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Biomedical subjects

E Wyllie

Publications and source records attributed to E Wyllie.

At least 73 records · Page 4Linked to original sources

Epilepsy surgery outcome: comprehensive assessment in children.

The effect of extratemporal and temporal lobe cortical resection on children with intractable epilepsy is not well understood. We evaluated a comprehensive array of outcome variables in 33 consecutive children who received epilepsy surgery at 12 years of age or younger. Twenty-two (67%) children were seizure-free, three (9%) had a greater than 90% reduction in seizures, and four had no improvement. Antiepileptic drugs (AEDs) were not required in 10 (30%) children and were reduced in number in another 10. Six (29%) of 21 tested children had an improvement of greater than 10 points in Verbal or Performance IQ after surgery, while one (4%) had a decrease greater than 10 points in Verbal IQ. One mild hemiparesis and one inferior quadrantanopsia occurred; both were anticipated. We used the Child Health Questionnaire (CHQ), a valid and reliable instrument for children, to assess health-related quality of life (HRQOL). Six of 12 subscale scores of the CHQ were significantly lower in the surgical group compared with 410 age-matched control subjects. Parents were satisfied with surgical results in 28 (85%) cases. Pathologic tissue diagnosis and site of resection were not associated significantly with any outcome measure. We conclude that surgery eliminates seizures and reduces AED requirements in most children with intractable epilepsy selected by currently available methods. Further investigation is needed to establish the nature and significance of inferior scores in the surgical group in the HRQOL domains of physical function, general health, and self-esteem.

Adolescent↗

Diagnostic testing of seizure disorders.

Although a thorough history and physical examination remain the basis for the evaluation of patients with a possible seizure disorder, electroencephalography (EEG) is a necessary extension of the neurologic examination. Most patients also require a magnetic resonance imaging (MRI) scan to identify a potentially epileptogenic lesion. This article reviews the technical considerations, common findings, and potential pitfalls related to the use of EEG and MRI. Adjunctive test such as ambulatory EEG, video/EEG, positron emission tomography, single photon emission computed tomography, and serum evaluation also are discussed for use in specific circumstances.

Adolescent↗

Epilepsy surgery in infants.

PURPOSE: We report 12 infants who had frontal (n = 3), temporal (n = 2), or temporoparieto-occipital (n = 2) resection or functional hemispherectomies (n = 5) at age 2.5-29 (mean 15.3) months for catastrophic epilepsy due to focal cortical dysplasia (n = 5), Sturge-Weber syndrome (n = 3), ganglioglioma (n = 3), or hemimegalencephaly (n = 1). Seizures began at 1 day to 14 months (mean, 4.0 months) after birth, occurred frequently (often many times per day, and were refractory to antiepileptic drugs. Patients were evaluated for surgery at 2.5-24 (mean 12.4) months of age. Seven patients had hemiparesis and eight had slowed cognitive development. Seizures were characterized by arrest or marked reduction of behavioral motor activity with nuclear level of consciousness (n = 4, with temporal or temporoparietal EEG seizures), focal clonic activity (n = 3, with perirolandic EEG seizures), generalized tonic stiffening (n = 3, with temporoparieto-occipital, parietal, or frontal EEG seizures), or infantile spasms and hypsarrhythmia (n = 2, with a frontal tumor or temporoparieto-occipital cortical dysplasia). METHODS: Magnetic resonance imaging (MRI) revealed the epileptogenic lesion in all but two patients, both with cortical dysplasia localized by interictal positron-emission tomography (PET) and other clinical or EEG features and confirmed on histopathologic examination of resected tissue. RESULTS: At follow-up 4-86 (mean 32) months after surgery, six patients were seizure free (Engel outcome class I), three had rare seizures with none in at least the previous 6 months (Engel class II), and two had worthwhile improvement (Engel class III). Except for the severely developmentally delayed infant with hemimegalencephaly, several patients had marked "catch-up" developmental progress after class I, II, or III outcome. Postoperative complications included subdural hematoma over the contralateral hemisphere (one patient) and entrapment and enlargement of the ipsilateral temporal horn (one patient) after hemispherectomy, both corrected uneventfully with a second surgical procedure. One patient died of unexplained causes several hours after frontal lobectomy. No patients had new neurologic deficits after surgery, and one patient had resolution of progressive fluctuating hemiparesis after resection of temporoparieto-occipital cortical dysplasia. CONCLUSIONS: Our results agree with previous reports that epilepsy surgery can provide relief from catastrophic epilepsy in carefully selected infants.

