scholarly journals Secondary late-onset Lennox-Gastaut syndrome: a critical view

1984 ◽  
Vol 42 (2) ◽  
pp. 95-104
Author(s):  
Amilton Antunes Barreira ◽  
Michel Pierre Lison
Keyword(s):  
Late Onset ◽  
Clinical Manifestations ◽  
Epileptic Seizures ◽  
Psychomotor Development ◽  
Average Period ◽  
Partial Seizures ◽  
Simple Complex ◽  
Early Form ◽  
Eeg Abnormalities ◽  
Interictal Eeg

From a group of 66 patients with the Lennox-Gastaut syndrome, 12 whose manifestations had started after the 6th year of life were selected for study. These patients were observed clinically and electroencephalographically for an average period of 2.5 years. We concluded that the late-onset syndrome can: occur after a long interval between diffuse encephalopathy and the first clinical manifestations, with or without previous alterations in psychomotor development; be associated from the onset with serious mental retardation; exhibit simple, complex and mixed seizures similar to those observed in the early form. These patients can also: suffer complex and mixed epileptic seizures previously unreported; paroxismal interictal EEG abnormalities that overlap those of the early form; and spike-slow wave complexes in the EEG that can be actived by hyperpnea. Our results demonstrate that the incidence of LGS after 6 years of age does not necessarily imply a lower frequency of organic antecedents, or beter neu-ropsychomotor development up to the onset of the syndrome or the presence of a higher rate of nonspecific seizures (generalized or partial seizures, and mainly those with elaborate symptomatolgy). The critical and encephalographic expression of the syndrome, which is secondary and starts after the 6th year of age, may depend at least in part on the age when diffuse encephalopathy started.

scholarly journals Childhood epileptic seizures imitating migraine and encephalitis

2012 ◽  
Vol 140 (9-10) ◽  
pp. 558-562
Author(s):  
Ruzica Kravljanac ◽  
Milena Djuric
Keyword(s):  
Antiepileptic Drugs ◽  
Clinical Presentation ◽  
Late Onset ◽  
Clinical Manifestations ◽  
Epileptic Seizures ◽  
Childhood Epilepsy ◽  
Visual Hallucinations ◽  
Seizure Recurrence ◽  
Acute Therapy ◽  
Epileptic Discharges

Introduction. Paroxismal events can resemble epileptic seizures, however, some epileptic seizures, especially benign occipital childhood epilepsies can imitate migraine, cycling vomiting or encephalitis. Objective. The aim of this study was evaluation of clinical and electroencephalographic (EEG) features and outcome in children with benign occipital childhood epilepsies. Methods. Investigation included 18 patients with benign occipital childhood epilepsies hospitalized in the period from 2007 to 2010. The diagnosis was based on clinical and EEG characteristics of seizures, while treatment included acute therapy for seizures and chronic antiepileptic drugs. Prognosis was analyzed in terms of neurological outcome and seizure recurrence rate. Results. Benign occipital childhood epilepsy with early onset was diagnosed in 15 children. Vegetative symptoms, mostly ictal vomiting (13), eye deviation and loss of consciousness (13) dominated in the clinical presentation. The most frequent EEG findings showed occipital epileptic discharges. Benign occipital childhood epilepsy with late onset was diagnosed in three cases. Seizures were manifested by visual hallucinations, headache and secondary generalized convulsions. All three patients were administered chronic antiepileptic drugs and had good outcome. Conclusion. In our patients, clinical manifestations of benign occipital epilepsies had some similarities with clinical features of migraine and encephalitis. It could explain misdiagnosis in some of them. Knowledge about main features and differences between each of these disorders is crucial for making appropriate diagnosis.

scholarly journals Mouse Models Characterize GNAO1 Encephalopathy as a Neurodevelopmental Disorder Leading to Motor Anomalies: from a Severe G203R to a Milder C215Y Mutation

