Biomedical subjects

Shelly K Weiss

Publications and source records attributed to Shelly K Weiss.

At least 19 recordsLinked to original sources

PubMed2006

Efficacy of dexamathasone on cerebral swelling and seizures during subdural grid EEG recording in children.

PURPOSE: To evaluate the impact of steroid treatment on cerebral swelling and seizures during subdural grid EEG (SGEEG) monitoring. METHODS: We reviewed data from 37 pediatric patients with intractable epilepsy who underwent SGEEG monitoring and divided the patients into those who received dexamethasone and those who did not. We then correlated administration of steroids to incidence of cerebral swelling on computed tomography (CT) scans and to frequency of seizures during SGEEG. RESULTS: Twenty-three patients received dexamethasone prophylactically every 6 hours (dosage range, 1-7.5 mg; mean, 3.2 mg) from the first day of SGEEG placement (group A); 14 patients received no dexamethasone (group B). Eight (21.6%) of 37 patients experienced cerebral swelling on CT: two (9%) were in group A, and six (42.9%) were in group B (p < 0.05). SGEEG monitoring time for recording habitual seizures that localized cortical areas for surgical excision was longer in group A (1-6 days; mean, 3.0) than it was in group B (1-3 days; mean, 2.2), (p < 0.05). Habitual seizures were recorded in 36 patients. One group A patient experienced obtundation due to cerebral swelling, and monitoring in this patient was discontinued. CONCLUSIONS: The prophylactic administration of steroids to pediatric patients during SGEEG monitoring is efficacious for reducing cerebral swelling. Although it decreases the frequency of habitual seizures and increases seizure-monitoring time, dexamethasone reduces the risk of complications from cerebral swelling during the SGEEG procedure.

Adolescent↗

PubMed2006

Topographic movie of ictal high-frequency oscillations on the brain surface using subdural EEG in neocortical epilepsy.

PURPOSE: To understand the rapid dynamic changes of ictal intracranial high-frequency oscillations (HFOs) in neocortical epilepsy. METHODS: We integrated multiple band frequency analysis and brain-surface topographic maps of HFOs from ictal subdural EEG (SDEEG) recordings. We used SDEEG to record partial seizures consisting of right-arm jerks with secondary generalization in a 17-year-old right-handed girl. We selected 20-s EEG sections that included preclinical seizure recordings. We averaged the HFO power between 60 and 120 Hz for 25 selected electrodes, made topographic maps from these averaged powers, and superimposed the maps on the brain-surface image. We filmed consecutive HFO maps at a 10-ms frame rate. RESULTS: Before clinical seizure onset, high-power HFOs emerged at the superior portion of the left precentral gyrus, then appeared in the middle of the left postcentral gyrus, and subsequently reverberated between both regions as well as the posterior portion of the left postcentral gyrus. Right-arm extension and facial grimacing started as the HFO power decreased. As generalized tonic-clonic seizures evolved, HFO power increased but remained within the central region. CONCLUSIONS: Topographic movies of intracranial HFOs on the brain surface allow visualization of the dynamic ictal changes in neocortical epilepsy.

Adolescent↗

PubMed2006

Preoperative simulation of intracerebral epileptiform discharges: synthetic aperture magnetometry virtual sensor analysis of interictal magnetoencephalography data.

