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Biomedical subjects

W Donald Shields

Publications and source records attributed to W Donald Shields.

At least 19 recordsLinked to original sources
PubMed2006

Hippocampal volume in childhood complex partial seizures.

PURPOSE: This study compared hippocampal volume in children with cryptogenic epilepsy, all of whom had complex partial seizures (CPS), and age and gender matched normal children controlling for between group differences in IQ and demographic variables (e.g., age, gender, ethnicity, socioeconomic status). It also examined the relationship between hippocampal volumes and seizure variables in the patients. METHODS: Using quantitative magnetic resonance imaging (MRI), we compared the hippocampal volumes of 19 medically treated children with CPS, aged 6-14 years, to 21 age and gender matched normal children. RESULTS: The children with CPS had significantly smaller total hippocampal volumes than the normal children. This finding was accounted for primarily by significantly smaller anterior hippocampal volumes. Within the CPS group, smaller total and posterior hippocampus volumes were significantly associated with longer duration of illness. Anterior hippocampal volumes, however, were unrelated to seizure variables. CONCLUSIONS: These findings imply impaired development of the hippocampus, particularly the anterior hippocampus, and a differential effect of the underlying illness and on-going seizures on hippocampal development in medically controlled pediatric CPS.

Adolescent↗
PubMed2006

Children with ESES: variability in the syndrome.

OBJECTIVE: We undertook a retrospective study of children who present with significant activation of paroxysmal discharges during sleep to examine the clinical spectrum of disorders that present with such an EEG abnormality. BACKGROUND: Electrical status epilepticus in sleep (ESES) is an electrographic pattern characterized by nearly continuous spike-wave discharges in slow wave sleep, usually with a frequency of 1.5-3 Hz and usually diffuse and bilateral in distribution. A variety of neurocognitive and behavioral problems have been associated with this EEG pattern. METHODS: We conducted a retrospective review of 1497 EEG records of patients admitted to University of California, Los Angeles (UCLA) for overnight video-EEG monitoring during a 5 year interval. Demographic, clinical and electroencephalographic variables were evaluated. RESULTS: EEG records for 102 patients meeting criteria were identified. Clinical information was available for 90 of those patients. Eighteen of these patients could be diagnosed with Landau-Kleffner syndrome (LKS). Key findings include: (1) neuroimaging abnormalities were uncommon in our LKS patients; (2) among children who do not fit the specific diagnostic criteria for LKS, a spike-wave index (SWI) >50% was more likely to be associated with global developmental disturbances than SWI < or =50% (p<0.05); (3) Children with generalized discharges were more likely to experience severe or global developmental disturbance than those with focal abnormalities, without reaching statistical significance (p=0.07). CONCLUSIONS: Severity of ESES can vary over time between and within patients and clinical status does not always directly correlate with SWI. However, the prognosis of LKS is substantially better than CSWS and these two disorders could be classified in a dichotomous manner rather than be seen as two points along a continuum.

Adolescent↗
PubMed2006

Thought disorder: A developmental disability in pediatric epilepsy.

This study compared thought disorder (i.e., impaired use of language to formulate and organize thoughts) in 93 children with complex partial seizures (CPSs) and 56 children with primary generalized epilepsy with absence (PGE) and its relationship to age, seizure, cognitive, and linguistic variables. By the use of psychopathology, social competence, academic achievement, and school problem measures, the functional implications of thought disorder in these two groups were compared. When demographic variables were controlled for, there were no significant differences in thought disorder scores between the CPS and PGE groups. However, the profile of age, gender, seizure, and cognitive variables related to thought disorder differed in the CPS and PGE groups. Within each group, different aspects of thought disorder were associated with different seizure variables. Thought disorder was related to psychopathology, school problems, decreased academic achievement, and poor peer interaction in the CPS group, but with school problems in the PGE group. These findings suggest that CPS and PGE affect the normal maturation of children's discourse skills, albeit through different mechanisms. The relationship of thought disorder to behavioral, academic, and social problems implies that these discourse deficits are one component of the developmental disabilities or comorbidities associated with pediatric CPS and PGE.

Adolescent↗
PubMed2006

Recurrent de novo mutations of SCN1A in severe myoclonic epilepsy of infancy.