Age Factors↗

Parental assessment of functional outcome after corpus callosotomy.

PURPOSE: We wished to evaluate functional outcome and parental satisfaction after corpus callosotomy. METHODS: We studied a cohort of parents of 17 patients who had received an anterior corpus callosotomy > 7 months earlier (mean 26 months) for severe, intractable seizures. Parental satisfaction with the overall surgical results was the primary outcome measure. In addition, the parents assessed behavior changes that had been emphasized preoperatively as important aspects of their children's function, including alertness and responsiveness, falls and injuries, and special care needs. Degree of seizure reduction and extent of corpus callosum were also measured. RESULTS: The parents of 15 (88%) patients reported satisfaction with the surgical outcome. All parents who expressed satisfaction would recommend callosotomy to another family in a similar situation. The behavior change most closely associated with satisfaction was improved alertness and responsiveness (p < 0.03). Although 9 patients experienced > 80% reduction in targeted seizures, satisfaction was not reported at a greater rate as compared with the 6 patients with 50-80% reduction. CONCLUSIONS: Most parents report satisfaction with the outcome of their child's corpus callosotomy, but are influenced by improvement in aspects of function and behavior in addition to seizure reduction.

Adolescent↗

Surgery for catastrophic localization-related epilepsy in infants.

Cortical resection or hemispherectomy has been reported to result in cessation or dramatic reduction of seizures for small numbers of highly selected infants with severe, intractable epilepsy and developmental delay. However, identification of potential surgical candidates during infancy can be especially challenging because seizure semiology and EEG may sometimes give limited localizing information, e.g., in patients with infantile spasms and hypsarrythmia due to focal cortical dysplasia or a tumor. In infants as well as older patients, the location of a potentially resectable epileptogenic lesion must be defined by convergence of results from video EEG, anatomic and functional neuroimaging, and clinical examination. Reported outcomes after surgery in small series include 78% of 23 infants seizure-free or with at least 90% seizure reduction (1993 University of California at Los Angeles series), and 75% of 12 infants seizure-free or with rare seizures (1995 Cleveland Clinic series). A tendency of "catch-up" developmental progress after surgery was observed in both series. A few reports of smaller groups of infants noted similar results. Prospective studies are in progress to better define the potential risks and benefits of early surgical intervention for infants and catastrophic localization-related epilepsy.

Age Factors↗

Epilepsy surgery in the setting of periventricular leukomalacia and focal cortical dysplasia.

We report an infant who had successful epilepsy surgery for intractable infantile spasms in the setting of bilateral periventricular leukomalacia and remote germinal matrix hemorrhage. Although MRI gave evidence of a diffuse cerebral insult and EEG showed hypsarrhythmia, focal epileptogenicity was suggested by previous partial seizures with onset over the right temporoparietal-occipital region and PET hypometabolism in that same area. Right temporoparietal and lateral occipital resection at 15 months resulted in seizure freedom and dramatic developmental progress at 1-year follow-up. Histopathologic examination of resected tissue showed cortical dysplasia, possibly due to the same insult that also resulted in the bilateral periventricular leukomalacia. From an etiologic perspective, this is one of very few reported cases strongly implicating acquired focal cortical dysplasia in response to a prenatal insult. From a clinical perspective, the case illustrates that the spectrum of potential surgical candidacy in infants may be broader than usually suspected.

Brain↗

Supplementary sensorimotor area seizures in children and adolescents.

