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Epilepsia Open Sep 2022This retrospective study was designed to observe differences in ictal movements of epileptic spasm (ES) before and after corpus callosotomy (CC). We hypothesized that...
Asymmetric epileptic spasms after corpus callosotomy in children with West syndrome may be a good indicator for unilateral epileptic focus and subsequent resective surgery.
OBJECTIVE
This retrospective study was designed to observe differences in ictal movements of epileptic spasm (ES) before and after corpus callosotomy (CC). We hypothesized that asymmetric expression of ES is more clarified after CC and would be a good indicator for the epileptic hemisphere.
METHODS
We selected 16 patients with intractable ES in West syndrome who were seizure-free after CC and subsequent resection or disconnective surgery of the unilateral hemisphere. We retrospectively reviewed their behavioral ES recorded at video-electroencephalography monitoring before and after CC. Asymmetric neck flexion (NF) and involuntary muscular contraction of the upper and lower extremities (MCU and MCL, respectively) were primarily described correlating their laterality and the responsible hemisphere proved by surgical resection.
RESULTS
Asymmetric NF, MCU, and MCL could be found both before and after CC. However, the percentage of those movements to the total number of ES increased after CC; asymmetric NF, 82.9% vs. 20.1%; unilaterally predominant MCU, 81% vs. 39.3%; and unilaterally predominant MCL, 77.6% vs. 29.9%. Regarding asymmetric NF, the direction in which the neck flexed or the head turned was significantly ipsilateral to the responsible hemisphere in 9 of 12 patients after CC (75%). The predominant side of MCU and MCL were significantly contralateral to the responsible hemisphere in 11 of 11 and 7 of 9 patients (100% and 77.8%, respectively).
SIGNIFICANCE
Asymmetric NF, MCU, and MCL were clarified in patients with ES who were successfully treated with CC and subsequent surgery. Those changes in ictal behaviors after CC may indicate the lateralization of epileptic activity and encourage more curative surgical treatment.
Topics: Child; Corpus Callosum; Epilepsy; Humans; Retrospective Studies; Spasm; Spasms, Infantile
PubMed: 35869791
DOI: 10.1002/epi4.12631 -
Epilepsia Jun 2013By combining electroencephalography (EEG) with functional magnetic resonance imaging (fMRI) it is possible to describe blood oxygenation level-dependent (BOLD) signal... (Review)
Review
By combining electroencephalography (EEG) with functional magnetic resonance imaging (fMRI) it is possible to describe blood oxygenation level-dependent (BOLD) signal changes related to EEG patterns. This way, EEG-pattern-associated networks of hemodynamic changes can be detected anywhere in the brain with good spatial resolution. This review summarizes EEG-fMRI studies that have been performed in children with epilepsy. EEG-fMRI studies in focal epilepsy (structural and nonlesional cases, benign epilepsy with centrotemporal spikes), generalized epilepsy (especially absence epilepsy), and epileptic encephalopathies (West syndrome, Lennox-Gastaut syndrome, continuous spike and waves during slow sleep, and Dravet syndrome) are presented. Although EEG-fMRI was applied mainly to localize the region presumably generating focal interictal discharges in focal epilepsies, EEG-fMRI identified underlying networks in patients with generalized epilepsies and thereby contributed to a better understanding of these epilepsies. In epileptic encephalopathies a specific fingerprint of hemodynamic changes associated with the particular syndrome was detected. The value of the EEG-fMRI technique for diagnosis and investigation of pathogenetic mechanisms of different forms of epilepsy is discussed.