Mutations in the voltage-gated sodium channel gene SCN1A are a major cause of severe myoclonic epilepsy of infancy (Dravet syndrome) and generalized epilepsy with febrile seizures plus. This study reports the identification of six de novo SCN1A mutations in patients with severe myoclonic epilepsy of infancy, including a tetranucleotide deletion in exon 26. The same deletion was previously observed in two unrelated patients and appears to result from slipped-strand mispairing of a direct repeat during deoxyribonucleic acid replication. Review of the literature indicates that recurrent mutations account for 25% of SCN1A mutations in severe myoclonic epilepsy of infancy, including six sites of deamination at CpG dinucleotides.

Adolescent↗
PubMed2006

Valproate-induced chorea and encephalopathy in atypical nonketotic hyperglycinemia.

Nonketotic hyperglycinemia is a disorder of amino acid metabolism in which a defect in the glycine cleavage system leads to an accumulation of glycine in the brain and other body compartments. In the classical form it presents as neonatal apnea, intractable seizures, and hypotonia, followed by significant psychomotor retardation. An important subset of children with nonketotic hyperglycinemia are atypical variants who present in a heterogeneous manner. This report describes a patient with mild language delay and mental retardation, who was found to have nonketotic hyperglycinemia following her presentation with acute encephalopathy and chorea shortly after initiation of valproate therapy.

Anticonvulsants↗
PubMed2005

Status epilepticus and frequent seizures: incidence and clinical characteristics in pediatric epilepsy surgery patients.

PURPOSE: The literature suggests that pediatric epilepsy surgery cases that present in status epilepticus (SE) are an unusual occurrence. However, this concept is based on case reports, and the incidence and clinical characteristics of these patients have not been systematically assessed. METHODS: The cohort consisted of resective epilepsy surgery cases from 2000 to 2005 (n = 115), and they were classified as presenting with continuous SE requiring medical suppression therapy (n = 6) or intermittent SE (greater than 3 seizures/hour; n = 17). The SE categories were compared with extratemporal surgery patients without SE (non-SE; n = 64) for differences in clinical variables abstracted from the medical record. RESULTS: Continuous SE was noted in 5% and intermittent SE in 15% of resective surgery cases, and all had extratemporal cortical involvement. Compared with continuous SE and non-SE cases, intermittent SE patients were younger at surgery with shorter duration of seizures, and had an increased incidence of active infantile spasms during video scalp EEG monitoring. Compared with non-SE cases, the continuous and intermittent SE groups required a larger number of antiepileptic medications presurgery and 6-months postsurgery, underwent hemispherectomy more frequently, and had an increased incidence of hemimegalencephaly and Rasmussen encephalitis and a lower occurrence of infarct/ischemia and infectious etiologies. Seizure control was over 71% up to 2 years postsurgery, and there were no differences between patient groups. Finally, seizure frequency per hour was greater in continuous SE cases compared with the intermittent SE group. CONCLUSIONS: Children presenting with continuous or intermittent SE are not rare in pediatric epilepsy surgery centers, and such cases are more commonly associated with infantile spasms, Rasmussen's syndrome, and hemimegalencephaly pathologies. Seizure outcome after surgery was not altered in pediatric patients because they had presented with continuous or intermittent SE.

Adolescent↗
PubMed2005

Depression and anxiety disorders in pediatric epilepsy.

PURPOSE: This study examined affective disorders, anxiety disorders, and suicidality in children with epilepsy and their association with seizure-related, cognitive, linguistic, family history, social competence, and demographic variables. METHODS: A structured psychiatric interview, mood self-report scales, as well as cognitive and language testing were administered to 100 children with complex partial seizures (CPSs), 71 children with childhood absence epilepsy (CAE), and 93 normal children, aged 5 to 16 years. Parents provided behavioral information on each child through a structured psychiatric interview and behavior checklist. RESULTS: Significantly more patients had affective and anxiety disorder diagnoses (33%) as well as suicidal ideation (20%) than did the normal group, but none had made a suicide attempt. Anxiety disorder was the most frequent diagnosis among the patients with a diagnosis of affective or anxiety disorders, and combined affective/anxiety and disruptive disorder diagnoses, in those with suicidal ideation. Only 33% received some form of mental health service. Age, verbal IQ, school problems, and seizure type were related to the presence of a diagnosis of affective or anxiety disorder, and duration of illness, to suicidal ideation. CONCLUSIONS: These findings together with the high rate of unmet mental health underscore the importance of early detection and treatment of anxiety disorders and suicidal ideation children with CPSs and CAE.

