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Biomedical subjects

Elaine Wyllie

Publications and source records attributed to Elaine Wyllie.

18 recordsLinked to original sources

Seizures during intracarotid methohexital and amobarbital testing.

BACKGROUND: Methohexital and amobarbital have been used as agents for Wada testing in the presurgical evaluation of patients with epilepsy. Previous experience with methohexital as an anesthetic indicates that methohexital may decrease seizure threshold and may trigger seizures. METHODS: A retrospective chart review of 760 intracarotid amobarbital and methohexital tests was performed to determine the frequency of seizures associated with preoperative intracarotid barbiturate testing for language and memory lateralization. RESULTS: Sixteen patients (2.1%) who had seizures were found. In 3 patients, seizures occurred prior to barbiturate injection, and in 13, following barbiturate injection. After injection of amobarbital, 4 of 538 patients (0.7%) had a seizure. Nine of 222 patients had a seizure after methohexital injection (4.1%) (P=0.001). CONCLUSION: Patients with a previous history of epilepsy may be at higher risk for seizures after methohexital injection as compared with amobarbital injection.

Adolescent↗

Risk factors for carbamazepine elevation and toxicity following epilepsy surgery.

UNLABELLED: A proportion of patients undergoing epilepsy surgery, and receiving carbamazepine (CBZ), experience significant elevations in CBZ plasma concentrations, some with associated CBZ toxicity. The objective of this study was to identify significant risk factors for elevations (>12 microg/ml) in CBZ concentrations and CBZ-induced toxicity following epilepsy surgery. METHODS: We retrospectively examined charts of 74 inpatients (31 children and 43 adults) chronically receiving CBZ and undergoing epilepsy surgery between January 1996 and June 2000. Patient demographics, medications, type of surgery, seizure history, adverse events, CBZ doses and concentrations were evaluated. RESULTS: 51.2% of adults and 51.6% of pediatric patients had drug elevations. In the pediatric group, 12.9% had symptoms of toxicity compared to 9.3% in the adult group. Five risk factors-pre-operative CBZ dose, fentanyl dose, surgery day CBZ concentration, body weight, and blood loss-were related to post-operative CBZ concentrations. Three risk factors: age <18 years, pre-operative CBZ dose, and the surgery day CBZ (immediate pre-operative) concentration, were significantly related to the outcome measure of abnormal CBZ concentration (>12 microg/ml). Two variables significantly related to toxicity were average post-operative CBZ dose and the surgery day CBZ concentration. Increases in concentrations occurred at a mean 33+/-13.7 h (range: 11-74 h) after surgery. DISCUSSION: Based upon our results in patients with one or more risk factors, we suggest that reduction of post-operative CBZ doses be considered.

Adolescent↗

Hemispherectomy for catastrophic epilepsy in infants.

PURPOSE: To report our experience with hemispherectomy in the treatment of catastrophic epilepsy in children younger than 2 years. METHODS: In a single-surgeon series, we performed a retrospective analysis of 18 patients with refractory epilepsy undergoing hemispherectomy (22 procedures). Three different surgical techniques were performed: anatomic hemispherectomy, functional hemispherectomy, and modified anatomic hemispherectomy. Pre- and postoperative evaluations included extensive video-EEG monitoring, magnetic resonance imaging, and positron emission tomography scanning. Seizure outcome was correlated with possible variables associated with persistent postoperative seizures. The Generalized Estimation Equation (GEE) and the Barnard's exact test were used as statistical methods. RESULTS: The follow-up was 12-74 months (mean, 34.8 months). Mean weight was 9.3 kg (6-12.3 kg). The population age was 3-22 months (mean, 11.7 months). Thirteen (66%) patients were seizure free, and four patients had >90% reduction of the seizure frequency and intensity. The overall complication rate was 16.7%. No deaths occurred. Twelve (54.5%) of 22 procedures resulted in incomplete disconnection, evidenced on postoperative images. Type of surgical procedure, diagnosis categories, persistence of insular cortex, and bilateral interictal epileptiform activity were not associated with persistent seizures after surgery. Incomplete disconnection was the only variable statistically associated with persistent seizures after surgery (p<0.05). CONCLUSIONS: Hemispherectomy for seizure control provides excellent and dramatic results with a satisfactory complication rate. Our results support the concept that early surgery should be indicated in highly selected patients with catastrophic epilepsy. Safety factors such as an expert team in the pediatric intensive care unit, neuroanesthesia, and a pediatric epilepsy surgeon familiar with the procedure are mandatory.

