Assessment of MR blood-oxygen-level-dependent (BOLD) cerebrovascular reactivity under general anesthesia in children with moyamoya.

BACKGROUND: Moyamoya is a progressive, non-atherosclerotic cerebral arteriopathy that may present in childhood and currently has no cure. Early diagnosis is critical to prevent a lifelong risk of neurological morbidity. Blood-oxygen-level-dependent (BOLD) MRI cerebrovascular reactivity (CVR) imaging provides a non-invasive, in vivo measure of autoregulatory capacity and cerebrovascular reserve. However, non-compliant or younger children require general anesthesia to achieve BOLD-CVR imaging. OBJECTIVE: To determine the same-day repeatability of BOLD-CVR imaging under general anesthesia in children with moyamoya. MATERIALS AND METHODS: Twenty-eight examination pairs were included (mean patient age = 7.3 ± 4.0 years). Positive and negatively reacting voxels were averaged over signals and counted over brain tissue and vascular territory. The intraclass correlation coefficient (ICC), Wilcoxon signed-rank test, and Bland-Altman plots were used to assess the variability between the scans. RESULTS: There was excellent-to-good (≥ 0.59) within-day repeatability in 18 out of 28 paired studies (64.3%). Wilcoxon signed-rank tests demonstrated no significant difference in the grey and white matter CVR estimates, between repeat scans (all p-values > 0.05). Bland-Altman plots of differences in mean magnitude of positive and negative and fractional positive and negative CVR estimates illustrated a reasonable degree of agreement between repeat scans and no systematic bias. CONCLUSION: BOLD-CVR imaging provides repeatable assessment of cerebrovascular reserve in children with moyamoya imaged under general anesthesia.

PANDAS/PANS in childhood: Controversies and evidence.

Since first defined in 1998, paediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) and its later, broader iteration, paediatric acute-onset neuropsychiatric syndrome (PANS), have garnered significant attention and controversy. The role of streptococcal infection in children with explosive onset obsessive-compulsive disorder and new onset tics, the natural history of this entity, and the role of symptomatic and disease-modifying therapies, including antibiotics, immunotherapy, and psychoactive drugs, are all issues that have yet to be definitively addressed. While definitive proof of the autoimmune hypothesis of PANDAS is lacking, given the heightened attention to this entity and apparent rise in use of this diagnostic category, addressing questions around diagnosis, treatment, and etiology is imperative. In this paper, we review current working definitions of PANDAS/PANS, discuss published evidence for interventions related to this entity, and propose a clinical approach to children presenting with acute symptoms satisfying criteria for PANDAS/PANS.

Measurement of cerebrovascular reactivity in pediatric patients with cerebral vasculopathy using blood oxygen level-dependent MRI.

BACKGROUND AND PURPOSE: Cerebrovascular reactivity (CVR) is an indicator of cerebral hemodynamics. In adults with cerebrovascular disease, impaired CVR has been shown to be associated with an increased risk of stroke. In children, however, CVR studies are not common. This may be due to the difficulties and risks associated with current CVR study methodologies. We have previously described the application of precise control of end-tidal carbon dioxide partial pressure for CVR studies in adults. Our aim is to report initial observations of CVR studies that were performed as part of a larger observational study regarding investigations in pediatric patients with cerebral vascular disease. METHODS: Thirteen patients between the ages of 10 and 16 years (10 with a diagnosis of Moyamoya vasculopathy and 3 with confirmed, or suspected, intracranial vascular stenosis) underwent angiography, MRI, and functional blood oxygen level-dependent MRI mapping of CVR to hypercapnia. The results of the CVR study were then related to both the structural imaging and clinical status. RESULTS: Sixteen blood oxygen level-dependent MRI CVR studies were performed successfully in 13 consecutive patients. Twelve of the 13 patients with angiographic abnormalities also had CVR deficits in the corresponding downstream vascular territories. CVR deficits were also seen in 8 of 9 symptomatic patients and 2 of the asymptomatic patients. Notably, in patients with abnormalities on angiography, the reductions in CVR extended beyond the ischemic lesions identified with MR structural imaging into normal-appearing brain parenchyma. CONCLUSIONS: This is the first case series reporting blood oxygen level-dependent MRI CVR in children with cerebrovascular disease. CVR studies performed so far provide information regarding hemodynamic compromise, which complements traditional clinical assessment and structural imaging.

