hypercholesterolaemia were more frequent in the female patients.
Adult↗
Descriptive epidemiology of motor neuron disease in Benghazi, Libya.
A total of 23 patients with motor neuron disease (MND), encompassing 17 cases of amyotrophic lateral sclerosis, 4 of progressive muscular atrophy and 2 of progressive bulbar palsy, was diagnosed in Benghazi, north-eastern Libya, between 1980 and 1985. The male to female ratio was 2.3:1. The average incidence of MND was 0.89/100,000 population/year (0.87 when age and sex-adjusted to the Libyan population). Eighteen patients were alive on the prevalence day, September 15, 1985, which provided a prevalence rate of 3.47/100,000 population (3.42 if adjusted). Age-specific incidence rates were highest in the 50- to 59-year-old age group, 8.14/100,000/year for men and 6.10/100,000/year for women. The median age at the time of diagnosis was 51 years, and the median duration for the 5 dead MND patients after the onset of the disease was 30 months. The median survival time for all MND cases combined was 42 months.
Adult↗
Pseudotumour cerebri: incidence and pattern in North-Eastern Libya.
The present documentation of 23 female patients with pseudotumour cerebri seen in Benghazi, North-Eastern Libya, over a period of 2 years constituted 21 cases with idiopathic pseudotumour and 2 patients with associated endocrine diseases. The clinical, laboratory and CT scan findings indicated idiopathic hypoparathyroidism in 1 case. The association of pseudotumour and hypothyroidism in a 40-year-old woman is suggested to be more than coincidental. The patient group was significantly more obese. Moderate to severe visual loss occurred in 9 patients. The incidence and extent of visual impairment did not correlate with the duration of symptoms, degree of obesity, use of contraceptive pills, steroid treatment, the presence of systemic hypertension, menstrual irregularity or empty sella. The incidence of 1.7 per 100,000 for the total and 3.6 per 100,000 for the female population in Benghazi observed in the present study is believed to be more than the frequency (as assessed by the hospital incidence) reported from other regions. A high prevalence of obesity may partly explain the increased occurrence of pseudotumour cerebri in North-Eastern Libya.
Adult↗
Visual evoked potentials in ataxia telangiectasia.
Pattern reversal visual evoked potentials (VEPs) elicited in four patients with ataxia telangiectasia revealed normal results in two and absent responses in two. The pathogenesis of the VEP abnormalities is discussed. It is surmised that the VEP changes reflect progressive degeneration of the nerve fibres in the anterior visual pathway, as in Friedreich's ataxia.
Ataxia Telangiectasia↗
Prevalence and pattern of multiple sclerosis in Benghazi, north-eastern Libya.
A search for Libyan patients with multiple sclerosis (MS) was made in Benghazi, located on the southern Mediterranean coast at a latitude of 32 degrees N. Twenty-one clinically definite and probable cases were detected during the period July 1982-June 1984. On the basis of 2 probable incidental cases, the incidence for 1983 was 0.8 per 100 000 of the population at risk (10-50 years). On July 1st, 1984, the rough prevalence rate for the total population was 4 per 100 000 and the age-adjusted prevalence rate was 5.9 per 100 000. This study suggests that Benghazi falls within the medium frequency band for MS. High prevalence of brainstem involvement and cerebellar dysfunction and infrequent occurrence of the severe optic-spinal form and sphincter disturbance indicates that the present group of patients resembles Western pattern of MS as opposed to Asian MS.
Adolescent↗
Syphilitic spinal neuroarthropathy with paraplegia.
The case of a 46-year-old male with syphilitic spinal neuroarthropathy complicated by paraplegia is reported. Marked sclerosis and osteophytes restricted to lumbo-dorsal spine, absence of ligamentous calcification and lack of long standing spinal symptoms in spite of the florid radiologic changes should raise the suspicion of neurogenic spinal arthropathy.
Cauda Equina↗
Prevalence and pattern of spinocerebellar degenerations in northeastern Libya.
An intensive search over a two-year period for cases of cerebellar and spinocerebellar degenerations in Benghazi, Libya, made through polyclinics, university hospitals and a centre for the handicapped, revealed a total of 52 patients, among whom 30 were index cases; the remainder were detected on family study. Twenty-five patients lived in Benghazi, giving a crude prevalence of 4.8/100 000 population. There were 24 patients (10 families) with hereditary spastic paraplegia (HSP), 13 (9 families) with early onset cerebellar ataxia with retained tendon reflexes (EOCA), 3 with Friedreich's ataxia (FA), 5 (1 family) with late onset cerebellar ataxia (LOCA) with pigmentary retinal degeneration and autosomal dominant inheritance, 6 single cases of LOCA and 1 with ataxia telangiectasia. There were 14 families with definite autosomal recessive inheritance and only 2 with dominant transmission. The large family size (average of 6.2 children per married woman in the patient group) and the high rate of consanguineous marriages contribute to the high incidence of familial cases, especially those with autosomal recessive inheritance. Nerve conduction studies were normal in HSP and abnormal in EOCA and FA. Computed tomographic scans revealed atrophy of the brainstem and cerebellum in 3 cases of EOCA and 2 with LOCA. No indigenous forms of the disease were observed and the clinical features differed little from the descriptions in literature. However, the relative rarity of patients with FA, in comparison with other types of hereditary ataxias, is striking.
Adolescent↗
Periodic EEG pattern in meningovascular syphilis.
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Adult↗
Visual evoked potentials in spinocerebellar degenerations.
Pattern reversal visual evoked potentials were studied in 21 patients with spinocerebellar ataxias among whom 6 had Friedreich's ataxia, 10 had hereditary spastic ataxia and 5 had spinocerebellar degeneration with slow eye movements (olivopontocerebellar degeneration). The VEP abnormalities found in 4 cases of Friedreich's ataxia and one with spinocerebellar degeneration with slow eye movements, consisted of, bilaterally absent VEP in 3 patients and bilaterally abnormal responses with asymmetry in two. All the patients with spastic ataxia had normal VEP latencies. The N 70 - P 100 amplitudes, in patients with hereditary ataxias were significantly reduced compared to controls (P less than 0.001). The VEP abnormalities correlated best with neuroophthalmic findings, but had no relation to age, sex, inheritance or duration of illness. The VEP findings are probably suggestive of progressive nerve fibre loss in the visual pathways with associated slowing of conduction. The higher incidence of visual pathway involvement in Friedreich's ataxia compared to other hereditary ataxias as reported in recent studies is confirmed.
Adult↗
Opsoclonus-palatal myoclonus syndrome.
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Eye Movements↗
Adult aqueductal stenosis with Recklinghausen's neurofibromatosis.
A 28-year-old man with von Recklinghausen's neurofibromatosis was first seen with a spastic-ataxic gait and epileptic seizures of recent onset. computerized tomographic scan established the diagnosis of aqueductal stenosis, rarely reported in patients with von Recklinghausen's disease. The value of computerized tomography in the etiological diagnosis of hydrocephalus in von Recklinghausen's disease is emphasized and the pathogenesis of aqueductal narrowing in neurofibromatosis is discussed.
Adult↗