Adolescent↗
PubMed2004

Effects of epilepsy surgery on psychiatric and behavioral comorbidities in children and adolescents.

When considering surgery in children, it is important to think about the potential effect on psychiatric and behavioral outcome. A growing body of evidence suggests that the type of epilepsy (benign or catastrophic) and the age of the patient affect developmental outcome. In younger children with catastrophic epilepsy, the primary goal of therapy is not only to control seizures, but also to offer the child the opportunity for the best possible development. In older children with partial seizures, the therapeutic goal is to control seizures, which may allow a more normal life and improve behavior. Epilepsy surgery generally appears to have a positive effect on behavior and development in many younger children with catastrophic epilepsy. Psychiatric and behavioral outcomes in older children with complex partial epilepsy are less clear; many patients improve, but there does appear to be a small risk of developing new psychiatric or behavioral disorders postoperatively.

Adolescent↗
PubMed2004

Diagnosis of infantile spasms, Lennox-Gastaut syndrome, and progressive myoclonic epilepsy.

The epilepsies of childhood are distinguished by an interesting dichotomy between the benign and catastrophic disorders. Approximately 50% of children outgrow childhood epilepsy as they mature; although the disorder is disruptive for children and families alike, it is not considered a medical disaster. The catastrophic epilepsies of childhood, in contrast, are associated with significant morbidity and mortality. Infantile spasms, Lennox-Gastaut syndrome, and the progressive myoclonic epilepsies are correlated with significant disability and a multiplicity of underlying etiologies. Accurate diagnosis of both the syndrome and the etiology is very important for treatment purposes, as well as for family education, since many of the disorders have a significant genetic component.

Adolescent↗
PubMed2004

Five or more acute postoperative seizures predict hospital course and long-term seizure control after hemispherectomy.

PURPOSE: Acute postoperative seizures (APOSs) are those that occur in the first 7 to 10 days after surgery, and previous studies in temporal lobe epilepsy patients support the notion that APOSs may foretell failure of long-term seizure control. It is unknown whether APOSs also predict seizure outcome or hospital course after hemispherectomy. METHODS: Hemispherectomy patients (n = 114) were studied retrospectively and subdivided into the following groups: No APOSs, 1 to 5 APOSs, or >5 APOSs. Intensive care unit (ICU) nursing staff or family members reported and described the APOS events. APOS categories were compared with pre- and postsurgery clinical variables abstracted from the medical record. RESULTS: APOSs occurred in 22.6% of hemispherectomy patients. Compared with the 0 and 1 to 5 APOS groups, patients with >5 APOS showed (a) longer seizure durations before surgery, (b) longer hospitalizations, (c) later oral food intake, (d) more frequent lumbar punctures, (e) worse seizure control at 0.5 and 1 year after surgery, (f) more antiepileptic drug (AED) use at 2 and 5 years after surgery, and (g) higher reoperation rate. No similar differences were found between the 0 and 1 to 5 APOS groups. The day of the APOS, whether the APOS was typical of preoperative seizures, and postsurgery scalp EEG did not predict long-term seizure control. APOS patients in the 1 to 5 and >5 groups had lower pre- and postsurgery Vineland developmental quotients compared with those without an APOS. CONCLUSIONS: Hemispherectomy patients with >5 APOSs had a more prolonged and complicated hospital course and worse postsurgery seizure control, more AED use, and higher reoperation rate than did patients with 0 or 1 to 5 APOSs. Thus the number of APOSs was a predictor of postsurgery seizure control and can be used to counsel patients and families about prognosis after hemispherectomy.

Acute Disease↗
PubMed2004

Psychopathology and pediatric complex partial seizures: seizure-related, cognitive, and linguistic variables.

PURPOSE: This study examined the role of cognition, language, seizure-related, and demographic variables in the psychopathology of children with complex partial seizure disorder (CPS) of average intelligence. METHODS: One-hundred one CPS and 102 normal children, aged 5.1 to 16.9 years, had a structured psychiatric interview and cognitive and language testing. Parents provided demographic, perinatal, and seizure-related information, as well as behavioral information through the Child Behavior Checklist (CBCL) and a structured psychiatric interview about the child. RESULTS: Significantly more CPS patients had psychopathology, cognitive deficits, and linguistic deficits than did those in the normal group. Among the patients, Verbal IQ predicted the presence of a psychiatric diagnosis, as well as CBCL scores in the borderline/clinical range. Seizure, linguistic, and demographic variables were unrelated to psychopathology. The cognitive and linguistic deficits of the CPS group, however, were predicted by seizure factors (e.g., prolonged seizures/febrile convulsions; seizure frequency/number of antiepileptic drugs) and demographic factors (e.g., minority status). CONCLUSIONS: Because subtle verbal cognitive deficits predict behavioral disturbances in pediatric CPSs, the study's findings highlight the importance of assessing behavior, cognition, and language in these children. They also underscore the negative impact of prolonged seizures, febrile convulsions, seizure frequency, and antiepileptic drug polytherapy on cognition and language in pediatric CPSs.

