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Biomedical subjects

W A Hauser

Publications and source records attributed to W A Hauser.

At least 145 records · Page 8Linked to original sources

Risk factors for absence seizures: a population-based case-control study in Rochester, Minnesota.

To our knowledge, this is the first population-based case-control study of risk factors for absence seizures (AS). Diagnosis of AS was based on clinical criteria. The complete medical history of potential cases, available through the records-linkage system for residents of Rochester, MN, was independently reviewed by three neurologists who agreed upon the diagnosis. All AS patients who were residents of Rochester at time of diagnosis between 1935 and 1979, and who were born in this community, were included (N = 30). Two population controls (born in Rochester) were matched to each patient, and for both patients and controls, the records-linkage system was used to obtain information about possible risk factors. The only factor significantly more common in cases than in controls was a history of febrile seizures (odds ratio = 12; p less than 0.01). We suggest that these febrile seizures represent either an early manifestation of the convulsive diathesis or the symptom of a preexisting brain dysfunction. None of the other factors investigated reached statistical significance, including those that have been previously suggested such as twin pregnancy, breech presentation at delivery, being first-born, and perinatal asphyxia. Sample size limitations should be considered in interpreting these findings.

Adolescent↗

Risk factors for generalized tonic-clonic seizures: a population-based case-control study in Rochester, Minnesota.

This investigation is, to our knowledge, the first population-based case-control study of prenatal, perinatal, and postnatal risk factors for generalized tonic-clonic seizures (GTCS). The clinical diagnosis of GTCS was confirmed through the independent review of the complete medical history of potential cases by three neurologists. All subjects with onset of GTCS before age 30 who were residents of Rochester, Minnesota at time of diagnosis between 1935 and 1979, and who were born in this community, were included (N = 53). Two controls were matched to each patient, and for both patients and controls, the unique records-linkage system for residents of Rochester was used to obtain information about possible risk factors. A history of convulsions in the mother, febrile seizures, and head trauma were significantly more common in cases than in controls. However, factors previously suggested such as: advanced age of the mother, previous miscarriages, gestational toxemia or eclampsia, bleeding during the index pregnancy, low birth weight, asphyxia, or postmaturity were not confirmed. Based on the present and previous studies, we suggest that different types of seizures have different risk factors and should, therefore, be investigated separately.

Adolescent↗

Complex segregation analysis of febrile convulsions.

Complex segregation analysis was performed on 467 nuclear families ascertained through febrile-convulsion probands. The probands were identified as having their first febrile convulsion while residents of Rochester, MN, during the years 1935-64. Parents and first- and second-degree relatives of probands were identified through the Olmsted County, MN, record-linkage system. Diagnoses of convulsive activity were made from review of medical records. The genetic models investigated included both single-major-locus and polygenic models, with likelihoods computed jointly on children and parents as well as being conditioned on parental phenotype. Possible heterogeneity was investigated by means of analyses of frequency of febrile convulsions in the proband. Analyses of the entire data set indicated that the single-major-locus models could be rejected. The most parsimonious model for these data was the pure polygenic (or common familial environment) model with a large heritable component (68% +/- 7%). However, when families were partitioned on the basis of frequency of febrile convulsions in the proband, significant heterogeneity was present. Our results indicated that the polygenic model was strongly corroborated in families of probands with a single febrile convulsion. In families of probands with multiple febrile convulsions, evidence was consistent with a single-major-locus model with nearly dominant seizure susceptibility.

Child, Preschool↗

Epidemiology of central nervous system infections in Olmsted County, Minnesota, 1950-1981.

We identified all diagnosed cases of infections of the central nervous system (CNS), excluding poliomyelitis, in the population of Olmsted County, Minnesota, from 1950 to 1981 and described incidence, time trends, etiologic agents, and mortality for these infections. The adjusted incidence rate for bacterial meningitis was 8.6/100,000 person-years (with a case fatality ratio of 10%) and was highest in children less than five years of age; in this age-group, rates more than doubled from 1950 to 1981. The adjusted incidence rate of brain abscess was 1.1, with a case fatality ratio of 37%. The adjusted incidence rate of aseptic meningitis was 10.9/100,000 person-years. Age-specific rates were highest in children less than one year of age and in men, and increased during the study period. The adjusted incidence rate of viral encephalitis was 7.4, with a case fatality ratio of 3.8%. Rates were highest in children less than 10 years of age and in men. By 10 years of age, 0.9% of the men and 0.7% of the women were affected by a CNS infection. Cumulative incidence (risk) through age 80 was 2.3% for men and 1.5% for women.

Age Factors↗

Seizures and myoclonus in patients with Alzheimer's disease.

