Search

Sleep-related movement disorders constitute a diverse group of diseases that include restless leg, nocturnal leg cramps, rhythmic movement disorder, bruxism, and nocturnal seizures, particularly frontal lobe epilepsy. These disorders may occur predominantly during sleep, but also occasionally during wakefulness (e.g., nocturnal leg cramps) or sleep-wake transition (e.g., rhythmic movement disorder). These conditions can lead to significant sleep onset insomnia, sleep disruption, and excessive daytime somnolence, with possible behavioral and cognitive disturbances consequence. Psychiatric disorders and medications used to control psychiatric symptoms can provoke or aggravate movement disorders during sleep. It is therefore essential when evaluating an individual with psychiatric disease to assess for these disorders and understand their pathophysiology and management.

Objective: Medial thalamic stroke in adults commonly results in severe learning and memory impairments and executive dysfunction, particularly during the acute phase. However, there is limited research on the cognitive recovery from thalamic stroke in physically healthy adolescents. This study aimed to fill this gap in the literature by utilizing a monozygotic twin control to investigate the neuropsychological outcomes of bilateral thalamic stroke in adolescence. Method: We evaluated an otherwise healthy 17-year-old male with a history of premature birth, developmental delay, and learning disability 2 and 7 months after he sustained a bilateral medial/anterior thalamic stroke of unknown etiology. His identical twin brother served as a case control. Results: The patient presented with improvements in many cognitive skills between assessments, most notably processing speed. Despite some mild improvement, however, he presented with significant deficits in fine motor speed/coordination, spatial perception, and rapid naming. Additionally, he exhibited persistent, severe deficits in verbal learning and memory. Relative sparing of executive functions (i.e., planning and set-shifting) and attention on standardized measures in this case may be explained by good underlying health, limited extra-thalamic damage, and/or recovery of function. The effects of thalamic injury resulted in minimal adaptive dysfunction or deterrence from academic or athletic success for the presented case. Conclusions: These results suggest risk for deficits in encoding of new verbal information following bilateral thalamic stroke in adolescence, as well as risk for persistent cognitive deficits despite initial improvements. This is consistent with descriptions of anterograde memory impairments in adults with similar lesions.

We aimed to study cost-effectiveness of seizure evaluation of children with epilepsy in the emergency department (ED). We reviewed epilepsy patients seen at our ED for 1 year. Age, laboratory and neuroimaging results, treatment, disposition, and usefulness of the visit (need for hospitalization, clinical improvement) were analyzed. We identified 330 patients, aged 23 days–21 years, 190 (57.5%) had blood tests, 45 (13.6%) urinalysis, 2 (0.6%) cerebrospinal fluid testing, and 44 neuroimaging studies (13.3%). Tests’ positive yield were 41%, 11%, 0%, and 4.5%, respectively. One-third of patients (n = 122) were treated with antiepileptic drugs. Other treatments were administered to 44 (13.3%). One hundred eighteen patients (35.7%) were admitted to our hospital, 208 (63%) discharged to home. Two hundred eight visits were useful (63%). One-third of visits did not provide useful patient care. Their visits were expensive and not very cost-effective. Investment in patient education could decrease unnecessary ED visits.

Brivaracetam is a new antiepileptic drug with limited data in children. The objective of this study was to assess the efficacy/tolerability of brivaracetam. This is a retrospective chart review of children/adolescents with refractory epilepsy treated with brivaracetam from 2016 to 2018. The primary outcome was seizure reduction (decrease in seizure frequency >50%). Twenty-three patients were identified. Mean age at initiation was 12.5 years. Fourteen were females. Epilepsy was focal in 11, generalized in 6, and mixed in 3. Average dose was 3.9 mg/kg/d. The mean duration of treatment was 8.2 months. Eight had greater than 50% decrease in seizure frequency, of which 7 had focal epilepsy, and 1 had Lennox-Gastaut/mixed epilepsy. Two had drowsiness and 3 behavioral complaints. One experienced tingling and dizziness. Our retrospective review suggests that brivaracetam is an effective therapy for refractory focal epilepsy in children older than 4 years of age.

Randomized controlled trials have shown that stimulants reduce symptoms of impulsivity and hyperactivity in children with attention deficit/hyperactivity disorder (ADHD); however, these rigid protocols show no advantage of one medication over another. Our study examined the question of differential efficacy and tolerability of five medications used for ADHD, in the open-label setting of our outpatient child neurology clinic. This retrospective study identified 137 children and adolescents (109 boys and 28 girls), with a mean age of 10 years (range 4 to 19 years) treated for ADHD. Treatment options were amphetamine/dextroamphetamine extended release (adderall XR) in 19.0%, amphetamine/dextroamphetamine (adderall), osmotic controlled-released (OROS) formulation of methylphenidate (OROS-MPH, concerta) in 29.2%, atomoxetine (strattera) in 21.9% and methylphenidate standard release (MPH) in 16.8%. Global effectiveness was assessed for each medication. Overall, 78% of patients improved with medication, with no significant statistical difference in efficacy among the five medications. Side effects included decreased appetite (14.6%), insomnia (10.2%), headaches (7.3%), and tics (3.7%). The only difference in side effects was with atomoxetine showing a significantly lower incidence of headaches than amphetamine/dextroamphetamine XR, amphetamine/dextroamphetamine or OROS-MPH. In conclusion, our results in the open-label setting were comparable to those found in randomized controlled trials; the medications we examined were equally effective with minimal differences in side effect profiles.

Background Multiple sulfatase deficiency is an autosomal recessive lysosomal storage disorder characterized by the absence of several sulfatases and resulting from mutations in the gene encoding the human C (alpha)-formylglycine-generating enzyme. There have been a variety of biochemical and clinical presentations reported in this disorder. Patient Description We present a 4-year-old girl with clinical findings of microcephaly, spondylolisthesis and neurological regression without ichthyosis, coarse facies, and organomegaly. Results The child's magnetic resonance imaging demonstrated confluent white matter abnormalities involving the periventricular and deep cerebral white matter with the U-fibers relatively spared. Biochemical testing showing low arylsulfatase A levels were initially thought to be consistent with a diagnosis of metachromatic leukodystrophy. The diagnosis of multiple sulfatase deficiency was pursued when genetic testing for metachromatic leukodystrophy was negative. Conclusion This child illustrates the clinical heterogeneity of multiple sulfatase deficiency and that this disorder can occur without the classic clinical features.

