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Biomedical subjects

S Shinnar

Publications and source records attributed to S Shinnar.

At least 55 records · Page 3Linked to original sources
PubMed1999

Treatment of newly diagnosed pediatric epilepsy: a community-based study.

OBJECTIVE: To determine the patterns and frequency of treatment and use of specific drugs for newly diagnosed pediatric epilepsy. DESIGN AND SETTING: Prospective, community-based study. Children were recruited from physicians in Connecticut from 1993 to 1997. PATIENTS: Children aged 1 month through 15 years at the time of their first seizure, who had 2 or more unprovoked seizures, and who were newly diangosed during the recruitment period were eligible. MAIN OUTCOME MEASURE: Initiation of treatment at diagnosis and within 1 year after diagnosis of epilepsy. RESULTS: Of 613 children, 482 (78.6%) were treated at the time of initial diagnosis. By 6 months another 10.3% were treated, and by 12 months 90% of the cohort had been treated. The most commonly prescribed antiepileptic drug (AED) was carbamazepine (38.8%) followed by sodium valproate (18.4%). Only 1 child received an investigational drug and none received any of the most recently approved drugs as a first AED. Children with idiopathic and secondarily generalized forms of epilepsy were most likely to be treated (90%-100%), whereas children with idiopathic localization-related epilepsy were least likely to be treated (50.8%). Approximately 80% of those with other forms of epilepsy were treated at the time of diagnosis. Use of specific medications reflected current guidelines and recommendations for treatment of specific seizure types and syndromes. CONCLUSIONS: In Connecticut, approximately 20% of children with epilepsy are not treated at the time of initial diagnosis, and around 10% continue to be untreated after 1 year. This most likely reflects the increased understanding of the nature of pediatric epilepsy and concerns regarding the adverse effects of AEDs. The most commonly used first drugs are carbamazepine and valproate. Follow-up of this cohort may help provide information to guide the use of recently approved AEDs.

Adolescent↗
PubMed1999

Status epilepticus in children with newly diagnosed epilepsy.

Status epilepticus (SE) constitutes a neurological emergency and may be of prognostic value in individuals with epilepsy. Little is known about the associations between other prognostic factors in epilepsy and the occurrence of SE. The following study examines associations between clinical characteristics of children with newly diagnosed epilepsy and the occurrence of SE when epilepsy is first diagnosed. Children were recruited prospectively from the practices of physicians throughout Connecticut. Information was collected via standardized interviews with parents and review of pertinent records. Analyses were performed for any SE, unprovoked SE only, and previous provoked SE. Of 613 children, 56 (9.1%) had one or more episodes of SE by the time the diagnosis of epilepsy was established. Factors correlated with SE during an unprovoked seizure were partial seizures and previous craniotomy. For SE during a provoked seizure, correlates were primarily young age at onset of epilepsy and nonidiopathic epilepsy syndrome. To date, subsequent SE has occurred in 4.3% of children without and in 19.6% of those with SE at diagnosis. By the time epilepsy is first diagnosed in children, SE has already occurred in a substantial minority. It is correlated with specific clinical characteristics in the children, which differ depending on whether the SE was provoked or unprovoked. SE has a high risk of recurring.

Child↗
PubMed1999

Epilepsy syndromes in patients with childhood-onset seizures in Finland.

Childhood-onset epilepsy is a common disorder. There is insufficient data on the distribution of epilepsy syndromes in the population and their effect on long-term prognosis. This report presents the data on epilepsy syndromes in a childhood-onset epilepsy cohort. A population-based active-prevalence cohort of all children under 16 years of age residing in the catchment area of Turku University Hospital with active epilepsy between 1961 and 1964 was monitored prospectively until 1992. Etiology, seizure type, and epilepsy syndromes were classified according to the recent guidelines of the International League Against Epilepsy. The etiology of seizures was idiopathic in 28%, cryptogenic in 22%, and remote symptomatic in 50%. Seizures were classifiable in 235 patients (96%) and included 157 (64%) with partial seizures and 88 (36%) with generalized seizures. Epilepsy syndromes could be classified in 223 patients (91%) and included 152 (62%) localization-related syndromes, 61 (25%) generalized epilepsy syndromes, and 10 (4%) with syndromes not determined as being focal or generalized. Prognosis for both survival and remission was dependent on etiology and on the specific epilepsy syndrome. The authors conclude that the International League Against Epilepsy guidelines can be successfully applied to a population-based cohort with childhood-onset epilepsy. Accurate classification of syndromes is important because in many cases long-term outcome may be largely determined by the underlying epilepsy syndrome.

Adult↗
PubMed1999

A 5-month-old with intractable epilepsy.

