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1.
Int J Pediatr Otorhinolaryngol ; 102: 154-156, 2017 Nov.
Article in English | MEDLINE | ID: mdl-29106865

ABSTRACT

Paradoxical vocal fold motion (PVFM) is characterized by vocal fold adduction during respiration. Benign Rolandic epilepsy (BRE) is the most common childhood epilepsy and can cause oropharyngolaryngeal or facial manifestations. A 9-year-old male presented with intermittent apnea lasting 30-60 seconds and presumed PVFM. The patient's physical and fiberoptic exam were normal. He was admitted and found to have episodes of oxygen desaturation, neck twitching, and tongue burning. An EEG revealed focal epilepsy. After starting anti-epileptic medications, he had resolution of symptoms. Our patient was eventually diagnosed with BRE, a focal onset epilepsy that can mimic primary otolaryngologic disease.


Subject(s)
Anticonvulsants/therapeutic use , Epilepsy, Rolandic/diagnosis , Vocal Cord Dysfunction/diagnosis , Apnea/etiology , Child , Diagnosis, Differential , Electroencephalography , Epilepsy, Rolandic/drug therapy , Fiber Optic Technology , Humans , Male , Vocal Cords
2.
J Neurosurg Pediatr ; 19(1): 56-62, 2017 Jan.
Article in English | MEDLINE | ID: mdl-27791702

ABSTRACT

OBJECTIVE Electrical status epilepticus of sleep (ESES) is a rare electrographic pattern associated with global regression, which is often poorly responsive to traditional epilepsy treatments and can have a devastating and permanent neurocognitive outcome. The authors analyzed clinical, electroencephalographic, and neuropsychological outcomes in 9 patients with refractory ESES treated with functional hemispherotomy to illustrate the wide clinical spectrum associated with the disease and explore the role of hemispherotomy in its treatment. METHODS During the period between 2003 and 2015, 80 patients underwent hemispherotomy at the authors' institution. Video electroencephalography (EEG) reports were reviewed for ESES or continuous spikes and waves during sleep (CSWS). Patients with preoperative ESES (> 85% slow-wave sleep occupied by spike waves), a unilateral structural lesion amenable to surgery, and more than 6 months of follow-up data were included in the analysis. Clinical data, EEG recordings, neuropsychological testing, and parental and clinician reports were retrospectively reviewed. RESULTS Nine patients were eligible for study inclusion. Age at seizure onset ranged from birth to 4.2 years (mean 1.9 years), age at ESES diagnosis ranged from 3.5 to 8.8 years (mean 6.0 years), and age at hemispherotomy ranged from 3.7 to 11.5 years (mean 6.8 years). All patients had drug-resistant epilepsy. The duration of epilepsy prior to hemispherotomy ranged from 2.7 to 8.9 years (mean ± SD, 5.0 ± 2.2 years). Engel Class I seizure outcome was observed in all 9 children, with a mean follow-up of 3.0 years (range 0.5-6.1 years). Hemispherotomy terminated ESES in 6 of 6 patients with available postoperative sleep EEG. All children had preoperative neuropsychological impairments. Developmental regression was halted postoperatively, but none of the children returned to their original pre-ESES baseline. Four children demonstrated academic gains, 2 of whom transitioned to mainstream classes. CONCLUSIONS Children with drug-resistant ESES and a unilateral structural lesion should be evaluated for hemispherotomy as they may experience the cessation of seizures, termination of ESES, and improvement in neuropsychological status.


