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1.
Neurocase ; 25(3-4): 91-97, 2019.
Article in English | MEDLINE | ID: mdl-31094654

ABSTRACT

Background: fMRI of mental phenomena is quite difficult to perform because lack of patient's cooperation or because the symptoms are stable. In some exceptional cases, however, fMRI and DTI are capable to provide insights on the anatomy of organic hallucinations. Methods: In this report we describe a 14-year-old boy with a left fronto-dorsal tumor who experienced chronic complex brief, frequent and repetitive complex visual and auditory hallucinations. His clinical picture included multiple and severe social and mood problems. During a presurgical fMRI mapping the patient complained of having the visual and auditory hallucinations. A block-design FMRI paradigm was obtained from the event timecourse. Deterministic DTI of the brain was obtained seeding the lesion as ROI. The patient underwent surgery and electrocorticography of the lesional area. Results: The fMRI of the hallucinations showed activation in the left inferior frontal gyrus (IFG) and the peri-lesional area. The tractography of the tumor revealed structural aberrant connectivity to occipital and temporal areas in addition to the expected connectivity with the IFG via the aslant fasciculus and homotopic contralateral areas. Intraoperative EEG demonstrated epileptic discharges in the tumor and neighboring areas. After resection, the patient's hallucinations stopped completely. He regained his normal social life and recover his normal mood. He remained asymptomatic for 90 days. Afterwards, hallucinations reappeared but with less intensity. Conclusions: To our knowledge, this is the first reported case of combined functional and structural connectivity imaging demonstrating brain regions participating in a network involved in the generation of complex auditory and visual hallucinations.


Subject(s)
Brain Neoplasms/diagnostic imaging , Brain/diagnostic imaging , Functional Neuroimaging , Hallucinations/diagnostic imaging , Magnetic Resonance Imaging , Adolescent , Brain/physiopathology , Brain/surgery , Brain Neoplasms/complications , Brain Neoplasms/physiopathology , Brain Neoplasms/surgery , Electroencephalography , Hallucinations/etiology , Hallucinations/physiopathology , Hallucinations/surgery , Humans , Male , Treatment Outcome
2.
Epilepsia ; 58(6): 1005-1014, 2017 06.
Article in English | MEDLINE | ID: mdl-28387951

ABSTRACT

OBJECTIVE: Evaluate the seizure-reduction response and safety of brain-responsive stimulation in adults with medically intractable partial-onset seizures of neocortical origin. METHODS: Patients with partial seizures of neocortical origin were identified from prospective clinical trials of a brain-responsive neurostimulator (RNS System, NeuroPace). The seizure reduction over years 2-6 postimplantation was calculated by assessing the seizure frequency compared to a preimplantation baseline. Safety was assessed based on reported adverse events. Additional analyses considered safety and seizure reduction according to lobe and functional area (e.g., eloquent cortex) of seizure onset. RESULTS: There were 126 patients with seizures of neocortical onset. The average follow-up was 6.1 implant years. The median percent seizure reduction was 70% in patients with frontal and parietal seizure onsets, 58% in those with temporal neocortical onsets, and 51% in those with multilobar onsets (last observation carried forward [LOCF] analysis). Twenty-six percent of patients experienced at least one seizure-free period of 6 months or longer and 14% experienced at least one seizure-free period of 1 year or longer. Patients with lesions on magnetic resonance imaging (MRI; 77% reduction, LOCF) and those with normal MRI findings (45% reduction, LOCF) benefitted, although the treatment response was more robust in patients with an MRI lesion (p = 0.02, generalized estimating equation [GEE]). There were no differences in the seizure reduction in patients with and without prior epilepsy surgery or vagus nerve stimulation. Stimulation parameters used for treatment did not cause acute or chronic neurologic deficits, even in eloquent cortical areas. The rates of infection (0.017 per patient implant year) and perioperative hemorrhage (0.8%) were not greater than with other neurostimulation devices. SIGNIFICANCE: Brain-responsive stimulation represents a safe and effective treatment option for patients with medically intractable epilepsy, including adults with seizures of neocortical onset, and those with onsets from eloquent cortex.