We reviewed 81 patients with dementia and autopsy findings of Alzheimer's disease (AD) to identify patients with seizures or myoclonus after onset of dementia. Eight (10%) had seizures, and eight others (10%) had myoclonus. The incidence of seizures was 10 times more than expected in a reference population. Seizures occurred in any stage of AD, but myoclonus was often a late manifestation. Both seizures and myoclonus, individually or together, are manifestations of AD and may be seen at any time in the course of the illness.

Aged↗

Cancer incidence in a cohort of patients with seizure disorders.

The incidence of cancer was evaluated in a population-based cohort of 959 patients diagnosed with seizure disorders while residents of Rochester, MN, between 1935 and 1979. For all cancer sites combined, there were 65 incidence cases for a standard morbidity ratio of 1.4. Most of the excess was attributable to a 22-fold increase in the incidence of primary brain tumors among these patients. Most of the brain tumors occurred within 5 years of the seizure disorder diagnosis, suggesting that the seizure disorder was due to the brain tumor. The incidence of cancer of other sites, exclusive of brain, was not elevated. There was no evidence of an association of cancer incidence with duration of seizures or with the use of anticonvulsant drugs.

Adolescent↗

Genetic heterogeneity in the epilepsies.

There is ample evidence for genetic and other heterogeneity in the mechanisms leading to epilepsy. Animal models of epilepsy show that genetic factors can influence the hypersensitivity of neurons. In the human, there are over 140 Mendelian traits (including disorders of amino acids, enzymes, hormones, and vasculature) that increase the risk of seizures. Furthermore, systems with an intermediate optimum (such as blood clotting and blood glucose) involve a number of mechanisms under independent genetic control, and it is reasonable to assume that the same principle applies to neuronal excitability. Finally, genetic variation can be expected in any of the factors that are altered in the origin of seizures: neuronal inhibition, inactivation of excitatory neurotransmitters, feedback control, and seizure generalization. One goal of future research is to define etiological subtypes on the basis of biochemical data or other factors. Meanwhile, it is possible to analyze currently available indicators of phenotypic variability (age at onset of seizures, family history of seizures, seizure type, EEG pattern, and history of antecedent factors such as fever or trauma) to address the following questions: Do any phenotypic groups have different sibling risks for seizures? How much phenotypic variability is seen among affected siblings of each defined group of probands (index cases)? Do any groups of probands show significant biochemical differences? Within a specific group, do isolated and familial cases show the same phenotype? Within a presumed single entity, will linkage marker studies show further heterogeneity? With such data in hand, certain strategies can be recommended. Complex segregation analysis of family data will permit a test of alternative models for genetic transmission. Linkage studies of selected large families (using recombinant DNA probes) will establish the genetic map location of any single-locus major factor. Selected samples of multiplex families (with several affected siblings) will concentrate the likelihood of genetic factors and will permit the detection of biochemical factors that might be significant in only a few families. Biochemical and other hypotheses can be tested in a panel of twin pairs concordant or discordant for epilepsy. The search for genetic heterogeneity clearly has implications for diagnosis, prognosis, therapy, and genetic counseling, as well as for other research studies on the basic mechanisms of the epilepsies.(ABSTRACT TRUNCATED AT 400 WORDS)

Age Factors↗

Transient global amnesia: a case-control study.

The clinical findings and laboratory results from 18 patients with clear-cut transient global amnesia (TGA) were compared with the results from 90 nonischemic neurological control patients using case-control analysis. A prior episode of cerebral ischemia, generally in the posterior circulation, was the most significant risk factor for TGA. Other risk factors for cerebrovascular disease were more prominent in the TGA group, but this association became less significant when cerebral ischemia was controlled for. Migraine, epilepsy, and psychiatric disorders were not significantly associated with TGA. In 5 of 13 patients, computed tomography showed focal thalamic and temporal lobe abnormalities. The recurrence rate was 7.0% for both TGA and subsequent cerebral ischemia. We conclude that TGA is closely linked to cerebrovascular disease. Further, prior damage to anatomical structures critical to memory may be necessary for the expression of this syndrome.

Aged↗

The risk of seizure disorders among relatives of children with febrile convulsions.

We studied the risk for seizure disorders among relatives of probands with febrile convulsions. The risk for febrile convulsions was raised in siblings, offspring, and nieces and nephews of probands. Risks to siblings were higher if one or both parents also had febrile convulsions, or the proband had no identified neurologic abnormality before the febrile convulsion, if the proband had recurrent febrile convulsions, or the febrile convulsions were complex. The risk for epilepsy was raised in siblings of probands, but not in other relatives. If the proband had febrile convulsions followed by epilepsy, risk to siblings for febrile convulsions and for epilepsy was significantly higher than when probands had either condition alone.