Spatiotemporal patterns of oscillatory brain activity during auditory word recognition in children: a synthetic aperture magnetometry study.

OBJECTIVE: We studied the task-induced spatiotemporal evolution and characteristics of cortical neural oscillations in children during an auditory word recognition task. METHODS: We presented abstract nouns binaurally and recorded the MEG response in eight healthy right-handed children (6-12 years). We calculated the event-related changes in cortical oscillations using a beamformer spatial filter analysis technique (SAM), then transformed each subject's statistical maps into standard space and used these to make group statistical inferences. RESULTS: Across subjects, the cortical response to words could be divided into at least two phases: an initial event-related synchronization in both the right temporal (100-300 ms, 15-25 Hz; 200-400 ms, 5-15 Hz) and left frontal regions (200-400 ms; 15-25 Hz); followed by a strong left-lateralized event-related desynchronization in the left temporal region (500-700 ms; 5-15 Hz). CONCLUSIONS: We found bilateral event-related synchronization followed by later left lateralized event-related desynchronization in language-related cortical areas. These data demonstrate the spatiotemporal time course of neural activation during an auditory word recognition task in a group of children. As well, this demonstrates the utility of SAM analyses to detect subtle sequential task-related neural activations.

Contrast sensitivity is reduced in children with infantile spasms.

PURPOSE: To investigate whether visual deficits in children with infantile spasm (IS) are the result of seizure activity or of treatment with the anticonvulsant drug vigabatrin (VGB). METHODS: Vision function was determined in three experiments by determining peak contrast sensitivity (CS) and grating acuity (GA) with the sweep visual evoked potential. Cross-sectional study A: 34 children, including 11 patients with childhood epilepsy with exposure to VGB for at least 6 months, 10 with childhood epilepsy exposed to antiepileptic drugs other than VGB, and 13 normally developing children. Cross-sectional study B: 32 children, including 16 with IS naïve to VGB and 16 normally developing children. Longitudinal study: seven children with IS naïve to VGB, with subsequent follow-up 5 to 10 months after starting VGB. RESULTS: In cross-sectional study A, the median CS was reduced by 0.5 log units (P = 0.025) in children with epilepsy exposed to VGB compared with those exposed to other antiepileptic drugs and normally developing children. In cross-sectional study B, the median CS was reduced by 0.25 log units (P = 0.0015) in children with IS (VGB naïve) compared with normally developing children. Longitudinal assessment showed no decrease in CS in children with IS who were followed up 5 to 10 months after starting VGB. There was no difference in GA among groups in any of the experiments. CONCLUSIONS: Patients with IS have CS deficits, but a sparing of GA. This deficit is present before VGB treatment and does not worsen with treatment onset. Results suggest that visual dysfunction is largely the result of the seizures themselves.

Cortical reorganization after modified constraint-induced movement therapy in pediatric hemiplegic cerebral palsy.

Constraint-induced movement therapy improves motor function in the affected hand of children with hemiplegic cerebral palsy and results in cortical changes in adults with stroke. This study measured clinical improvement and cortical reorganization in a child with hemiplegia who underwent modified constraint-induced movement therapy for 3 weeks. Clinical, functional magnetic resonance imaging and magnetoencephalography measurements were done at baseline, after therapy, and 6 months after therapy. Modified constraint-induced movement therapy resulted in clinical improvement as measured by the Pediatric Motor Activity Log. Functional magnetic resonance imaging showed bilateral sensorimotor activation before and after therapy and a shift in the laterality index from ipsilateral to contralateral hemisphere after therapy. Magnetoencephalography showed increased cortical activation in the ipsilateral motor field and contralateral movement evoked field after therapy. Cortical reorganization was maintained at the 6-month follow-up. This is the first study to demonstrate cortical reorganization after any version of constraint-induced movement therapy in a child with hemiplegia.

Placebo-controlled study examining effects of selegiline in children with attention-deficit/hyperactivity disorder.

