Focal features in West syndrome indicating candidacy for surgery

doi:10.1016/S0887-8994(97)00013-1

Pediatric Neurology

Volume 16, Issue 3, April 1997, Pages 213-217

https://doi.org/10.1016/S0887-8994(97)00013-1 Get rights and content

Abstract

A subgroup of patients with West syndrome (WS) also have focal features, and some of these patients respond to surgical intervention. To determine the incidence of focal features in patients with WS and to establish the correlation of these features with outcome, all patients with WS treated in the Epilepsy Unit of the Children's Hospital (Boston, MA) during the years 1989 to 1992 were retrospectively evaluated. Focal features included partial seizures, hemiparesis, focal radiologic findings, asymmetric spasms, and lateralized hypsarrhythmia. The different focal features were later correlated with the natural history of the disease. Sixty-seven patients were included in the study. Sixty-six percent had asymmetric manifestations. Asymmetric spasms, hemihypsarrhythmia, and partial seizures combined with infantile spasms had significant association with asymmetric brain pathology. Focal features did not correlate with age of onset and outcome, but were associated with etiology. Eight of the nine patients who underwent epilepsy surgery had at least two focal features or more. Two of the operated children had focal features in the absence of magnetic resonance imaging abnormalities, thus indicating the necessity for positron emission tomography, the results of which justified surgery.

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Cited by (53)