RATIONALE: Some types of seizures in children may be difficult to recognize; the diagnosis of seizures arising near the mesial posterior frontal supplementary sensorimotor area (SSMA) may be especially challenging. Such seizures have been well described in adults, but few pediatric cases have been reported even though onset is typically in childhood. METHODS: We studied 11 children and adolescents with SSMA seizures diagnosed by prolonged video electroencephalography (EEG). RESULTS: Mean age at onset was 5.8 years, and diagnosis by video EEG was made at a mean age of 12 years. Children had at least borderline intelligence and few abnormal findings on neurologic examination. Seizures were typically brief, frequent, and predominantly nocturnal, with bilateral tonic posturing, gross proximal limb movements, and preserved consciousness. Magnetic resonance imaging showed normal findings or a superior or mesial posterior frontal tumor or cortical dysplasia. Routine EEG findings were usually normal, but prolonged EEG showed epileptiform discharges over the vertex. Seizure onset in the region of the SSMA was confirmed by subdural EEG in six patients who were studied for epilepsy surgery. Five patients had seizure-free outcome or worthwhile improvement after operation. Two nonoperated patients are seizure-free on antiepileptic medication, and three are undergoing surgical evaluation. CONCLUSIONS: Diagnosis of SSMA seizures in children may be challenging because of unusual symptoms (bilateral tonic limb involvement with preserved consciousness) and frequently normal routine EEG findings. Video EEG may be necessary for diagnosis, and magnetic resonance imaging may reveal a focal lesion. Increased recognition of this seizure type in pediatric patients should lead to earlier diagnosis for optimal medical or surgical treatment.

Adolescent↗

Frontal lobe seizures: electroclinical syndromes.

To define further the electroclinical manifestations of frontal lobe epilepsy (FLE), we studied 150 seizures manifested by 24 patients; 18 patients had subdural electrode arrays (SEA). The findings in these patients clearly overlapped presumably reflecting the interconnections between functionally related frontal zones; yet the manner in which the symptoms clustered and the sequence in which they occurred generally indicated the anatomic site of the epileptogenic zone. We divided the patients into three major groups: (a) those with supplementary motor seizures, (b) those with focal motor seizures, and (c) those with complex partial seizures (CPS, psychomotor seizures). Supplementary motor seizures began with tonic posturing of the extremities. Focal motor seizures generally began with conscious contralateral version or unilateral clonic focal motor activity; tonic posturing was noted only late in the seizure. CPS (psychomotor) began with unresponsiveness at onset, followed by staring or unconscious contraversion. We compared frontal lobe seizures with temporal lobe seizures reported previously; oral-alimentary automatisms, repetitive hand movements, or looking around, were more common in temporal lobe seizures, whereas tonic posturing and bicycling movements were more common in frontal lobe psychomotor seizures.

Adolescent↗

Childhood ganglioglioma and medically intractable epilepsy. A clinicopathological study of 15 patients and a review of the literature.

Gangliogliomas are an increasingly recognized cause of epilepsy in children. Several studies have shown that early surgical treatment is beneficial, but controversy exists regarding the type of surgical treatment required for optimal seizure control and to prevent tumor recurrence. To address this issue, we performed a retrospective review of 15 children operated on at the Cleveland Clinic during a 7-year period with medically intractable epilepsy who were found to harbor a ganglioglioma during the course of their work-up. Nine patients with mostly nondominant hemisphere tumors underwent tumor resection without the use of electrocorticography to guide additional resection of epileptogenic foci, while 6 other patients with dominant hemisphere tumors had subdural electrode grids placed to extraoperatively map zones of ictal onset and eloquent areas. The extent of tumor resection was then correlated to seizure outcome and tumor recurrence. Of the 11 patients who received a gross total resection, 9 are seizure-free while 2 have a greater than 90% reduction in their seizure frequency (100% 'good' outcome). There was no tumor recurrence in this group. Of the 4 patients who underwent a subtotal tumor resection, 1 is seizure-free while 3 have persistent seizures (25% 'good' outcome). All patients in this group have stable disease on follow-up magnetic resonance imaging. In those patients who received subdural electrode grids, the extent of resection of the zones of ictal onset did not correlate with seizure outcome. The mean follow-up was 42 months. We conclude that complete tumor resection is the most important factor for optimal seizure control and to prevent tumor recurrence.