There is evidence suggesting a role for dopamine in attention-deficit/hyperactivity disorder (ADHD). Pharmacological treatments that act on the dopamine system have been successful in reducing ADHD symptoms. However, unlike traditional stimulants (i.e., methylphenidate), selegiline is a monoamine oxidase inhibitor (MAOI) that has been shown to reduce ADHD symptoms without producing undesirable side effects. In this study using a randomized, double- blind, placebo-controlled, crossover design, cognitive tasks and behavioral rating scales were administered to measure the effectiveness of selegiline in treating different symptoms of ADHD in 11 children aged 6-13. Results indicate that selegiline may target specific symptoms of ADHD including: sustained attention, the learning of novel information, hyperactivity, and peer interactions. Because the drug was not associated with negative side effects and did not specifically reduce symptoms of impulsivity, selegiline may be a preferred treatment for individuals who present with the primarily inattentive subtype of ADHD.

Reduced visual function associated with infantile spasms in children on vigabatrin therapy.

PURPOSE: To use visual evoked potential (VEP) testing to determine whether visual deficits are present in children with a history of vigabatrin use. METHODS: Contrast sensitivity and visual acuity were assessed by visual evoked potential testing and compared between 28 children (mean age, 4.90 +/- 4.92 years) with seizure disorders who had taken vigabatrin and 14 typically developing children (mean age, 3.14 +/- 1.70 years). Exclusion criteria were heritable eye disease, suspected cortical visual impairment, nystagmus, and prematurity >2 weeks. The effects of the following factors on contrast sensitivity and visual acuity were examined: type of seizure (infantile spasms versus other), ERG result, duration of vigabatrin therapy, cumulative dosage of vigabatrin, and other seizure medications (other versus no other medication). RESULTS: Contrast sensitivity and visual acuity were reduced in vigabatrin-treated children with infantile spasms compared with vigabatrin-treated children with other seizure disorders and typically developing control subjects. The other factors examined had no significant effect on contrast sensitivity or visual acuity, with adjustment for seizure type. CONCLUSIONS: Children with infantile spasms on vigabatrin may have compromised visual function, even in the absence of suspected cortical visual impairment. The children tested in the present study have reduced vision, probably associated with infantile spasms rather than vigabatrin.

Characteristic retinal atrophy with secondary "inverse" optic atrophy identifies vigabatrin toxicity in children.

OBJECTIVE: To describe the clinical pattern of retinal atrophy in children caused by the anticonvulsant vigabatrin. DESIGN: An interventional case series report. PARTICIPANTS: One hundred thirty-eight patients, mainly infants, were evaluated regularly for evidence of possible vigabatrin toxicity in the Eye and Neurology clinics at the Hospital for Sick Children, Toronto. METHOD: Sequential clinical and electroretinographic (International Society for Clinical Electrophysiology of Vision standards) evaluations every 6 months. MAIN OUTCOME MEASURES: Presence of recognizable retinal and optic atrophy in the presence of abnormal electroretinogram (ERG) and other clinical findings. RESULTS: Three children being treated for seizures with vigabatrin showed definite clinical findings of peripheral retinal nerve fiber layer atrophy, with relative sparing of the central or macular portion of the retina and relative nasal optic nerve atrophic changes. Some macular wrinkling was evident in 1 case. Progressive ERG changes showing decreased responses, especially the 30-Hz flicker response, supported the presence of decreased retinal function. CONCLUSIONS: A recognizable and characteristic form of peripheral retinal atrophy and nasal or "inverse" optic disc atrophy can occur in a small number of children being treated with vigabatrin. The changes in superficial light reflexes of the retina in children facilitate the clinical recognition of nerve fiber layer atrophy. The macula is relatively spared, although superficial retinal light reflexes indicating wrinkling of the innermost retina suggest early macular toxicity as well. Because these changes are accompanied by electrophysiologic evidence of retinal dysfunction, discontinuation of vigabatrin should be strongly considered.

Unusual presentation of Lafora's disease.

Lafora's disease is a progressive myoclonus epilepsy with onset in adolescence and a gradual decline in cognitive functions and increase in seizure intractability. We present the case of a 16-year-old with precipitous dementia within 6 months of onset. Peripheral biopsies and EPM2A mutation analysis were negative. The diagnosis could be established only by brain biopsy.

Functional MRI study of verbal fluency in a patient with subcortical laminar heterotopia.