There is increasing evidence documenting activation of inflammatory processes in focal epilepsies. This review article summarizes current data regarding immune mediated inflammatory processes in patients with symptomatic partial epilepsies such as mesial temporal sclerosis, focal cortical dysplasia, and Rasmussen's encephalitis. We have also reviewed several neuronal surface antibody-associated syndromes, which have been recently described with focal seizures as an important part of clinical presentation, such as antibody-associated limbic encephalitis and N-methyl-D-aspartic acid receptor antibody syndrome. An autoimmune mechanism may be one pathogenic factor in some symptomatic epilepsies acting as a triggering event in the process leading to the development of epilepsy.

We describe a case of 5,10-methenyltetrahydrofolate synthetase (MTHFS) deficiency characterized by microcephaly, global developmental delay, epilepsy, and cerebral hypomyelination. Whole exome sequencing (WES) demonstrated homozygosity for the R74X mutation in the MTHFS gene. The patient had the unexpected finding of elevated cerebrospinal fluid (CSF) neopterin. The novel finding of macrocytic anemia in this patient may provide a clue to the diagnosis of this rare neurometabolic disorder. Keywords: 5,10-methenyltetrahydrofolate synthetase, MTHFS, Folate, Cerebral hypomyelination

Epilepsy is the most common neurologic disorder worldwide and is characterized by recurrent unprovoked seizures. The mitochondrial (mt) respiratory chain is the final common pathway for cellular energy production through the process of oxidative phosphorylation. As neurons are terminally differentiated cells that lack significant regenerative capacity and have a high energy demand, they are more vulnerable to mt dysfunction. Therefore, epileptic seizures have been well described in several diseases such as mt encephalomyopathy, lactic acidosis, and stroke-like episodes and myoclonic epilepsy and ragged red fibers, which are caused by gene mutations in mtDNA, among others. Mutations in nuclear DNA regulating mt function are also being described (eg, POLG gene mutation). The role of mitochondria (mt) in acquired epilepsies, which account for about 60% of all epilepsies, is equally important but less well understood. Oxidative stress is one of the possible mechanisms in the pathogenesis of epilepsy resulting from mt dysfunction gradually disrupting the intracellular Ca 2+ homeostasis, which modulates neuronal excitability and synaptic transmission, making neurons more vulnerable to additional stress, and leading to energy failure and neuronal loss in epilepsy. Antiepileptic drugs (AEDs) also affect mt function in several ways. There must be caution when treating epilepsy in patients with known mt disorders as some AEDs are toxic to the mt. This review summarizes our current knowledge of the effect of mt disorders on epilepsy, of epileptic seizures on mt, and of AEDs on mt function and the implications of all these interactions for the management of epilepsy in patients with or without mt disease.

Lacosamide is a US Food and Drug Administration (FDA)–approved antiepileptic drug for patients 17 years or older with partial epilepsy. There are sparse data on children. The objective of our study was to evaluate its efficacy/safety in children with refractory epilepsy. Forty children (mean age 14.3 years) were treated with lacosamide at our institution (adjunctive therapy in 36, monotherapy in 4). Fifteen patients had symptomatic focal epilepsy, 2 had cryptogenic focal epilepsy, 20 had symptomatic generalized epilepsy, and 3 had cryptogenic generalized epilepsy. Two had juvenile myoclonic epilepsy and 5 had Lennox-Gastaut syndrome. Forty-two percent had at least >50% reduction in seizure frequency, and 6 became seizure free. Average dose was 7 mg/kg/d and average follow-up was 9.2 months. Responders had a 76.5% mean decrease in seizures. Fifteen children experienced an adverse reaction and 7 discontinued lacosamide (4: Ineffective, I: insurance denial, 1: tremor, 1: behavior). Lacosamide is effective and well-tolerated in children with refractory epilepsy.

Interictal occipital epileptiform abnormalities have not been well characterized. The objective of this pilot study was to assess their significance in children.A search was performed on the EEG database for the keywords "occipital", "spike", "sharp wave" and "epileptiform". Patients were divided into two groups based on the absence of all (group 1) or presence of any (group 2) of the following criteria: mental retardation, cerebral palsy, neurological deficits, abnormal MRI and/or intractable epilepsy. Special attention was given to the spike/sharp wave amplitude/duration and background slowing.A total of 44 children (eight months to 15 years) were studied. Groups 1 and 2 were each composed of 22 children. Background slowing was more frequent in group 2 (10/22, 45%) compared to group 1 (1/22, 4.5%; p = 0.002). In group 2, 8/22 (36%) had spikes or sharp waves with amplitudes below 50 microV or above 150 microV with a positive predictive value of 89%, and a negative predictive value of 39%. Only 1/22 (4.5%) in group 1 had epileptiform activity outside of the 50-150 microV range.The presence of very high or low-amplitude occipital epileptiform abnormalities or background slowing may be indicative of encephalopathy.

This report describes a 9-year-old child with status epilepticus and cat scratch disease. This patient's focal seizures and electroencephalographic changes persisted for 18 months after status epilepticus. This patient represents the third reported case of persistent focal seizures or electroencephalographic changes after status epilepticus secondary to cat scratch disease. This finding suggests that cat scratch encephalopathy may be a cause of localization-related epilepsy, and should be investigated when evaluating a patient with new-onset partial seizures.

Levetiracetam is an antiepileptic drug approved for use by the US FDA as adjunctive therapy in partial-onset seizures in adults and children 4 years or older. It has also been approved as adjunctive treatment for adults and children 12 years and over with juvenile myoclonic epilepsy and for the treatment of primary generalized tonic–clonic seizures in adults and children over the age of 6 years with idiopathic generalized epilepsy. In Europe, in addition to these indications, it is also approved for use as monotherapy in the treatment of partial-onset seizures in patients from the age of 16 years with newly diagnosed epilepsy. It has a unique mechanism of action different to that of other antiepileptic drugs. The recent development of a parenteral intravenous formulation allows for its use when oral administration is not feasible. The most common serious side effects in children are behavioral. Its broad spectrum of action, lack of significant systemic side effects and the absence of interaction with other drugs makes it an attractive choice for use in children with epilepsy.