The nosology of early infantile seizures and epilepsy syndromes is controversial. There are two age-related early infantile epileptic encephalopathy syndromes that are characterized by early onset of spasms, a burst-suppression EEG pattern, poor response to treatment, and poor prognosis. This paper examines the early myoclonic epilepsies.

Brain↗
PubMed1999

Evidence for linkage of adolescent-onset idiopathic generalized epilepsies to chromosome 8-and genetic heterogeneity.

Several loci and candidate genes for epilepsies or epileptic syndromes map or have been suggested to map to chromosome 8. We investigated families with adolescent-onset idiopathic generalized epilepsy (IGE), for linkage to markers spanning chromosome 8. The IGEs that we studied included juvenile myoclonic epilepsy (JME), epilepsy with only generalized tonic-clonic seizures occurring either randomly during the day (random grand mal) or on awakening (awakening grand mal), and juvenile absence epilepsy (JAE). We looked for a gene common to all these IGEs, but we also investigated linkage to specific subforms of IGE. We found evidence for linkage to chromosome 8 in adolescent-onset IGE families in which JME was not present. The maximum multipoint LOD score was 3.24 when family members with IGE or generalized spike-and-waves (SW) were considered affected. The LOD score remained very similar (3.18) when clinically normal family members with SW were not considered to be affected. Families with either pure grand mal epilepsy or absence epilepsy contributed equally to the positive LOD score. The area where the LOD score reaches the maximum encompasses the location of the gene for the beta3-subunit of the nicotinic acetylcholine receptor (CHRNB3), thus making this gene a possible candidate for these specific forms of adolescent-onset IGE. The data excluded linkage of JME to this region. These results indicate genetic heterogeneity within IGE and provide no evidence, on chromosome 8, for a gene common to all IGEs.

Adolescent↗
PubMed1999

Classification of childhood epilepsy syndromes in newly diagnosed epilepsy: interrater agreement and reasons for disagreement.

PURPOSE: The International League Against Epilepsy (ILAE) classification of the epilepsies is in increasingly widespread use. The following analysis was done to assess the interrater agreement in classifying epilepsy syndromes in children with newly diagnosed epilepsy. METHODS: In a prospective, community-based study, 613 children with newly diagnosed epilepsy were recruited. Based on information available at diagnosis or generated as part of the initial diagnostic assessment, three pediatric neurologists independently classified epilepsy syndromes. Interrater agreement was assessed with kappa. RESULTS: Interrater agreement was extremely good, with kappa scores > or = 0.80 for almost all comparisons. Relatively limited quality of the EEG and seizure information in some cases, as well as discrepancies between the two, were associated with a tendency for more disagreement. CONCLUSIONS: A high degree of interrater agreement was obtained in this study, indicating that the system for classifying syndromes can be meaningfully used in a community-based sample. Quality of the information, which is often, by necessity, less than optimal in newly diagnosed epilepsy, is a potential barrier to identification of syndromes. A substantial proportion of children were classified into relatively nonspecific syndromes. Over time, additional information may come to light to allow more precise identification of their forms of epilepsy. In an epidemiologic setting, the ILAE classification of the epilepsies can be successfully used with a high degree of reliability to classify newly diagnosed epilepsy in children.

Age Factors↗
PubMed1999

Newly diagnosed epilepsy in children: presentation at diagnosis.

PURPOSE: The current understanding of epilepsy has changed significantly in the past 2 decades. This report presents a description of newly diagnosed childhood-onset epilepsy, with a special emphasis on epilepsy syndromes, in a large, prospectively ascertained community-based cohort evaluated and diagnosed in the mid-1990s. METHODS: Children, aged 0 through 15 years at the time of the first seizure, were prospectively identified at the time of diagnosis of epilepsy through the practices of 16 of the 17 child neurologists in Connecticut as well as five adult neurologists and seven pediatricians from January 1993 through December 1997. Parents were interviewed, and all relevant medical records were reviewed. Classification of seizures and of epilepsy syndromes was done for each child by each of three pediatric neurologists. Discrepancies were resolved in conference. RESULTS: A total of 613 children was recruited into the study. The median age at time of the first seizure was 5.3 years. Half the cohort was boys. Eighteen percent had a remote symptomatic etiology. Epilepsy syndromes were classifiable in all but four children, although some syndromes are, by definition, relatively nonspecific. In this childhood-onset cohort, 58.6% of the syndromes were localization related, 29.0% generalized, and 12.4% undetermined as to whether focal or generalized. Benign rolandic epilepsy occurred in 10% of the cohort. Primarily generalized syndromes accounted for 20.6%, with childhood absence being the single most common syndrome in this subgroup (12.1% of the cohort). Secondarily generalized syndromes accounted for 8.5% of the total, with infantile spasms being the most common in this grouping (3.9% of the cohort). CONCLUSIONS: This study presents a description of childhood- and adolescent-onset epilepsy as it is diagnosed and evaluated in the 1990s in one state in the United State and based on current classification guidelines. The results should be generalizable to the rest of the country. The prognostic value of early identification of epilepsy syndromes will be determined through subsequent follow-up of this cohort.