Subject(s)
Cerebral Cortex/physiopathology , Deep Brain Stimulation/methods , Drug Resistant Epilepsy/physiopathology , Drug Resistant Epilepsy/therapy , Electric Stimulation Therapy/methods , Electroencephalography , Neocortex/physiopathology , Adolescent , Adult , Brain Mapping , Deep Brain Stimulation/instrumentation , Electric Stimulation Therapy/instrumentation , Electrodes, Implanted , Epilepsies, Partial/physiopathology , Epilepsies, Partial/therapy , Epilepsy, Complex Partial/physiopathology , Epilepsy, Complex Partial/therapy , Epilepsy, Partial, Motor/physiopathology , Epilepsy, Partial, Motor/therapy , Epilepsy, Tonic-Clonic/physiopathology , Epilepsy, Tonic-Clonic/therapy , Feasibility Studies , Female , Follow-Up Studies , Humans , Male , Middle Aged , Young Adult
3.
Epilepsia ; 57(3): 436-44, 2016 Mar.
Article in English | MEDLINE | ID: mdl-26778272

ABSTRACT

OBJECTIVE: We observed several children with medically resistant epilepsy demonstrating focal positron emission tomography (PET) hypermetabolism, a finding rarely reported and of questionable significance. We therefore retrospectively reviewed the incidence of hypermetabolic PET, and its relationship to electroencephalography (EEG) and magnetic resonance imaging (MRI) findings, and to the outcome of epilepsy surgery. METHODS: We retrospectively reviewed 498 PET brain studies in patients with medically resistant childhood epilepsy for evidence of hypermetabolism. In patients with PET hypermetabolism, we correlated metabolic abnormality with the scalp EEG and MRI findings. In a subset of patients who underwent surgical resection, we further correlated the PET findings with histopathologic and surgical outcomes. RESULTS: Focal PET hypermetabolism was identified in 33 (6.6%) of 498 studies. The region of hypermetabolism correlated with a spike count of ≥10 per minute in 26 of 32 concomitant scalp EEG studies and 18 of 21 lesions evident on MRI. In 17 patients who underwent surgical resection, PET hypermetabolism further correlated with regions revealing almost continuous epileptiform discharges on the intracranial EEG and with histopathologically malformative tissue. At a minimum follow-up of 1 year postsurgery (median 33 months), 7 (50%) of 14 patients had Engel's class I outcome, 4 patients had class II, and 2 had class III outcome, whereas one patient was unchanged. At last follow-up, seizure freedom was noted in five of seven patients with focal PET hypermetabolism alone versus three of eight patients with PET hypometabolism. SIGNIFICANCE: Focal PET hypermetabolism is associated with high spike frequency on scalp EEG and can occur in the absence of ictal events during the peri-injection period. Correlation with intracranial EEG usually corroborates the highly epileptogenic pathophysiologic state. Cortical malformations constitute the most common pathologic substrate, and resection of the hypermetabolic PET region may facilitate favorable outcomes. These observations indicate that focal PET hypermetabolism is an important marker of the epileptogenic zone and may represent its epicenter.


Subject(s)
Drug Resistant Epilepsy/diagnostic imaging , Drug Resistant Epilepsy/metabolism , Positron-Emission Tomography/methods , Adolescent , Child , Child, Preschool , Cohort Studies , Drug Resistant Epilepsy/epidemiology , Drug Resistant Epilepsy/surgery , Electroencephalography/methods , Humans , Incidence , Infant , Retrospective Studies , Treatment Outcome , Young Adult
4.
Epilepsia ; 57(9): 1450-7, 2016 09.
Article in English | MEDLINE | ID: mdl-27387565