Topics: Brain; Child; Electroencephalography; Epilepsies, Partial; Epilepsy; Epilepsy, Generalized; Functional Neuroimaging; Humans; Infant; Magnetic Resonance Imaging; Spasms, Infantile
PubMed: 23647021
DOI: 10.1111/epi.12197 -
Seizure Feb 2023The molecular mechanisms leading to infantile epileptic spasm syndrome (IESS) remain obscure. The only common factor seems to be that the spasms are restricted to a... (Review)
Review
The molecular mechanisms leading to infantile epileptic spasm syndrome (IESS) remain obscure. The only common factor seems to be that the spasms are restricted to a limited period of infancy, during a certain maturational state. Here the current literature regarding the biochemical mechanisms of brain maturation in IESS is reviewed, and various hypotheses of the pathophysiology are put together. They include: (1) imbalance of inhibitory (NGF, IGF-1, ACTH, GABA) and excitatory factors (glutamate, nitrites) which distinguishes the different etiological subgroups, (2) abnormality of the hypothalamic pituitary adrenal (HPA) axis linking insults and early life stress, (3) inflammation (4) yet poorly known genetic and epigenetic factors, and (5) glucocorticoid and vigabatrin action on brain development, pinpointing at molecular targets of the pathophysiology from another angle. An altered maturational process may explain why so many, seemingly independent etiological factors lead to the same clinical syndrome and frequently to developmental delay. Understanding these factors can provide ideas for novel therapies.
Topics: Humans; Infant; Spasms, Infantile; Epilepsy; Vigabatrin; Glucocorticoids; Spasm; Anticonvulsants
PubMed: 36634586
DOI: 10.1016/j.seizure.2023.01.004 -
Epilepsia 2002Neonatal seizures frequently accompany neonatal encephalopathies. Seizures occur in approximately 1.8-5/1,000 live births in this country and are caused by virtually any... (Review)
Review
Neonatal seizures frequently accompany neonatal encephalopathies. Seizures occur in approximately 1.8-5/1,000 live births in this country and are caused by virtually any condition that affects neonatal brain function. This review provides a simple classification of seizures and emphasizes that many abnormal intermittent behaviors in this age group are not accompanied by ictal EEG patterns. Additionally, < or =50% of neonatal seizures are not associated with abnormal clinical behavior. This is a common phenomenon, particularly after anticonvulsant treatment in which the clinical seizures are suppressed but electrographic seizures continue unabated. Seizures also may be caused by genetic disorders, several of which are benign, familial, and caused by channelopathies involving potassium channels. The review also discusses the epileptic syndromes seen in neonates, including early myoclonic encephalopathy, Ohtahara syndrome, pyridoxine dependency, and glucose transporter type 1 syndrome.
Topics: Age Factors; Electroencephalography; Epilepsy; Humans; Infant, Newborn; Seizures; Spasms, Infantile; Syndrome
PubMed: 12060001
DOI: 10.1046/j.1528-1157.43.s.3.11.x -
Journal of Clinical Neurophysiology :... Oct 2012Neonatal seizures are common, often require EEG monitoring for diagnosis and management, may be associated with worse neurodevelopmental outcome, and can often be... (Review)
Review
Neonatal seizures are common, often require EEG monitoring for diagnosis and management, may be associated with worse neurodevelopmental outcome, and can often be treated with existing anticonvulsants. A neonatal electrographic seizure is defined as a sudden, repetitive, evolving, and stereotyped event of abnormal electrographic pattern with amplitude of at least 2 μV and a minimum duration of 10 seconds. The diagnosis of neonatal seizures relies heavily on the neurophysiologist's interpretation of EEG. Consideration of specific criteria for the definition of a neonatal seizure, including seizure duration, location, morphology, evolution, semiology, and overall seizure burden, has utility for both the clinician and the researcher. The importance of EEG in the diagnosis and management of neonatal seizures, the electrographic characteristics of neonatal seizures, the impact of neonatal seizures on outcome, and tools to aid in the identification of neonatal seizures are reviewed.
Topics: Anticonvulsants; Brain; Brain Mapping; Brain Waves; Electroencephalography; Humans; Infant, Newborn; Periodicity; Predictive Value of Tests; Spasms, Infantile; Status Epilepticus; Time Factors; Treatment Outcome
PubMed: 23027101
DOI: 10.1097/WNP.0b013e31826bd90d -
Epilepsia Nov 2004To reach a broad consensus on case definitions, outcomes, and outcome measures that will ease future study design and facilitate comparison of data from different... (Comparative Study)
Comparative Study Review
PURPOSE
To reach a broad consensus on case definitions, outcomes, and outcome measures that will ease future study design and facilitate comparison of data from different studies of infantile spasms and West syndrome.