Intravenous levetiracetam recently became available for use in patients aged >16 years. There are few data about its safety and efficacy in children. We retrospectively analyzed data from children treated with intravenous levetiracetam. Ten patients (6 female, 4 male), aged 3 weeks to 19 years, were treated with intravenous levetiracetam at a mean dose of 50.5 mg/kg/day for a mean duration of 4.9 days. Four patients received intravenous levetiracetam for acute repetitive seizures/status epilepticus, and three as replacement for oral levetiracetam because administration of oral levetiracetam was temporarily infeasible. One patient each received intravenous levetiracetam for seizure prophylaxis during brain biopsy, as maintenance treatment after acute seizures, and as substitute for sodium valproate. Three of four patients with acute repetitive seizures/status epilepticus became seizure-free; the fourth patient had a partial reduction in seizure frequency. All three patients who received intravenous levetiracetam as substitute for oral levetiracetam tolerated the switch well. The other three patients were seizure-free on intravenous levetiracetam. No serious adverse effects were observed, and all patients completed treatment with intravenous levetiracetam for the intended period. Intravenous levetiracetam may be effective in various clinical situations requiring intravenous administration of an antiepileptic drug.

Objective: The purpose of this study was to determine the relationship between epilepsy and respiratory chain defects in children with mitochondrial encephalopathies (ME). Study Design: We conducted a retrospective review of the medical records of children referred for evaluation of an ME. Only patients assigned a definite diagnosis of ME using modified Walker criteria and with a respiratory chain defect were included. Clinical data pertaining to the ME and epilepsy type were collected. Mitochondria were isolated by subcellular fractionation from a vastus lateralis muscle biopsy and studies were performed using polarographic and spectroscopic techniques for the quantitative determination of NADH and cytochrome components of the respiratory chain. Results: A total of 38 children with ME were identified. Seizures were present in 61%. Sixteen of 23 children with epilepsy (70%) had refractory epilepsy associated with a progressive encephalopathy. Children with epilepsy had a significantly higher incidence of complex I defects than children without epilepsy (p

Summary Purpose: Exacerbation of epilepsy may occur following initiation of therapy with antiepileptic drugs (AEDs). The aim of this study is to analyze the clinical and EEG characteristics of a group of pediatric patients with worsening of seizures and/or EEG deterioration while on oxcarbazepine (OXC). Methods: A retrospective analysis of a clinical database was performed to identify patients with epilepsy treated with OXC over the past 3 years. History, neurological examination, and EEG findings were reviewed to identify any who had developed exacerbation of seizures or new abnormalities on EEG. Results: Of 290 patients on OXC, we identified 12 patients with new onset seizures, all with initial normal neurological exam and normal EEG, who developed either worsening of preexisting seizures, new seizure types, and/or EEG deterioration following introduction of OXC monotherapy. EEG changes were primarily characterized by new onset of generalized epileptiform activity not reported on the initial baseline EEG. Following substitution of OXC with a broad spectrum AED, significant improvement of seizure control and improvement in the EEG was observed. Conclusions: These findings suggest that OXC can aggravate seizures and/or worsen EEG features in children. Following initiation of therapy with OXC, monitoring of patients with follow-up EEGs may be important, especially in patients who do not show adequate response to therapy.

Although levetiracetam has shown efficacy in children with epilepsy, when used as adjunctive therapy, limited data are available regarding its use as monotherapy. The objective of this study is to evaluate the efficacy and tolerability of levetiracetam monotherapy in a cohort of pediatric patients with epilepsy. A retrospective analysis of pediatric epilepsy patients receiving levetiracetam at a single institution was performed over a 3-year period. Eighty-one patients were identified, 18 of whom received levetiracetam as monotherapy (mean age, 9.6 years). Epilepsy types were partial in 14 and generalized in 4. Conversion to levetiracetam monotherapy occurred in 16 patients due to lack of efficacy or adverse events, and 2 patients were initially started on monotherapy. Dose range of levetiracetam was 14-60 mg/kg, and duration of therapy ranged from 2-24 months. Eleven patients became seizure free on levetiracetam, one had at least 50% reduction in seizures, and six others had no change in seizure frequency. Adverse events included worsening of behavior, irritability, and possible cognitive changes, seen in 4 patients. Levetiracetam was discontinued in seven patients overall. Levetiracetam monotherapy appeared to be effective and well tolerated in this group of children with epilepsy and warrants further investigation in a well-controlled, prospective study.

Epilepsy, a common problem in child neurology, affects the entire family. There is a potential for such psychosocial consequences as parental chronic sorrow and alterations in coping. In this study, 67 parents completed brief questionnaires about their sorrow and coping styles. Results demonstrated chronic sorrow as measured by the Adapted Burke Questionnaire (10.45 +/- 7.9). Interestingly, the total score was not significantly different between parents of children with refractory and nonrefractory epilepsy or parents of children with comorbid or without comorbid conditions. Selection of the individual item disbelief, however, was significantly increased in parents of children with nonrefractory epilepsy, and selection of the item anger was significantly increased in parents of children with comorbid conditions. Parental coping styles were similar to those reported in the normative data for the instrument used, the Coping Health Inventory for Parents (CHIP). The correlation between chronic sorrow and coping was significant between the grief component of sorrow and Coping Pattern II of the CHIP. Implications for practice include earlier identification of parental feelings of sorrow and coping styles, which may contribute to a positive outcome.

Lacosamide is FDA-approved in patients 17 years or older with partial-onset epilepsy. We evaluated the efficacy and tolerability of lacosamide in children with refractory generalized epilepsy. We retrospectively reviewed records of 21 children with refractory generalized epilepsy treated with lacosamide in our institution from 2009–2013 divided into 2 subgroups- I, Lennox-Gastaut Syndrome, and II, other generalized epilepsies. Efficacy was defined as seizure freedom or ≥50% seizure reduction. Descriptive data analysis including seizure freedom was compared using c2 analysis. There were eleven females and ten males with a mean age, of 11.9 years. Five patients became seizure free, nine had ≥50% seizure reduction, and seven had no response. Group I: seven had ≥50% improvement, one did not respond. Group II: five became seizure free, two had ≥50% improvement, five had no response. Lacosamide is effective and well tolerated in children with refractory generalized epilepsy particularly patients with Lennox-Gastaut Syndrome.