RATIONALE: Double cortex syndrome is a malformation in which there is a band of subcortical heterotopic grey matter separated from the cortex by white matter. The functional activity of the heterotopic neurons is unclear. PATIENT: A 13-year-old female was evaluated for seizures. The EEG showed bifrontal spike wave disturbance. Band heterotopia, in association with mild reduction of sulcation of the cerebral hemispheres, was found on MRI. Psychological assessment indicated the presence of variable cognitive abilities, with verbal IQ [82] generally better than nonverbal IQ [59], and specific difficulties in language comprehension and mathematics. METHOD: Functional MRI was used to localize the areas of language and motor activation. The language activation paradigm was a visual verb generation task with a visual fixation baseline. The motor paradigm consisted of alternating blocks of sequential finger tapping and rest. Coronal functional and anatomical images were obtained. RESULTS: The motor paradigm produced activation of the primary motor cortex, the band heterotopia and the supplementary motor cortex. The language paradigm produced activation of the left inferior frontal gyrus and left supplementary motor area, but not of the band heterotopia. CONCLUSIONS: The activation of heterotopic grey matter during a motor task demonstrates a hemodynamic association with motor activity and suggests that this tissue may be functional. Such association was not seen with the language task. We speculate that later maturing functions such as language are restricted in their development to the normal situated superficial cortex in our patient.

Pediatric constraint-induced movement therapy is associated with increased contralateral cortical activity on functional magnetic resonance imaging.

The mechanism behind constraint-induced movement therapy (constraint therapy) success is unknown. Study objectives were to evaluate cortical change after modified constraint therapy and explore a novel approach to quantify developmental disregard. Five participants underwent modified constraint therapy. Functional magnetic resonance imaging (MRI) and clinical measures were done pretreatment and posttreatment. Developmental disregard indices were calculated. Four participants showed clinical improvement posttreatment. Functional MRI laterality indices were variable pretreatment and exclusively contralateral among participants posttreatment. The disregard index range was -12.9 to 62.6 among participants. Disregard indices were correlated with change scores after treatment on the Pediatric Motor Activity Log amount of use domain (r = .93, P = .02), Assisting Hand Assessment (r = .93, P = .02), and grip strength (r = .92, P = .03). Study results suggest that a shift to or persistence of contralateral cortical activity for affected hand movement is important for constraint therapy mechanism of action; and developmental disregard may be a predictor of positive response to treatment.

Cerebral arteriopathy in children with neurofibromatosis type 1.

OBJECTIVE: Cerebrovascular abnormalities are serious but underrecognized complications of neurofibromatosis type 1 (NF1). The aim of this study was to investigate the prevalence, clinical presentation, imaging findings, and prognosis of cerebral arteriopathies in childhood NF1. METHODS: Patients followed at the NF1 clinic at the Hospital for Sick Children, Toronto, Ontario, Canada, between 1990 and 2007 were studied. Patients with confirmed NF1 diagnosis and neuroimaging results were included. All neuroimaging studies were reviewed for the presence of arteriopathy by 2 study pediatric neuroradiologists blinded to clinical information. Clinical records of children with cerebral arteriopathy were reviewed. RESULTS: Among 419 children with confirmed NF1, 266 (63%) received neuroimaging. Among children with neuroimaging results, 17 had cerebral arteriopathy (minimum prevalence rate of 6%). Among the 35 patients who received magnetic resonance angiography (MRA), arteriopathy was more common in patients with NF1 with optic gliomas (11 of 21) compared with those without optic glioma (4 of 14). Forty-seven percent of children developed focal deficits months to years after the diagnosis of the arteriopathy. Follow-up at a mean of 7 years after diagnosis of arteriopathy showed that 35% (6 of 17) had progressive arteriopathy requiring revascularization surgery. Seven patients received aspirin for primary stroke prevention. On retrospective review of imaging studies, a mean delay of 51 months to clinical radiographic reporting of these findings was observed. CONCLUSIONS: The prevalence of cerebral arteriopathy in children with NF1 in this study was at least 6% and was associated with young age and optic glioma. Arteriopathy causes stroke with resultant neurologic deficits. Medical and/or surgical interventions may prevent these complications. Therefore, the addition of vascular imaging (MRA/conventional angiography) to brain imaging studies for early detection of arteriopathy should be considered for children with NF1, particularly young patients with optic glioma.

Intrahemispheric reorganization of language in children with medically intractable epilepsy of the left hemisphere.