Subject(s)
Electroencephalography/trends , Hemispherectomy/trends , Sleep/physiology , Status Epilepticus/diagnosis , Status Epilepticus/surgery , Child , Child, Preschool , Female , Follow-Up Studies , Humans , Male , Status Epilepticus/physiopathology
3.
Childs Nerv Syst ; 32(9): 1703-8, 2016 Sep.
Article in English | MEDLINE | ID: mdl-27465677

ABSTRACT

PURPOSE: Dravet syndrome (DS), also known as severe myoclonic epilepsy of infancy (SMEI), is a rare genetic disorder that results in severe childhood-onset epilepsy. Children with DS initially present with seizures in the first year of life that are often associated with fevers. With age, multiple seizure types develop. There are few reports and no guidelines regarding palliative surgical treatment for DS. Therefore, we reviewed our surgical experience with DS. METHODS: We conducted a retrospective review of all patients with genetically confirmed DS who underwent either vagal nerve stimulator (VNS) implantation or corpus callosotomy (CC) from May 2001 to April 2014 at our institution. All inpatient and outpatient relevant documentation were reviewed. Demographic information, genetic mutation, operation performed, and preoperative and postoperative seizure frequency were recorded. Inclusion criteria required greater than one-year postoperative follow-up. RESULTS: Seven children with DS were assessed. Six patients were treated with VNS and one patient was treated with CC. In one child, VNS was followed by CC as a secondary procedure. Therefore, in total, eight surgeries were performed on seven patients during the study period. At least 1 year elapsed from presentation to our hospital and surgery for all patients. Average time after the first seizure to VNS was 4.1 years, and the average time after the first seizure to CC was 7.6 years. The mean age of patients undergoing VNS implantation was 4.3 years, and the mean age for patients undergoing CC was eight. Average follow-up for all seven patients was 6.6 years. Seizures were decreased in five of the six patients with VNS and decreased in the two patients after CC. Four of the six patients who had VNS implanted had a greater than 50 % reduction in seizure frequency, and one of the six patients who had VNS implanted had a less than 50 % reduction in seizure frequency. One patient did not respond effectively to the VNS and had very limited change in seizure frequency. Both patients who had a CC had a greater than 50 % reduction in seizure frequency. CONCLUSIONS: Both VNS and CC in patients with DS can be effective at reducing seizure frequency. Patients with DS may benefit from earlier and more aggressive surgical intervention. Studies using larger patient cohorts will help clarify the role that surgery may play in the multidisciplinary approach to controlling seizures in DS. Further studies will help determine the appropriate timing of and type of surgical intervention.


Subject(s)
Corpus Callosum/surgery , Epilepsies, Myoclonic/diagnosis , Epilepsies, Myoclonic/surgery , Palliative Care/trends , Vagus Nerve Stimulation , Electroencephalography/trends , Epilepsies, Myoclonic/physiopathology , Epilepsy/diagnosis , Epilepsy/physiopathology , Epilepsy/surgery , Female , Humans , Infant , Male , Vagus Nerve Stimulation/trends
4.
Epilepsy Behav ; 37: 26-31, 2014 Aug.
Article in English | MEDLINE | ID: mdl-24967697

ABSTRACT

PURPOSE: Up to 14% of children with epilepsy continue to experience seizures despite having appropriate medical therapy and develop medically refractory epilepsy (MRE). Assessing clinical outcomes and therapeutic efficacy in children with MRE undergoing palliative epilepsy surgery has been challenging because of the lack of a quantitative instrument capable of estimating the clinical status of these patients. The ideal instrument would at once consider seizure control, neurodevelopment, caregiver burden, and quality of life. The purpose of this study was to develop and pilot the Pediatric Refractory Epilepsy Questionnaire (PREQ), a quantitative instrument to assess the severity and individual burden of epilepsy in children with MRE undergoing palliative epilepsy treatments. METHODS: The caregivers of 25 patients with MRE completed the PREQ and the Quality of Life in Childhood Epilepsy (QOLCE) measure and participated in a semistructured interview. Medical records of the patients were reviewed, an Early Childhood Epilepsy Severity Scale (E-CHESS) score was calculated, and a Global Assessment of Severity of Epilepsy (GASE) score was obtained for each patient. KEY FINDINGS: The initial PREQ was modified based on the analysis of responses, association with previously validated scales, comments from caregivers, and expertise of the PREQ panelists. Pediatric Refractory Epilepsy Questionnaire subscale scores were calculated based on clinical paradigm and compared with independent measures of seizure severity and quality of life. Significant correlations were observed between the seizure severity subscale and the GASE score (r=0.55) and between the mood subscale and the well-being score (r=0.61) on the QOLCE. Significant correlations were also observed between the caregiver rating of seizure severity and the GASE score (r=0.53), the social activity score (r=0.57), and the behavior score (r=0.43) on the QOLCE. Correlations between the caregiver rating of quality of life and the quality of life score (r=0.58) and the number of AEDs used (r=0.45) were also significant. SIGNIFICANCE: This pilot study is an initial, critical step in the development of the PREQ. The significant correlations between the PREQ subscales and the external epilepsy severity and quality of life measures lend preliminary support to our hypothesis that the PREQ is assessing the severity of epilepsy along with other important domains, such as mood, neurodevelopment, and quality of life. A larger prospective study of this modified PREQ is currently underway to further develop the PREQ.