OBJECT: Magnetoencephalography (MEG) has been used for the preoperative localization of epileptic equivalent current dipoles (ECDs) in neocortical epilepsy. Spatial filtering can be applied to MEG data by means of synthetic aperture magnetometry (SAM), and SAM virtual sensor analysis can be used to estimate the strength and temporal course of the epileptic source in the region of interest. To evaluate the clinical usefulness of this approach, the authors compare the results of SAM virtual sensor analysis to the results of ECD analysis, subdural electroencephalography (EEG) findings, and surgical outcomes in pediatric patients with neocortical epilepsy. METHODS: Ten pediatric patients underwent MEG, invasive subdural EEG, and cortical resection for neocortical epilepsy. The authors compared the morphological characteristics, quantity, location, and distribution of the epileptiform discharges assessed using SAM and ECD analysis, and subdural EEG findings (interictal discharges and ictal onset zones). In nine patients, MEG revealed clustered ECDs. The region exhibiting the maximum percentage (> or = 70%) of spikes/sharp waves on SAM was colocalized to clustered ECDs in seven patients. In six patients, SAM demonstrated focal spikes; in two, diffuse spikes; and in two others, focal rhythmic sharp waves. These epileptiform discharges were similar to those recorded on subdural EEG. In nine patients, concordant regions containing the maximum percentage of spikes/sharp waves were revealed by SAM and subdural EEG data. The region of the maximum percentage of spikes/sharp waves as demonstrated by SAM was colocalized to the ictal onset zone identified by subdural EEG findings in seven patients and partially colocalized in two. CONCLUSIONS: The SAM virtual sensor analysis revealed morphological characteristics, location, and distribution of epileptiform discharges similar to those shown by subdural EEG recordings. By using SAM it is possible to predict intracerebral interictal epileptiform discharges in the region of interest from noninvasively collected preoperative MEG data. The maximum interictal discharge zone identified by SAM virtual sensors correlated to clustered ECDs and the ictal onset zone on subdural EEG findings. Complementary analyses of ECDs and SAM on three-dimensional MR images can improve delineation of epileptogenic zones and lesions in neocortical epilepsy.

Adolescent↗

PubMed2005

Focal cortical high-frequency oscillations trigger epileptic spasms: confirmation by digital video subdural EEG.

OBJECTIVE: To localize high-frequency oscillations (HFOs) on the cortex during epileptic spasms using video subdural EEG and Multiple Band Frequency Analysis (MBFA). METHODS: Using video subdural EEG sampled at 1 kHz, we studied a 14-year-old boy with asymmetric epileptic spasms of possible left frontal origin. We identified HFOs, then analyzed and localized their distributions by MBFA. We correlated HFO distribution to clinical spasm intensity. RESULTS: Ictal subdural EEG recorded HFOs at 60-150 Hz lasting 0.3-4 s. MBFA showed extensive but noncontiguous distribution of HFOs predominantly over the left frontal and temporal regions. HFOs began and became quasiperiodic before manifestation of clinical spasms. As clinical spasms intensified, HFOs persisted in regions where they initiated subclinically but were of higher frequency and greater power than HFOs in other regions. We performed cortical resections over the left frontal and temporal regions with predominant HFOs. Six months after surgery, the patient remained seizure free. CONCLUSIONS: HFOs were present over the ictal onset zone during epileptic spasms. Periodic spasms in this patient had the characteristics of partial seizures. SIGNIFICANCE: We show that HFOs occurred over the cerebral cortex during epileptic spasms, and we suggest that these focal cortical HFOs triggered the spasms.

Adolescent↗

PubMed2005

Reactive EEG patterns in pediatric coma.

This study was designed to determine whether the observed reactive electroencephalographic patterns in comatose children were associated with a better outcome. All electroencephalograms performed in comatose children aged 2 months to 18 years during the period 1996-2003 were retrospectively analyzed and classified according to modified Young's classification. Reactivity to painful/auditory stimuli and passive eye closure (at least two modalities) was checked in all electroencephalograms. The clinical outcome at 1 year or during the last clinic/inpatient follow-up was scored according to the Pediatric Cerebral and Overall Performance Category Scale. Outcomes were then compared using Fisher exact test and the Mann-Whitney test. Thirty-three patients had electroencephalography within 72 hours after the onset of coma. Fourteen of 33 electroencephalograms revealed reactive patterns. Outcome was unfavorable in 4 (28.6%) of these patients. Three children had no residual neurologic impairment. Among the 19 children with nonreactive electroencephalogram, 13 (65%) had unfavorable outcome, which included 10 deaths. All the survivors had residual neurologic impairment. Outcome was better in children with reactive electroencephalographic patterns (Fisher exact test; P = 0.023). Comatose children with reactive electroencephalographic patterns have better clinical outcome in terms of morbidity and mortality. A careful assessment of electroencephalographic reactive patterns in all comatose children is required for better understanding of the clinical outcome.

Adolescent↗

PubMed2005

A reappraisal of rhythmic coma patterns in children.