ABSTRACT

OBJECTIVE: This study investigates whether a combined rotating dipole (RD) and moving dipole (MD) solution enhances three-dimensional electroencephalography (EEG) source imaging (3D-ESI) localization in magnetic resonance imaging (MRI)-negative pediatric patients with focal cortical dysplasia (FCD). METHODS: We retrospectively selected 14 MRI-negative patients with FCD from a cohort of 60 pediatric patients previously used to evaluate the diagnostic utility of 3D-ESI in epilepsy surgery. Patients were younger than 18 years at time of surgery and had at least 1 year of outcome data. RD and MD models were constructed for each interictal spike or sharp wave, and it was determined whether each inverse algorithm localized within the surgical resection cavity (SRC). We also compared the 3D-ESI findings and surgical outcome with positron emission tomography (PET) and ictal single photon emission computed tomography (iSPECT). RESULTS: RD analyses revealed a high concordance with the SRC (78.6%), particularly for temporal lobe resection (100.0%), and showed superior localization compared to PET and iSPECT, with the highest correlation in FCD type I and temporal lobe resection. Furthermore, the RD method was superior to iSPECT in FCD type II cases and to PET in extratemporal resections. RD and MD results were comparable, but in 18.2% of patients with FCD type I with localizing RDs, the MD solution was only partially within the SRC; in all of these patients 3D-ESI also correlated with superior surgical outcome compared to PET and iSPECT, especially when RD and MD solutions were analyzed together. SIGNIFICANCE: 3D-ESI in MRI-negative cases showed superior localization compared to iSPECT or PET, especially in FCD type I and temporal lobe epilepsy, and correlated with superior surgical outcome compared to iSPECT and PET at 1 year and 2 years postoperatively, especially when RD and MD solutions were analyzed together. These findings suggest that 3D-ESI based on a combined RD-MD solution improves surgical accuracy in MRI-negative patients with FCD.


Subject(s)
Brain Mapping , Electroencephalography , Epilepsy/diagnostic imaging , Epilepsy/etiology , Imaging, Three-Dimensional , Malformations of Cortical Development, Group I/complications , Adolescent , Algorithms , Child , Child, Preschool , Cohort Studies , Epilepsy/complications , Epilepsy/surgery , Female , Humans , Infant , Magnetic Resonance Imaging , Male , Neurosurgical Procedures , Positron-Emission Tomography , Tomography, Emission-Computed, Single-Photon , Treatment Outcome
5.
Epilepsia ; 57(1): 24-31, 2016 Jan.
Article in English | MEDLINE | ID: mdl-26696504

ABSTRACT

OBJECTIVE: The aim of this study was to investigate the utility of three-dimensional electroencephalography source imaging (3D-ESI) with low-resolution electroencephalographic data in the pediatric noninvasive presurgical evaluation, and to compare the findings with positron emission tomography (PET) and ictal single-photon emission computed tomography (iSPECT). METHODS: We retrospectively selected 60 patients from a database of 594 patients who underwent excisional surgery for drug-resistant epilepsy. Patients were <18 years at time of surgery, had at least one presurgical volumetric brain magnetic resonance imaging (MRI), and at least 1 year of outcome data. 3D-ESI was performed with NeuroScan software CURRY V.7.0. For each patient the surgical resection was planned utilizing 3D-ESI as an adjunctive tool to supplement MRI and electrocorticographic data. Our analyses addressed three critical variables: pathology (focal cortical dysplasia vs. other pathologies), imaging (MRI negative vs. positive cases), and surgery (temporal resection vs. extratemporal and multilobar resections). We also compared the localizing utility and surgical outcome of 3D-ESI findings with PET, iSPECT, and the colocalized surgical resection. Statistical analyses were performed using the Statistical Package for the Social Sciences, Version 20. RESULTS: Mean age at surgery was 11.18 years (range 1-18 years). 3D-ESI showed a strong correlation with the surgical resection cavity (65.0%), particularly within the temporal lobe. 3D-ESI demonstrated better localization in MRI-negative cases (78.6%), which was not statistically significant. 3D-ESI also correlated with a superior surgical outcome profile compared to PET and iSPECT. SIGNIFICANCE: Our findings demonstrate that 3D-ESI data obtained with low-resolution electroencephalography achieves reasonably accurate noninvasive localization of epileptic spikes in pediatric focal epilepsy, especially in temporal lobe and MRI-negative cases, and is comparable to iSPECT and PET. Given its lesser expense and lack of radiation exposure, 3D-ESI is a useful and efficient tool for evaluating surgical candidacy in pediatric epilepsy surgery centers, particularly if PET and iSPECT are unavailable.