Subject(s)
Epilepsy/complications , Quality of Life , Severity of Illness Index , Surveys and Questionnaires , Adolescent , Caregivers/psychology , Caregivers/statistics & numerical data , Child , Child, Preschool , Epilepsy/psychology , Female , Humans , Male , Pilot Projects , Prospective Studies , Reproducibility of Results , Seizures , Sickness Impact Profile
5.
Epilepsia ; 55(8): 1292-300, 2014 Aug.
Article in English | MEDLINE | ID: mdl-24836831

ABSTRACT

OBJECTIVE: FOXG1-related disorders are associated with severe intellectual disability, absent speech with autistic features, and epilepsy. Children with deletions or intragenic mutations of FOXG1 also have postnatal microcephaly, morphologic abnormalities of the corpus callosum, and choreiform movements. Duplications of 14q12 often present with infantile spasms, and have subsequent intellectual disability with autistic features. Long-term epilepsy outcome and response to treatment have not been studied systematically in a well-described cohort of subjects with FOXG1-related disorders. We report on the epilepsy features and developmental outcome of 23 new subjects with deletions or intragenic mutations of FOXG1, and 7 subjects with duplications. METHODS: Subjects had either chromosomal microarray or FOXG1 gene sequencing performed as part of routine clinical care. Development and epilepsy follow-up data were collected from medical records from treating neurologists and through telephone parental interviews using standardized questionnaires. RESULTS: Epilepsy was diagnosed in 87% of the subjects with FOXG1-related disorders. The mean age of epilepsy diagnosis in FOXG1 duplications was significantly younger than those with deletions/intragenic mutations (p = 0.0002). All of the duplication FOXG1 children with infantile spasms responded to hormonal therapy, and only one required long-term antiepileptic therapy. In contrast, more children with deletions/intragenic mutations required antiepileptic drugs on follow-up (p < 0.0005). All subjects with FOXG1-related disorders had neurodevelopmental disabilities after 3 years of age, regardless of the epilepsy type or intractability of seizures. All had impaired verbal language and social contact, and three duplication subjects were formally diagnosed with autism. Subjects with deletion/intragenic mutations, however, had significantly worse ambulation (p = 0.04) and functional hand use (p < 0.0005). SIGNIFICANCE: Epilepsy and developmental outcome characteristics allow clinicians to distinguish among the FOXG1-related disorders. Further genotype-phenotype studies of FOXG1 may help to elucidate why children develop different forms of developmental epilepsy.