Age Factors↗
PubMed1999

The association between seizure clustering and convulsive status epilepticus in patients with intractable complex partial seizures.

PURPOSE: We examined the association between seizure clustering and convulsive status epilepticus (SE) in patients with intractable complex partial seizures, to identify whether patients whose seizures typically cluster are at high risk for convulsive SE (CSE). METHODS: Seventy-six patients with intractable complex partial epilepsy who underwent presurgical evaluation in the Montefiore Epilepsy Management Unit from 1993 to 1997 were contacted and interviewed about typical seizure frequency and distribution and history of CSE. Seizure clustering was defined as three or more complex partial seizures within a 24-h period, with return to baseline between seizures. RESULTS: Of the 76 patients contacted, 21 (28%) had experienced at least one episode of CSE, and 36 (47%) typically experienced clustered seizures. SE occurred in 16 (44%) of 36 patients with clustered seizures, and in five (12.5%) of 40 patients with nonclustered seizures (p < 0.002). Of 53 patients with temporal lobe epilepsy, CSE occurred in 13 (50%) of 26 patients with clustered seizures, and four (14.8%) of 27 patients with nonclustered seizures (p < 0.006). CONCLUSIONS: Patients with intractable complex partial or localization-related epilepsy who typically experience seizure clustering are at a significantly higher risk for CSE than are patients with nonclustered seizures.

Adolescent↗
PubMed1999

Distribution of epilepsy syndromes in a cohort of children prospectively monitored from the time of their first unprovoked seizure.

PURPOSE: To assess the distribution of epilepsy syndromes and their stability in children. METHODS: A cohort of 407 children with a first unprovoked seizure was prospectively recruited and followed up for a mean of 9.4 years. Etiology and epilepsy syndromes were classified by using the International League Against Epilepsy (ILAE) guidelines in the 182 children with two or more seizures. Classification was done both at time of second seizure and at last follow-up. Two-year terminal remission also was analyzed by etiology and epilepsy syndrome. RESULTS: Etiology of epilepsy syndromes was idiopathic in 45 (25%), cryptogenic in 89 (49%), and remote symptomatic in 48 (26%). In the initial classification, 114 (63%) children had a localization-based epilepsy syndrome including idiopathic in 26, cryptogenic in 34, and symptomatic based on localization or etiology in 54. Twenty-one (12%) children had a generalized epilepsy syndrome, including 19 with primary generalized epilepsy. Forty-seven (26%) cases were in the category of undetermined if focal or generalized. At last follow-up, there was a change in either etiology (n = 16) or the final epilepsy syndrome classification (n = 33) or both (n = 15) in 34 (19%) cases. At time of last follow-up, 144 (79%) of the children with epilepsy were in 2-year terminal remission, and 108 (59%) were in 2-year terminal remission without medications. Factors associated with a favorable prognosis included an idiopathic or cryptogenic etiology and having a localization-based idiopathic epilepsy syndrome. CONCLUSIONS: After two seizures, childhood-onset epilepsy can be classified by etiology and epilepsy syndrome. Prognosis is favorable in the majority of cases. However, the apparent syndrome may change with longer follow-up. The ability to classify these cases early in the clinical course is important if they are to be used for prognostic purposes.

Adolescent↗
PubMed1998

Long-term prognosis of seizures with onset in childhood.