Resective epilepsy surgery for West syndrome: The Hypsarrhythmic Asymmetric Scoring Scheme is a determining predictor of seizure outcome
2022, Seizure
It has been suggested that asymmetric hypsarrhythmia is associated with structural etiology. We devised the Hypsarrhythmic Asymmetric Scoring Scheme (HASS) to quantify the degree of hypsarrhythmic asymmetry in a retrospective series of patients who underwent surgical treatment at our center. The present study aimed to investigate the role of HASS in predicting the postsurgical seizure outcomes.
We retrospectively analyzed the records of 46 children with hypsarrhythmia who underwent resective epilepsy surgery between 2018 and 2020 and were followed up for at least 1 year after surgery. Hypsarrhythmia severity in each hemisphere was quantified and scored. The HASS score was calculated as the difference between the two hemispheres. Univariate results were submitted to logistic regression models to identify independent predictors for favorable surgical outcomes.
Of the 46 patients who underwent resective surgery, Engel's class I–Ⅱ outcomes were achieved in 34 (73.9%). The Engel I–Ⅱ group had a significantly higher HASS score than the Engel Ⅲ–Ⅳ group (p<0.001). Multivariate analysis showed that the HASS score was the only significant predictor of good outcomes (p = 0.011). Further receiver operating characteristic analysis showed that a threshold of 7 yielded a better seizure outcome with a sensitivity of 97.06% and specificity of 83.33%.
As the first hypsarrhythmia scoring system specially designed for presurgical evaluation, the HASS score may contribute to predicting the postsurgical seizure outcome from the electroencephalography perspective.
Focal signs in infantile spasms
2022, Seizure
Citation Excerpt :
Kramer et al. studied focal clinical signs, together with EEG and imaging techniques. In this study focal symptoms only appeared in patients with a unilateral focus and in addition, the sensitivity of MRI was compared with Positron emission tomography–computed tomography (PET-CT), showing the superiority of PET-CT [9]. This leads to another relevant point, as in our study focal lesions were diagnosed on imaging with MRI only.
Infantile spasms belong to the group of epileptic encephalopathies that typically occur in early infancy and are often associated with severe developmental delay. Little is known about whether focal features are part of the syndrome and thus occur independently of etiology, or whether focal features always indicate a cerebral lesion.
In our study we included all patients with infantile spasms documented by prolonged video-electroencephalogram (EEG) monitoring between 7/2003 and 11/2020 and analysed symptoms such as tonic posturing, clonic movements, deviation of the eyes and unilateral deviation of the mouth. These symptoms were classified as lateralizing or non-lateralizing and the correlation to the presence of a lesion was investigated.
Eighteen patients (9 w/9 m) were included in the study. Lateralizing tonic posturing was found in 66.6% of the patients. Deviation of the eyes to one side and unilateral deviation of the mouth were detected in 61.1% and 11.1% of patients, respectively. Taking into account all symptoms (tonic posturing, clonic movements, deviation of the eyes, unilateral deviation of the mouth), focal signs were observed in a total of 94.4%, with only half of the total patient population having a cerebral lesion.
In our study, lateralizing symptoms in infantile spasms occurred independently of the presence of a lesion. In contrast, focal symptoms in older children or adults usually correlate with the presence and localization of a lesion. A possible hypothesis could be that the brain is still maturing in infancy.
Surgical treatment in children with intractable epilepsy after viral encephalitis
2020, Epilepsy Research
To investigate the surgical outcome in children with epilepsy after viral encephalitis (VE), we studied the prognostic factors for surgery and summarized the surgical strategies of children with epilepsy secondary to VE.
We retrospectively analyzed 23 surgically treated children with VE. The subjects were divided into two groups according to their surgical outcome. All presurgical evaluation data were collected and analyzed.
Among the 23 operated children, the mean age at surgery was 6.1 years. Surgeries were hemispherotomy (n = 12), temporal-parietal-occipital disconnection (n = 4), whole corpus callosotomy (n = 3), lobectomy (n = 3), and vagus nerve stimulation (n = 1). The mean patient follow-up time was 37.2 months, and 13 children had a good outcome (ILAE classification 1−3). Univariate analyses revealed that the latency from infection to the first unprovoked seizure, MRI laterality, concordance of PET and MRI abnormalities, and acute postoperative seizure (APOS) were prognostic factors of seizure outcomes (P < 0.05). No correlation was found between generalized seizures and poor outcome (P = 0.229).
We concluded that the children who achieve favorable surgical outcomes are those with longer latency, unilateral abnormalities on MRI, consistency of PET and MRI abnormalities, and no APOS. Without invasive studies, epilepsy surgery may be successful for selected children with epilepsy after VE, despite diffuse interictal epileptiform discharges on scalp EEG. In addition, children with generalized seizures were not an absolute contraindication for surgery.
Evolution of Surgical Management for Intractable Epileptic Spasms
2020, Seminars in Pediatric Neurology
The understanding and management of epileptic spasms has considerably evolved since the mid 19th century. The realization that epileptic spasms can be generated from a focal brain lesion played a pivotal role in the development of neurosurgical management for intractable forms of this epilepsy. During pre-surgical planning, the addition of functional FDG PET imaging has further refined the electroencephalographic localization of epileptogenic lesions. In some cases, neurosurgical resection of a focus that is co-localized by the FDG PET scan and electroencephalography can lead to partial or complete reversal of developmental delay along with reduced seizure frequency or seizure freedom. In cases where near-complete hemispheric cortex is implicated in spasm generation, subtotal hemispherectomy has shown encouraging results. Moreover, palliative resection of the major perpetrating focus in carefully chosen patients with bilateral multifocal spasms has also led to favorable outcomes. However, in patients with tuberous sclerosis with high tuber burden, the localizing value of FDG PET imaging may be limited. In such cases, employment of AMT PET technology has become a valuable tool for localization of actively epileptogenic tubers. This article highlights the historic steps in the successful advancements of neurosurgical interventions for the treatment of intractable epileptic spasms.
Generalized epilepsies
2019, Handbook of Clinical Neurology
Idiopathic generalized epilepsies (IGE) are characterized by normal background EEG activity and generalized interictal spike-and-wave discharges in the absence of any evidence of brain lesion. Absence epilepsies are the prototypes of IGEs. In childhood and juvenile absence epilepsies, by definition, all patients manifest absence seizures associated with an EEG pattern of generalized spike-wave (GSW) discharges. In juvenile myoclonic epilepsy, myoclonic jerks, usually affecting shoulders and arms bilaterally and appearing upon awakening, are the most characteristic clinical feature. Myoclonic jerks are accompanied on the EEG by generalized spike/polyspike-and-wave (GSW, GPWS) complexes at 3.5–6 Hz. Idiopathic generalized epilepsy with generalized tonic–clonic seizures only is a broad and nonspecific category including all patients with generalized tonic–clonic seizures and an interictal EEG pattern of GSW discharges. Despite the strong heritability and the recent advances in genetic technology, the genetic basis of IGEs remains largely elusive and only in a small minority of patients with classic IGE phenotypes is a monogenic cause identified. Early myoclonic encephalopathy (EME), early infantile encephalopathy with suppression bursts, West syndrome, and Lennox–Gastaut syndrome, once classified among the generalized epilepsies, are now considered to be epileptic encephalopathies. Among them, only Lennox–Gastaut syndrome is characterized by prominent generalized clinical and EEG features.
Epileptic spasms — 175 years on: Trying to teach an old dog new tricks
2017, Seizure
This text provides an overview of how the condition "infantile spasms" has evolved in the last 175 years.
Key references are summarised to assimilate this review. Results: Infantile spasms, first described by Dr West in 1841, has undergone extensive investigation to understand the pathogenesis, aetiologies, optimal intervention and most likely prognosis for the affected child. The terminology has recently evolved such that the preferred term for the condition is now “epileptic spasms” in recognition of the fact that cases can present outside infancy. The aetiologies are diverse and can be structural, genetic, metabolic or acquired. Increasing numbers of presumed causative genetic mutations are now being identified. The condition is an epileptic encephalopathy such that without adequate control of the clinical seizures and correction of the abnormal EEG, ongoing neurological damage occurs. In some cases neuroregression is inevitable despite intervention. First-line treatments are either hormonal therapies, adrenocortcotrophic hormone or prednisolone, or vigabatrin. In the sub-group of patients with tuberous sclerosis complex, vigabatrin is the preferred treatment. High dose prednisolone may be a more viable option in resource limited settings. Recent research has suggested that combining hormonal therapies with vigabatrin will result in more patients achieving spasm cessation.
Despite extensive study, the pathogenic mechanisms remain an area of debate and in need of further exploration. The enigma, however, may be explained as the role of resting state and dysfunctional brain networks are elucidated further.