2021 ◽  
Author(s):  
Denis Silachev ◽  
Alexey Koval ◽  
Mikhail Savitsky ◽  
Guru Padmasola ◽  
Charles Quairiaux ◽  
...  
Keyword(s):  
Mouse Models ◽  
De Novo ◽  
Late Onset ◽  
Wide Spectrum ◽  
Neurodevelopmental Disorder ◽  
Point Mutations ◽  
Clinical Manifestations ◽  
Epileptic Seizures ◽  
Motor Dysfunction ◽  
Neuronal Precursor Cells

Abstract GNAO1 encephalopathy characterized by a wide spectrum of neurological deficiencies in pediatric patients originates from de novo heterozygous mutations in the gene encoding Gαo, the major neuronal G protein. Efficient treatments and even the proper understanding of the underlying etiology are currently lacking for this dominant disease. Adequate animal models of GNAO1 encephalopathy are urgently needed. Here we describe establishment and characterization of mouse models of the disease based on two point mutations in GNAO1 with different clinical manifestations. One of them is G203R leading to the early-onset epileptic seizures, motor dysfunction, developmental delay and intellectual disability. The other is C215Y producing much milder clinical outcomes, mostly – late-onset motor hyperactivity. The resultant mouse models show distinct phenotypes: severe neonatal lethality in GNAO1[G203R]/+ mice vs. normal vitality in GNAO1[C215Y]/+. The latter model further revealed strong hyperactivity and hyperlocomotion in a panel of behavioral assays, without signs of epilepsy, recapitulating the patients’ manifestations. Importantly, despite these differences the two models similarly revealed prenatal brain developmental anomalies, such as enlarged lateral ventricles and decreased numbers of neuronal precursor cells in the cortex. Thus, our work unveils GNAO1 encephalopathy as to a large extent neurodevelopmental malady. We expect that this understanding and the tools we established will be instrumental for future therapeutic developments.

Seizure Types, Epilepsy Syndromes, Etiology, and Diagnosis

CNS Spectrums ◽  
2001 ◽  
Vol 6 (9) ◽  
pp. 750-755 ◽  
Author(s):  
Jonathan C. Edwards
Keyword(s):  
Classification System ◽  
Clinical Manifestations ◽  
Epileptic Seizures ◽  
Conscious State ◽  
Cortical Region ◽  
Partial Seizures ◽  
Complex Partial Seizures ◽  
Metabolic Derangement ◽  
Surgical Evaluation ◽  
Recent Classification

ABSTRACTThe clinical manifestation of epileptic seizures may vary widely from patient to patient, depending on the region of the brain involved. Over the centuries, many seizure classific systems have been used, and the current most widely used classification system is that of the International League Against Epilepsy (ILAE). The ILAE system divides seizures into those of partial onset and those of generalized onset, depending on whether the initial clinical manifestations indicate that one cortical region or both hemispheres are involved at the onset of the seizure. Partial seizures are then divided into simple partial seizures, in which a fully conscious state is retained, or complex partial seizures, in which consciousness is impaired. A more recent classification system based purely on symptom features and signs has been proposed, and this system may provide advantages for localization, and especially for surgical evaluation. Epilepsy is a condition characterized by recurrent unprovoked seizures. Epilepsy may be idiopathic, cryptogenic, or symptomatic. Idiopathic epilepsies are generally genetic, and while man such syndromes have been described, advances in molecular genetics will undoubtedly reveal many more syndromes in near future. Cryptogenic epilepsies are those in which an underlying cause is suspected, but the etiology remains undetected. Epilepsies for which there is an underlying structural cause or major metabolic derangement are considered symptomatic. Common causes and diagnostic evaluation are described in this article.

scholarly journals Aggravation of symptomatic occipital epilepsy of childhood by carbamazepine

2014 ◽  
Vol 71 (4) ◽  
pp. 404-407 ◽  
Author(s):  
Fadil Skrijelj ◽  
Mersudin Mulic
Keyword(s):  
Slow Wave ◽  
Clinical Manifestations ◽  
Brain Magnetic Resonance Imaging ◽  
Poor Performance ◽  
Epileptic Seizures ◽  
High Amplitude ◽  
Psychomotor Development ◽  
Vacuum Extraction ◽  
Sharp Waves ◽  
Normal Limits