Cerebral Cortex↗

Treatment options and paradigms in childhood temporal lobe epilepsy.

Temporal lobe epilepsy in adults is a relatively homogenous syndrome with hippocampal sclerosis being its most common pathologic substrate. In the pediatric age group, low-grade neoplasms and cortical dysplasia are much more common than hippocampal sclerosis. Pediatric temporal lobe epilepsy has distinct semiologic, electrophysiologic and imaging characteristics as compared with its adult counterpart. The various treatment options for pediatric temporal lobe epilepsy include antiepileptic drugs, resective surgery, vagal nerve stimulation and the ketogenic diet. In spite of the multiple antiepileptic drugs currently available, 5-10% of all newly diagnosed cases will remain intractable to medical therapy and should be referred for presurgical evaluation. Resective surgery offers the best chance of seizure freedom in carefully selected patients. Future areas of research include new drug development, better imaging and localization techniques, and brain stimulation.

Anticonvulsants↗

A proposal for a five-dimensional patient-oriented epilepsy classification.

The recent proposal by the ILAE Task Force for Epilepsy Classification consists of a multi-axial syndrome-oriented approach. Epilepsy syndromes, as defined by the ILAE, group patients according to various, poorly defined parameters. The resulting syndromes have frequently no biological significance, with overlap among different syndromes and syndromes changing with age. Additionally, only a minority of patients can be classified syndromatically, and the axes of this classification system convey redundant information. We propose a five-dimensional, patient-oriented approach to classifying epilepsies. This approach shifts from the syndrome-oriented approach to a standard, neurological, methodological, patient-oriented approach, using independent criteria in each of the five dimensions. Similar to general neurology, the first step in each patient-physician encounter in epileptology is to take a history of the presenting symptoms and generate a hypothesis regarding the localization and etiology of the symptom within the nervous system. Therefore, the main dimensions of this classification consist of: 1) localization of the epileptogenic zone, 2) seizure semiology classified according to the semiological seizure classification, 3) etiology, 4) seizure frequency, and 5) related medical conditions. These dimensions characterize all of the information necessary for patient management, are independent parameters, and include more pertinent information with regards to patient management than the ILAE axes. All patients can be classified according to this five-dimensional system even at the initial patient encounter when no detailed test results are available. Information from clinical tests, such as MRI and EEG, are translated into the best possible working hypothesis at the time of classification, allowing for increasing precision of the classification as additional information becomes available. This patient-oriented classification envisions an epileptic seizure as an independent symptom of a central nervous system dysfunction due to different causes, with various cortical localizations, occurring at various frequencies, and in conjunction with other diseases and clinical symptoms.

Adult↗

Hemispheric malformations of cortical development.

The term hemispheric malformations of cortical development (MCDs) is used in this article to represent congenital malformations that predominantly or exclusively involve complete or substantial portion of one cerebral hemisphere. Hemispheric MCDs usually present during early childhood with intractable epilepsy, cognitive delay, and contralateral hemiparesis. Early identification and selection of children who may be candidates for epilepsy surgery are important during diagnostic evaluation of hemispheric MCD. Surgical intervention at an appropriate time, whenever possible, offers the best chance for seizure freedom and improved cognitive outcome. In addition, making an accurate genetic diagnosis is imperative to offer genetic testing and counseling to the family. In this article, the authors discuss the developmental and genetic mechanisms, pathologic features, diagnosis, and treatment of hemispheric MCD that are substrates of catastrophic childhood epilepsy amenable to surgical treatment.

Brain Diseases↗

Lateralizing signs during seizures in infants.

OBJECTIVE: To identify lateralizing features during seizures in infants and assess their reliability. METHODS: Infants were included if they had video-EEG monitoring at our institution, and were seizure-free for at least 12 months after epilepsy surgery. Lateralizing signs and seizure symptomatology were classified based on blinded video review. RESULTS: We analyzed 100 seizures from 19 infants (1 to 32, mean 13 months of age) (1 to 14 seizures per patient). Potential lateralizing signs were seen in 58 seizures from 12 infants, including unilateral clonic jerking (8 patients); forced, sustained tonic version of the eyes to one side (7 patients); predominantly unilateral infantile spasms (5 patients); unilateral tonic stiffening of an arm and leg (2 patients); nystagmus (2 patients) and postictal hemiparesis (1 patient). Except for tonic eye version, each of the signs was contralateral to the hemisphere of seizure-onset in all but one patient who had predominantly ipsilateral spasms and clonic arm activity. Tonic eye version was contralateral in 3 patients, ipsilateral in 1 patient, and toward either side in different seizures in 3 patients. CONCLUSION: Reliable lateralizing signs included focal clonic activity and predominantly unilateral spasms. Focal tonic activity, nystagmus and postictal hemiparesis were also consistently contralateral but were observed only in few patients. Tonic eye version was unreliable and could not be used to lateralize seizure onset. The sequence of eye and head version evolving to generalized tonic clonic convulsions was not seen in this age group.

