Computerized Tomography in the Study of West's Syndrome

Gastaut, H; Gastaut, J. L.; Régis, H; Bernard, R; Pinsard, N; Saint-Jean, M.; Roger, J; Dravet, Charlotte

doi:10.1111/j.1469-8749.1978.tb15178.x

Cited by 51 publications

(7 citation statements)

References 5 publications

(1 reference statement)

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“…There are other previous articles of epilepsy cases associated with striatal lesions such as a tumor. 12 These findings may indicate that the striatum of these cases had epileptogenesity.…”

Section: Discussionmentioning

confidence: 77%

Anterior striatum with dysmorphic neurons associated with the epileptogenesis of focal cortical dysplasia

Kaido

Otsuki

Kaneko

et al. 2010

Seizure

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The epileptogenesis of the striatum is unknown. We describe the case of a 12-year-old girl with intractable epilepsy who was treated by surgical interventions. Magnetic resonance imaging (MRI) showed ambiguous corticomedullary boundary in the left frontal lobe, and magnetoencephalography (MEG) revealed spike dipoles in the vicinity of the left ventral striatum. The epileptic seizures disappeared after partial resection of the frontal lobe, but recurred within 2 months and remained intractable. Neuropathological examination confirmed the presence of focal cortical dysplasia in the resected brain tissue. Ictal single photon emission computed tomography at this period displayed hyperperfusion of the left anterior striatum. At the second surgery, intraoperative electrocorticography exhibited spike discharges from the anterior striatum. After the removal of this structure and adjacent brain tissues, the patient remains seizure-free for 33 months, without any neurological deficits. Histopathological examination of the resected tissue revealed a large number of dysmorphic neurons distributed widely in the cerebral cortex, subcortical white matter, striatum, and insular cortex. These findings suggest that microscopic dysplasia of basal ganglia can accompany certain cases of focal cortical malformations, and may play a critical role in the epileptogenesis through their interaction with cortical structures.

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“…There are other previous articles of epilepsy cases associated with striatal lesions such as a tumor. 12 These findings may indicate that the striatum of these cases had epileptogenesity.…”

Section: Discussionmentioning

confidence: 77%

Anterior striatum with dysmorphic neurons associated with the epileptogenesis of focal cortical dysplasia

Kaido

Otsuki

Kaneko

et al. 2010

Seizure

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“…Patients with primary generalized epilepsy[234] and benign rolandic epilepsy[5] have a low incidence of abnormality on CT scan, while an abnormal finding on CT scan is common in children with infantile spasms. [67] Conventionally, during the evaluation of partial seizures, computed tomogram is carried out only if other neurological symptoms or signs are present. In their absence, the indication for CT has been less certain.…”

Section: Introductionmentioning

confidence: 99%

Clinico - diagnostic and therapeutic relevance of computed tomography scan of brain in children with partial seizures

Patel

Jain

Iyer

et al. 2013

Ann Indian Acad Neurol

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Background:Therapeutic relevance of computed tomography (CT) in children with partial seizures is reported to be remarkably low (1-2%). However, in the developing countries where infections involving the nervous system are common, routine CT scan of brain may help in finding treatable causes of seizures.Objective:Aim of this study was to evaluate the significance of CT scan of brain in the management of children with partial seizures.Materials and Methods:Children with partial epilepsy, whose predominant seizure type was focal motor seizures, were included in the study. CT scan of brain was done in all children aged between 1 month and 12 years with partial seizures of unknown etiology prospectively. The clinical findings of these children were noted along with the CT findings.Results:Between August 2001 and July 2002, of the 200 children with seizure disorder 50 children who satisfied the inclusion criteria were included in the study. CT scan of brain was normal in 16 children (32%) and was abnormal in 34 children (68%). Twenty children (~60% of abnormal scan) had potentially correctable lesions: Tuberculoma (n = 13), neurocysticercosis (n = 3), and brain abscess (n = 4). Five children had changes representing static pathology that did not influence patient management. The clinical features correlated with CT findings in 78% children.Conclusion:Children with partial motor seizures have high probability of having abnormal findings on CT scan of brain, especially, neuro-infections which are potentially treatable. Therefore, CT scan brain should be carried out in all children with partial motor seizures especially, in developing countries.

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“…75,76 Computerized tomography scan provided a great addition to the understanding of the etiology in patient with epileptic spasms and hypsarrhythmia. 77,78 Diagnoses like cortical malformations, tuberous sclerosis, and Aicardi syndrome were no longer confirmed postmortem.Synchronized recording of video and EEG added to the electroclinical and semiological characteristic of infantile spasms. 75,79 Genetic studies of infantile spasms and hypsarrhythmia considered a multifactorial etiology and studied the familial risk.…”

Section: Other Significant Landmarks For the Classification Diagnosis...mentioning

confidence: 99%

Historical Overview of Hypsarrhythmia and Its Association to Epileptic Spasms: A Review of the Medical Literature From 1952 to 1982

Pestana-Knight

Mani

2022

Journal of Clinical Neurophysiology

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Summary:The initial description of infantile spasms and its association to developmental abnormalities was attributed to Dr. Williams J. West in 1841 but the clinical scenario at the time had also been seen by other physicians. French physician Henry Gastaut proposed the eponym of West syndrome in the 9th Colloquium de Marseille in 1960. The description of hypsarrhythmia in 1952 by Gibbs and Gibbs added the EEG component to the triad of infantile spasms. The hypsarrhythmia discovery led to a sudden interest in understanding the etiology and developing treatments for this devastating disease affecting infants and young children. It was in the 1950s when cases of infantile spasms with absence of hypsarrhythmia were initially observed. Also, the treatment with adrenocorticotrophic hormone was initially reported as efficacious for treating infantile spasms and hypsarrhythmia in the late 1950s. Adrenocorticotrophic hormone remains the best treatment option for these epilepsy types. This article will provide a historical review of knowledge developments about hypsarrhythmia and infantile spasms, emphasizing the period 1952 to 1982. The goal of the article was to highlight clinical elements that were discovered then and remain clinically relevant today.

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Computerized Tomography in the Study of West's Syndrome

Cited by 51 publications

References 5 publications

Anterior striatum with dysmorphic neurons associated with the epileptogenesis of focal cortical dysplasia

Anterior striatum with dysmorphic neurons associated with the epileptogenesis of focal cortical dysplasia

Clinico - diagnostic and therapeutic relevance of computed tomography scan of brain in children with partial seizures

Historical Overview of Hypsarrhythmia and Its Association to Epileptic Spasms: A Review of the Medical Literature From 1952 to 1982

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