BACKGROUND: The long-term prognosis of seizures that begin in childhood is uncertain. METHODS: We prospectively studied 245 children from the catchment area of Turku University Hospital in Turku, Finland, who had active epilepsy diagnosed between 1961 and 1964. Sixty-eight patients (28 percent) had idiopathic seizures (presumed to have a genetic origin), 54 (22 percent) had cryptogenic seizures (occurring in otherwise normal persons with no clear cause), and 123 (50 percent) had remote symptomatic seizures (with no immediate cause but occurring in persons with a prior brain injury or a static encephalopathy). RESULTS: At the final follow-up in 1992, we had sufficient data on 220 patients (90 percent), 176 of whom were alive and 44 of whom had died; the remaining 25 had emigrated, could not be traced, or declined to participate. Thirty-nine patients who died were not free of seizures at the time of death, and 33 had remote symptomatic seizures. Among the surviving patients, 112 (64 percent) had been seizure-free for at least five years, including 83 (47 percent) who were not taking antiepileptic medications. The most important predictors of being seizure-free for at least five years were a rapid response to therapy (defined as a reduction in the frequency of seizures of 75 to 100 percent within three months of beginning treatment) and a diagnosis of idiopathic seizures. As compared with a matched control group, 99 patients with epilepsy but no other initial neurologic impairment were of similar socioeconomic status and had similar rates of passing an examination given after 12 years of school. Significantly more patients, however, had completed only six years of school (relative risk, 2.13), were unemployed (relative risk, 3.76), were not married (relative risk, 3.50), and did not have children (relative risk, 3.00). CONCLUSIONS: Although the majority of patients with epilepsy in childhood are free of seizures by the time they become adults, they are at increased risk for social and educational problems. Patients whose epilepsy does not remit also have an increased risk of death.

Adolescent↗
PubMed1998

A multicenter study of the efficacy of the ketogenic diet.

OBJECTIVE: To determine the efficacy of the ketogenic diet in multiple centers. DESIGN: A prospective study of the change in frequency of seizures in 51 children with intractable seizures who were treated with the ketogenic diet. SETTING: Patients were enrolled from the clinical practices of 7 sites. The diet was initiated in-hospital and the patients were followed up for at least 6 months. PATIENTS: Fifty-one children, aged 1 to 8 years, with more than 10 seizures per week, whose electroencephalogram showed generalized epileptiform abnormalities or multifocal spikes, and who had failed results when taking at least 2 appropriate anti-epileptic drugs. INTERVENTION: The children were hospitalized, fasted, and a 4:1 ketogenic diet was initiated and maintained. MAIN OUTCOME MEASURES: Frequency of seizures was documented from parental calendars and efficacy was compared with prediet baseline after 3, 6, and 12 months. The children were categorized as free of seizures, greater than 90% reduction, 50% to 90% reduction, or lower than 50% reduction in frequency of seizures. RESULTS: Eighty-eight percent of all children initiating the diet remained on it at 3 months, 69% remained on it at 6 months, and 47% remained on it at 1 year. Three months after initiating the diet, frequency of seizures was decreased to greater than 50% in 54%. At 6 months, 28 (55%) of the 51 initiating the diet had at least a 50% decrease from baseline, and at 1 year, 40% of those starting the diet had a greater than 50% decrease in seizures. Five patients (10%) were free of seizures at 1 year. Age, sex, principal seizure type, and electroencephalogram were not statistically related to outcome. CONCLUSION: The ketogenic diet is effective in substantially decreasing difficult-to-control seizures and can successfully be administered in a wide variety of settings.

Child↗
PubMed1998

Issues related to caring for infants to adults on an integrated epilepsy unit.

We integrated the care of patients of all ages (ranging thus far from 4 weeks to 73 years) in our dedicated 8-bed Epilepsy Unit. Administrative issues pertaining to admission and discharge criteria, unit policies and procedures and an interdisciplinary quality assurance plan were examined in relation to the impact of combining both pediatric and adult patients. Clinical considerations included the diversified abilities needed to care for pediatric and adult patients both in relation to the technical skills as well as psychosocial skills required. The advantages of integrating patients of all ages on one unit include having a staff highly trained in assessment and intervention skills for a particular disorder. The psychosocial issues that arise in these patients, regardless of age, tend to encompass the entire family; therefore a holistic approach is appropriate for both children and adults. An autonomous nursing practice was established with the development of critical pathways and patient care protocols. Our experience suggests that integrated specialized units can enhance the care of patients with intractable seizures.

Adolescent↗
PubMed1998

Complications of the ketogenic diet.

PURPOSE: The ketogenic diet has been successfully used in treatment of pediatric epilepsy for >70 years. Few serious complications caused by the diet have been reported. We report complications that have been experienced by children receiving the ketogenic diet. METHODS: In a 22-month period, we treated 52 children with the classic ketogenic diet and monitored them in a prospective manner. RESULTS: Five children (10%) experienced serious adverse events (AE) after initiation of the diet. Four patients (80%) were treated with valproate (VPA) in addition to the diet, as compared with 25 (53%) of the other 47 children. Two patients developed severe hypoproteinemia within 4 weeks of initiation of the diet, and 1 of them also developed lipemia and hemolytic anemia. A third child developed Fanconi's renal tubular acidosis within 1 month of diet initiation. Two other children manifested marked increases in liver function tests, 1 during the initiation phase and the other 13 months later. CONCLUSIONS: Clinicians who wish to use the ketogenic diet must be aware of the potential of serious AE and possible interactions of the diet with VPA.