Child, Preschool↗

Ictal SPECT in children with partial epilepsy due to focal cortical dysplasia.

We studied the usefulness of ictal single-photon emission computed tomography in the presurgical evaluation of children with partial epilepsy resulting from focal cortical dysplasia. Fifteen children, age 1-18 years, were identified with partial epilepsy caused by focal cortical dysplasia (confirmed by histology) who underwent subtraction ictal single-photon emission computed tomography during presurgical evaluation. All children later underwent surgery at the Cleveland Clinic Epilepsy Center between 1996 and 2000. The findings of ictal single-photon emission computed tomography and brain positron emission tomography were classified as localized when "localizing and concordant" with the surgical resection site, nonconcordant when "localizing but not concordant" with the surgical resection, or nonlocalized when "no well-localized region of ictal hyperperfusion was observed on the difference image". In 15 patients, age 1.5-18 years (median age 8 years), epilepsy was classified as frontal in 7, posterior temporal/occipital in 3, temporal in 2, multilobar in 2, and parietal in 1. Of 15 patients, preoperative magnetic resonance imaging revealed focal cortical dysplasia in 11, positron emission tomography was localized in 9, and ictal single-photon emission computed tomography was localized in 8 patients. In 4 patients with normal magnetic resonance imaging but scalp electroencephalographic findings of partial epilepsy, ictal single-photon emission computed tomography and positron emission tomography were localized in 3 each. Fourteen patients were monitored for 6-39 months (mean 20 months). Six of 7 patients (85%) with localized ictal single-photon emission computed tomography compared with 4 of 7 (57%) with nonconcordant/nonlocalized ictal single-photon emission computed tomography had no seizures at follow-up. In 4 patients with normal magnetic resonance imaging, 3 patients with localized ictal single-photon emission computed tomography were free of seizures compared with 1 with nonconcordant ictal single-photon emission computed tomography who continued to have seizures. Ictal single-photon emission computed tomography is a useful adjunctive test in presurgical evaluation of children with refractory partial epilepsy due to focal cortical dysplasia, especially when brain magnetic resonance imaging is normal.

Adolescent↗

Specific epileptic syndromes are rare even in tertiary epilepsy centers: a patient-oriented approach to epilepsy classification.

PURPOSE: To assess the practicability and reliability of a five-dimensional patient-oriented epilepsy classification and to compare it with the International League Against Epilepsy (ILAE) classification of epilepsy and epileptic syndromes. The dimensions consist of the epileptogenic zone, semiologic seizure type(s), etiology, related medical conditions, and seizure frequency. METHODS: The 185 epilepsy patients (94 adults, 91 children, aged 18 years or younger) were randomly selected from the database of a tertiary epilepsy center and the general neurological department of a metropolitan hospital (28 adults). The charts were reviewed independently by two investigators and classified according to both the ILAE and the patient-oriented classification. Interrater reliability was assessed, and a final consensus among all investigators was established. RESULTS: Only four (4%) adults and 19 (21%) children were diagnosed with a specific epilepsy syndrome of the ILAE classification. All other patients were in unspecific categories. The patient-oriented classification revealed that 64 adults and 56 children had focal epilepsy. In an additional 34 adults and 45 children, the epileptogenic zone could be localized to a certain brain region, and in 14 adults and five children, the epileptogenic zone could be lateralized. Fourteen adults and 21 children had generalized epilepsy. In 16 adults and 14 children, it remained unclear whether the epilepsy was focal or generalized. Generalized simple motor seizures were found in 66 adults and 52 children, representing the most frequent seizure type. Etiology could be determined in 40 adults and 45 children. Hippocampal sclerosis was the most frequent etiology in adults (10%), and cortical dysplasia (9%), in children. Seven adults and 31 children had at least daily seizures. Seventeen adults and 26 children had rare or no seizures at their last documented contact. The most frequent related medical conditions were psychiatric disorders and mental retardation. Interrater agreement was high (kappa values of 0.8 to 0.9) for both the patient-oriented and the ILAE classification. CONCLUSIONS: Specific epilepsy syndromes included in the current ILAE classification are rare even in a tertiary epilepsy center. Most patients are included in unspecific categories that provide only incomplete information. In contrast, all of the patients could be classified by the five-dimensional patient-oriented classification, providing all essential information for the management of the patients with a high degree of interrater reliability.