Subject(s)
Brain/pathology , Brain/surgery , Drug Resistant Epilepsy/diagnosis , Drug Resistant Epilepsy/surgery , Electroencephalography , Imaging, Three-Dimensional/methods , Adolescent , Analysis of Variance , Brain/diagnostic imaging , Child , Child, Preschool , Cohort Studies , Female , Humans , Infant , Magnetic Resonance Imaging , Male , Positron-Emission Tomography , Tomography, Emission-Computed, Single-Photon , Treatment Outcome
6.
Epilepsy Behav ; 58: 91-6, 2016 05.
Article in English | MEDLINE | ID: mdl-27064828

ABSTRACT

This study evaluated language organization in children with intractable epilepsy caused by temporal lobe focal cortical dysplasia (FCD) alone or dual pathology (temporal lobe FCD and hippocampal sclerosis, HS). We analyzed clinical, neurological, fMRI, neuropsychological, and histopathologic data in 46 pediatric patients with temporal lobe lesions who underwent excisional epilepsy surgery. The frequency of atypical language representation was similar in both groups, but children with dual pathology were more likely to be left-handed. Atypical receptive language cortex correlated with lower intellectual capacity, verbal abstract conceptualization, receptive language abilities, verbal working memory, and a history of status epilepticus but did not correlate with higher seizure frequency or early seizure onset. Histopathologic substrate had only a minor influence on neuropsychological status. Greater verbal comprehension deficits were noted in children with atypical receptive language representation, a risk factor for cognitive morbidity.


Subject(s)
Drug Resistant Epilepsy/diagnostic imaging , Epilepsy, Temporal Lobe/diagnostic imaging , Language , Magnetic Resonance Imaging/methods , Temporal Lobe/diagnostic imaging , Adolescent , Cerebral Cortex/diagnostic imaging , Cerebral Cortex/physiology , Cerebral Cortex/surgery , Child , Child, Preschool , Drug Resistant Epilepsy/psychology , Drug Resistant Epilepsy/surgery , Epilepsy, Temporal Lobe/psychology , Epilepsy, Temporal Lobe/surgery , Female , Humans , Male , Memory, Short-Term/physiology , Temporal Lobe/physiology , Temporal Lobe/surgery , Young Adult
7.
Epilepsia ; 61(2): 228-229, 2020 02.
Article in English | MEDLINE | ID: mdl-32030740
8.
Epilepsia ; 56(7): 1065-70, 2015 Jul.
Article in English | MEDLINE | ID: mdl-25959880

ABSTRACT

OBJECTIVE: There are limited data on the indications for the use of chronic invasive electroencephalography (EEG) monitoring (IEM) for pediatric epilepsy surgery. METHODS: We retrospectively studied 102 children who underwent intracranial monitoring to map critical cortex, localize the epileptogenic region, or resolve divergent findings. We assessed IEM utility based on changes to the resection plan following analysis of noninvasive data. RESULTS: IEM was judged useful in 87% of cases and had greatest utility for resolving discordant data and localizing extratemporal and multilobar epileptogenic zones. IEM data were least useful for seizure onset in the temporal lobe and had little utility for direct cortical stimulation mapping unless functional magnetic resonance imaging (fMRI) revealed atypical language representation or the epileptogenic zone was in proximity to critical cortex. SIGNIFICANCE: IEM utility was demonstrated for a majority of cases with well-defined indications. The method of assessing utility will facilitate multicentric studies toward developing future consensus and practice guidelines.


Subject(s)
Electrodes, Implanted , Electroencephalography/statistics & numerical data , Epilepsy/diagnosis , Epilepsy/surgery , Monitoring, Intraoperative/statistics & numerical data , Adolescent , Child , Child, Preschool , Electroencephalography/methods , Epilepsy/physiopathology , Female , Humans , Male , Monitoring, Intraoperative/methods , Retrospective Studies , Young Adult
9.
Epilepsia ; 56(10): 1590-8, 2015 Oct.
Article in English | MEDLINE | ID: mdl-26249524

ABSTRACT

OBJECTIVE: To report the feasibility, safety, and clinical outcomes of an exploratory study of MR-guided Laser Interstitial Thermal Therapy (MRgLITT) as a minimally invasive surgical procedure for the ablation of epileptogenic foci in children with drug-resistant, lesional epilepsy. METHODS: Retrospective chart review of all MRgLITT procedures at a single tertiary care center. All procedures were performed using a U.S. Food and Drug Administration (FDA)-cleared surgical laser ablation system (Visualase Thermal Therapy System). Predefined clinical and surgical variables were extracted from archived medical records. RESULTS: Seventeen patients underwent 19 MRgLITT procedures from May 2011 to January 2014. Mean age at seizure onset was 7.1 years (range 0.1-14.8 years). Mean age at surgery was 15.3 years (range 5.9-20.6 years). Surgical substrates were mixed but mainly composed of focal cortical dysplasia (n = 11). Complications occurred in four patients. Average length of hospitalization postsurgery was 1.56 days. Mean follow-up was 16.1 months (n = 16; range 3.5-35.9 months). Engel class I outcome was achieved in seven patients (7/17; 41%), Engel class II in one (1/17; 6%), Engel class III in three (3/17; 18%), and Engel class IV in six (6/17; 35%). Three patients (3/8; 38%) with class I and II outcomes and five patients (5/9; 56%) with class III and IV outcomes had at least one prior resection. Fisher's exact test was not statistically significant for the association between Engel class outcome and previous resection (p = 0.64). SIGNIFICANCE: This study provides descriptive results regarding the use of MRgLITT in a mixed population of pediatric, lesional, drug-resistant epilepsy cases. The ability to classify case-specific outcomes and reduce technical complications is anticipated as experience develops. Further multicenter, prospective studies are required to delineate optimal candidates for MRgLITT, and larger cohorts are needed to more accurately define outcome and complication rates.