We investigated language representation in nine children (six male, three female; 5.6-17.7 years of age) who underwent surgical treatment of medically intractable epilepsy of the left hemisphere. Although interhemispheric reorganization has been previously documented in similar groups, this is the first study to systematically evaluate possible intrahemispheric effects of early insult. All cases had left hemisphere seizure foci and underwent extraoperative stimulation mapping (ESM) for language localization prior to receiving cortical resections. To compare ESM findings across subjects and to assess intrahemispheric reorganization, we developed a novel coregistration technique whereby independent raters plotted two-dimensional (2D) ESM findings in 3D standard space. Expressive language sites identified with ESM were compared with a structural probability map of pars opercularis, or Broca's area. The average difference between independent raters' estimates of 28 language sites was 3.9 mm (SD = 2.0), indicating excellent agreement; the coregistration procedure permitted assessment of 2D ESM findings in 3D standard space. We observed language sites in regions substantially anterior and superior to canonical Broca's area, possibly reflecting intrahemispheric reorganization. Findings suggest that left hemisphere insult in young children may result in anterior displacement of language within the frontal cortex.

The Hospital for Sick Children, Toronto, Longitudinal ERG study of children on vigabatrin.

The purpose of this longitudinal study was to identify changes in ERG responses associated with vigabatrin treatment. We accomplished this by recording longitudinally ERGs in children before and during vigabatrin treatment and comparing results between children on vigabatrin monotherapy and those taking additional anticonvulsive medications. Thirty-three children on vigabatrin therapy were tested; the duration between visits was approximately 6 months. Thirteen children were assessed initially before starting vigabatrin therapy and seven were assessed soon after (age range 1.5-126 months, median 6 months). The remaining 13 patients were already on vigabatrin at the time of initial visit (age range 6.5-180 months, median 16 months). ERGs were tested using the standard protocol established by the International Society for Clinical Electrophysiology of Vision, with Burian-Allen bipolar contact-lens electrodes. In addition to standard responses we recorded photopic oscillatory potentials (OPs). All 33 patients were tested longitudinally on at least two occasions and 11 were tested on three occasions. For children whose only anticonvulsive drug was vigabatrin there was a significant curvature (quadratic function, p < 0.05) of the predicted cone b-wave amplitude with time; exhibited as increase in b-wave amplitude followed by subsequent decrease. Descriptive data demonstrated the same pattern in the group taking anticonvulsive medications in addition to vigabatrin. In most children the flicker amplitude declined between 6 months and 1 year of vigabatrin treatment. Our data demonstrated that rod responses, which may be abnormal before initiation of vigabatrin, did not change substantially with vigabatrin treatment.

Longitudinal changes in photopic OPs occurring with vigabatrin treatment.

PURPOSE: Vigabatrin (gamma-vinyl-GABA) is an antiepileptic drug successful in the management of infantile spasms. Photopic ERGs were tested in children followed longitudinally before and during vigabatrin treatment. METHODS: Subjects were 26 infants (age range 1.5-24 months, median 7.6 months) on vigabatrin treatment who had been tested on multiple visits (two to four visits; mean, three visits). Eighteen of these were assessed initially before starting vigabatrin therapy and eight were assessed within 1 week of initiation of the drug. ERGs were recorded at 6-month intervals. Standard ISCEV protocol with Burian-Allen bipolar contact-lens electrodes (standard flash 2.0 cd.s/m2) was used. Although ISCEV standards were followed, a higher flash intensity (set at 3.6 cd.s/m2) was chosen for single-flash cone assessment to provide a better definition of OPs. Photopic OPs were divided into categories of early OPs and late OP (OP4). Responses were compared with age corrected limits extrapolated from our lab control database. RESULTS: Results showed differential effects of vigabatrin on the summed early OP amplitudes versus the late OP (OP4) and cone b-wave amplitude. The early OPs showed significant decrease (p = 0.0005, repeated measures analysis of variance) after 6 months and remained decreased for the duration of treatment. There was no significant change seen in the late OP. The cone b-wave amplitude showed initial increase (p = 0.04) after 6 months, followed by a decrease after 18 months; a trend similar to that of the late OP. CONCLUSION: Early photopic OPs were disrupted more than the late OP, suggesting relative deficit in the ON (depolarizing) retinal pathways.

Changes in the electroretinogram resulting from discontinuation of vigabatrin in children.