Adolescent↗

Benign focal epileptiform discharges of childhood and hippocampal sclerosis.

PURPOSE: Benign focal epileptiform discharges of childhood (BFEDCs) are common EEG findings between ages 4 and 14 years. This epoch of maturational development overlaps with the age at presentation of temporal lobe epilepsy (TLE) due to hippocampal sclerosis (HS) in children. METHODS: From our series of 17 preadolescent children who eventually underwent anteromesial temporal resection for medically refractory TLE due to HS, we identified two children, plus one thereafter, who were initially dismissed as candidates for epilepsy surgery because of abundant extratemporal sharp waves, which were bilateral in two cases. The sharp waves had the distinctive morphology, distribution, and sleep activation suggestive of BFEDCs, but the medical intractability and seizure symptoms were unusual for benign focal epilepsy of childhood. RESULTS: In each case, surgical candidacy was clarified when magnetic resonance imaging (MRI) showed unilateral HS and video-EEG demonstrated seizure onset in the ipsilateral anteromesial temporal region. The postoperative freedom from seizures in each case (follow-up, 2 to 4 years) confirmed that HS was the primary epileptogenic process, and that the BFEDCs were incidental or an atypical secondary manifestation. CONCLUSIONS: These cases illustrate the need for more extensive study of children with BFEDCs when medical intractability and seizure symptoms speak against a simple diagnosis of benign focal epilepsy of childhood. In addition, we observed that the BFEDCs in two of our children had an unusual bilateral occipitofrontal distribution, and we speculate that the coexistence of the BFEDCs in children with HS may not be an incidental finding.

Child↗

Syringomyelia presenting as paroxysmal arm posturing resembling seizures.

A 9-year-old female presented with daily episodes of medically refractory paroxysmal bilateral arm posturing, which had long been thought to be epileptic seizures. She also had other types of episodes, including daily staring spells and infrequent generalized tonic-clonic convulsions. Neurologic examination was normal except for delayed cognitive development. The results of previous electroencephalograms (EEG) were normal, and magnetic resonance imaging of the head revealed a Chiari I malformation. Video EEG monitoring revealed no EEG changes during the attacks, and magnetic resonance imaging of the spine revealed a large cervical syrinx associated with the Chiari malformation. The episodes of paroxysmal bilateral dystonic arm posturing resolved after surgical intervention for the syrinx. This report illustrates that cervical cord disease is an unusual although potentially treatable condition to be considered in the differential diagnosis of paroxysmal episodes with dystonic movements of the arms, even in the absence of other physical findings of myelopathy.

Arm↗

Outcome after surgical treatment.

Approximately 50% of patients will become seizure free following surgery for intractable epilepsy caused by malformations of cortical development. Another 20% have only rare seizures. The outcome is dependent on the completeness of the resection as determined by MR imaging and electroencephalography. The surgical procedures performed for malformations of cortical development in infants, children, and adults include lobar and multilobar resections or hemispherectomy.

Cerebral Cortex↗

Focal cortical dysplasias in eloquent cortex: functional characteristics and correlation with MRI and histopathologic changes.

PURPOSE: Focal cortical dysplasia (CD) is increasingly recognized as a common pathologic substrate of medically intractable epilepsy. As these lesions are often localized in the frontal lobe (therefore in potentially eloquent cortex), an understanding of the functional status of the involved region(s) and of its anatomic and pathologic correlates is of prime importance. The purpose of this study is to assess the function of focal CD in relation to magnetic resonance imaging (MRI) and histopathologic features. METHODS: Eight patients operated on for medically intractable epilepsy with histologically proven focal CD involving putative eloquent cortex in the frontal lobe (perirolandic and Broca's areas) were included in the study. Functional regions (motor and language) and epileptogenic areas were assessed by extraoperative electrocorticographic recording and electrical cortical mapping. Cortical functions were correlated with the extent of epileptogenicity on electrocorticographic recordings, MRI features, and histologic characteristics. RESULTS: Language or motor areas were colocalized with epileptogenic regions (n=6 of 8, 75%), but were not mapped in regions of increased signal on fluid-attenuated inversion recovery (FLAIR) MRI (when they were identified) on preoperative MRI (n=5 of 5, 100%). Histologically, balloon cells were almost exclusively found in nonfunctional regions with FLAIR MRI abnormalities. When resected, regions of motor cortex were characterized by cortical dyslamination, columnar disorganization, and dysmorphic neurons, but were devoid of balloon cells. CONCLUSIONS: We found an absence of language or motor functions in perirolandic and Broca's areas that showed decreased epileptogenicity, histopathological evidence of CD with balloon cells and FLAIR MRI signal increase. Language and motor functions were present in epileptogenic and dysplastic areas with no balloon cells and no FLAIR signal abnormalities. These findings have implications on options for epilepsy surgery in patients with CD.