Subject(s)
Drug Resistant Epilepsy/therapy , Laser Therapy/methods , Neuroimaging , Adolescent , Child , Electroencephalography , Female , Humans , Male , Retrospective Studies , Video Recording , Young Adult
10.
Epilepsia ; 56(11): 1669-86, 2015 Nov.
Article in English | MEDLINE | ID: mdl-26434565

ABSTRACT

Our inability to adequately treat many patients with refractory epilepsy caused by focal cortical dysplasia (FCD), surgical inaccessibility and failures are significant clinical drawbacks. The targeting of physiologic features of epileptogenesis in FCD and colocalizing functionality has enhanced completeness of surgical resection, the main determinant of outcome. Electroencephalography (EEG)-functional magnetic resonance imaging (fMRI) and magnetoencephalography are helpful in guiding electrode implantation and surgical treatment, and high-frequency oscillations help defining the extent of the epileptogenic dysplasia. Ultra high-field MRI has a role in understanding the laminar organization of the cortex, and fluorodeoxyglucose-positron emission tomography (FDG-PET) is highly sensitive for detecting FCD in MRI-negative cases. Multimodal imaging is clinically valuable, either by improving the rate of postoperative seizure freedom or by reducing postoperative deficits. However, there is no level 1 evidence that it improves outcomes. Proof for a specific effect of antiepileptic drugs (AEDs) in FCD is lacking. Pathogenic mutations recently described in mammalian target of rapamycin (mTOR) genes in FCD have yielded important insights into novel treatment options with mTOR inhibitors, which might represent an example of personalized treatment of epilepsy based on the known mechanisms of disease. The ketogenic diet (KD) has been demonstrated to be particularly effective in children with epilepsy caused by structural abnormalities, especially FCD. It attenuates epigenetic chromatin modifications, a master regulator for gene expression and functional adaptation of the cell, thereby modifying disease progression. This could imply lasting benefit of dietary manipulation. Neurostimulation techniques have produced variable clinical outcomes in FCD. In widespread dysplasias, vagus nerve stimulation (VNS) has achieved responder rates >50%; however, the efficacy of noninvasive cranial nerve stimulation modalities such as transcutaneous VNS (tVNS) and noninvasive (nVNS) requires further study. Although review of current strategies underscores the serious shortcomings of treatment-resistant cases, initial evidence from novel approaches suggests that future success is possible.


Subject(s)
Drug Resistant Epilepsy/diagnosis , Drug Resistant Epilepsy/therapy , Malformations of Cortical Development/diagnosis , Malformations of Cortical Development/therapy , Anticonvulsants/therapeutic use , Drug Resistant Epilepsy/epidemiology , Electroencephalography/methods , Humans , Magnetic Resonance Imaging/methods , Malformations of Cortical Development/epidemiology , Treatment Outcome
11.
Epilepsia ; 55 Suppl 3: 34-6, 2014 Aug.
Article in English | MEDLINE | ID: mdl-25209084

ABSTRACT

Children with intractable epilepsy benefit from early surgical treatment when possible to reduce long-term morbidity. Issues for transition are variable according to type and timing of surgery, and the outcome. When surgery is successful, cognitive and behavioral improvements may not be apparent for several years. The value of continuing antiepileptic drug (AED) treatment after successful surgery remains unclear, and the adjustment to a life without seizures may be challenging.