OBJECTIVE: This study was designed to determine the prevalence of rhythmic coma patterns in comatose children and to ascertain the prognostic significance of reactive rhythmic coma patterns. METHODS: We retrospectively analyzed and classified electroencephalogram (EEGs) in comatose children between two months and 18 years of age during the period 1996 - 2003 according to modified Young's classification. Outcome at one-year was scored according to the Paediatric Cerebral and Overall Performance Category Scale. Outcomes were compared using Fisher's exact test and Mann-Whitney test. RESULTS: Analysis of 63 electroencephalogram (EEG) records in 38 patients showed rhythmic patterns in 19 records (30.2%; 9 alpha, 4 spindle, 4 theta and 2 beta coma patterns, total number of children = 14). Aetiology and outcome of alpha coma patterns and other rhythmic coma patterns were similar. In five children, one type of rhythmic pattern changed to another. Records with reactive rhythmic coma 66.7% (6/9), were associated with favourable outcome. Sixty percent of the records (6/10 records in seven children) with non-reactive pattern were associated with unfavourable outcome. This clinically significant difference did not reach statistical significance (lower Paediatric Cerebral and Overall Performance Category Scale score p= 0.14; favourable outcome p=0. 19). CONCLUSION: Rhythmic coma patterns in comatose children are not uncommon. Aetiology, reactivity and outcome of individual patterns are similar and thus make the rhythmic coma patterns distinct EEG signatures in comatose children. There was a clinically significant better outcome with reactive rhythmic coma patterns.

Adolescent↗

PubMed2005

The prevalence of autistic spectrum disorder in children surveyed in a tertiary care epilepsy clinic.

UNLABELLED: It is well documented that children with autistic spectrum disorder (ASD) have an increased prevalence of seizures; however, studies have not been done to evaluate the prevalence of ASD in children with epilepsy. This comorbidity is important to define as early diagnosis and intervention in some children with ASD has been shown to improve outcome. METHOD: Children with epilepsy seen in a tertiary care epilepsy clinic were evaluated using validated autism screening questionnaires (ASQ). In addition, questions about sleep-related disorders, behavior, seizure characteristics, antiepileptic agents, and body mass index (BMI) were requested. An attempt was then made to determine if there was a correlation between the factors identified and ASD. RESULTS: Of the 107 questionnaires returned, 97 ASQ's were properly completed and used in this study. Approximately 32% of children fit the ASQ criteria for having ASD. Most children had not been previously diagnosed. Worst behavior and daytime sleepiness was seen in those at greater risk (p < 0.01). Seizures also occurred earlier (approximately 2 years) in children at risk of having ASD. CONCLUSION: Though confirmatory diagnostic evaluations are needed, this questionnaire-based study suggests that children with epilepsy are at greater risk of having ASD, and illustrates the need for more clinical vigilance. Behavioral difficulties and daytime sleepiness identified in these children could potentially affect their ability to learn. It is of interest that the age of seizure onset identified in those at greater risk corresponds with the approximate age of regression identified in some children with ASD.

Adolescent↗

PubMed2005

Characterizing magnetoencephalographic spike sources in children with tuberous sclerosis complex.

PURPOSE: Tuberous sclerosis complex (TSC) often causes medically intractable seizures. Magnetoencephalography (MEG) localizes epileptiform discharges. To evaluate the use of MEG spike sources (MEGSSs) for localizing epileptic zones in TSC patients, we characterized MEGSSs and correlated them to EEG and magnetic resonance imaging (MRI) results. METHODS: We analyzed data from seven children who underwent prolonged video-EEG, MEG, and MRI. We classified MEGSSs as clusters (six or more spike sources, 1 cm between sources regardless of number of sources). RESULTS: A single, unilateral cluster with additional scatters occurred in two patients; these predominantly lateralized dipoles correlated to prominent tubers on MRI and ictal/interictal EEG zones. Bilateral clusters with scatters existed in two patients; cluster locations partly overlapped multiple prominent tubers. These patients also had bilateral or diffuse interictal discharges, bilateral or generalized seizures, and changing seizure types and EEG findings. Only bilateral scatters occurred in three patients; scatters partly overlapped EEG interictal/ictal-onset regions; one patient had coexisting generalized seizures. In one patient with equally bilateral scatters, scatters overlapped a prominent tuber and interictal/ictal-onset zones in the right frontal region. CONCLUSIONS: MEG contributes to information from EEG and MRI for localizing epileptogenic zones in children with TSC. A single cluster with scatters in a unilateral hemisphere predicts a primary epileptogenic zone or hemisphere; bilateral or multiple clusters indicate bilateral primary or potential epileptogenic zones; and bilateral scatters without clusters may indicate epileptogenic zones that are hidden within extensive areas of scattered MEGSSs.

