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Biomedical subjects

M Duchowny

Publications and source records attributed to M Duchowny.

At least 19 recordsLinked to original sources
PubMed2004

Practice parameter: medical treatment of infantile spasms: report of the American Academy of Neurology and the Child Neurology Society.

OBJECTIVE: To determine the current best practice for treatment of infantile spasms in children. METHODS: Database searches of MEDLINE from 1966 and EMBASE from 1980 and searches of reference lists of retrieved articles were performed. Inclusion criteria were the documented presence of infantile spasms and hypsarrhythmia. Outcome measures included complete cessation of spasms, resolution of hypsarrhythmia, relapse rate, developmental outcome, and presence or absence of epilepsy or an epileptiform EEG. One hundred fifty-nine articles were selected for detailed review. Recommendations were based on a four-tiered classification scheme. RESULTS: Adrenocorticotropic hormone (ACTH) is probably effective for the short-term treatment of infantile spasms, but there is insufficient evidence to recommend the optimum dosage and duration of treatment. There is insufficient evidence to determine whether oral corticosteroids are effective. Vigabatrin is possibly effective for the short-term treatment of infantile spasm and is possibly also effective for children with tuberous sclerosis. Concerns about retinal toxicity suggest that serial ophthalmologic screening is required in patients on vigabatrin; however, the data are insufficient to make recommendations regarding the frequency or type of screening. There is insufficient evidence to recommend any other treatment of infantile spasms. There is insufficient evidence to conclude that successful treatment of infantile spasms improves the long-term prognosis. CONCLUSIONS: ACTH is probably an effective agent in the short-term treatment of infantile spasms. Vigabatrin is possibly effective.

Administration, Oral↗
PubMed2001

Absence of hippocampal sclerosis in children with multiple daily seizures since infancy.

We report a series of nine children with multiple daily seizures since infancy who underwent functional hemispherectomy that included en bloc resection of the hippocampus and the temporal neocortex. In all cases, the hippocampi were normal by conventional histology despite the fact that these patients had suffered from recurrent seizures over a long period of time. This observation suggests that extremely frequent seizures in childhood are not invariably associated with the development of hippocampal sclerosis.

Child↗
PubMed2001

Outcome of corpus callosotomy and other pediatric epilepsy surgery: parental perceptions.

PURPOSE: Parental perceptions regarding the outcome of epilepsy surgery in their children were assessed preoperatively via telephone survey. METHODS: Twenty-seven parents - sixteen mothers (MO), eleven fathers (FA) - of sixteen children (nine boys, seven girls; ages 6 months-18 years) with intractable epilepsy (refractory to > or = 3 anticonvulsants) were interviewed. Twenty-four questions were posed during single, 20-minute sessions to both parents. RESULTS: A majority of parents (63% MO, 82% FA) would consider epilepsy surgery successful if it achieved > or = 75% seizure reduction. Incomplete seizure control would even be acceptable if the overall number of seizures were reduced (69% MO, 18% FA). In order to gain seizure remissions, behavioral deterioration would be accepted (57% MO, 50% FA). Less than total seizure control would be accepted if surgery produced behavioral improvement (57% MO, 51% FA, Kappa > 0.6). For seizure reduction, parents would accept: visual field deficits (82% MO, 67% FA), short-term memory deficits (33% MO, 75% FA), and speech problems (56% MO, 17% FA). CONCLUSION: Parents of children with medically-resistant seizures perceive adverse physical and behavioral consequences of epilepsy surgery to be acceptable if there is adequate seizure control. Their acceptance of postoperative deficits underscores the magnitude of the handicap produced by chronic childhood epilepsy.

Adolescent↗
PubMed2000

Aberrant neural circuits in malformations of cortical development and focal epilepsy.

Malformations of cortical development (MCD) account for a high proportion of medically resistant partial seizures in children and figure prominently in pediatric surgical series. In contrast to the results of epilepsy surgery for postnatally acquired lesions, seizure freedom in patients with MCD is less certain owing to difficulties in defining the epileptic zone, and fully excising the epileptogenic cortex. The authors present evidence that, compared with postnatally acquired lesions such as those due to tumors or trauma, focal epileptogenesis associated with MCD is best conceptualized as a disorder of widespread and patchy disturbance of cortical networks. This developmental perspective implies that the epileptogenic region in MCD is rarely discrete even in patients with focal anatomic lesions, and may include remote cortical or subcortical areas. Preoperative investigative protocols based on this model have improved surgical results, but outcome remains far from optimal and further progress in understanding the complex developmentally based features of MCD is required.

Cerebral Cortex↗
PubMed2000

Predictors of outcome in pediatric epilepsy surgery.