Subject(s)
Epilepsy/diagnosis , Epilepsy/genetics , Forkhead Transcription Factors/genetics , Mutation/genetics , Nerve Tissue Proteins/genetics , Adolescent , Adult , Child , Child, Preschool , Cohort Studies , Female , Follow-Up Studies , Humans , Infant , Male , Treatment Outcome , Young Adult
6.
Neurosurgery ; 74(1): 17-28; discussion 28, 2014 Jan.
Article in English | MEDLINE | ID: mdl-24089047

ABSTRACT

BACKGROUND: Corpus callosotomy (CC) is a valuable palliative surgical option for children with medically refractory epilepsy due to generalized or multifocal cortical seizure onset. OBJECTIVE: To investigate the extent of CC resulting in optimal seizure control in a pediatric patient population and to evaluate the modification of seizure profile after various CC approaches. METHODS: The records of 58 children (3-22 years of age at the time of surgery) with medically refractory epilepsy who underwent CC between 1995 and 2011 were retrospectively reviewed. RESULTS: Anterior two thirds callosotomy resulted in resolution of absence (P = .03) and astatic (P = .03) seizures, whereas anterior two thirds callosotomy followed by second-stage completion resulted in resolution of generalized tonic-clonic (GTC) (P = .03), astatic (P = .005), and myoclonic (P = .03) seizures in addition to a trend toward resolution of absence seizures (P = .08). Single-stage upfront complete callosotomy resulted in resolution of absence (P = .002), astatic (P < .0001), myoclonic (P = .007), and complex partial (P = .008) seizures in addition to a trend toward resolution of GTC (P = .06). In comparing a composite of subjects who underwent anterior two thirds callosotomy alone or 2-stage complete callosotomy before the second stage to complete the callosotomy with subjects who underwent upfront complete CC, a more favorable outcome was found in those with the upfront complete CC (P = .02). CONCLUSION: Single-stage upfront complete callosotomy is effective in relieving a broader spectrum of seizure types than anterior two thirds callosotomy or 2-stage complete callosotomy in children. The advantages of single-stage complete callosotomy must be weighed against the potentially higher risk of neurological and operative complications.


Subject(s)
Corpus Callosum/surgery , Epilepsy/surgery , Split-Brain Procedure/methods , Adolescent , Child , Child, Preschool , Female , Humans , Male , Postoperative Complications/epidemiology , Retrospective Studies , Split-Brain Procedure/adverse effects , Treatment Outcome , Young Adult
7.
J Neurosurg Pediatr ; 7(2): 179-88, 2011 Feb.
Article in English | MEDLINE | ID: mdl-21284465

ABSTRACT

OBJECT: Patients undergoing epilepsy surgery without evidence of a lesion on MR imaging and without a temporal source for seizure onset generally have less favorable outcomes than patients with structural lesions or temporal onset. However, many of these patients are viable candidates for invasive monitoring and subsequent resection or multiple subpial transections (MSTs). The purpose of this study was to evaluate the surgical treatment of pediatric patients with extratemporal, nonlesional epilepsy in order to better understand the clinical and neuropsychological outcomes expected in this patient group. METHODS: Forty-three pediatric patients with negative results on MR imaging and lateralized, extratemporal findings on electroencephalography underwent invasive monitoring with grid and/or strip electrodes. Thirty-three subsequently had resection of an epileptogenic focus and/or MSTs. RESULTS: Outcome was classified as Engel class I or II in 54.5% of the patients who underwent resection/MSTs and Engel class III or IV in 45.5%. Use of MSTs was associated with poor outcome. Neuropsychological evaluation showed significant improvement in immediate auditory attention following surgery and revealed several significant results on subgroup analysis. Complications occurred in 14% of patients (a 7% rate per procedure). Ten patients (23%) underwent invasive monitoring without proceeding to therapeutic surgery because no epileptogenic region was amenable to resection. Neuropsychological outcomes were generally stable. CONCLUSIONS: Patients with extratemporal, nonlesional seizures are viable candidates for invasive monitoring with grid/strip electrodes, and good outcomes can be obtained with resective surgery. The use of MSTs may correlate with worse outcome. This study also provides additional data to assist in counseling patients on the risks of negative invasive monitoring, deficits resulting from resection/MSTs, and possible operative complications.


