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The goal of this review is to discuss the comorbidities reported in specific epilepsy syndromes to examine possible underlying causes or associations and to present data on current therapies for these conditions. Comorbid conditions including cognitive impairment, neuropsychiatric problems, and social difficulties are common in children with epilepsy, and often more disabling than the seizures themselves. Biological factors associated with a greater risk of comorbidity in epilepsy include younger age at seizure onset, cognitive impairment, temporal or frontal lobe onset, and intractability. Social factors correlating with greater risk include lower socioeconomic status, lower parental education level, and poorer family function. These comorbid conditions not only have a significant impact on the child but also are a source of increased stress and burden for families. Increased awareness and early diagnosis of these conditions may affect therapeutic intervention and long-term outcome as well as assist in better understanding of potential risk factors and biological mechanisms. [ABSTRACT FROM AUTHOR]

We aimed to determine variation in treatment of newly diagnosed infantile spasms, focusing on details of adrenocorticotropic hormone (ACTH) administration using a Redcap questionnaire sent to members of the Child Neurology Society. Two hundred fifty-seven members responded. Eighty-four percent prescribers used ACTH to treat infantile spasms. Seventy-six percent always admit patients. There is no difference between prescriber type (epileptologist or other) and prescriber location (state-funded or non-state-funded hospital) for decision to admit. Electroencephalographic (EEG) confirmation of spasms and education for injection were the commonest reasons to admit. Only 45% of prescribers accurately estimated the cost of ACTH. Participants in the hospital vial program were significantly more likely to always admit patients for ACTH than those who did not participate in such a program (P = .02). Although having the hospital sample vial allows time to complete investigation of infantile spasms and eliminates delays in initiating ACTH, it adds significantly to the cost of therapy., (© The Author(s) 2015.)

Seizures in the newborn period are common and frequently indicate serious underlying brain injury. Although accumulating evidence suggests that they may impair brain development, there are currently no evidence-based guidelines for evaluation and management of neonatal seizures. In this review, we will address some of the current controversies facing child neurologists and neonatologists, including how to define, monitor, and treat neonatal seizures.

The feasibility and clinical utility of early electroencephalogram (within 48 hours) was studied in 127 children (age, 1 month-17 years) referred for a "first seizure." The electroencephalogram was considered late after 48 hours. Electroencephalogram abnormalities were classified as nonepileptiform or epileptiform. Children were classified as having an "epileptic" or "nonepileptic" event. An early electroencephalogram was obtained in 23 (18%). Late referral (n = 36), weekend event (n = 23), difficulty contacting families (n = 11), parental schedules (n = 9), and laboratory scheduling (n = 11) resulted in late electroencephalograms. All 94 children with an epileptic event had an electroencephalogram, 19 (20%) within 48 hours. Results were abnormal in 9 (47%) early (7 epileptiform, 2 nonepileptiform) and 35 (44%) late (30 epileptiform, 5 nonepileptiform). Increased abnormalities were not seen with early electroencephalography (P = .50). Early electroencephalograms may not be feasible in the pediatric population and did not show a higher yield of abnormalities.

Sixty-four children, aged 0-17 years, undergoing ambulatory electroencephalography (EEG) were prospectively recruited during a 12-month period. The diagnostic yield of ambulatory electroencephalography was determined for each of the following groups: group 1: differentiation of seizures from nonepileptic events; group 2: determination of seizure/interictal discharge frequency; and group 3: classification of seizure type or localization. The ambulatory electroencephalography answered the clinical question in 61% of group 1 (27/44) and 100% of groups 2 (16/16) and 3 (4/4). Of 44 cases in Group 1, clinical events were recorded in 61%; the ambulatory electroencephalography result changed the diagnosis from epileptic to nonepileptic or vice versa in 27%. When clinicians suspected that events were epileptic, ambulatory electroencephalography changed the clinical impression in 50%, whereas when events were suspected to be nonepileptic, ambulatory electroencephalography confirmed that impression in 83%.

The purpose of this study was to determine if reduction in spike frequency in children with benign epilepsy of childhood with centrotemporal spikes treated with sulthiame correlates with improved neuropsychologic function. Six untreated children (5 boys, 1 girl) with a mean (standard deviation) age of 9.1 years (1.5 years) underwent overnight ambulatory electroencephalograph recording and neuropsychologic evaluation at baseline and after 6 months of sulthiame monotherapy. The Reliable Change Index was used to determine whether a statistically reliable change in neuropsychologic function occurred. All children continued receiving sulthiame monotherapy (dose range, 8.0-9.3 mg/kg per day) for the study period without further seizures. Although spike frequency was reduced in all 6 children (3 showing a decrease to less than 10% of baseline), they all demonstrated significant deterioration in their reading ability, 5 had significant declines in general memory, and more than half had reduction in attention skills and mathematics ability. Although sulthiame was effective at seizure control and significantly reduced spike frequency in children, it was also associated with deterioration in cognitive function.