Subject(s)
Anterior Temporal Lobectomy , Epilepsies, Partial/physiopathology , Transition to Adult Care , Adult , Age of Onset , Anterior Temporal Lobectomy/methods , Child , Epilepsies, Partial/surgery , Humans , Risk , Treatment Outcome
12.
Epilepsy Behav ; 31: 117-22, 2014 Feb.
Article in English | MEDLINE | ID: mdl-24397914

ABSTRACT

The current definition of epilepsy emphasizes the importance of cognitive impairment for a complete understanding of the disorder. Cognitive deficits have distinct functional manifestations that differentially impact the daily life experiences of children and adolescents with epilepsy and are a particular concern as they frequently impair academic performance. In particular, memory impairment and executive dysfunction are common disabilities in adults with temporal lobe epilepsy but are less easily recognized and studied in the pediatric population. This review focuses on the consequences of early-onset temporal lobe epilepsy for the development of memory and executive function and discusses current theories to explain these deficits.


Subject(s)
Cognition Disorders/etiology , Epilepsy, Temporal Lobe/complications , Memory Disorders/etiology , Child , Humans , Neuropsychological Tests
13.
Epilepsia ; 54(12): 2060-70, 2013 Dec.
Article in English | MEDLINE | ID: mdl-24304432

ABSTRACT

Nearly one third of patients with epilepsy become medically intractable, and the likelihood of achieving seizure freedom decreases with each additional medication trial. For appropriately chosen patients, epilepsy surgery affords the opportunity to achieve seizure freedom and potentially wean off medications. Epilepsy surgery, as with medical management, is not without adverse effects; to counsel patients wisely, practitioners need to understand the advantages and disadvantages of both. Randomized controlled trials in temporal lobe epilepsy reveal that epilepsy surgery achieves superior outcome compared to continued medical management. Although seizure freedom is the ultimate goal of any therapy, it represents a single outcome measure among a variety of other domains that affect patient welfare. It is imperative that providers understand the patient variables that affect these outcome measures and how these measures impact each other. Because the data comparing surgical therapy versus medical management for refractory epilepsy are limited, we review the available evidence comparing outcomes beyond seizure freedom including quality of life, cognition, psychosocial function, mortality, and financial costs.


Subject(s)
Epilepsy/surgery , Anticonvulsants/therapeutic use , Cognition , Epilepsies, Partial/drug therapy , Epilepsies, Partial/surgery , Epilepsy/drug therapy , Epilepsy/economics , Epilepsy/mortality , Epilepsy, Temporal Lobe/surgery , Health Care Costs , Humans , Seizures/prevention & control , Treatment Outcome
14.
Epilepsia ; 54(2): 351-8, 2013 Feb.
Article in English | MEDLINE | ID: mdl-23293926

ABSTRACT

PURPOSE: To assess the predictive value of ictal single-photon emission computed tomography (SPECT) for outcome after excisional epilepsy surgery in a large population of children with focal cortical dysplasia (FCD). METHODS: One hundred seventy-three ictal SPECT studies in 106 children with histologically proven FCD were retrospectively analyzed. The extent and location of ictal hyperperfusion and completeness of surgical removal were assessed. Completeness of resection of epileptogenic regions defined by ictal SPECT, electroencephalography (EEG), and magnetic resonance imaging (MRI) were compared and correlated with postoperative seizure outcome. In addition, subcortical activation of the cerebellum, basal ganglia, and thalamus were analyzed. KEY FINDINGS: The extent of hyperperfusion was focal or lobar in 58%, whereas multilobar activations occurred in only 32%; hemispheric or bilateral findings were rare. Favorable postsurgical seizure outcome was achieved in 67% patients with nonlocalized SPECT findings, 45% with nonresected ictal hyperperfusion, 36% with partially resected ictal hyperperfusion, and 86% when the zone of ictal hyperperfusion was completely resected (p = 0.000198). The favorable postsurgical outcome after complete removal of the SPECT hyperperfusion zone surpassed the 75% rate of seizure freedom in patients with removal of MRI/EEG-defined epileptogenic region. A similar predictive value of ictal SPECT for seizure outcome was found in nonoperated patients and subjects who were undergoing reoperation. Subcortical activation conferred no predictive value. SIGNIFICANCE: Ictal SPECT helps to define the epileptogenic zone in a high proportion of children with FCD undergoing surgical evaluation. Complete removal of both SPECT and MRI/EEG-defined regions is a strong predictor of surgical success and has important implications for surgical planning.