Introduction. Carbamazepine can lead to aggravation of epileptic seizures in generalized epilepsies (primary or secondary) with clinical manifestations of absence (typical or atypical) and/or myoclonic seizures. However, some focal epilepsies can be also aggravated by the introduction of carbamazepine. Case report. We presented a 10-year-old boy born after a complicated and prolonged delivery completed by vacuum extraction, of early psychomotor development within normal limits. At the age of 8 years he had the first epileptic seizure of simple occipital type with generalization and urination. Brain magnetic resonance imaging (MRI) showed focal cortical reductions in the left parietal and occipital regions. Interictal EEG recorded slowed basic activities above the posterior regions of the left hemisphere, with intermittent occurrence of occipital sharp waves and bioccipital sharp and slow-wave complexes. Initially, treatment with valproate was administered; however, the addition of carbamazepine into therapy induced aggravation of seizures and EEG findings, changed behavior and poor performance at school. By withdrawal of carbamazepine the condition improved both clinically and in EEG findings. Conclusion. Childhood occipital epilepsy lesions show deterioration due to carbamazepine, which if administered induces aggravation of seizures, behavior changes, cognition with occurrence of long-term bilateral discharges, and posterior sharp and slow wave high amplitude complexes recorded by EEG.

scholarly journals Epilepsy in Down Syndrome: A Highly Prevalent Comorbidity

2021 ◽  
Vol 10 (13) ◽  
pp. 2776
Author(s):  
Miren Altuna ◽  
Sandra Giménez ◽  
Juan Fortea
Keyword(s):  
Down Syndrome ◽  
Functional Outcomes ◽  
Clinical Presentation ◽  
Late Onset ◽  
Current Knowledge ◽  
Epileptic Seizures ◽  
Myoclonic Epilepsy ◽  
Increased Risk ◽  
Number Of Individuals

Individuals with Down syndrome (DS) have an increased risk for epilepsy during the whole lifespan, but especially after age 40 years. The increase in the number of individuals with DS living into late middle age due to improved health care is resulting in an increase in epilepsy prevalence in this population. However, these epileptic seizures are probably underdiagnosed and inadequately treated. This late onset epilepsy is linked to the development of symptomatic Alzheimer’s disease (AD), which is the main comorbidity in adults with DS with a cumulative incidence of more than 90% of adults by the seventh decade. More than 50% of patients with DS and AD dementia will most likely develop epilepsy, which in this context has a specific clinical presentation in the form of generalized myoclonic epilepsy. This epilepsy, named late onset myoclonic epilepsy (LOMEDS) affects the quality of life, might be associated with worse cognitive and functional outcomes in patients with AD dementia and has an impact on mortality. This review aims to summarize the current knowledge about the clinical and electrophysiological characteristics, diagnosis and treatment of epileptic seizures in the DS population, with a special emphasis on LOMEDS. Raised awareness and a better understanding of epilepsy in DS from families, caregivers and clinicians could enable earlier diagnoses and better treatments for individuals with DS.

Ictal and interictal EEG abnormalities in ADHD children recorded over night by video-polysomnography

2007 ◽  
Vol 75 (2-3) ◽  
pp. 130-137 ◽  
Author(s):  
Rosalia Silvestri ◽  
Antonella Gagliano ◽  
Tiziana Calarese ◽  
Irene Aricò ◽  
Clemente Cedro ◽  
...  
Keyword(s):  
Eeg Abnormalities ◽  
Adhd Children ◽  
Interictal Eeg

scholarly journals Etiology of epilepsy a prospective study of 210 cases

1991 ◽  
Vol 49 (3) ◽  
pp. 251-254 ◽  
Author(s):  
Walter Oleschko Arruda
Keyword(s):  
Ct Scan ◽  
Neurological Examination ◽  
Epileptic Seizures ◽  
Partial Seizures ◽  
Complex Partial Seizures ◽  
Blood Tests ◽  
Generalized Seizures ◽  
A Prospective Study ◽  
Csf Examination