Adolescent↗

Reye's syndrome: incidence and time trends in Olmsted County, MN, 1950-1981.

Incidence and time trends of Reye's syndrome have been determined in Olmsted County, MN, using the facilities of the Rochester Epidemiology Program Project at Mayo Clinic. Incidence rates in individuals under 18 years of age were 1.1 per 100,000 person-years in the 1970-75 time interval and 1.7 in the 1976-81 interval. Failure to identify any case in the earliest time interval yields a rate of zero, and an incidence higher than 0.6 per 100,000 can be excluded with 95% probability. This finding is consistent with an increasing incidence over time in this community and does not exclude the possibility that Reye's syndrome did not exist in Olmsted County before 1970.

Adolescent↗

Encephalitis and aseptic meningitis, Olmsted County, Minnesota, 1950-1981: I. Epidemiology.

All cases fulfilling stated criteria for encephalitis and aseptic meningitis in Olmsted County, Minnesota, for the period 1950 through 1981 were identified. This is, to our knowledge, the first such incidence and trend study in a delineated population, providing rates per 100,000 person-years of 7.4 for encephalitis (189 cases) and 10.9 for aseptic meningitis (283 cases). These are about twelve and six times higher, respectively, than the rates reported by the Centers for Disease Control. The rates have been stable over successive 5- or 10-year periods except for a recent increase in aseptic meningitis. Both conditions were more common in the summer months, in childhood, and among males. Viral identification using conventional laboratory tests has improved with time; in the period 1970 through 1981, virus type was specified in about one-fourth of the cases. The most common agents identified were California and mumps viruses in encephalitis, and entero and mumps viruses in aseptic meningitis. Antecedent and/or concurrent infections were noted in 42 and 35% of encephalitis and aseptic meningitis cases, respectively. No case due to mumps, measles, or rubella viruses has occurred since 1972, reflecting the impact of immunizations. Recovery was reported at the end of the acute phase in 95% of patients with aseptic meningitis, and there were no deaths. Seventy-eight percent of encephalitis patients recovered completely; the case fatality rate was 3.8%. Of the encephalitis cases, 2% were diagnosed initially postmortem.

Adolescent↗

Heart disease mortality and morbidity in patients with epilepsy.

All-cause and heart disease mortality and ischemic heart disease incidence among patients with an initial diagnosis of epilepsy while residents of Rochester, MN, from 1935 through 1979 were determined. Death rates from heart disease were slightly elevated for persons with epilepsy. The increased death rate from heart disease was confined to persons less than 65 years of age. The incidence of ischemic heart disease and of sudden cardiac death as the initial manifestation of ischemic heart disease was significantly increased in persons with epilepsy, but the increase was primarily limited to those with symptomatic epilepsy attributed to cerebrovascular disease. The occurrence of ischemic heart disease and sudden cardiac death was not related to anticonvulsant medication status.

Adolescent↗

Seizures and head injury in an urban community.

Among 811 patients hospitalized for head injury (HI) in the Bronx, 14 (1.7%) had HI caused by a seizure, 47 (5.8%) had a history of seizures of some type unrelated to the index HI episode, and 40 (4.9%) had a history of single or recurrent unprovoked seizures. The 40 patients who had unprovoked seizures and HI not caused by a seizure tended to have more severe HI and were more likely to abuse drugs and alcohol, but were otherwise similar to HI patients without a history of epilepsy. The number of cases observed is increased threefold over that expected if rates were similar to those in the predominantly white middle-class community of Rochester, MN. We estimate the prevalence of epilepsy in the Bronx to be between 2.4 and 4.7%.

Adult↗

The logic of noncompliance: management of epilepsy from the patient's point of view.

Studies of patient compliance with medical regimens reveal that one-quarter to one-half of patients act at variance with their physician's suggestions. Using anthropological methods of long-term participant observation, seven epileptic patients were intensively studied for ten months in a variety of social contexts, including visits to health resources. Their conceptions of proper management of epilepsy were elicited, and their communication of these conceptions to providers of health care was observed. Although most of these patients were noncompliant, no single pattern of noncompliance encompassed their behavior as a group, and some of them had more than one drug consumption strategy. Furthermore, some patients were found to be noncompliant with biomedical regimens, yet actively and consistently pursuing alternative regimens. A range of such alternative therapies were identified both for low-income and middle-income patients. These case histories suggest that patients actively assess the quality of care given by their providers of health care. From the patient's point of view, biomedical strategies form one facet of many coping mechanisms in the management of epilepsy.

Adolescent↗