Subject(s)
Electroencephalography/methods , Magnetic Resonance Imaging/methods , Malformations of Cortical Development/diagnostic imaging , Malformations of Cortical Development/pathology , Tomography, Emission-Computed, Single-Photon/methods , Adolescent , Adult , Brain/diagnostic imaging , Brain/pathology , Cerebrovascular Circulation/physiology , Child , Child, Preschool , Epilepsy/diagnostic imaging , Epilepsy/pathology , Female , Humans , Infant , Male , Malformations of Cortical Development/surgery , Neurosurgical Procedures , Retrospective Studies , Young Adult
15.
Epilepsia ; 54(11): 1913-21, 2013 Nov.
Article in English | MEDLINE | ID: mdl-24117179

ABSTRACT

PURPOSE: Variable predictors of postsurgical seizure outcome have been reported in children with tuberous sclerosis complex (TSC). We analyzed a large surgical series of pediatric TSC patients in order to identify prognostic factors crucial for selection of subjects for epilepsy surgery. METHODS: Thirty-three children with TSC who underwent excisional epilepsy surgery at Miami Children's Hospital were retrospectively reviewed. A total of 29 clinical, neuropsychological, electroencephalography (EEG), magnetic resonance imaging (MRI), and surgical variables were analyzed and related to seizure outcomes. Univariate Barnard's exact test, Wilcoxon's rank-sum test, and multivariate statistical Cox's model were used to examine the significance of associations between the variables and seizure outcome. KEY FINDINGS: Eighteen patients (55%) have been seizure-free 2 years after (final) surgery; postoperative complications occurred in five subjects (15%). Complete removal of epileptogenic tissue detected by both MRI and intracranial EEG, regional scalp interictal EEG patterns, and agreement of interictal and ictal EEG localization were the most powerful predictors of seizure-free outcome. Other significant predictors included occurrence of regional scalp ictal EEG patterns, fewer brain regions affected by tubers, presence of preoperative hemiparesis, and one-stage surgery. Remaining factors such as age at seizure onset, incidence of infantile spasms or other seizure types, duration of epilepsy, seizure frequency, mental retardation, as well as types and extent of resections did not influence outcome. SIGNIFICANCE: Perioperative features rather than preoperative variables are the most important determinants of postsurgical seizure outcome in patients with TSC. Our findings may assist in the surgical management of these patients.


Subject(s)
Electroencephalography , Epilepsy/surgery , Tuberous Sclerosis/surgery , Child, Preschool , Electroencephalography/methods , Epilepsy/complications , Female , Humans , Infant , Infant, Newborn , Magnetic Resonance Imaging/methods , Male , Retrospective Studies , Treatment Outcome , Tuberous Sclerosis/complications
16.
Epileptic Disord ; 15(4): 383-91, 2013 Dec.
Article in English | MEDLINE | ID: mdl-24342862

ABSTRACT

AIMS: To identify variables that influence the extent of ictal single-photon emission computed tomography (SPECT) findings in paediatric patients with focal cortical dysplasia (FCD). METHODS: We visually evaluated 98 ictal SPECT studies from 67 children treated surgically for intractable epilepsy caused by FCD. SPECT findings were classified as "non-localised", "well-localised", and "extensive" and compared with parameters of injected seizures (seizure type and duration, injection time, and scalp EEG ictal pattern), presence of structural pathology on MRI, type of surgery performed after SPECT study, and histological findings. RESULTS: A shorter injection time and duration of injected seizure was associated with more localised SPECT hyperperfusion. SPECT findings were not significantly influenced by type of injected seizure. Widespread ictal scalp EEG patterns were associated with extensive SPECT findings. Larger zones of hyperperfusion were more common in patients with lesional MRI and patients undergoing multilobar resections. SPECT studies demonstrating good localisation were more common in patients with mild malformations of cortical development. CONCLUSION: Early ictal SPECT radiotracer injection is crucial for successful localisation of the epileptogenic zone. Seizure duration, type of scalp EEG findings, and presence of structural pathology on MRI may influence the extent of ictal SPECT hyperperfusion, which was associated with certain types of epilepsy surgery as well as histopathological findings.