This single-center analysis evaluated the efficacy of oxcarbazepine monotherapy in children and adolescents. A retrospective chart review identified 60 patients (male = 33, female = 27) aged 6 months to 17.8 years (mean age 8.2 ± 4.7 years) with partial onset epilepsy receiving oxcarbazepine monotherapy. The range of oxcarbazepine dose was 6-71 mg/kg/day (mean 26.3 ± 11.4 mg/kg/day). The duration of therapy ranged from 3 months to 8 years (mean duration 16.7 ± 14.3 months). Fifty-one patients (85%) achieved ≥50% reduction in seizure frequency, and 25 of 60 patients (42%) achieved seizure freedom. Ten patients (16.67%) reported adverse events including drowsiness, aggressive behavior, ataxia, dizziness, diplopia, and leg cramps. No hyponatremia or skin rash was observed. Twenty-four patients were switched from carbamazepine to oxcarbazepine monotherapy. In these patients carbamazepine was discontinued because of incidence of adverse events, poor seizure control, or both. Seventy-nine percent of patients switched from carbamazepine to oxcarbazepine monotherapy had ≥50% reduction in seizure frequency, and 37.5% became seizure-free. These findings suggest that oxcarbazepine monotherapy is effective and well tolerated in children and adolescents with partial epilepsy.

Oxcarbazepine is indicated for use as monotherapy or adjunctive therapy in the treatment of partial seizures in adults and children >or=4 years of age. The purpose of this retrospective chart review was to assess efficacy and tolerability of oxcarbazepine in children

Summary: Purpose: Benign partial epilepsy in infancy (BPEI) was first described by Watanabe in 1987. The aim of this study is to describe a series of infants from the United States to characterize this entity further. Methods: Among patients with the diagnosis of epilepsy followed up at our institution between 2002 and 2004, those satisfying the criteria for BPEI were included in a retrospective study. Results: Sixteen (10.2%) of 150 patients with new onset of epilepsy younger than 2 years were identified. The mean age at seizure onset was 8 months. Four (25%) infants had a family history of benign seizures. All infants were neurologically and developmentally normal at the onset of seizures. The seizures occurred in clusters in 75% of patients, predominantly in wakefulness. The initial manifestation was behavioral arrest with staring (69%) and apnea with cyanosis or pallor (37.5%). These symptoms were followed by deviation of eyes or head or both (56%), mild clonic movements (31%), or increased limb tone (35%). Secondary generalization was noticed in 37.5% of patients. All infants had normal interictal EEGs and brain MRIs. Ictal EEGs disclosed electrographic seizures in 50% of patients (temporal origin in 62% and central in 38%). Fifteen (94%) patients were treated with AEDs with good response. The mean duration of treatment was 12.4 months. The final developmental assessment of all patients was normal. Conclusions: We believe that BPEI exists as a unique entity and should be included in the differential diagnosis of epilepsies in infancy with partial origin.

Policies of administration, availability, and utility of ordering emergency electroencephalograms (EEGs) during nonbusiness hours vary widely among different EEG laboratories. In an attempt to explore further the importance of performing such emergency procedures in children, we analyzed the utility of not only emergency EEGs but also emergency long-term bedside EEGs and emergency video-EEGs at our institution in 1 year. The number of EEG studies performed in 1 year at our neurophysiology laboratory was 1821: 1212 routine EEGs, 387 24-hour ambulatory EEGs, 81 video-EEGs, and 141 long-term bedside EEGs. The number of emergency studies during the same period of time was 32 (1.8% of the total studies): 18 emergency EEGs, 8 emergency long-term bedside EEGs, and 6 emergency video-EEGs. The reasons for ordering the 18 emergency EEGs included the evaluation of (1) altered mental status ( n = 10), (2) paroxysmal movement (including cluster of seizures) ( n = 6), and (3) prolonged febrile or afebrile seizures prior to being discharged on a weekend ( n = 2). The eight emergency long-term bedside EEGs were done to evaluate (1) altered mental status ( n = 6) and (2) frequently occurring paroxysmal events ( n = 2). Four of the eight emergency long-term bedside EEGs were done after an abnormal emergency EEG. The six emergency video-EEGs were done to evaluate frequently occurring paroxysmal events ( n = 5) and altered mental status ( n = 1). Overall, emergency EEGs and emergency video-EEGs were useful in decision making in 30 of 32 (94%) studies. This might be related to the fact that a neurologist approved all of the studies. Appropriate strategies need to be developed to make this essential service available for patient care. ( J Child Neurol 2005;20:416—419).

Relapse of herpes simplex virus (HSV) encephalitis following acyclovir therapy has been reported infrequently in children beyond the neonatal period. The pathogenic mechanism of the recurrence is not fully understood. We report two new cases that support a mechanism of latent HSV infection with reactivation of the disease. Our patients were 2 years (#1) and 8 months (#2) old at initial infection. Both presented with fever, lethargy, focal seizures, and focal motor abnormalities. Serum HSV antibodies (Abs) were negative. The patients were treated with acyclovir for 14 and 21 days, respectively. They were readmitted at 1 month, and 4 days after discharge, respectively, with recurrent lethargy, seizures, and choreo-athetoid movements. Serum and CSF HSV Abs were significantly increased. CSF PCR was positive. In patient # 2 acyclovir-sensitive HSV was isolated from a brain biopsy. Both patients were re-treated with acyclovir, but progressed to a neurovegetative state. In our cases, latent HSV infection and reactivation is the most likely explanation for recurrent encephalitis. The immuno-pathogenic mechanisms of the infection recurrence are discussed. Based on the reported cases in the literature, patients younger than 2 years of age and with lower total dose of acyclovir treatments have a higher risk of recurrence.

Occurrence of hypsarrhythmia after the age of 3 years is rare. The objective of this study is to describe a group of patients who have persistence of hypsarrhythmia after the age of 3 years. The authors retrospectively reviewed the EEGs of 24 patients with hypsarrhythmia. Electroencephalographies (EEGs) were scored using a hypsarrhythmia scale. The clinical data of 7 patients with EEG scores greater than 9 at ages ≥3 years were analyzed. The mean age was 5.7 years (range, 3-8.7 years). EEG background amplitudes ranged from 200 to 500 µV in 5 patients and it was greater than 500 µV in the other 2. Six patients had electrodecremental responses. The etiology was developmental in 3 patients, mitochondrial disease in 2, and hypoxic ischemic encephalopathy in 2. Our study suggests that a subgroup of patients with hypsarrhythmia may not transition to a Lennox-Gastaut pattern or normalization after the age of 3 years.