The objectives of this study were to determine if (1) children with migraine experience greater sleep disturbances than their siblings, (2) those with more severe migraine have greater levels of sleep disruption, and (3) these sleep disturbances lead to greater behavioral problems and more missed school. Children aged 6 to 18 years with a diagnosis of migraine for > 6 months, who had at least one sibling without migraine in the same age range, were identified through our neurology clinic database or at the time of the clinic visit. Parents completed the (1) demographic, general health, and migraine information questionnaire; (2) Child Sleep Habits Questionnaire; and (3) Behavior Assessment System for Children: Second Edition (BASC-2) Parent Rating Scales for each child. Cases with migraine had higher total sleep (P < .02), sleep delay (P < .03), and daytime sleepiness scores (P < .001) than controls. Cases with more severe migraines had higher total sleep (P < .01) and sleep duration scores (P < .03) than those with milder headaches. In cases, higher total sleep scores predicted greater behavior problems on all four composite scales on the Behavior Assessment System for Children: Second Edition (Externalizing Problems, P < .05; Internalizing Problems, P < .005; Behavior Systems Index, P < .003; and Adaptability Skills, P < .006). We conclude that children with migraine are prone to greater sleep and behavioral disturbances than children without headache. Sleep disorders should be routinely queried and appropriate advice on sleep hygiene provided.

Adolescent perception of physical and social impact of chronic illness was assessed to determine (1) if there is greater prejudice toward epilepsy than other chronic disease and (2) if adolescents with chronic disease have less prejudice toward similarly affected peers with all types of chronic disease or just their specific chronic disease. Cognitively normal teens aged 13 to 18 years without chronic disease (n = 41) and with epilepsy (n = 32), asthma (n = 38), diabetes (n = 21), and migraine (n = 17) were interviewed in the outpatient clinics of a tertiary care pediatric center regarding their perceptions of the physical and social impact of eight chronic diseases (epilepsy, asthma, diabetes, Down syndrome, arthritis, migraine, leukemia, human immunodeficiency virus [HIV] infection). Epilepsy was perceived to have a more adverse physical impact than all chronic illnesses except Down syndrome. The perception was that it more frequently caused mental handicap, injured the afflicted individual and bystanders, and led to death. Epilepsy was also perceived to have a more negative social impact, particularly on behavior, honesty, popularity, adeptness at sports, and fun. Significantly more adolescents expressed reluctance to befriend peers with epilepsy, both from their own and their perceived parental perspectives. Having a chronic disease did not generally alter the adolescents' perceptions of peers with chronic disease. However, cases with epilepsy ranked this disease to have less social impact than teens with other chronic diseases. In conclusion, adolescents consider epilepsy to have a greater physical and social impact than most chronic diseases. Educational efforts should focus on the "normality" of most persons with epilepsy and emphasize the low risk of injury when proper first aid is followed.

A structured interview of 84 families of children with epilepsy followed through the neurology clinic of a tertiary care children's hospital was conducted to assess the epilepsy-specific information sources accessed and the perceived accuracy of these sources. Families accessed a mean of 3.5 sources from or specifically recommended by the clinic or family doctor and 4.1 sources outside these areas. Families of children with intractable epilepsy and higher-educated parents, but not those of higher socioeconomic status, consulted more extensively. The perceived accuracy of information rated highest for clinic-recommended Internet sites (100%), the clinic nurse (97%), and the neurologist (93%). Sources external to the clinic had variable ratings; those with the greatest perceived accuracy included other Internet sites or family members within the medical profession (85% for both) and lay organizations (84%). Friends within the medical profession, other families, and complementary health care providers also ranked highly. Recommendation of sites and books by epilepsy clinics is more helpful than general handouts.

Acute necrotizing encephalopathy is a fulminant neurologic disease seen predominantly in Japan and Taiwan. We present two cases diagnosed at a Canadian center within the same year in Caucasian children. Both were previously well, developed an acute viral illness with fever and vomiting, and progressed to brain death within 2 to 4 days. Neuroimaging and postmortem examination demonstrated the unique features of bilateral and severe necrosis of deep gray- and subcortical white-matter structures. The first case was associated with extensive, but transient, hepatic involvement, recent varicella and rotavirus infections, and detailed metabolic studies, including mitochondrial functional analysis, were normal. The second case tested positive for influenza A infection, whereas evidence of liver damage was lacking. Both children demonstrated early lymphopenia and myocardial necrosis, two features not previously associated with acute necrotizing encephalopathy. These cases are unique in their occurrence in non-Japanese children and are among the first published reports in Canada.