Subject(s)
Epilepsy/diagnostic imaging , Tomography, Emission-Computed, Single-Photon , Child , Child, Preschool , Electroencephalography/methods , Epilepsy/pathology , Epilepsy/physiopathology , Female , Humans , Magnetic Resonance Imaging/methods , Male , Predictive Value of Tests , Tomography, Emission-Computed, Single-Photon/methods
18.
Brain ; 139(Pt 10): 2583-2586, 2016 Oct.
Article in English | MEDLINE | ID: mdl-27671027
19.
J Neurosurg ; : 1-8, 2022 Jan 14.
Article in English | MEDLINE | ID: mdl-35171812

ABSTRACT

OBJECTIVE: Magnetic resonance-guided focused ultrasound (MRgFUS) is an incisionless procedure capable of thermoablation through the focus of multiple acoustic beams. Although MRgFUS is currently approved for the treatment of tremor in adults, its safety and feasibility profile for intracranial lesions in the pediatric and young adult population remains unknown. METHODS: The long-term outcomes of a prospective single-center, single-arm trial of MRgFUS at Nicklaus Children's Hospital in Miami, Florida, are presented. Patients 15-22 years of age with centrally located lesions were recruited, clinically consistent with WHO grade I tumors that require surgical intervention. This cohort consisted of 4 patients with hypothalamic hamartoma (HH), and 1 patient with tuberous sclerosis complex harboring a subependymal giant cell astrocytoma (SEGA). RESULTS: In each case, high-intensity FUS was used to target the intracranial lesion. Real-time MRI was used to monitor the thermoablations. Primary outcomes of interest were tolerability, feasibility, and safety of FUS. The radiographic ablation volume on intra- and postoperative MRI was also assessed. All 5 patients tolerated the procedure without any complications. Successful thermoablation was achieved in 4 of the 5 cases; the calcified SEGA was undertreated due to intratumor calcification, which prevented attainment of the target ablation temperature. The HHs underwent target tissue thermoablations that led to MR signal changes at the treatment site. For the patients harboring HHs, FUS thermoablations occurred without procedure-related complications and led to improvement in seizure control or hypothalamic hyperphagia. All 5 patients were discharged home on postoperative day 1 or 2, without any readmissions. There were no cases of hemorrhage, electrolyte derangement, endocrinopathy, or new neurological deficit in this cohort. CONCLUSIONS: This experience demonstrates that FUS thermoablation of centrally located brain lesions in adolescents and young adults can be performed safely and that it provides therapeutic benefit for associated symptoms.

20.
Epilepsia ; 52(9): 1699-704, 2011 Sep.
Article in English | MEDLINE | ID: mdl-21762450

ABSTRACT

PURPOSE: Medically refractory epilepsy caused by cortical tubers resulting from tuberous sclerosis complex (TSC) often requires surgical intervention. The locations of cortical tubers generally are determined by preoperative magnetic resonance imaging (MRI). In this pilot study, we explored the feasibility of using the optical characteristics of cortical tubers as a potential means to guide their resection intraoperatively. METHODS: Optical characteristics of normal cortex and cortical tubers were measured intraoperatively using diffuse reflectance spectroscopy in three children undergoing epilepsy surgery for drug-resistant seizures. Unique diffuse reflectance spectroscopic features of cortical tubers were identified and their physiologic associations determined. KEY FINDINGS: Diffuse reflectance spectra revealed several features that distinguish cortical tubers from normal cortex. In tubers, diffuse reflectance intensities at oxy- and deoxyhemoglobin isobestic points, like 500, 530, and 570 nm, were consistently higher than those in normal cortex. According to the profile of diffuse reflectance spectra from 520-580 nm, hemoglobin oxygenation in tubers often was lower than in normal cortex. SIGNIFICANCE: Albeit preliminary, our findings suggest that the optical characteristics of cortical tubers differ from intervening normal cortex, likely reflecting the lower cerebral blood volumes and reduced hemoglobin oxygenation of cortical tubers. The results of this study can be used to design biomedical instruments that aid tuberectomies.


Subject(s)
Cerebral Cortex/pathology , Tuberous Sclerosis/pathology , Analysis of Variance , Cerebral Cortex/diagnostic imaging , Child , Child, Preschool , Female , Fluorodeoxyglucose F18 , Humans , Magnetic Resonance Imaging/methods , Male , Mass Spectrometry , Pilot Projects , Positron-Emission Tomography , Spectrum Analysis , Tuberous Sclerosis/diagnostic imaging
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