Current American Clinical Neurophysiology Society guidelines require a minimum of 20 minutes of artifact-free EEG recording; however, the optimum duration for routine EEGs is not established. Our hypothesis was that an EEG recording of 40 minutes’ duration would yield more information than a 20-minute EEG in capturing epileptiform abnormalities and in obtaining sleep. We retrospectively studied 150 consecutive EEGs of 40 minutes’ duration performed at St Christopher’s Hospital for Children. Although the majority (89%) of interictal EEG abnormalities can be identified within the first 20 minutes of a routine EEG, extending the time of a routine EEG increases the yield significantly by identifying an additional 11% of abnormal studies ( P = .0001), precluding the need for further long-term monitoring in these patients. Forty-three percent of interictal epileptiform abnormalities were found during sleep. We recommend that routine EEGs be performed for 40 minutes, whenever possible, to improve yield in a cost-effective manner.

A 19-month-old, white, Pennsylvanian boy, with an unremarkable medical history, presented to our hospital with a 3-week history of nonbloody, nonbilious emesis up to 5 times a day and nonbloody diarrhea. Ten days before admission, his gait became progressively unsteady, until he finally refused to walk. A day before admission, he found it difficult to move his eyes. The patient was hypoactive. History, physical and neurologic examination, blood and cerebrospinal (CSF) fluid studies, and neuroimaging studies ruled out the most frequent causes of acute ataxia. The etiology of bilateral, complete ophthalmoplegia was also taken into consideration. Magnetic resonance imaging (MRI) findings of bilateral thalami and mammillary bodies provided diagnostic clues. Additional history and specific tests established the final diagnosis and treatment plan. The patient improved to a normal neurologic state. This case provides important practical information about an unusual malnutrition cause of acute ataxia, particularly in young children of developing countries.

A 7-year-old child presented with atypical absence epilepsy. He also had autism and severe cognitive deficit. As part of his diagnostic workup, a chromosomal microarray analysis was performed, which showed novel biallelic deletions in the neurexin 1 gene (NRXN1). His fraternal twin sister, who also had autism and cognitive impairment, was subsequently found to have the same biallelic deletions. Deletions included a 272-282kb loss at band 2p16.3 in one allele and a smaller 135-174-kb loss on the second allele. Neurexin 1 (NRXN1) is a cell adhesion protein, forming a synaptic complex with neuroligin. This signals a pathway that is critical for activity-dependent synaptic transmission. Mutations in this gene have been associated with autism and neurodevelopmental delay. Although there are many reports of heterozygous mutations with variable expressivity, only 3 cases with biallelic NRXN1 mutations have been previously reported, all of which have a more severe phenotype. We report 2 siblings with biallelic deletions, both of which affect the promoter region and exons 1-5 in the α-NRXN1 isoform, which has a role in the Ca(2+)-dependent release of neurotransmitters in the central nervous system. Our cases expand the phenotype of biallelic α NRXN 1 mutations and emphasize the important role of NRXN1 in autism and intellectual disability. Chromosomal microarray analysis should be the clinical standard in all specialties for first-tier genetic testing in autistic spectrum disorders.

2q37 microdeletion syndrome is a rare syndrome characterized by neurodevelopmental delay, bone, cardiovascular, and neurological alterations. This syndrome is typically associated with loss of genetic material of approximately 100 genes in the 2q37 band. However, the genes associated with neurodevelopmental phenotype in this syndrome are still unknown. We identified a deleted region of 496 kb by whole genome array CGH in a patient who fulfilled criteria for 2q37 microdeletion syndrome with developmental delay, microcephaly, hypoplasia of the corpus callosum, hand wringing, toe walking, and seizures. The deleted segment contains genes that are highly expressed in the developing human cortical plate and the subventricular zone (SVZ) in vivo and human neural progenitors in vitro, including SEPT2, THAP4, ATG4B, PPP1R7, and STK25. Network analysis revealed that STK25 was the most interacting gene associated with neural development in this deletion. Our report narrows the likely causative genomic region for microcephaly and neurodevelopmental delay in 2q37 microdeletion syndrome to a small genomic region enriched with neural progenitor genes that may represent an important locus for the development of the human cortex and corpus callosum. © 2015 Wiley Periodicals, Inc.

Myoclonus is a brief, rapid, involuntary muscle jerk originating in the central nervous system that can be physiological or a symptom of disease. We report a group of five children with excessive myoclonic jerks, only during sleep, and abnormal EEG during the events. Although only one third of the events had EEG epileptiform correlate, the presence of myoclonus without epileptiform EEG correlate has been described in patients with benign myoclonic epilepsy of infancy. We hypothesize that these findings may represent a variant of benign myoclonic epilepsy of infancy.

The Publisher regrets that this article is an accidental duplication of an article that has already been published, http://dx.doi.org/10.1016/j.spen.2014.04.015. The duplicate article has therefore been withdrawn. The full Elsevier Policy on Article Withdrawal can be found at http://www.elsevier.com/locate/withdrawalpolicy.

The interpretation of QT interval is often neglected during electroencephalography (EEG) reading. We compared the incidence of prolonged QT interval, as seen in the electrocardiography (ECG) recording lead of the EEG, in children presenting with seizure, syncope, or attention-deficit hyperactivity disorder (ADHD). Abnormal QT was defined as >460 ms. The incidence of prolonged QT in the seizure, syncope, and ADHD groups was 1/50 (2%), 7/50 (14%), and 2/50 (4%), respectively (P = .036, chi-square). The mean ± SD of QT were 405 ± 34, 424 ± 39, and 414 ± 36, respectively (P = .035, analysis of variance [ANOVA], syncope group, compared with seizure group). The incidence of prolonged QT as measured in the EEG was unexpectedly high in children presenting with seizure, syncope, or ADHD. These data support the concept that QT evaluation should be emphasized during routine EEG reading, as it may aid in identifying cases of undiagnosed cardiac conduction abnormalities. Prospective studies comparing EEG-ECG tracings with 12-lead ECG are warranted.

Thirty-two children with refractory partial epilepsy received open-label gabapentin as an additional medication to their antiepileptic drug regimen. Gabapentin was given in a dose ranging from 10 to 50 mg/kg per day (mean dose, 26.7 mg/kg daily). All patients had partial seizures with or without secondary generalization. Compared with baseline, 11 patients (34.4%) had a greater than 50% decrease in seizure frequency, and 4 (12.5%) had a 25% to 50% decrease in seizure frequency. Of the seven children who received the medication for 6 months or longer, two were seizure free and four were almost seizure free (having one seizure every few months). Mean gabapentin concentration was 4.8 micrograms/ml, and mean apparent clearance was 372 ml/kg per hour. The major reported side effects were behavioral. These consisted of hyperactivity, irritability, and agitation that occurred in patients with baseline mental retardation with attention deficit. We conclude that gabapentin can be a useful adjunctive medication in the treatment of refractory partial epilepsy in children.