Adolescent↗
PubMed2004

Management of epilepsy in mentally retarded children using the newer antiepileptic drugs, vagus nerve stimulation, and surgery.

Clusters of seizures, prolonged seizures, and status epilepticus occur more frequently in children with multiple disabilities, and chronic seizures are more likely to be refractory to treatment. In many patients, the seizures appear to contribute to the mental retardation. Thus, if the lives of these children are to improve, seizure control is essential. However, medical treatment can interfere with cognition and cause behavioral disturbances, making life very difficult for the child and the child's family. With the introduction of 10 new antiepileptic drugs in the last decade, the treatment of epilepsy in multiply handicapped children has significantly advanced. These new antiepileptic drugs may improve seizure control, medication tolerance, or both. Although the ultimate therapeutic goal is to keep children seizure free and alert, compromises regarding medication choice and dosage are still necessary in many cases. Novel treatment options, such as the vagus nerve stimulator, may decrease seizure frequency without behavioral or cognitive side effects. In carefully selected children with specific epilepsy syndromes, epilepsy surgery can provide partial or complete relief from seizures.

Anticonvulsants↗
PubMed2004

Cerebral hemispherectomy in pediatric patients with epilepsy: comparison of three techniques by pathological substrate in 115 patients.

OBJECT: Cerebral hemispherectomy for intractable seizures has evolved over the past 50 years, and current operations focus less on brain resection and more on disconnection. In addition, cases involving cortical dysplasia and Rasmussen encephalitis are being identified and surgically treated in younger individuals. Few studies have been conducted to compare whether there are perioperative differences based on hemispherectomy technique and/or pathological substrate in pediatric patients with epilepsy. METHODS: In this study the authors compared, stratified by disease, anatomical (37 cases) and Rasmussen functional hemispherectomy (32 cases) with a new modified lateral hemispherotomy (46 cases). Pathological processes included cortical dysplasia (55 cases), Rasmussen encephalitis (21 cases), infarction/ischemia (27 cases), and other/miscellaneous (12 cases). The authors found differences in perioperative clinical factors based on operative technique and/or pathological substrate. In terms of technique, the lateral hemispherotomy was associated with the least intraoperative blood loss, shortest intensive care unit stay, and lowest complication rate. The anatomical hemispherectomy was associated with the longest hospital stay, delayed oral food intake, highest postsurgery fevers, and the highest incidence of shunt requirement. The functional hemispherectomy was associated with the highest reoperation rate for recurrent seizures (25%). In terms of pathology, patients with cortical dysplasia were the youngest at surgery, suffered the greatest amount of blood loss, and required the longest operative/anesthesia times compared with the other pathologically defined groups. Postoperative seizure control (range 0.5-2 years) was not statistically different according to technique or disease process and was similar to that in cases of pediatric temporal lobe epilepsy. CONCLUSIONS: The authors found differences in perioperative risks and hospital course but not postsurgery seizure control, which vary by hemispherectomy technique and/or disease process. The modified lateral hemispherotomy approach offers various advantages related to operative blood loss and reoperation compared with anatomical and functional hemispherectomies that are especially relevant in younger patients with cortical dysplasia and Rasmussen encephalitis with small and/or malformed ventricles.

Cerebral Cortex↗
PubMed2003

Behavioral disorders in pediatric epilepsy: unmet psychiatric need.