Adolescent↗
PubMed1998

Seizures with fever after unprovoked seizures: an analysis in children followed from the time of a first febrile seizure.

PURPOSE: To determine how the onset of unprovoked seizures influences recurrence of seizures with fever in children followed from the time of a first febrile seizure. METHODS: In a prospective cohort of children (n = 428) identified at the time of a first febrile seizure, predictors of a second seizure with fever were identified. The occurrence of a first unprovoked seizure was treated as a time-dependent covariate in a Cox regression model rather than as a censoring point as it traditionally has been in the past. RESULTS: One hundred forty-three (33.4%) children had further seizures. Seven had further seizures with fever only after onset of unprovoked seizures. After adjustment was made for the four previously described predictors of recurrent febrile seizures (age at onset, family history, height of fever, and duration of fever), the onset of unprovoked seizures was associated with a rate ratio of 3.47 (p = 0.0015), indicating a large increase in the risk of further seizures with fever after onset of unprovoked seizures. CONCLUSIONS: Young children who develop unprovoked seizures after a febrile seizure are at substantial risk for further seizures with fever. This may represent part of the spectrum of benign febrile seizures or it may represent the so-called "epilepsy triggered by fever" spectrum. It affects only a small proportion of children with febrile seizures; however, in some children, it may be useful information to consider when making treatment decisions.

Age of Onset↗
PubMed1997

Predictors of recurrent febrile seizures. A prospective cohort study.

OBJECTIVES: To define the risk and identify predictors of single and multiple recurrent febrile seizures. METHODS: Children (n = 428) with first febrile seizures were prospectively identified and followed for 2 or more years. Parents were interviewed soon after their children's first febrile seizure and were called every 3 months to ascertain recurrent febrile seizures. Medical records of first and recurrent seizures were reviewed for additional information. RESULTS: A total of 136 children (31.8%) experienced recurrent seizures: 73 (17.1%) had only 1 recurrence, 38 (8.9%) had 2 recurrences, and 25 (5.8%) had 3 or more recurrences. Young age at onset, a history of febrile seizures in a first-degree relative, low degree of fever while in the emergency department, and a brief duration between the onset of fever and the initial seizure were strong independent predictors of recurrent febrile seizures. With these 4 factors combined, it is possible to define groups of children having very high and very low probabilities of having any recurrences (> 70% vs < 20%), having 2 or more recurrences (> 60% vs < 10%), and having 3 or more recurrence (12% vs about 0%). In children who had at least 1 recurrence, age at the time of the first recurrence and a family history of epilepsy were predictors of subsequent recurrences. CONCLUSIONS: In children who have had a first febrile seizure, recurrences are common. The risk for 1 or more recurrences can be meaningfully predicted at the time of the initial febrile seizure with a combination of the 4 factors identified in this study.

Child, Preschool↗
PubMed1997

Tiagabine.

Tiagabine is a novel antiepileptic drug that was designed to block gamma-aminobutyric acid uptake by presynaptic neurons and glial cells. It has been shown to be effective against partial seizures in adults and adolescents. Preliminary pediatric data are also encouraging. This article reviews the available animal and human data on the pharmacokinetics, efficacy, and safety of tiagabine.

Adult↗
PubMed1997

Do seizures beget seizures? An assessment of the clinical evidence in humans.

For more than a century, epilepsy was characterized as a chronic disease, with little chance of remission or cure. It was also considered a progressive disease in which seizures led to more seizures. Experimental work in animals provided additional support for the notion that seizures could beget seizures. However, the earliest clinical observations in humans were based on highly selected, largely refractory patients. Furthermore, the experimental work in animals bore little relation to naturally occurring seizures and epilepsy in humans. Evidence from multiple sources regarding the nature and natural history of seizures and epilepsy in humans has repeatedly demonstrated that in most cases of occurrence of seizures itself does not influence the long-term outcome of epilepsy. Consequently, interventions to prevent seizures early in the course of a seizures disorder do not alter the natural history of seizure disorders with respect to whether remission will occur in the long term. That outcome is largely predetermined by other factors, many of which are not currently amenable to intervention. In some rare syndromes, deterioration is progressive. In these instances, it is the underlying syndrome, not the seizures, that is primarily responsible for the deterioration. In addition, extremely prolonged seizures (which are rare) may also directly cause damage. These are exceptions rather than, as previously believed, the rule. The available human data strongly suggest that seizures do not beget seizures and that epilepsy in humans is usually not a progressive disorder.

Anticonvulsants↗