Vertebral artery dissection is an uncommon cause of stroke in children. Accuracy of diagnosis by magnetic resonance angiography (MRA) instead of invasive transfemoral angiography (TFA) has been controversial. The need for anticoagulation and duration of such therapy is also arguable. We report 2 boys with vertebral artery dissection: one, aged 7 years, presented with hemiparesis and seizures and the other, aged 4 years, presented with ataxia. Each boy's initial MRA was not interpreted as delineating occlusive lesions to explain the posterior circulation infarcts visualized on computed tomography and magnetic resonance imaging scans. However, subsequent MRAs were suspicious for vertebral artery dissection, which was confirmed by TFA. Both children were treated with anticoagulation therapy. The first patient continued to manifest evidence of new infarcts despite treatment (initially with aspirin alone, followed by anticoagulation with heparin and warfarin), and is now maintained on a combination of high dose warfarin and aspirin. The second patient is now maintained on aspirin alone after initial anticoagulation for 6 months with heparin followed by warfarin. A high index of suspicion for vertebral artery dissection may allow diagnosis on the basis of MRA alone. Previous reports have indicated good outcomes of vertebral artery dissection in children and adults irrespective of anticoagulation treatment. Our experience suggests that anticoagulation may be beneficial in preventing further strokes caused by the dissection.

Childhood absence epilepsy (CAE) typically starts between four and seven years of age. Onset before three years is rare and has not been previously reported from North America. We retrospectively reviewed the electroencephalography laboratory database and paediatric neurology clinic records (from January 2000 to June 2009) at our institution in order to identify patients with absence seizures beginning before age three. Information was collected for age, gender, neurodevelopment, antiepileptic drugs (AEDs) used, seizure control, follow-up, and side effects. Of 12 patients identified, mean age at onset was 20.5 months (range: 11 months to two years; follow-up: six months to 11 years). Seven of 12 patients had normal neurodevelopment and five had speech delay. Four patients were seizure-free without AEDs, three were seizure-free with a single AED, and five still had seizures with multiple AEDs. Three patients had recurrences after medication withdrawal. Other previously published series have identified better seizure control than that reported here, however, 16% of the 130 patients so far documented are reported to have poorly controlled epilepsy, indicating that early-onset CAE is not a homogeneous condition. The debate as to whether early-onset CAE is a distinct epilepsy syndrome therefore continues. We believe that early-onset CAE may be a distinct epilepsy syndrome, with some features that overlap with those of typical CAE, as well as unique distinguishing features. Large prospective multicentric studies would be necessary to definitely resolve this matter.

Measurements of brainstem auditory evoked potentials (BAEP) and middle latency auditory evoked potentials (MLAEP) are readily available neurophysiologic assessments. The generators for BAEP are believed to involve the structures of cochlear nerve, cochlear nucleus, superior olive complex, dorsal and rostral pons, and lateral lemniscus. The generators for MLAEP are assumed to be located in the subcortical area and auditory cortex. BAEP are commonly used in evaluating children with autistic and hearing disorders. However, measurement of MLAEP is rarely performed in young children. To explore the feasibility of this procedure in young children, we retrospectively reviewed our neurophysiology databank and charts for a 3-year period to identify subjects who had both BAEP and MLAEP performed. Subjects with known or identifiable central nervous system abnormalities from the history, neurologic examination and neuroimaging studies were excluded. This cohort of 93 children up to 3 years of age was divided into 10 groups based on the age at testing (upper limits of: 1 week; 1, 2, 4, 6, 8, 10 and 12 months; 2 years; and 3 years of age). Evolution of peak latency, interpeak latency and amplitude of waveforms in BAEP and MLAEP were demonstrated. We concluded that measurement of BAEP and MLAEP is feasible in children, as early as the first few months of life. The combination of both MLAEP and BAEP may increase the diagnostic sensitivity of neurophysiologic assessment of the integrity or functional status of both the peripheral (acoustic nerve) and the central (brainstem, subcortical and cortical) auditory conduction systems in young children with developmental speech and language disorders.

Making a diagnosis of mitochondrial disease (MD) is extremely challenging and often employs the analysis of respiratory complex (RC) activities in biopsied skeletal muscle. Given both the invasive nature and expense of biopsied-muscle based testing for mitochondrial defects, buccal swab enzyme analysis has been explored as an alternative approach to the more invasive muscle biopsy. Case studies have recently suggested that buccal swabs from patients can be used to accurately assess mitochondrial enzyme activities including RC I and RC IV using a dipstick methodology combined with spectrophotometric analysis. In this study, forty patients with suspected MD who have previously been found to have significant defects in either RC I or RC IV in skeletal muscle were assessed by buccal swab analysis and compared to enzyme values obtained with unaffected controls (n=106) in the same age range. Buccal citrate synthase was used as an indicator of overall mitochondrial content, correlating well with overall buccal mitochondrial frataxin levels and was found to be elevated above control levels in 28% of the patients in this cohort. Of 26 cases with significant muscle RC I deficiency, 20 displayed significantly reduced levels of buccal RC I activity. All 7 of the patients with muscle RC IV deficiency showed significant buccal RC IV defect and 6 of the 7 patients with combined defects in muscle RC I and IV activity levels also exhibited analogous deficiencies in both buccal RC I and RC IV activities. In conclusion, the relatively high correlation (over 82%) of buccal and muscle RC deficiencies further supports the validity of this non-invasive approach as a potentially useful tool in the diagnosis of MD.

Long QT syndrome can present with neurological manifestations, including syncope and seizure-like activity. These patients often receive an initial neurologic evaluation, including electroencephalography (EEG). Our previous retrospective study suggested an increased prevalence of prolonged corrected QT interval (QTc) measured during the EEG of patients with syncope. The aim of the current study is to assess the accuracy of the EEG QTc reading compared with the nonsimultaneous 12-lead electrocardiography (ECG) in children with syncope. Abnormal QTc was defined as ≥450 ms in boys, ≥460 ms in girls. Forty-two children were included. There was no significant correlation between QTc readings in the EEG and ECG. EEG failed to identify 2 children with prolonged QTc in the ECG and overestimated the QTc in 3 children with normal QTc in the ECG. This study suggests that interpretation of the QTc segment during an EEG is limited. Further studies with simultaneous EEG and 12-lead ECG are warranted.