Subject(s)
Epilepsy/surgery , Child , Child, Preschool , Female , Humans , Infant , Male , Neuropsychological Tests , Retrospective Studies , Treatment Outcome
8.
Eur J Hum Genet ; 19(1): 102-7, 2011 Jan.
Article in English | MEDLINE | ID: mdl-20736978

ABSTRACT

Genome-wide high-resolution array analysis is rapidly becoming a reliable method of diagnostic investigation in individuals with mental retardation and congenital anomalies, leading to the identification of several novel microdeletion and microduplication syndromes. We have identified seven individuals with duplication on chromosome 14q11.2q13.1, who exhibited idiopathic developmental delay and cognitive impairment, severe speech delay, and developmental epilepsy. Among these cases, the minimal common duplicated region on chromosome 14q11.2q13.1 includes only three genes, FOXG1, C14orf23, and PRKD1. We propose that increased dosage of Forkhead Box G1 (FOXG1) is the best candidate to explain the abnormal neurodevelopmental phenotypes observed in our patients. Deletions and inactivating mutations of FOXG1 have been associated with a Rett-like syndrome characterized by hypotonia, irritability, developmental delay, hand stereotypies, and deceleration of head growth. FOXG1, encoding a brain-specific transcription factor, has an important role in the developing brain. In fact, in vivo studies in chicken brain demonstrated that overexpression of FOXG1 results in thickening of the neuroepithelium and outgrowth of the telencephalon and mesencephalum, secondary to a reduction in neuroepithelial cell apoptosis.


Subject(s)
Chromosomes, Human, Pair 14/genetics , Developmental Disabilities/genetics , Epilepsy/genetics , Forkhead Transcription Factors/genetics , Gene Duplication , Intellectual Disability/genetics , Language Development Disorders/genetics , Nerve Tissue Proteins/genetics , Adult , Child, Preschool , Comparative Genomic Hybridization , Female , Humans , Infant , Male , Oligonucleotide Array Sequence Analysis
9.
J Neurosurg Pediatr ; 4(4): 323-32, 2009 Oct.
Article in English | MEDLINE | ID: mdl-19795963

ABSTRACT

OBJECT: Hemispherotomy generally is performed in hemiparetic patients with severe, intractable epilepsy arising from one cerebral hemisphere. In this study, the authors evaluate the efficacy of hemispherotomy and present an analysis of the factors influencing seizure recurrence following the operation. METHODS: The authors performed a retrospective review of 49 patients (ages 0.2-20.5 years) who underwent functional hemispherotomy at their institution. The first 14 cases were traditional functional hemispherotomies, and included temporal lobectomy, while the latter 35 were performed using a modified periinsular technique that the authors adopted in 2003. RESULTS: Thirty-eight of the 49 patients (77.6%) were seizure free at the termination of the study (mean follow-up 28.6 months). Of the 11 patients who were not seizure free, all had significant improvement in seizure frequency, with 6 patients (12.2%) achieving Engel Class II outcome and 5 patients (10.2%) achieving Engel Class III. There were no cases of Engel Class IV outcome. The effect of hemispherotomy was durable over time with no significant change in Engel class over the postoperative follow-up period. There was no statistical difference in outcome between surgery types. Analysis of factors contributing to seizure recurrence after hemispherotomy revealed no statistically significant predictors of treatment failure, although bilateral electrographic abnormalities on the preoperative electroencephalogram demonstrated a trend toward a worse outcome. CONCLUSIONS: In the present study, hemispherotomy resulted in freedom from seizures in nearly 78% of patients; worthwhile improvement was demonstrated in all patients. The seizure reduction observed after hemispherotomy was durable over time, with only rare late failure. Bilateral electrographic abnormalities may be predictive of posthemispherotomy recurrent seizures.