Adolescent↗

Language dominance in patients with early childhood tumors near left hemisphere language areas.

RATIONALE: Language function often develops in the right hemisphere following an acute left hemispheric insult in early childhood. Little is known, however, about lateralization of language function in patients with early childhood tumors near potential left hemisphere language areas. Issues of language dominance are important in the strategy for resection of left temporal and frontal tumors. METHODS: We studied 12 patients who had determination of hemispheric language dominance by the intracarotid amobarbital procedure prior to resection of a left inferolateral frontal or left mid or posterior temporal tumor that (1) was near a classic language area and (2) first manifested with partial seizures by age 6 years (mean, 3.7). RESULTS: Hemispheric language dominance was left in 10 patients (83%), right in one patient, and bilateral in one patient. Six patients with left temporal tumors had localization of Wernicke's area with cortical stimulation, and in five the language area was posterior to the tumor. In one patient, the tumor infiltrated Wernicke's area. Eleven of the 12 patients had complete tumor resection (mean age at operation, 11.8 years) and were seizure-free at follow-up (mean, 3.1 years). Neuropsychological testing showed a tendency for improved language function after operation, and no patient had new overt language deficits. CONCLUSIONS: Early low-grade left frontal and temporal tumors usually did not result in transfer of language dominance to the contralateral hemisphere. Tumors may grow slowly along with the developing brain in young children, with continued left hemisphere language development in regions separate from the neoplasm. Successful tumor resection can be accomplished, but it may require cortical stimulation for localization and sparing of nearby language areas.

Adolescent↗

Ictal amaurosis: MRI, EEG, and clinical features.

We studied seven consecutive patients who experienced amaurosis as a prominent feature of their seizures. The mean age of seizure onset was 4 years. Six patients had MRI abnormalities including ischemic, traumatic, and space-occupying lesions, with five limited to the parietal-occipital region. Six patients had unilateral, posterior interictal spike and slow wave complexes. Four patients experienced severe postictal headache and nausea. Absence of a family history of seizures distinguished the patients with MRI lesions from previously presented series of benign occipital lobe epilepsy. We conclude that the constellation of ictal amaurosis, occipital paroxysms, and postictal migrainous symptoms does not necessarily signify a benign, nonlesional epilepsy; MRI is recommended for such patients without a family history of similar seizures.

Adolescent↗

Cortical electrical stimulation in humans. The negative motor areas.

Summarizing, we have presented evidence in humans for two "negative motor areas" which we had speculated play a significant role in the planning of voluntary motor movements. A review of the more recent experimental literature shows that histological, physiological, and electrical stimulation studies in animals reveal the existence of two frontal regions that from the experimental data also seem to play an essential role in the preparation (as opposed to execution) of voluntary movements. Current available evidence suggests that these two areas (areas F5 and F6 of Rizzolatti et al.) correspond to the negative motor areas we have described in human studies. Also of interest is that Broca's area in the dominant hemisphere overlaps the corresponding negative motor area. This observation suggests that Broca's area has evolved from area F5 of monkeys specializing in the planning of fine movements necessary for speech production. We feel that current evidence suggests the existence of three mechanisms by which cortical stimulation (by electrical stimulation or by epileptic activation) can generate negative motor phenomena: 1. The "silent period," which is consistently contralateral, has a somatotopic distribution, and tends to affect predominantly muscles involved in fine movements. It is of relatively short duration and seems to be generated by the activation of cortical areas in the primary sensorimotor region. The H-reflex is not inhibited during the silent period, suggesting that the silent period is generated by a decrease in the excitatory input through direct corticospinal neurons on the spinal alpha motoneurons. It is possible that in normal individuals this system is used for fine tuning of fine distal movements. The negative myoclonus seen in some patients with focal cortical epilepsy is probably generated by this mechanism. The primary and supplementary "negative motor areas" described in this chapter. This effect also has a somatotopic distribution but can affect muscle bilaterally even if there is a clear predominance contralaterally. The negative motor effect does not influence postural tone and can be prolonged. The negative motor effect is probably produced by activation of agranular cortex immediately in front of the primary and supplementary face motor area. These cortical areas are probably used for organization and integration of fine motor movement. Activation of these areas would produce an apraxia of fine movements. Focal atonic seizures are probably generated by this mechanism. 3. The fast-conducting corticoreticulospinal pathways, which by activation of the brainstem inhibitory centers (NRPo, n.r. magnocellularis dorsal beta, and NRGc), tend to produce bilateral atonia of axial, postural muscles. This system probably does not depend on the presence of the direct corticospinal pathways. By analogy with cataplexy, which is probably produced by activation of similar brainstem inhibitory systems, we would expect the H-reflex to be markedly diminished or even to disappear during the atomic phase (64). This pathway would be used normally for postural adjustments and locomotion. The bilateral massive atonic seizures, seen most frequently in patients with severe and diffuse cortical lesions, are probably produced by this mechanism. However, the bilateral atonic seizures occasionally seen in patients with focal cortical lesions may also be produced by a similar mechanism.