Brain Mapping↗

PubMed2005

Neurophysiologic findings of neuronal migration disorders: intrinsic epileptogenicity of focal cortical dysplasia on electroencephalography, electrocorticography, and magnetoencephalography.

We define specific neurophysiologic characteristics for focal cortical dysplasia, a neuronal migration disorder. We reviewed data from published reports and our patients with focal cortical dysplasia. Our patients underwent preoperative scalp video-electroencephalography (EEG), magnetic resonance imaging (MRI), magnetoencephalography, and intraoperative or extraoperative electrocorticography monitoring. Scalp EEG showed trains of rhythmic epileptiform spike or sharp waves. Positive spikes correlated with early seizure onset, MRI lesion around the rolandic fissure, hemiparesis, and a less favorable outcome. Interictal electrocorticography showed continuous epileptogenic discharges: repetitive electrographic seizures and bursting discharges or continuous or quasicontinuous rhythmic spiking. Ictal electrocorticography showed paroxysmal fast and/or repetitive spiking. Magnetoencephalography showed clustered spike sources within and extending from the lesion. Cortical stimulation gave more frequent, lower-threshold afterdischarges and higher-threshold primary motor function. Focal cortical dysplasias are highly and intrinsically epileptogenic. For surgical seizure control, EEG, electrocorticography, and magnetoencephalography must delineate the intrinsic epileptogenic zone within and extending from the focal cortical dysplasia identified by MRI.

Brain Mapping↗

PubMed2005

Recurrent intractable seizures in children with cortical dysplasia adjacent to dysembryoplastic neuroepithelial tumor.

The purpose of this study was to identify the pathologic features that predict postoperative outcome in children with cortical dysplasia adjacent to dysembryoplastic neuroepithelial tumors. We reviewed the records of children with dysembryoplastic neuroepithelial tumor who underwent epilepsy surgery and who had at least 1 year of surgical follow-up. We divided the dysembryoplastic neuroepithelial tumors into three pathology classes (simple, complex, and nonspecific), categorized adjunctive cortical dysplasia into four types, and compared histopathology with seizure outcomes. We identified 26 children with dysembryoplastic neuroepithelial tumors. Dysembryoplastic neuroepithelial tumors were complex in 19 patients (73%), simple in 6 (23%), and nonspecific in 1 (4%). Cortical dysplasia was adjacent to dysembryoplastic neuroepithelial tumors in 18 patients. Six patients had type IA cortical dysplasia, 5 had type IB, 3 had type IIA, and 1 had type IIB. The 3 remaining patients had repeated surgeries; of these, 2 patients had cortical dysplasias of type IA/IB and 1 was type IIA/IIB. Eight (39%) of 18 patients with dysembryoplastic neuroepithelial tumors and cortical dysplasia required further surgery for recurrent intractable seizures (P < .05), whereas none of 8 patients without cortical dysplasia required additional surgery. Of 13 patients with type I cortical dysplasia, only 4 had a poor seizure outcome, whereas all 5 patients with type II had a poor seizure outcome postoperatively (P < .05). Children with dysembryoplastic neuroepithelial tumor and cortical dysplasia often had recurrent intractable seizures postoperatively and required further epilepsy surgery. Cortical dysplasia adjacent to dysembryoplastic neuroepithelial tumor can play a role in the epileptogenicity of dysembryoplastic neuroepithelial tumor. Complete resection of a dysembryoplastic neuroepithelial tumor and its adjacent cortical dysplasia should be considered.

Adolescent↗

PubMed2005

Identifying the primary epileptogenic hemisphere from electroencephalographic (EEG) and magnetoencephalographic dipole lateralizations in children with intractable epilepsy.

We used electroencephalographic (EEG) and magnetoencephalographic dipole lateralizations to identify the primary epileptogenic hemisphere in 41 children with intractable localization-related epilepsy. We compared EEG and magnetoencephalographic dipole lateralizations, EEG ictal onsets, and magnetic resonance images (MRIs). Concordant lateralization of EEG and magnetoencephalographic dipoles (> 50% of each lateralizing to the same hemisphere) occurred in 34 patients, with EEG ictal onsets in the same hemisphere in 23 (68%) and concordant MRI lesions in 23 (68%). Focal resection in 16 of 20 patients resulted in a good surgical outcome. Of the seven children with nonconcordant magnetoencephalographic and EEG lateralizations, one (14%) had EEG ictal onset and one (14%) had MRI lesions that lateralized; none had surgery. The relationship between lateralized EEG and magnetoencephalographic dipoles forecasts surgical candidacy. Concordant lateralizations predict good seizure control after surgery by identifying the primary epileptogenic hemisphere. Discordant lateralizations signify an undetermined epileptogenic hemisphere and contraindicate surgery without further testing.