Subacute sclerosing panencephalitis (SSPE) typically presents with progressive mental deterioration, behavioral changes, and myoclonic jerks. Atypical presentations are not unknown and may result in diagnostic delays. A 9-year-old girl presented with poor balance and ataxia following an episode of upper respiratory tract infection. Neurological examination revealed mild hemiparesis and ataxia. Brain magnetic resonance imaging revealed scattered areas of T2 and fluid-attenuated inversion recovery hyperintensities in the white matter consistent with acute disseminated encephalomyelitis. Despite treatment with intravenous methylprednisolone, intravenous immunoglobulins, and plasmapheresis, progressive neurological worsening occurred. Later during the course of her illness, subacute sclerosing panencephalitis was suspected from the appearance of burst-suppression pattern on electroencephalogram, and the diagnosis confirmed by elevated titers of measles antibodies in cerebrospinal fluid. Physicians taking care of children need to be aware of atypical presentations of subacute sclerosing panencephalitis and must have a high index of suspicion to prevent diagnostic delays and avoid unnecessary diagnostic and therapeutic interventions.

About 5-10% of school-age children manifest migraine headaches. Treatment options for pediatric migraine are limited. Topiramate is approved for migraine prophylaxis in adults, but its use in children is limited. We retrospectively reviewed the records of 37 patients, i.e., 22 (60%) girls and 15 (40%) boys (mean age, 14 years; range, 7.3-20.5 years), diagnosed with migraine without aura in 30 (81%), with aura in four (11%), and abdominal, ophthalmoplegic, and catamenial in one each. The mean follow-up was 12 +/- 5 months standard deviation (S.D.). Clinical response was qualified as excellent, good, no change, or worse. Numbers of headaches per month were 15 +/- 7 S.D. prior to treatment and 3 +/- 3.4 S.D. (P0.001) after treatment. An excellent or good response (50% migraine reduction) was attained in 28 patients (76%). Ten (27%) patients exhibited adverse effects. Patients taking2 mg/kg/day were more likely to demonstrate side effects. The mean dose for patients without adverse effects was 1.27 +/- 0.7 mg/kg/day S.D. Those who reported adverse effects were taking a mean dose of 2.8 +/- 1.5 mg/kg/day S.D. This study demonstrated that topiramate is an effective, safe alternative for the prophylaxis of pediatric migraine. An acceptable risk/benefit maintenance dose wasor =2 mg/kg/day.

The study objective was to describe polysomnographic findings in children with attention deficit hyperactivity disorder (ADHD) with diverse sleep problems. Polysomnographic data were retrospectively analyzed for 33 children (age 3-16 years) with ADHD who had sleep studies performed for diverse sleep complaints. Eight patients (24%) had obstructive sleep apnea, 10 (30%) had periodic limb movements of sleep, 8 (24%) had upper airway resistance syndrome, and 5 (15%) had obstructive hypoventilation. The ADHD group showed decreased sleep efficiency, increased arousal index, increased wake after sleep onset, decreased oxygen saturation nadir, and increased snoring, compared with control subjects. Compared with ADHD children without sleep disordered breathing, those who had sleep disordered breathing were significantly more obese and had more sleep architectural abnormalities (including increased sleep latency, increased rapid eye movement latency, increased wake after sleep onset, and increased arousal index with more oxygen desaturations), although total sleep time and sleep efficiency were not significantly different. Sleep disordered breathing and periodic limb movements of sleep appear to be common among children with ADHD who have symptoms of disturbed sleep.

This study sought to evaluate polysomnographic abnormalities in a cohort of 40 children with epilepsy who underwent a sleep study because of various sleep complaints. Retrospective analyses included polysomnographic variables, antiepileptic drugs, type of epilepsy, and seizure control. The subgroup with epilepsy and obstructive sleep apnea syndrome was compared with 11 children who manifested uncomplicated obstructive sleep apnea syndrome. Thirty-three patients (83%) exhibited snoring (42.5%), sleep-disordered breathing (obstructive hypoventilation, 12.5%; obstructive sleep apnea, 20%; and upper-airway resistance syndrome, 7.5%), or periodic limb movements of sleep (10%). Children with poor seizure control demonstrated significantly lower sleep efficiency, a higher arousal index, and a higher percentage of rapid-eye-movement sleep compared with children who were seizure-free or exhibited good seizure control. Patients with epilepsy and obstructive sleep apnea had significantly a higher body mass index, longer sleep latency, a higher arousal index, and a lower apnea-hypopnea index, but significantly more severe desaturation compared with patients with uncomplicated obstructive sleep apnea. A significant proportion of children with epilepsy referred for polysomnography with diverse sleep problems manifest sleep-disordered breathing, including obstructive sleep apnea syndrome.

Ocular compression (OC) is a maneuver performed during EEG to demonstrate increased vagal reactivity in children with suspected syncope including breath-holding spells. We examined the relationship between the simulated OC pressure exerted by different physicians and the cardiac slowing responses that they had historically obtained as per EEG records. Simulated OC was performed by each physician using a sphygmomanometer. EEGs were reviewed for the rate of positive cardiac slowing per physician. Among three physicians who performed a total of 73 OC, the mean +/- SD of applied pressure were 29.0 +/- 2.4, 60.7 +/- 3.5 and 42.4 +/- 2.5 mmHg, respectively. There was good intra-physician consistency for the OC pressures exerted. The mean pressure exerted was significantly different between physicians (p0.001, ANOVA). The positive response rate for cardiac slowing among these physicians was 11/37 (29.7%), 10/21 (47.6%) and 8/15 (53.3%) respectively. The difference in positive OC responses between physicians was not significant (p = 0.127, chi-square). Higher OC pressures did not translate into more positive responses. A pressure of 30 mmHg is as good as 60 mmHg in demonstrating cardiac slowing during OC.