Brain↗

Functional anatomy of the human supplementary sensorimotor area: results of extraoperative electrical stimulation.

Electrical stimulation studies have demonstrated that a "supplementary motor area" (SMA) exists in humans. However, its precise functional organization has not been well defined. We reviewed the extraoperative electrical stimulation studies of 15 patients with intractable epilepsy who were evaluated with chronically implanted interhemispheric subdural electrodes. SMA-type positive motor responses were elicited not only from the mesial portion of the superior frontal gyrus but also from its dorsal convexity, and from the paracentral lobule, cingulate gyrus, and precuneus. Sensory symptoms, that could not be attributed to stimulation of the primary sensory area, were elicited from the superior frontal and cingulate gyri in addition to the precuneus. Therefore, human SMA, as defined by electrical stimulation, is not always confined to the mesial portion of the superior frontal gyrus as described previously. It is also not strictly "motor" but "sensorimotor" in representation. We propose referring to this region as the "supplementary sensorimotor area" (SSMA). We observed a somatotopic organization within the SSMA with an order of lower extremity, upper extremity, and head from posterior to anterior. Sensory representation in an individual was either anterior or posterior to the positive motor representation but never both. There was a supplementary eye field within the head representation. A supplementary negative motor area was noted at the anterior aspect of the SSMA. No language area was demonstrated within the SSMA. The physiologic significance of the SSMA and functional consequences of its resection must be addressed in further studies.

Adolescent↗

Children with seizures: when can treatment be deferred?

The decision whether or not to recommend chronic antiepileptic drug treatment for a child with seizures requires a risk-benefit analysis tailored to each individual case. Because all of the available antiepileptic medications have some potential adverse effects, the analysis may weigh in favor of a decision not to treat. Three clinical scenarios in pediatrics in which the no-treatment option may often be appropriate include a single unprovoked seizure, febrile seizures, and benign focal epilepsy of childhood.

Adolescent↗

Comparison of scalp electroencephalogram with subdural electrocorticogram recordings and functional mapping in frontal lobe epilepsy.

We compared the findings of scalp electroencephalogram with subdural electrode array (SEA) recordings in 19 patients with refractory frontal lobe epilepsy. Prolonged scalp interictal recordings localized the epileptogenic zone in 12 patients; seven had no interictal sharp waves. The SEAs showed multifocal interictal sharp waves in all patients. Seven patients with localized seizure onset on scalp recording showed extensive ictal onset on the SEA recording. Five patients with lateralized seizure onset to one hemisphere on scalp recording were found to have ictal onset on SEA restricted to a smaller area. Because of the large epileptogenic zone found on SEA recordings, a complete resection was possible in only five (33%) of the 15 patients who had resections. Eight (53%) of the 15 patients benefited from surgery (mean follow-up, 4.6 years). The SEAs also allowed functional localization in most patients. From these data, we suggest that a localizing scalp electroencephalogram in patients with frontal lobe epilepsy may be misleading because SEA recordings show larger epileptogenic zones than anticipated. Furthermore, we postulate that the larger extensive epileptogenic zone may account for the poorer surgical outcome in patients with frontal lobe epilepsy compared with patients with temporal lobe epilepsy.

Adolescent↗