METHODS
Persons who had recently presented or published first-author original research in this field were invited to participate in an e-mail Delphi process and to invite other investigators or clinicians who they thought might participate.
RESULTS
The process consisted of six rounds, anonymous except to the facilitator. In total, responses were received from 46 participants. The final statement was approved by 31 participants from 15 countries. It concluded that the primary clinical outcome, cessation of spasms, should denote absence of witnessed spasms from within 14 days of commencement of treatment, and for > or =28 consecutive days from the last witnessed spasm. Primary electroclinical outcome denotes cessation of spasms with resolution of hypsarrhythmia. West syndrome should be a defined subset of the syndrome of infantile spasms. An infantile spasms single-spasm variant should be recognized. Ways are suggested of handling subtle spasms in the context of clinical studies. It proposes a standard for reporting modifying and atypical features of hypsarrhythmia, a minimal set of baseline characteristics and outcomes that should be reported in trials of infantile spasms, and suggests a standard definition of relapse. Consensus was not reached on a definition of hypsarrhythmia.
CONCLUSIONS
We reached a clear consensus on many aspects of study design for the investigation of infantile spasms, although incomplete consensus was found on how to define EEG criteria.
Topics: Child, Preschool; Delphi Technique; Electroencephalography; Electronic Mail; Humans; Infant; Outcome Assessment, Health Care; Spasms, Infantile; Terminology as Topic; Treatment Outcome
PubMed: 15509243
DOI: 10.1111/j.0013-9580.2004.02404.x -
Epilepsia Apr 2015To prospectively evaluate the etiology of new-onset infantile spasms and evaluate the yield of genetic and metabolic investigations in those without obvious cause after...
OBJECTIVE
To prospectively evaluate the etiology of new-onset infantile spasms and evaluate the yield of genetic and metabolic investigations in those without obvious cause after initial clinical evaluation and magnetic resonance imaging (MRI).
METHODS
Twenty-one U.S. pediatric epilepsy centers prospectively enrolled infants with newly diagnosed West syndrome in a central database. Etiology and investigations performed within 3 months of diagnosis were documented.
RESULTS
From June 2012 to June 2014, a total of 251 infants were enrolled (53% male). A cause was identified in 161 (64.4%) of 250 cases (genetic,14.4%; genetic-structural, 10.0%; structural-congenital, 10.8%; structural-acquired, 22.4%; metabolic, 4.8%; and infectious, 2.0%). An obvious cause was found after initial clinical assessment (history and physical examination) and/or MRI in 138 of 161, whereas further genetic and metabolic studies were revealing in another 23 cases. Of 112 subjects without an obvious cause after initial evaluation and MRI, 81 (72.3%) had undergone genetic testing, which showed a causal abnormality in 23.5% and a variant of unknown significance in 14.8%. Although metabolic studies were done in the majority (serum, 79.5%; urine, 69.6%; and cerebrospinal fluid [CSF], 38.4%), these revealed an etiology in only five cases (4.5%). No correlation was found between type of health insurance (public vs. private) and either genetic or metabolic testing.
SIGNIFICANCE
Clinical evaluation and MRI provide a specific diagnosis in 55% of children presenting with West syndrome. We propose that a cost-effective workup for those without obvious cause after initial clinical evaluation and MRI includes an array comparative genomic hybridization (aCGH) followed by an epilepsy gene panel if the microarray is not definitive, serum lactate, serum amino acids, and urine organic acids.