PURPOSE: This study examined the relation between psychiatric diagnosis and mental health services in children with epilepsy and the associated demographic, cognitive, linguistic, behavioral, and seizure-related variables. METHODS: The Kiddie Schedule for Affective Disorders and Schizophrenia (K-SADS), the Child Behavior Checklist, the Test of Language Development, and the Wechsler Intelligence Scale for Children-Revised (WISC-R) were administered to 114 children, aged 5 to 16 years, with either complex partial seizures (CPS) or primary generalized with absence (PGE, petit mal). A Diagnostic and Statistical Manual of Mental Disorders (DSM-IV) diagnosis and information regarding mental health services were derived from the K-SADS. RESULTS: Although approximately 60% of the subjects had a DSM-IV psychiatric diagnosis, >60% received no mental health treatment. Absence of mental health care was associated with younger age, less parental education, limited number of antiepileptic drugs (AEDs; i.e., one or none), and higher verbal IQ. In addition, children with PGE and a single psychiatric diagnosis were less likely to have a history of mental health treatment. CONCLUSIONS: This is the first study to demonstrate unmet mental health need in a large sample of children with CPS and PGE. The study's findings suggest that parents and clinicians should be aware of the mental health needs of children with epilepsy, particularly if they have one or more of the identified risk factors.

Adolescent↗
PubMed2002

Developmental outcome with and without successful intervention.

A review of the literature on the developmental outcome of medically and surgically treated symptomatic infantile spasms (IS) indicates that poor seizure control, severe mental retardation, and marked behavioral disorders are found at long-term outcome of symptomatic IS. The 2-year outcome findings of the UCLA Pediatric Epilepsy Surgery Research Group in children with symptomatic infantile spasms and in children with early onset intractable symptomatic epilepsy other than IS (non-IS) demonstrate impaired development of cognition, language, and social communication despite improved seizure control. The social communication deficits of these children are similar to those found in autistic children. Finally, the underlying pathology of the resected brain, rather than ongoing seizures and seizure type, plays an important role in development of the remaining brain tissue and the outcome of early onset symptomatic IS and non-IS.

Child Behavior↗
PubMed2002

Efficacy and safety of levetiracetam in children with partial seizures: an open-label trial.

PURPOSE: To assess the efficacy and safety of levetiracetam (LEV) as adjunctive therapy in children with treatment-resistant partial-onset seizures. METHODS: Children (aged 6-12 years) with treatment-resistant partial-onset seizures receiving one standard antiepileptic drug (AED) were eligible. After a 4-week baseline period, children received LEV in a 6-week titration phase (target dose, 40 mg/kg/day) followed by an 8-week evaluation phase. Seizure frequency during the evaluation period with individualized LEV doses (20-40 mg/kg/day) were compared with the 4-week baseline seizure frequency. Plasma concentrations of LEV and other AEDs were determined to evaluate potential drug interactions. RESULTS: Twenty-four subjects enrolled and received LEV; 23 entered the evaluation phase, and 22 completed the evaluation phase. Compared with their baseline seizure frequency, 12 (52%) of 23 subjects entering the evaluation phase had their seizure frequency decrease by >50%. Two subjects remained seizure free during the entire evaluation period. LEV did not significantly affect plasma concentrations of any concomitant AED during this study, and no alteration of mean clinical laboratory values was observed. The most commonly reported adverse events were headache, infection, anorexia, and somnolence. CONCLUSIONS: This open-label study of adjunctive LEV therapy (at 20-40 mg/kg/day) suggests that LEV is effective, safe, and well tolerated in children ages 6-12 years with treatment-resistant partial-onset seizures. A randomized, placebo-controlled, double-blind trial of LEV adjunctive therapy in children with treatment-resistant partial-onset seizures is needed and ongoing to confirm these open-label findings.

Age Factors↗
PubMed2002

Social communication in children with epilepsy.

BACKGROUND: This study examined measures of social communication that involve the use of language in formulating and organizing thoughts and its relationship with seizure-related, developmental, cognitive, and behavioral variables in 92 children with complex partial seizure disorder (CPS), 51 with primary generalized epilepsy (PGE), and 117 normal children, aged 5.1-16.9 years. METHODS: Coding the children's speech samples with the Kiddie Formal Thought Disorder Rating Scale (Caplan et al., 1989) and Halliday and Hasan's (1976) analysis of cohesion demonstrated social communication deficits in both seizure disorder groups. RESULTS: The CPS patients had both formal thought disorder and cohesion deficits and the PGE group had mild cohesion deficits. IQ, as well as fronto-temporal and bilateral spike and wave activity were associated with the severity of the social communication deficits of the CPS group. The social communication deficits of the PGE group, however, were related to IQ and seizure control. CONCLUSIONS: Recurrent CPS and PGE and fronto-temporal localization of epileptic activity might impair the development of children's communication skills.

Adolescent↗