OBJECTIVE: To determine the correlation between pre- and perioperative variables on the outcome of children undergoing focal resections for medically intractable partial epilepsy. METHODS: Retrospective analysis of pre- and perioperative variables in a cohort of 75 patients younger than 12 years of age who underwent excisional surgery and had at least 1 year of follow-up. Outcome, measured by postoperative seizure frequency, was analyzed as a function of age at seizure onset, duration of epilepsy, presence of cognitive impairment, lobe of seizure origin, presence of a lesion, histopathology, and completeness of resection. Completeness of resection was defined on the basis of excising both the entire structural lesion if present and the region revealing prominent interictal and ictal abnormalities on intracranial EEG. RESULTS: Seventy-seven percent of patients had good outcomes (class 1 or 2), and 59% were seizure-free. Lesional status, site of resection, and pathologic diagnoses were not significant predictors of outcome except for in multilobar resection, for which overall outcome was relatively poor (44% class 3 or 4; 22% seizure-free). Completeness of resection was the only significant predictor of good outcome (p < 0.001), with 92% of patients who underwent complete resection of the epileptogenic zone achieving good outcome compared with 50% of patients who had incomplete resections. CONCLUSION: In this series of pediatric patients, complete resection of the lesion and the electrographically abnormal region was the main determinant of outcome after focal resections. Except for multilobar resections, other factors examined in this study did not significantly influence postoperative seizure prognosis and should not influence candidate selection for the surgical process.

Child↗
PubMed2000

Recent advances in candidate selection for pediatric epilepsy surgery.

Many patients who were once considered inoperable are candidates for a wide variety of surgical procedures. This trend is particularly striking at pediatric centers that serve patients with diverse forms of epilepsy that compromise development. Newer diagnostic techniques, including structural and functional imaging, EEG monitoring, and surgical technology, can provide a complete representation of the etiology and expression of the epileptic focus, including accurate cortical localization. This review focuses on several forms of epilepsy that, until recently, were not regarded to be surgically amenable. The adverse seizure and neurobehavioral prognosis in patients with these disorders provides the justification to search for innovative treatments. With more sophisticated tools for preoperative evaluation and an expanding knowledge base, it is likely that the number of surgical candidates will continue to increase in the future.

Brain↗
PubMed2000

Selection criteria and preoperative investigation of patients with focal epilepsy who lack a localized structural lesion.

Children with medically resistant epilepsy are often referred for surgical evaluation. The absence of a specific focal lesion on MRI may render the work-up difficult. In response to the need to localize the primary area of epileptogenesis, surgery protocols are being developed which rely on clinical semiology, EEG and functional imaging data. In selected cases, intracranial EEG monitoring may be required. While testing more often depends on the convergence of modalities, it is possible to localize seizure origin in the majority of children, and fully excise the epileptogenic region. This review presents the etiology and preoperative modalities available for children with intractable, non-lesional epilepsy.

Brain↗
PubMed1999

A placebo-controlled trial of lamotrigine add-on therapy for partial seizures in children. Lamictal Pediatric Partial Seizure Study Group.

OBJECTIVE: To compare the safety and efficacy of add-on lamotrigine and placebo in the treatment of children and adolescents with partial seizures. BACKGROUND: Add-on and monotherapy lamotrigine is safe and effective in adults with partial seizures, and reports of preliminary uncontrolled trials suggest similar benefits in children. METHODS: We studied 201 children with diagnoses of partial seizures of any subtype currently receiving stable conventional regimens of antiepileptic therapy at 40 study sites in the United States and France. After a baseline observation period (to confirm that more than four seizures occurred in each of two consecutive 4-week periods), patients were randomized to add-on lamotrigine or placebo therapy. A 6-week dose-escalation period was followed by a 12-week maintenance period. RESULTS: Compared with placebo, lamotrigine significantly reduced the frequency of all partial seizures and the frequency of secondarily generalized partial seizures in these treatment-resistant patients. The most commonly reported adverse events in the lamotrigine-treated patients were vomiting, somnolence, and infection; the frequency of these and other adverse events was similar to that in the placebo-treated group, with the exception of ataxia, dizziness, tremor, and nausea, which were more frequent in the lamotrigine-treated group. The frequency of withdrawals for adverse events was similar between groups. Two patients were hospitalized for skin rash, which resolved after discontinuation of lamotrigine therapy. CONCLUSIONS: Lamotrigine was effective for the adjunctive treatment of partial seizures in children and demonstrated an acceptable safety profile.

Adolescent↗
PubMed1999

Clinical spectrum of cortical dysplasia in childhood: diagnosis and treatment issues.

Disorders of cortical development form a spectrum of lesions produced by insults to the developing neocortex. These conditions typically first manifest in childhood with epilepsy, developmental delay, and focal neurologic signs. Although the clinical and electrophysiologic findings are often nonspecific, high-resolution magnetic resonance imaging facilitates diagnosis during life, and assists in delineating specific clinical syndromes. While many patients are dysmorphic and severely affected by mental retardation and epilepsy, some have normal or near-normal cognitive function and no seizures. Molecular studies of dysplastic cortex are providing new insights into the basic mechanisms of brain function and development, while pathologic analysis of tissue removed at surgery is helping to define epileptic circuitry. Treatment of the epilepsy associated with cortical dysplasia is often frustrating, but surgical approaches based on accurately defining epileptogenic regions are proving increasingly successful. Genetic diagnosis is important for accurate counseling of families.