Child↗

PubMed2005

Characterizing magnetic spike sources by using magnetoencephalography-guided neuronavigation in epilepsy surgery in pediatric patients.

OBJECT: The authors sought to validate magnetoencephalography spike sources (MEGSSs) in neuronavigation during epilepsy surgery in pediatric patients. METHODS: The distributions of MEGSSs in 16 children were defined and classified as clusters (Class I), greater than or equal to 20 MEGSSs with 1 cm or less between MEGSSs; small clusters (Class II), 6 to 19 with 1 cm or less between; and scatters (Class III), less than 6 or greater than 1 cm between spike sources. Using neuronavigation, the MEGSSs were correlated to epileptic zones from intra- and extraoperative electrocorticography (ECoG), surgical procedures, disease entities, and seizure outcomes. Thirteen patients underwent MEGSSs: nine had clusters; two had small clusters, one with and one without clusters; and three had scatters alone. All 13 had scatters. Clusters localized within and extended from areas of cortical dysplasia and at margins of tumors or cystic lesions. All clusters were colocalized to ECoG-defined epileptic zones. Four of 10 patients with clusters and/or small clusters underwent complete excisions, and six underwent partial excision with or without multiple subpial transections. In the three patients with scatters alone, ECoG revealed epileptic zones buried within MEGSS areas; these regions of scatters were completely excised and treated with multiple subpial transections. Coexisting scatters were left untreated in nine of 10 patients. Postoperatively, nine of 13 patients were seizure free; the four patients with residual seizures had clusters in unresected eloquent cortex. Three patients in whom no MEGSSs were demonstrated underwent lesionectomies and were seizure free. CONCLUSIONS: Magnetoencephalography spike source clusters indicate an epileptic zone requiring complete excision. Coexisting scatters remote from clusters are nonepileptogenic and do not require excision. Scatters alone, however, should be examined by ECoG; an epileptic zone may exist within these distributions.

Adolescent↗

PubMed2005

Partial seizures triggering infantile spasms in the presence of a basal ganglia glioma.

Infantile spasms associated with brain tumors have been reported. A focal cortical lesion can induce infantile spasms by triggering the brainstem and basal ganglia in this vulnerable age group. We report the case of a female infant with a low-grade glioma in the right basal ganglia, spreading to the cortical area. She presented at the age of five months with left hemiparesis and partial seizures. She developed infantile spasms at the age of 12 months. This is the first video clip report of partial seizures triggering symmetrical spasms in series, secondary to a basal ganglia glioma extending to the cortex. [Published with video sequences].

Anticonvulsants↗

PubMed2003

Characteristics of dipoles in clustered individual spikes and averaged spikes.

The aim of this study is to analyze the characteristics of dipoles in clustered individual spikes and averaged spikes, we compared electroencephalography (EEG) dipole localizations from patients with intractable extratemporal lobe epilepsy (IETLE) and from patients with benign epilepsy with centrotemporal spikes (BECTS). We studied 10 patients; five with IETLE who underwent epilepsy surgery after subdural EEG and five with BECTS. We recorded 19-channel digital scalp EEGs and used clustering analysis for individual spikes to characterize interictal spikes. We selected and averaged one representative spike group at the maximum negative peak electrode. We used a single dipole method with three-shell spherical head model. We compared dipole localizations of both averaged and individual spikes.IETLE data had more identifiable spike clusters and fewer spikes in each cluster than BECTS (P<0.05). Dipole sources with goodness-of-fit >or=95% in averaged spikes were less frequent in IETLE than in BECTS (P<0.05). For IETLE, averaged spikes showed no dipoles (two patients), while individual spikes gave dipole sources reliably in the epileptic region. For BECTS, individual and averaged spike sources were clustered. More than 80% of dipoles in averaged spikes were stable, in close proximity, for prolonged periods in BECTS. More spike groups after clustering and fewer acceptable dipoles from averaged spikes in IETLE reflect variable spike activity over extensive epileptic regions. Fewer spike groups producing more acceptable dipoles in BECTS correlate with stable spike sources within the isolated epileptic central region. Characteristics of clustered interictal spikes need careful examination before the use of dipole analysis of averaged spikes for epilepsy evaluation.