Lamotrigine (LTG) has shown to confer broad-spectrum, well-tolerated control of epilepsy. Monotherapy is preferable over polytherapy because of better compliance, fewer adverse events, less interactions, lower teratogenicity and lower cost. The aim of this study is to evaluate the efficacy and safety of LTG monotherapy on seizure control in a cohort of children and adolescents with epilepsy. We retrospectively reviewed the records of children and adolescents treated with LTG monotherapy at our institution between 2001 and 2006. Data collected included demographics, seizure type, etiology of seizures, age at onset of seizures and at initiation of LTG treatment, number of antiepileptic drugs (AEDs) prior to LTG, dose of LTG, length of follow-up, treatment response, and adverse events. Seventy-two children and adolescents were identified (mean age 12.1 years); 37.5% had mental retardation. Age at onset of epilepsy was 5.7 years (0-16). Twenty three percent had symptomatic focal epilepsy, 15.5% idiopathic focal epilepsy, 19.4% symptomatic generalized epilepsy and 41.6% idiopathic generalized epilepsy. LTG was used as first-line monotherapy in 26.4% of patients and as a second-line monotherapy in 73.6%. Age at initiation of LTG therapy was 10 years (2.8-19). Mean number of AEDs tried prior to LTG was 1.3 (0-6). Mean dose of LTG was 5.5mg/kg/day (1.1-13.7). Mean follow-up period was 33 months (3 weeks to 11.5 years). The degree of seizure reduction was as follows: seizure free in 42%, 75-90% reduction in 17.4%, 50-74% in 11.6%, 25-49% in 10%. Sixteen percent had no change in seizure control and 3% became worse. The most common adverse event was rash (6.9%). Six (8.3%) patients discontinued LTG because of the adverse events. No patient had Stevens-Johnson syndrome. In conclusion, LTG was effective and well-tolerated as monotherapy in children and adolescents for both focal and generalized epilepsies.

Coffin-Lowry syndrome is a well-defined clinical entity classically associated with moderate to severe mental retardation, characteristic facial features, skeletal deformities, and tapering fingers. A characteristic paroxysmal disorder was described in up to 10% patients with Coffin-Lowry syndrome, characterized by sudden loss of muscle tone induced by unexpected tactile or auditory stimuli. These events were given several names, including cataplexy, nonepileptic collapses with atonia, exaggerated startle responses, hyperekplexia, and stimulus-induced drop episodes. Various therapies were undertaken for these drop attacks, including clonazepam, tiagabine, felbamate, selective serotonin reuptake inhibitors, and tricyclics, with variable improvement. We report on a 22-year-old man with Coffin-Lowry syndrome with stimulus-induced drop episodes, who failed therapy with clonazepam, several antiepileptic drugs, and escitalopram, and who was given a trial of sodium oxybate with complete resolution of the drop attacks.

Vagus nerve stimulation (VNS) is approved for use in patients with refractory epilepsy over the age of 12 years. While this procedure is widely used, there is little data on adverse events in young children.A retrospective chart review was conducted on 26 children who had VNS implantation for refractory epilepsy from 1998 to 2004.Ages ranged from 3 to 17 years (16 boys and 10 girls). Seventy-seven percent had moderate to severe mental retardation. Sixty-five percent had more than 30 seizures per month. Symptomatic-generalized epilepsy was the predominant epilepsy syndrome seen in 77% of children. The duration of VNS treatment ranged from 1 month to 8 years (mean = 3.5 years). Twenty of 26 patients (77%) were on rapid-cycling mode. More than 50% reduction in seizure frequency was noted in 54% with two patients achieving seizure freedom. Twenty-three percent had less than 50% seizure reduction. Four patients were able to terminate seizures with use of the magnet. VNS was removed from one patient because of intractable cough persisting in spite of stimulation being turned off for 1 month. Another patient had it removed twice for infection. Obstructive sleep apnea (OSA) was observed in four patients (15%) after placement of VNS.VNS appears to be an effective treatment for children with refractory epilepsy. Development of intractable cough in one patient in spite of device being turned off and recurrent infection-related removal in another are unusual complications. Polysomnography before implantation of VNS should be considered to identify patients with pre-existing OSA.

Damage or loss of inhibitory cortical γ-aminobutyric acid (GABA)ergic interneurons is associated with impaired inhibitory control of neocortical pyramidal cells, leading to hyperexcitability and epileptogenesis. The calcium binding proteins parvalbumin and calbindin-D28k are expressed in subpopulations of GABAergic local circuit neurons in the neocortex and can serve as neuronotypic markers. Parvalbumin and calbindin-D28k facilitate the neuron's ability to sustain firing and provide neuroprotection. The goal of this study was to assess the hitherto unknown status of inhibitory interneurons in cortical tubers of human tuberous sclerosis complex. Surgically excised cortical tubers from three patients with tuberous sclerosis complex were evaluated immunohistochemically with antibodies to parvalbumin and calbindin-D28k. Cortical specimens from young patients with intractable seizures, including microdysgenesis (n = 3), postischemic cortical scarring (n = 1), porencephaly (n = 1), postictal gliosis (n = 3), and low-grade neuronal or glial tumors (n = 5), were also examined for comparison. In cortical tubers, calcium binding protein immunoreactivities (calbindin-D28k > parvalbumin) were present in medium or large-size dysplastic neurons, whereas giant or ballooned cells were parvalbumin or calbindin-D28k negative. In microdysgenesis, a nearly normal number of parvalbumin-positive neurons and a decreased number of calbindin-D28k-positive neurons were present. In peritumoral but more so in gliotic cortex, a coordinate decrease of parvalbumin and calbindin-D28k immunoreactivities was present. Our findings indicate that the expression of parvalbumin or calbindin-D28k by subpopulations of dysplastic neurons in cortical tubers is aberrant and denotes dysfunctional inhibitory circuits inept for excitoprotection.

Episodes of syncope or breath-holding spells are often misdiagnosed as epileptic events. The purpose of this study was to assess the usefulness of an electroencephalogram (EEG) with ocular compression to distinguish breath-holding spells and syncope from epileptic seizures. A retrospective analysis was performed on the EEG records of all children on whom ocular compression was performed from 2000 to 2003. Data from 116 patients with a clinical diagnosis consistent with either syncope or breath-holding spells were compared with a group of 46 patients with epilepsy. The RR interval during ocular compression was significantly higher in syncope patients compared with patients with epilepsy (P < .005). Using 2 seconds of asystole as the cutoff, the sensitivity of ocular compression was 26%, with 100% specificity. The change in RR interval from baseline to ocular compression also distinguished patients with breathholding spells and syncope from patients with epilepsy. Even a small increase of 0.5 seconds in the RR interval demonstrated a sensitivity of 46%, with a specificity of 98%. Ocular compression performed during an EEG is useful in distinguishing patients with breath-holding spells and syncope from those with epileptic seizures. A requirement of a 2-second period of asystole with ocular compression excludes many patients. Our data indicate that an RR interval increase of 0.5 seconds over baseline identifies additional patients with increased vagal tone. Prompt and accurate diagnosis of the etiology of loss of consciousness might preclude the need for further extensive and expensive evaluation and reduce patient and parental distress. (J Child Neurol 2006;21:907—910; DOI 10.2310/7010.2006.00209).