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Original articleFocal features in West syndrome indicating candidacy for surgery

Abstract

Lancet

Pediatr Neurol

Pediatr Neurol

Brain Dev

Rev Electroencephalogr Neurophysiol Clin

Surgery for infantile spasms: Neuroimaging perspective

Epilepsia

Asymmetric hypsarrhythmia and infantile spasms in West syndrome

J Child Neurol

Asymmetric hypsarrhythmia: Clinical electroencephalographic and radiological findings

Epilepsia

Asymmetric and asynchronous infantile spasms

Epilepsia

Proposal for revised classification of epilepsies and epileptic syndromes

Epilepsia

Sebaceous nevus of Jadassohn associated with infantile spasms

An Esp Pediatr

Infantile spasms, hypsarrhythmia and mental retardation. A study of etiology in 61 patients

Epilepsia

Infantile spasms. I: PET identifies focal cortical dysgenesis in cryptogenic cases for surgical treatment

Ann Neurol

Infantile spasms associated with hemihypsarrhythmia and hemimegalencephaly

Dev Med Child Neurol

Electron microscopic and Golgi study in a case of hemimegalencephaly

Acta Neuropathol

Twenty-four-hour ambulatory EEG monitoring in infantile spasms

Epilepsia

Epilepsy in patients with classical agyria-pachygyria

Boll Lega Ital Epil

Lissencephaly (agyria-pachygyria). Clinical findings and serial EEG studies

Dev Med Child Neurol

Facilitation of infantile spasms by partial seizures

Epilepsia

Infantile spasms and neuronal heterotopias

Acta Pediatr Scand

Original article
Focal features in West syndrome indicating candidacy for surgery