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Individuals with Lennox- Gastaut syndrome ( LGS) often do not respond to or become resistant to pharmacologic treatments. Ketogenic diets ( KDs) and vagus nerve stimulation ( VNS) are nonpharmacologic treatment options for these intractable patients. The classic KD, a high-fat, low-carbohydrate diet with 90% of calories derived from fat, has been used in the treatment of seizures for >90 years. About half of patients with LGS respond to the KD with a >50% reduction in seizures and some patients may achieve a >90% reduction. Vagus nerve stimulation therapy involves a surgically implanted generator that delivers intermittent electrical stimuli to the brain via an electrode wrapped around the left vagus nerve. It is utilized as adjunctive therapy for patients with drug-resistant epilepsy (including patients with LGS) who are not suitable candidates for resective surgery. Similar to the KD, about half of LGS patients respond to VNS therapy, with a >50% reduction in seizures, and the response may improve over time. Both the KD and VNS are options for patients with LGS. [ABSTRACT FROM AUTHOR]

A large randomized study was conducted in patients with newly diagnosed infantile spasms to compare 2 doses of vigabatrin in achieving spasm cessation. High (100-148 mg/kg/d) and low (18-36 mg/kg/d) oral doses of vigabatrin were evaluated in a randomized, single-blind study of 14 to 21 days with subsequent open-label treatment up to 3 years. Spasm cessation was defined as 7 consecutive days of spasm freedom beginning within the first 14 days, confirmed by video-electroencephalogram. A total of 221 subjects comprised the modified intent-to-treat cohort. More subjects in the high-dose group achieved spasm cessation compared with the low-dose vigabatrin group (15.9% [17/107] vs 7.0% [8/114]; P = .0375). During follow-up, 39 of 171 (23%) subjects relapsed; 28 of 39 (72%) regained spasm freedom. Adverse events were primarily mild to moderate in severity. Vigabatrin had a dose-dependent effect in spasm reduction. Spasm cessation occurred rapidly and was maintained in the majority of infants. [ABSTRACT FROM PUBLISHER]

Evidence for a poor psychiatric, social, and vocational adult outcome in childhood absence epilepsy (CAE) suggests long-term unmet mental health, social, and vocational needs. This cross-sectional study examined behavioral/emotional, cognitive, and linguistic comorbidities as well as their correlates in children with CAE. Sixty-nine CAE children aged 9.6 (SD = 2.49) years and 103 age- and gender-matched normal children had semistructured psychiatric interviews, as well as cognitive and linguistic testing. Parents provided demographic, seizure-related, and behavioral information on their children through a semi-structured psychiatric interview and the child behavior checklist (CBCL). Compared to the normal group, 25% of the CAE children had subtle cognitive deficits, 43% linguistic difficulties, 61% a psychiatric diagnosis, particularly attention deficit hyperactivity disorder (ADHD) and anxiety disorders, and 30% clinically relevant CBCL broad band scores. The most frequent CBCL narrow band factor scores in the clinical/borderline range were attention and somatic complaints, followed by social and thought problems. Duration of illness, seizure frequency, and antiepileptic drug (AED) treatment were related to the severity of the cognitive, linguistic, and psychiatric comorbidities. Only 23% of the CAE subjects had intervention for these problems. The high rate of impaired behavior, emotions, cognition, and language and low intervention rate should alert clinicians to the need for early identification and treatment of children with CAE, particularly those with longer duration of illness, uncontrolled seizures, and AED treatment. [ABSTRACT FROM AUTHOR]

Infantile spasms is one of the “catastrophic childhood epilepsies” because of the difficulty in controlling seizures and the association with mental retardation. However, early recognition, a careful diagnostic evaluation, and proper treatment may allow some children to attain seizure control and to achieve a normal, or at least much improved, level of development. Thus, there is the opportunity to have an important impact in the lives of these unfortunate children and their families. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

West's syndrome (infantile spasms) can be considered the classic disorder of the catastrophic childhood epilepsies. Although West's syndrome was identified 160 years ago, it is still not fully understood today. Because of the multiple etiologies associated with West's syndrome, the evaluation and treatment of the disease are complex, and, to date, there are no drugs approved by the US Food and Drug Administration specifically for the treatment of infantile spasms. The multiple etiologies associated with infantile spasms require that physicians take a broad look at all therapeutic options. There are, however, logical sequences that physicians should pursue when trying to treat this disorder. This article reviews the etiologies associated with West's syndrome, antiepilepsy drug therapies currently being used to treat the syndrome, and other treatment options available to clinicians. (J Child Neurol 2002;17:S76-S79). [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Abstract: Given the FDA's warning regarding the potential connection between suicidal behavior and antiepileptic drugs, this study examined methods by which to detect suicidal ideation in children with epilepsy. It compared the sensitivity, specificity, and area under the curve for identifying children with suicidal behavior using the Child Behavior Checklist (CBCL) and a structured psychiatric interview. Parent-completed CBCLs provided behavioral problem scores on 177 children with epilepsy, aged 5–16years. Psychiatric diagnoses were made based on separate child and parent structured psychiatric interviews about the child. The children answered questions on suicidal behaviors during the interview. A clinically elevated score in the CBCL Total Problems scale and having more than one psychiatric diagnosis, irrespective of the type of diagnosis, were significant predictors and correctly classified children with suicidal ideation in 79% of the cases based on the CBCL and 80% of the cases with more than one psychiatric diagnosis. These findings indicate that elevated CBCL Total Problems scores, a commonly used instrument, can screen and identify risk for suicidal behavior in children with epilepsy. Additionally, irrespective of diagnosis, if a child with epilepsy has more than one psychiatric diagnosis, further assessment of suicidal behavior is warranted. Importantly, the results underscore the utility of having parents complete a questionnaire in the waiting room in order to identify children with epilepsy at risk for suicidal behavior. [Copyright &y& Elsevier]