Electroretinograms (ERGs) have been recorded longitudinally in children before and during treatment with the antiepileptic drug vigabatrin for the past 3.5 years. Vigabatrin induced changes in ERG responses occur in children; the most dramatic changes occur in the oscillatory potentials. The purpose of this study was to identify changes in ERG responses associated with discontinuation of vigabatrin treatment. If vigabatrin-induced changes reverse after discontinuation of the drug we infer that the original change is not an indicator of toxicity. ERG data were analyzed from 17 children who discontinued vigabatrin therapy. The duration of treatment ranged from 5 to 52 months, the age for the first ERG ranged from 6 to 38 months (median 10 months). ERGs were tested using the standard protocol established by the International Society for Clinical Electrophysiology of Vision, with Burian-Allen bipolar contact-lens electrodes. In addition to standard responses we recorded photopic oscillatory potentials (OPs). During vigabatrin treatment OPs show a greater change than other ERG responses, with the early occurring wavelets from the photopic OPs showing the greatest change. With discontinuation of vigabatrin the amplitude of the early wavelets of the photopic OPs increased dramatically compared with amplitudes while taking the drug (paired t-test, p = 0.000075). The scotopic oscillatory potentials also show some recovery. Although changes in oscillatory potentials may occur with vigabatrin toxicity, a large change likely occurs with a non-toxic pharmacological effect of vigabatrin on GABAergic amacrine cells in the inner plexiform layer. Reduction of OPs in children on vigabatrin may not be related to toxicity.

Effect of intracranial bleeds on the health and quality of life of boys with hemophilia.

OBJECTIVES: To compare the health, physical function, and quality of life (QoL) of boys with hemophilia with and without a history of intracranial hemorrhage (ICH). STUDY DESIGN: Of 172 patients with hemophilia A or B, 18 (10%) had at least one episode of ICH. For outcome assessments, 16 of 18 (89%) boys with ICH and 32 controls, matched (1:2) for age and severity of hemophilia, were available. The outcome measures were neurologic function, physical function, and QoL. RESULTS: The median age of the boys at the first ICH was 5.9 months (range, 1 day to 2.7 years). Boys with ICH had a higher incidence of inhibitors and lower mean household income. Neurologic examination was abnormal in seven of 16 (44%) boys with ICH and nine of 32 (28%) controls (P=.3). The mean physical function in boys with ICH was lower (82%+/-25%) compared with controls (93.5%+/-12%, P=.045). The QoL was decreased in boys with ICH compared with controls (6.8+/-3.2 vs 8.5+/-1.4, P=.02), whereas health-related QoL was not significantly different between groups. CONCLUSION: The poorer long-term outcomes of boys with hemophilia appropriately treated for ICH, especially in the domain of QoL, suggest that new strategies to prevent ICH and to manage ICH effectively in this population are needed.

The significance of ear plugging in localization-related epilepsy.

PURPOSE: The localizing value of ear plugging in the treatment of auditory onset partial seizures, to our knowledge, has not been previously described. We propose that ear plugging is a clinical response to a sensory seizure manifested as an auditory hallucination and a tool for identifying the seizure focus in the auditory cortex on the superior temporal gyrus. METHODS: We report on three children who had prior epilepsy surgery for recurrent symptomatic localization-related epilepsy and who, subsequent to their surgery, displayed stereotyped unilateral or bilateral ear plugging at the onset of partial seizures. We studied scalp video electroencephalography (VEEG), magnetoencephalography (MEG), and magnetic resonance imaging (MRI) in all three. Additionally, we used electrocorticography (ECoG) in two patients, intracranial VEEG monitoring in one patient, and functional MRI language mapping in two patients. RESULTS: All three patients plugged their ears with their hands during auditory auras that localized to the superior temporal gyrus and were followed by partial seizures that spread to a wider field, as shown on scalp and intracranial VEEG. All three patients had MEG interictal discharges in the superior temporal gyrus. One patient who was nonverbal and unable to describe an auditory phenomenon plugged the ear contralateral to where temporal lobe-onset seizures and MEG interictal discharges occurred. CONCLUSIONS; Ear-plugging seizures indicate an auditory aura and may also lateralize seizure onset to the contralateral temporal lobe auditory cortex. Stereotyped behaviors accompanied by epileptic seizures in children who have poor communication skills are important in the seizure semiology of localization-related epilepsy.