Adolescent↗

Hypomotor seizures in infants and children.

PURPOSE: Hypomotor seizures (characterized by diminished behavioral activity with indeterminate level of consciousness) have been identified as an important seizure type in infants. Our goal was to investigate further the clinical and EEG features of hypomotor seizures. METHODS: We retrospectively reviewed 110 hypomotor seizures from 34 patients recorded with video-EEG. RESULTS: Twenty-seven (79%) patients were younger than 48 months, and seven (21%) were aged 4 to 15 years. Seventy-one (64%) seizures had regional or lateralized EEG onset, arising predominantly from temporal or parietal lobe regions. The other 39 (35%) seizures had generalized onset, usually with abrupt onset of diffuse rhythmic high-amplitude theta activity or diffuse electrodecrement and only rarely (two patients) with slow spike-wave complexes or 3-Hz spike-wave complexes. Hypomotor seizures with generalized EEG onset were significantly shorter than those with regional or lateralized onset (p = 0.01, GEE model). Unsustained head or eye movements and subtle mouth automatisms were commonly seen in hypomotor seizures with either focal or generalized onset. Seventeen percent of hypomotor seizures with focal onset evolved to include version of head and eyes or jerking of one arm, whereas 2% of generalized hypomotor seizures evolved to a cluster of spasms. CONCLUSIONS: Hypomotor seizures may be either focal or generalized. Regional EEG onsets were most often temporal or parietal, suggesting that focal hypomotor seizures may be a bland form of "complex partial" seizures with no or minimal automatisms, seen predominantly in infants. Generalized hypomotor seizures were rarely associated with an ictal pattern of generalized spike-wave complexes, suggesting a different mechanism from absence seizures seen later in life.

Adolescent↗

Paroxysmal nonepileptic events in children and adolescents.

OBJECTIVE: Paroxysmal nonepileptic events (PNEs) are frequently encountered in children and adolescents; however, there is little information concerning the relative frequency of various types of these disorders. We report our experience with PNEs in a group of children and adolescents who underwent prolonged video-electroencephalographic monitoring. METHODS: During a 6-year period, 883 patients were monitored in the Pediatric Epilepsy Monitoring Unit and 134 patients (15.2%) were documented to have PNEs on the basis of a typical spell recorded during monitoring. Their hospital charts were reviewed and videotapes of these events were analyzed. RESULTS: Patients were divided into 3 age groups: 1) the Infant, Toddler, and Preschool Group (2 months-5 years) that comprised 26 patients. The most common diagnoses were stereotyped movements, hypnic jerks, parasomnias, and Sandifer syndrome. Concomitant epilepsy was present in 12 patients (46%). 2) The School-Age Group (5-12 years) consisted of 61 patients. The most frequent diagnoses were conversion disorder (psychogenic seizures), inattention or daydreaming, stereotyped movements, hypnic jerks, and paroxysmal movement disorders. Fifteen patients (25%) had concomitant epilepsy. 3) The Adolescent Group (12-18 years) consisted of 48 patients, of whom 40 patients (83%) were diagnosed with conversion disorder. Nine patients (19%) had concomitant epilepsy. CONCLUSIONS: In our patients with PNEs, conversion disorder was seen in children >5 years old and its frequency increased with age, becoming the most common type of PNEs among adolescents. In adolescents, conversion disorder was more common in females, whereas males predominated in the school-aged group. Concomitant epilepsy with nonepileptic events occurred in all 3 age groups to a varying extent.

Adolescent↗

Implementing health checks for the over-75s.

Patients who are over the age of 75 can have multiple health problems, but medical or surgical intervention is not always appropriate. This article outlines a comprehensive holistic assessment tool for the over-75s that was developed by nurses in three GP practices in North Yorkshire. The project addressed the complex needs of a client group that is often denied access to wider health services.

Aged↗