The objective of this study was to establish the etiology of epilepsy in 210 chronic epileptics (110 female, 100 male), aged 14-82 years (34.2±13.3). Patients less than 10 years-old and alcoholism were excluded. All underwent neurological examination, routine blood tests, EEG and CT-scan. Twenty patients (10.5%) were submitted to spinal tap for CSF examination. Neurological examination was abnormal in 26 (12.4%), the EEG in 68 (45.5%), and CT-scan in 93 (44.3%). According to the International Classification of Epileptic Seizures (1981), 101 (48.1%) have generalized seizures, 66 (31.4%) partial seizures secondarily generalized, 25 (11.8%) simple partial and complex partial seizures, and 14 (6.6%) generalized and partial seizures. Four patients (2.0%) could not be classified. In 125 (59.5%) patients the etiology was unknown. Neurocysticercosis accounted for 57 (27.1%) of cases, followed by cerebrovascular disease 8 (3.8%), perinatal damage 5 (2.4%), familial epilepsy 4 (1.9%), head injury 4 (1.9%), infective 1 (0.5%), and miscelanea 6 (2.8%).

Late onset epileptic seizures A retrospective study of 250 patients

2009 ◽  
Vol 72 (4) ◽  
pp. 380-384 ◽  
Author(s):  
José Luis Pérez López ◽  
Jesús Longo ◽  
Fernando Quintana ◽  
Consuelo Diez ◽  
José Berciano
Keyword(s):  
Retrospective Study ◽  
Late Onset ◽  
Epileptic Seizures

scholarly journals Permutation Entropy-Based Interpretability of Convolutional Neural Network Models for Interictal EEG Discrimination of Subjects with Epileptic Seizures vs. Psychogenic Non-Epileptic Seizures

Entropy ◽  
2022 ◽  
Vol 24 (1) ◽  
pp. 102
Author(s):  
Michele Lo Giudice ◽  
Giuseppe Varone ◽  
Cosimo Ieracitano ◽  
Nadia Mammone ◽  
Giovanbattista Gaspare Tripodi ◽  
...  
Keyword(s):  
Neural Network ◽  
Discriminant Analysis ◽  
Convolutional Neural Network ◽  
Epileptic Seizures ◽  
Permutation Entropy ◽  
Diagnostic Tools ◽  
Support Vector ◽  
Feature Maps ◽  
Time Frequency ◽  
Interictal Eeg

The differential diagnosis of epileptic seizures (ES) and psychogenic non-epileptic seizures (PNES) may be difficult, due to the lack of distinctive clinical features. The interictal electroencephalographic (EEG) signal may also be normal in patients with ES. Innovative diagnostic tools that exploit non-linear EEG analysis and deep learning (DL) could provide important support to physicians for clinical diagnosis. In this work, 18 patients with new-onset ES (12 males, 6 females) and 18 patients with video-recorded PNES (2 males, 16 females) with normal interictal EEG at visual inspection were enrolled. None of them was taking psychotropic drugs. A convolutional neural network (CNN) scheme using DL classification was designed to classify the two categories of subjects (ES vs. PNES). The proposed architecture performs an EEG time-frequency transformation and a classification step with a CNN. The CNN was able to classify the EEG recordings of subjects with ES vs. subjects with PNES with 94.4% accuracy. CNN provided high performance in the assigned binary classification when compared to standard learning algorithms (multi-layer perceptron, support vector machine, linear discriminant analysis and quadratic discriminant analysis). In order to interpret how the CNN achieved this performance, information theoretical analysis was carried out. Specifically, the permutation entropy (PE) of the feature maps was evaluated and compared in the two classes. The achieved results, although preliminary, encourage the use of these innovative techniques to support neurologists in early diagnoses.

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