The physical manifestations a seizure produces provide critical information. It is assumed that all generalized convulsions are ostensibly the same, regardless of whether they are primary or secondary generalized seizures. We undertook a pilot study to determine if the clinical phenomenology of secondary generalized seizures in children with epilepsy is different from classic descriptions of generalized tonic-clonic convulsions. A data capture sheet was created and applied to the video-electroencephalographic (EEG) records of 64 secondary generalized seizures from 13 children with intractable and/or refractory epilepsy. Many features of secondary generalized seizures were different from traditional descriptions of generalized convulsions. In 100% of cases, the mouth either remained open or repeatedly opened and closed rather than slamming shut. In 77% of cases, a variety of late motor activities were seen to occur after the seizure activity had ceased and the EEG record was quiet. The clinical features of a generalized convulsion in a child, especially mouth opening and late motor events, can be useful in establishing the origin as either focal or primary generalized.

The objective of this study was to determine if cognitively normal children with epilepsy have higher accidental injury rates than their age- and sex-matched friends without epilepsy and what factors may predict this. Patients 5 to 16 years old, with a developmental quotient >70, without major motor or sensory impairments, with a 1-year history of epilepsy and who either had a seizure or had been on antiepileptic drugs within the past year, were identified from the pediatric neurology database of the Royal University Hospital. Twenty-five of 31 cases and their best friend controls agreed to participate. Seizure-related factors including type, duration, frequency, timing, date of diagnosis, antiepileptic drug initiation and discontinuation, and specific types and total antiepileptic drugs used were assessed by interview. Questionnaires about accidental injury including type, number, severity, and, if applicable, injuries resulting from seizures, as well as general safety practices, activity restrictions, and presence of attention-deficit hyperactivity disorder, were completed by cases and controls. No significant differences in injury numbers (specific types or total) or severity were found, although a small number of epileptic children were very predisposed to injury. Seizure-related factors did not predict injury in cases. Safety practices were similar, and restrictions in cases were not excessive. Children with attention-deficit hyperactivity disorder had a higher injury rate, both in cases and controls. Cognitively normal children with epilepsy do not have a higher injury rate than their nonepileptic peers. If consciousness is impaired in seizures, extra supervision for swimming and bathing and restricted climbing heights are suggested. All other safety restrictions for epileptic children should follow those appropriate to nonepileptic children to allow a normal lifestyle.

The aims of this study were to determine predictors of abnormal outcome, neurodevelopmental deterioration, new-onset epilepsy, refractory epilepsy, and recurrent status epilepticus in children presenting with status epilepticus. For all children presenting to Royal University Hospital, Saskatoon, Saskatchewan, Canada, with status epilepticus between January 1987 and December 1996, demographic data, details of status epilepticus (etiology, duration, treatment, and investigations), developmental milestones, seizures prior to and following status epilepticus, recurrent status epilepticus, and neurologic examination findings at status epilepticus and at follow-up were collected by chart review, patient interview, and neurologic examination. Neurodevelopmental outcome was determined for all subjects except those who died during the initial hospitalization. Predictors of new-onset epilepsy, refractory epilepsy, and recurrent status epilepticus were determined for children followed for 3 months or more after status epilepticus. At follow-up, 79% were abnormal neurologically. Predictors included etiology (nonfebrile or nonidiopathic), perinatal difficulties, preceding developmental delay, abnormal initial neurologic examination; and abnormal neuroimaging. Thirty-four percent showed neurodevelopmental deterioration; predictors included etiology (nonidiopathic or nonfebrile), young age at status epilepticus (12 months or less), and abnormal neuroimaging. Thirty-six percent with no history of seizures preceding status epilepticus developed epilepsy and 25% developed refractory epilepsy. Fifty percent of children had recurrent status epilepticus. In conclusion, very few children presenting in status epilepticus were normal at follow-up. Sequelae were seen predominantly in those with a nonidiopathic, nonfebrile etiology, whereas those with idiopathic or febrile status epilepticus did well.

The objective of this study was to determine the frequency of atypical clinical and electrographic features in children with benign rolandic epilepsy. A retrospective case series design was employed in the setting of a tertiary care pediatric hospital. Forty-two children with benign rolandic epilepsy were seen through our neurology department between January 1, 1991, and December 31, 1993. Their charts were reviewed for atypical clinical features, imaging studies and results, total number of seizures at initial presentation and last follow-up, and use of anticonvulsants. Atypical clinical features included status epilepticus, developmental delay, daytime-only seizures, screaming as a seizure component, and postictal Todd's paresis. All children had at least one electroencephalogram, and these records were reviewed for atypical electrographic features such as unusual location, atypical spike morphology, and abnormal background. Atypical clinical features were seen in 50% of patients and atypical electrographic features in 31%. Computed tomographic scans were performed in 15 patients and were consistently normal. Treatment with anticonvulsant medication was initiated in 40%. Although patients with atypical features did not have an increased seizure frequency, they were more likely to undergo imaging studies (P < .01) and to be commenced on anticonvulsant medication (P < .02). Our experience suggests that atypical clinical and electrographic features are the rule rather than the exception in benign rolandic epilepsy. Further work must be done to develop a reliable definition of this common entity.