Subject(s)
Epilepsy/surgery , Hemispherectomy/methods , Postoperative Complications/etiology , Adolescent , Anterior Temporal Lobectomy/methods , Child , Child, Preschool , Corpus Callosum/surgery , Epilepsy/diagnosis , Epilepsy/etiology , Female , Follow-Up Studies , Hippocampus/surgery , Humans , Image Processing, Computer-Assisted , Infant , Magnetic Resonance Imaging , Male , Microsurgery/methods , Postoperative Complications/diagnosis , Postoperative Complications/surgery , Recurrence , Reoperation , Retrospective Studies , Treatment Outcome , Young Adult
10.
Epilepsia ; 50(6): 1450-61, 2009 Jun.
Article in English | MEDLINE | ID: mdl-19054401

ABSTRACT

PURPOSE: Medically intractable seizures arising from the sensorimotor (rolandic) cortex present a formidable challenge for epileptologists and epilepsy surgeons. The objective of this study was to evaluate the safety and efficacy of surgical treatment for seizures in this region. METHODS: We retrospectively reviewed the medical records of 24 children and adolescents who underwent surgical management of refractory rolandic epilepsy at our institution. RESULTS: Patient age at the time of surgery ranged between 1.9 and 19.2 years, and median postoperative follow-up was 2.3 years. Three patients had clear lesions on preoperative magnetic resonance imaging (MRI) that correlated with noninvasive electroencephalography (EEG) and underwent single-stage lesionectomies. Of the 21 patients who underwent invasive monitoring, 19 underwent therapeutic procedures, including lesionectomy, topectomy, and multiple subpial transections. Engel class I and II outcomes were seen in five of six patients (84.0%) after lesionectomy and in seven of nine patients (77.0%) and four of six patients (66.7%) after sensorimotor corticectomy or multiple subpial transections (MSTs), respectively. At last follow-up, neurologic deficits were observed following 50.0% (n = 3 of 6) of lesionectomies and 67.0% (n = 7 of 9) of topectomies; no neurologic deficits were seen in patients undergoing motor cortex MSTs. No correlation existed between seizure outcome and duration of epilepsy, age at diagnosis, age at surgery, histopathology, or the presence or absence of a discrete lesion. DISCUSSION: Surgery for appropriately selected children with intractable seizures arising from rolandic cortex was effective and associated with minimal neurologic morbidity in many cases. Engel I-II outcome was achieved in 77% of patients overall, and neurologic deficits were often mild and improved over time. Lesionectomy resulted in the best seizure control, followed by topectomy, and then MSTs.


Subject(s)
Epilepsy, Rolandic/etiology , Epilepsy, Rolandic/surgery , Motor Cortex/pathology , Neurosurgical Procedures/methods , Seizures/physiopathology , Somatosensory Cortex/pathology , Adolescent , Child , Child, Preschool , Electroencephalography , Epilepsy, Rolandic/diagnostic imaging , Female , Follow-Up Studies , Humans , Infant , Magnetic Resonance Imaging/methods , Male , Motor Cortex/diagnostic imaging , Motor Cortex/surgery , Positron-Emission Tomography/methods , Retrospective Studies , Somatosensory Cortex/diagnostic imaging , Somatosensory Cortex/surgery , Tomography, Emission-Computed, Single-Photon , Videotape Recording/methods , Young Adult
11.
Arch Neurol ; 63(10): 1419-26, 2006 Oct.
Article in English | MEDLINE | ID: mdl-17030658