Adolescent↗

PubMed2003

Utility of antiepileptic drug monitoring in the pediatric emergency department.

Convulsive disorders are common in the pediatric age group, and measurement of serum concentration of an antiepileptic drug (AED) is frequently ordered for epileptic patients in the emergency department (ED). The objective of this study was to develop a better understanding of the indications for, and consequences of, monitoring AED serum concentrations in the pediatric ED. Charts of 116 patients who visited the ED and were tested for blood levels of AED were retrospectively reviewed. Main outcome measures were number and percentage of levels outside the therapeutic range, discontinuation of an AED or introduction of a new one, dosage modifications, and admission to hospital. Two pediatricians and a pediatric neurologist aware only of patients' age, weight, diagnosis, history, clinical presentation, and drug details reviewed each case and on the basis of predetermined criteria decided whether measurement of AED was indicated. Mean age (+/- SD) of the study population was 7 +/- 5 years (range, 2 months-17 years). Forty-two patients (36%) were on monotherapy, and 74 (64%) were on polytherapy. Sixty-eight patients (59%) presented with increased seizure frequency, 7 (6%) with status epilepticus, and 13 (11%) with suspected AED toxicity. The remainder of the children presented with problems unrelated to epilepsy. No significant difference was found between patients with AED levels within the therapeutic range and those with levels outside it in the proportion of children needing dosage change, change in medication, or hospital admission (P = 0.5, 0.8, and 0.8, respectively). None of the patients presenting with status epilepticus and only 15% of those with increased seizure activity had subtherapeutic levels. Review of the cases suggested that measuring serum AED level was not indicated in 57 (49.1%) patients. In the pediatric ED, abnormal AED levels do not correlate with clinical management. Before ordering tests, physicians should consider whether their results would alter patient treatment.

Adolescent↗

PubMed2003

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.

Adolescent↗

PubMed2003

Characteristics of prolonged afterdischarges in children with malformations of cortical development.

We investigated aberrant cortical excitability in malformations of cortical development From subdural electrodes, we recorded afterdischarges lasting > or = 6 seconds in 12 of 13 patients with malformations of cortical development and 6 of 10 pediatric patients with nonmalformations of cortical development and reviewed amperage thresholds, distribution of afterdischarges, and motor responses. In patients with malformation of cortical development, motor response thresholds were high; afterdischarge and motor response thresholds, which essentially overlapped, inversely correlated with age (P < .01); afterdischarge thresholds declined with age; and 8 patients showed afterdischarges in remote sites. In nonmalformation of cortical development, afterdischarge thresholds did not significantly correlate with age; motor response thresholds tended to decline with age; and 2 patients had remote afterdischarges. Adolescent patients with malformations of cortical development had lower afterdischarge thresholds than adolescents with nonmalformation of cortical development (P < .05). From their high afterdischarge (and motor response) thresholds, we concluded that preadolescent patients with malformation of cortical development have less excitable, immature cortices, whereas adolescent patients with malformation of cortical development with low afterdischarge thresholds have hyperexcitable cortices. Remote afterdischarges over focal dysplastic cortex suggest aberrant cortical excitability and neural circuits.

Adolescent↗

PubMed2002

A 15-year-old boy with central nervous system vasculopathy presenting with dysarthria-clumsy hand syndrome.

Dysarthria-clumsy hand stroke syndrome has been described frequently in adults but not in children. We report a 15-year-old right-handed boy with sudden onset of dysarthria, dysphagia, right facial weakness, and mild right-hand clumsiness. Computed tomographic scan and magnetic resonance imaging demonstrated infarction in the genu and posterior limb of the left internal capsule. Magnetic resonance angiography and conventional angiography demonstrated stenosis of the supraclinoid portion of the left internal carotid artery and the origin of the left ophthalmic artery. Lacunar infarction in an older adult is not the only mechanism leading to dysarthria-clumsy hand syndrome.

Adolescent↗