Abstract: This study examined the relationship between suicidal ideation and frontotemporal volumes, particularly orbital frontal gyrus volume, in 51 subjects with epilepsy with a mean age of 9.8 (2.1) years. Structured psychiatric interviews of the children and parents provided information on suicidal behavior and DSM-IV diagnoses. Tissue of 1.5-T MRI scans was segmented, and total brain, frontal lobe, frontal parcellations, and temporal lobe volumes were computed. The 11 subjects with epilepsy with suicidal ideation had significantly smaller right orbital frontal gyrus white matter volumes and larger left temporal lobe gray matter volumes than the 40 children without suicidal thoughts. Given the role of the orbital frontal gyrus in both emotional regulation and epilepsy, these findings highlight the biological underpinnings of suicidal ideation in pediatric epilepsy. [Copyright &y& Elsevier]

Abstract: Children with epilepsy and control children were followed over a 2-year interval. Comorbidities of epilepsy, often defined as problems related to IQ, academic achievement, language, and psychopathology, were evaluated prospectively. It was hypothesized that over time (1) the presence of comorbidities would predict worse outcomes, and (2) epilepsy variables would negatively impact comorbidities. The study included 39 children with complex partial seizures (CPS), 25 children with childhood absence epilepsy (CAE), and 27 healthy children, aged 7.6–16.1years. The findings were notable for stability over the interval in all three groups. Additionally, baseline seizure variables and change over the interval appear to play a role in IQ and math achievement scores of children with epilepsy with average IQ and in the reading achievement scores of those with below-average IQ. However, seizure variables at baseline and follow-up were not predictors of DSM-IV diagnoses, depression, anxiety, or behavioral problems. [Copyright &y& Elsevier]

Abstract: In this study the relationship between language skill and frontotemporal volumes was compared in 69 medically treated subjects with epilepsy and 34 healthy children, aged 6.1–16.6years. Also, whether patients with linguistic deficits had abnormal volumes and atypical associations between volumes and language skills in these brain regions was determined. The children underwent language testing and MRI scans at 1.5 T. Brain tissue was segmented and frontotemporal volumes were computed. Higher mean language scores were significantly associated with larger inferior frontal gyrus, temporal lobe, and posterior superior temporal gyrus gray matter volumes in the epilepsy group and in the children with epilepsy with average language scores. Increased total brain and dorsolateral prefrontal gray and white matter volumes, however, were associated with higher language scores in the healthy controls. Within the epilepsy group, linguistic deficits were related to smaller anterior superior temporal gyrus gray matter volumes and there was a negative association between language scores and dorsolateral prefrontal gray matter volumes. These findings demonstrate abnormal development of language-related brain regions, and imply differential reorganization of brain regions subserving language in children with epilepsy with normal linguistic skills and in those with impaired language. [Copyright &y& Elsevier]

Abstract: Abnormal amygdala volumes in pediatric mood–anxiety disorders and attention deficit hyperactivity disorder (ADHD), as well as high rates of these diagnoses in childhood absence epilepsy (CAE), prompted this study of amygdala volume in CAE. Twenty-six children with CAE and 23 normal children, aged 6.6–15.8years, underwent MRI at 1.5T. The tissue imaged with MRI was segmented, and amygdala volumes were obtained by manual tracings. There were no significant amygdala volume differences between the CAE and normal groups. Within the CAE group, however, the children with ADHD had significantly smaller amygdala volumes than the subjects with CAE with no psychopathology and those with mood/anxiety diagnoses. There was also a significant relationship between higher seizure frequency and greater amygdala asymmetry in the epilepsy group. Given ongoing development of the amygdala during late childhood and adolescence, despite the lack of significant group differences in amygdala volumes, the association of amygdala volume abnormalities with ADHD and seizure frequency implies a possible impact of the disorder on amygdala development and CAE-associated comorbidities, such as ADHD. [Copyright &y& Elsevier]

Abstract: 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. [Copyright &y& Elsevier]

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. [Copyright &y& Elsevier]

Discusses a study that examined psychopathology in a sample of complex partial seizures (CPS), primary generalized epilepsy and nonepileptic children using the Child Behavior Checklist (CBCL) and the Schedule for Affective Disorders and Schizophrenia (K-SADS). Demographics and seizure characteristics of the children; CBCL scores and K-SADS diagnoses; Clinical implications.