ABSTRACT

OBJECTIVE: To compare composite subtraction ictal single-photon emission computed tomography coregistered to magnetic resonance imaging (also known as SISCOM) patterns between right and left medial temporal-onset seizures to document neuroanatomical involvement in perfusion patterns. DESIGN: A retrospective comparative survey. SETTING: Epilepsy monitoring unit in a tertiary care referral center. PARTICIPANTS: Subjects with temporal lobe epilepsy (TLE) who underwent ictal single-photon emission computed tomography studies. MAIN OUTCOME MEASURES: Comparison of ictal perfusion pattern changes in subjects with right and left temporal seizures. RESULTS: Composite subtraction ictal single-photon emission computed tomography coregistered to magnetic resonance images showed similar regions of hyperperfusion change in the ipsilateral anteromedial temporal-corpus striatum-insula region in both groups. In the midbrain reticular formation, there was a significant difference in hyperperfusion between the left and right TLE groups. In addition, the right, but not the left, TLE group shows contralateral hypoperfusion of the temporoparietal junction. CONCLUSIONS: While anteromedial temporal-corpus striatum-insula perfusion patterns are similar, there are brainstem and hemispheric perfusion pattern differences in right and left TLE seizures, confirming pathophysiological differences between the groups. These findings help define neuronal network involvement in TLE seizures, and may explain the differences in clinical symptoms of right and left TLE seizures.


Subject(s)
Epilepsy, Temporal Lobe/diagnostic imaging , Epilepsy, Temporal Lobe/physiopathology , Magnetic Resonance Imaging/methods , Positron-Emission Tomography/methods , Temporal Lobe/diagnostic imaging , Temporal Lobe/physiopathology , Adult , Brain/anatomy & histology , Brain/diagnostic imaging , Brain/physiopathology , Brain Mapping/methods , Cerebrovascular Circulation , Female , Functional Laterality , Humans , Male , Middle Aged , Predictive Value of Tests , Retrospective Studies , Temporal Lobe/pathology
12.
J Nucl Med ; 46(1): 38-43, 2005 Jan.
Article in English | MEDLINE | ID: mdl-15632031

ABSTRACT

UNLABELLED: Our objective was to demonstrate common patterns of ictal cerebral blood flow changes in a group of patients with mesial temporal lobe epilepsy (MTLE) using the technique of composite subtraction ictal SPECT coregistered to MRI (SISCOM). METHODS: We retrospectively reviewed the video electroencephalography recordings, SPECT studies, and MR images of 32 MTLE patients and of a subgroup of 11 patients with pathologically verified mesial temporal sclerosis (MTS). Composite SISCOM studies were performed as previously described. The patients were grouped for analysis into those with right seizure onset and those with left seizure onset. The images of the right and left MTS subgroups were combined for analysis by rotating right MTS images to show changes on the left side. The SPECT subtractions were segmented to show regions of hyperperfusion at 1 SD above the mean. A rainbow color map was applied to the final composite SISCOM images to assist in the interpretation of results. Binomial probability was calculated to demonstrate the level of significance of perfusion changes. RESULTS: All patients demonstrated typical seizure semiology of MTLE. Seventeen patients had left MTLE and 15 patients had right MTLE. The levels of significance were set at 6 of 17 (P = 0.042) for the left MTLE group, 6 of 15 (P = 0.022) for the right MTLE group, and 5 of 11 (P = 0.021) for the MTS subgroup. Results among all groups were similar. The most-contiguous area of hyperperfusion was the anterior temporal area extending to include the insular cortex and basal ganglia, lateralizing to the side of seizure onset. CONCLUSION: Composite SISCOM studies in patients with well-localized MTLE most commonly show a region of hyperperfusion in the anterior temporal region, which often also involves the basal ganglia and insula, likely representing the primary regions of seizure propagation. Identifying this pattern of hyperperfusion as typical for mesial temporal onset seizures should assist in clinical interpretation and localization of ictal SPECT studies.