Brain Diseases↗
PubMed1999

The localizing value of ictal SPECT in children with tuberous sclerosis complex and refractory partial epilepsy.

Although multiple cortical tubers are a hallmark of tuberous sclerosis complex (TSC), seizures often originate from a single tuber, making excisional surgery a therapeutic option for intractable patients. To assess the role of ictal single photon emission computed tomography (SPECT) in defining the epileptogenic tuber, we reviewed videoelectroencephalography (V/EEG) data, magnetic resonance imaging (MRI) and SPECT scans of 15 patients (aged 3 months to 15 years, mean 5.1) with medically resistant partial seizures and TSC. SPECT scans were performed using Tc-99m-hexamethylpropyleneamine oxime (HMPAO) injected within 30 seconds of electrographic seizure onset and were graded on a scale from 1 to 5 (1 weakly perfused, 5 strongly perfused). The scalp EEG revealed localized seizure origin in ten patients; five had concordant hyperperfused SPECT regions consisting of comma-shaped areas surrounding hypoperfused areas in the candidate tuber. Strongly hyperperfused regions (grade 3-5) were noted in two patients whose ictal EEG patterns were characterized by sustained, rhythmic fast activity or spiking. The other five patients had nonlocalizing or poorly sustained ictal EEG patterns; one patient in this group had focal hyperperfusion. Invasive EEG recordings in two patients revealed propagated EEG patterns that correlated with the ictal SPECT findings. These findings indicate a strong correlation between ictal SPECT and ictal scalp EEG, especially when there are sustained rhythmic fast ictal EEG patterns.

Adolescent↗
PubMed1998

Epilepsy surgery in the first three years of life.

PURPOSE: Partial seizures in early postnatal life may be catastrophic and associated with poor long-term outcome. Epilepsy surgery can alleviate partial seizures in older children and adults, but there is little experience with surgical therapy in infancy apart from hemispheric epilepsy syndromes. METHODS: We analyzed the results of cortical resection to treat medically refractory partial epilepsy in 31 children (16 boys, 15 girls) aged <3 years (mean, 18.3 months). Subjects were included only if seizure relief was the primary indication for surgery. RESULTS: Follow-up of at least 1 year (mean, 4.6 years) in 26 patients revealed that 16 were seizure-free, 4 had >90% seizure reduction, and 6 had <90% reduction. There was no significant difference in seizure outcome between hemispherectomy/multilobar resections and lobar resections or temporal versus extratemporal resection. Seizure outcome was independent of the amount of cortex removed in nonlesional patients. Only the presence of a discrete lesion on preoperative neuroimaging correlated with a favorable outcome. Family perceptions of accelerated development in seizure-free patients were not confirmed on developmental assessment. CONCLUSIONS: We conclude that cortical resection often benefits very young children with catastrophic partial seizures, but does not guarantee enhanced neurological development. The location and extent of the excised cortex may not be critical as long as the entire epileptogenic region and lesion are removed.

Adult↗
PubMed1998

[Epilepsy centers: classification and basic requirements].

Over the last two decades the surgical treatment of epilepsies has become a commonly used and effective method for the treatment of intractable epilepsy. However, because of its wide use in various types of centers throughout the world the methodology involved varies significantly throughout the centers. With this article we propose the bases from which a multidisciplinary team of numerous centers can eventually develop an effective and acceptable international protocol for the surgical treatment of the epilepsies and the classifications of the centers.

Epilepsy↗
PubMed1997

Results and complications after reoperation for failed epilepsy surgery in children.

The seizure outcome and neurological outcome in children who undergo reoperation for failed epilepsy surgery have not been well documented. This retrospective study evaluated 20 children who underwent a second resective surgery for recurrent seizures. Four categories of patients were identified: (1) extension of the initial resection was performed in 8 patients; (2) 5 patients underwent lobectomy or corticectomy in a region remote from the original surgical site; (3) multilobar resection which may have included further resection of the initial procedure was accomplished in 4 patients; (4) hemispherectomy was performed in 3 patients. Patients with reoperation in the same lobe as the first procedure (group 1) had a 62% seizure-free rate, while 44% of patients in groups 2 and 3 were free from seizures at follow-up evaluation. Patients undergoing hemispherectomy had a 67% seizure-free rate. Significant unexpected neurological deficits occurred in 3 patients who underwent multilobar resection at reoperation. Complications included motor and language deficits. Reoperation for intractable partial epilepsy is beneficial in selected children. Patients who require multilobar resections may have higher risk of postoperative neurological deficit than those patients with reoperation in one lobe. These factors may be useful in counseling parents of children considering reoperation for recurrent epilepsy.

Adolescent↗