Topics: Child; Follow-Up Studies; Humans; Infant; Infant, Newborn; Prospective Studies; Spasms, Infantile; Treatment Outcome; United States
PubMed: 25779538
DOI: 10.1111/epi.12951 -
Medicina Aug 2022The International League Against Epilepsy (ILAE) recently socialized the proposed classification for epileptic syndromes of neonatal onset and up to the first 2 years of... (Review)
Review
The International League Against Epilepsy (ILAE) recently socialized the proposed classification for epileptic syndromes of neonatal onset and up to the first 2 years of age, dividing them into self-limited epileptic syndromes and epileptic and developmental encephalopathies (DEEs). In this review we will focus on DEEs, defined as disorders in which there is developmental impairment related to both the underlying aetiology independent of epileptiform activity and epileptic encephalopathy. These include early infantile epileptic encephalopathy or Ohtahara syndrome and early myoclonic encephalopathy in the neonatal period, now grouped under the name of epileptic and early childhood developmental encephalopathies (EIDEE). Infantile epileptic spasms syndrome, childhood epilepsy with migratory crises and Dravet syndrome are part of the infant-onset encephalopathies. The importance of early recognition of epileptic encephalopathies lies not only in the control of epileptic seizures, but also in stopping deterioration by trying to change the course of the disease. It is essential to know the etiology, avoiding medications that can exacerbate seizures and worsen the course, applying precision m edicine as well as identifying candidate patients for early epilepsy surgery.
Topics: Brain Diseases; Child, Preschool; Electroencephalography; Epilepsy; Epileptic Syndromes; Humans; Infant; Infant, Newborn; Seizures; Spasms, Infantile
PubMed: 36054851
DOI: No ID Found -
Archives of Disease in Childhood Apr 2005Corticosteroids (predominantly prednisolone and hydrocortisone) and adrenocorticotropic hormone (ACTH) have been used in the treatment of the epilepsies for over 50... (Review)
Review
Corticosteroids (predominantly prednisolone and hydrocortisone) and adrenocorticotropic hormone (ACTH) have been used in the treatment of the epilepsies for over 50 years. Over the past 30 years most reports have focused on epilepsy syndromes and epileptic encephalopathies resistant to treatment with the more conventional anticonvulsant and antiepileptic drugs (AEDs) and specifically West syndrome. There has been relatively little attention on the role of corticosteroids in treating other epilepsies.
Topics: Adrenal Cortex Hormones; Anticonvulsants; Child; Epilepsy; Humans; Infant; Spasms, Infantile; Syndrome
PubMed: 15781928
DOI: 10.1136/adc.2004.051375 -
Revista de NeurologiaWest's syndrome (WS), which is also known as infantile myoclonic encephalopathy with hypsarrhythmia, is one of the generalized epileptic syndromes with a cryptogenic or... (Review)
Review
West's syndrome (WS), which is also known as infantile myoclonic encephalopathy with hypsarrhythmia, is one of the generalized epileptic syndromes with a cryptogenic or symptomatic origin. It is an age-dependent epileptic syndrome. The latest neuroimaging techniques have enabled us to gain a better understanding of its physiopathology and to identify new aetiological factors responsible for the clinical symptoms. WS can be due to a number of aetiologies, the most notable of which are congenital errors of metabolism. The incidence of cases due to phenylketonuria or hypoglycaemia is currently diminishing, yet, there is a rise in the number of new metabolic diseases that are responsible for the symptoms of WS. These include the carbohydrate-deficient glycoprotein syndromes or biotinidase deficiency. In all cases, and especially so in those that are idiopathic, it is wise to conduct exhaustive aetiological studies, since on some occasions metabolic diseases will be shown to be responsible, and this will then modify the prognosis, therapy and genetic counselling. It is important to have a protocol for both study and therapy available for this syndrome. The therapeutic options available can be implemented after ruling out a neurometabolic disease as being responsible for the syndrome and quickly beginning treatment with vigabatrine, sodium valproate plus pyridoxine, ACTH or hydrocortisone. If there is no response then topiramate can be used. Other therapeutic options, such as the use of zonisamide, a ketogenic diet or even surgical treatment, are also analyzed.
Topics: Clinical Protocols; Humans; Infant; Spasms, Infantile
PubMed: 14533111
DOI: No ID Found