Subject(s)
Brain/blood supply , Brain/diagnostic imaging , Epilepsy, Temporal Lobe/diagnosis , Epilepsy, Temporal Lobe/physiopathology , Magnetic Resonance Imaging/methods , Subtraction Technique , Tomography, Emission-Computed, Single-Photon/methods , Adolescent , Adult , Blood Flow Velocity , Brain Mapping/methods , Cerebrovascular Circulation , Child , Female , Humans , Image Enhancement/methods , Image Interpretation, Computer-Assisted/methods , Male , Middle Aged
13.
Brain ; 127(Pt 8): 1731-40, 2004 Aug.
Article in English | MEDLINE | ID: mdl-15231583

ABSTRACT

MRI-based evaluation of the hippocampus is important in the assessment and treatment of patients with mesial temporal lobe epilepsy (MTLE). Using MRI-based large-deformation high-dimensional mapping (HDM-LD), which allows structural evaluation of regions of the hippocampus, we document the HDM-LD-defined pattern of hippocampal deformation in MTLE patients compared with matched controls. In 30 subjects with MTLE and confirmed medial temporal lobe sclerosis (MTS), we performed measurements of intracranial area, brain parenchymal volume and deformation-based hippocampal segmentations, and then grouped patients into right and left MTS groups (resulting in 15 subjects in each group). Using HDM-LD techniques, we compared the right and left MTS groups with a group of 15 matched controls. Analysis included both the MTS and contralateral hippocampi, and covariance for changes in brain parenchymal volume. Final results were interpreted using a segmentation showing normal hippocampal surface subfield anatomy. Comparing the MTS groups with controls, after covarying with brain parenchymal volume, the MTS hippocampi showed significant volume loss (P < 0.0001), contralateral hippocampi showed no significant volume loss. HDM-LD techniques showed significant shape changes, with marked inward deviation in the Sommer sector of the MTS hippocampi. In the contralateral hippocampi, the inferior surface of the hippocampal body showed inward deformation in the medial aspect of the subiculum, with minimal involvement of the Sommer sector. HDM-LD shows involvement of subregions of the hippocampus which are consistent with MTS histopathology. Contralateral hippocampi show different HDM-LD changes, suggesting that the underlying disease process in the contralateral hippocampi is different from MTS.


Subject(s)
Epilepsy, Temporal Lobe/pathology , Hippocampus/pathology , Adult , Brain/pathology , Brain Mapping/methods , Female , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Retrospective Studies , Sclerosis , Temporal Lobe/pathology
14.
Intensive Care Med ; 30(2): 207-212, 2004 Feb.
Article in English | MEDLINE | ID: mdl-14615839

ABSTRACT

OBJECTIVE: When emergent or continuous EEG monitoring is carried out to detect subclinical seizures, expert interpretation of the results is usually not immediately available. We assessed the ability to recognize epileptiform discharges among various nonexpert bedside caregivers. A second objective was to determine whether such skill improves with a simple educational intervention. DESIGN AND SETTING: A prospective cohort study with an educational intervention in a university-based tertiary medical center. PARTICIPANTS: All 125 neurology and neurosurgery residents, intensive care fellows, critical care and neurological floor nurses, and EEG technicians were offered participation. Of these, 50 completed the study and provided 2,398 EEG responses. INTERVENTIONS: A computer-based PowerPoint lecture designed to teach recognition of epileptiform discharges. MEASUREMENTS AND RESULTS: (a) Average number of correct responses in identifying epileptiform discharges in a computer-based pre-test consisting of 24 EEG questions. (b) Impact of the educational intervention as measured by change in the mean average score in a similar post-test. The rate of correct responses by group ranged from 46% for medical critical care nurses to 94% for EEG technicians. Overall mean correct response rate was 14.72 (61%) for the pre-test, and increased to 16.06 (67%) in for post-test (p=0.002). CONCLUSIONS: Recognition of epileptiform discharges by bedside caregivers is disturbingly low. A simple educational intervention only modestly improved such ability. Therefore we should not rely on nontrained personnel to interpret emergent or continuous recordings. EEG technicians are an exception, and their skill could be utilized during emergent studies.


Subject(s)
Electroencephalography , Epilepsy/diagnosis , Personnel, Hospital , Cohort Studies , Diagnosis, Computer-Assisted , Humans , Personnel, Hospital/standards , Prospective Studies
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