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 Expanding the Spectrum of EEG Periodic Discharges in Subacute Sclerosing Panencephalitis: A Case Report

2021 ◽  
Vol 11 (01) ◽  
pp. e245-e249
Author(s):  
Iliyana Aleksandrova ◽  
Asya Asenova ◽  
Daniela Deneva ◽  
Veneta Bojinova
Keyword(s):  
Case Report ◽  
Slow Wave ◽  
Subacute Sclerosing Panencephalitis ◽  
Epileptiform Activity ◽  
Epileptic Seizures ◽  
High Amplitude ◽  
The Body ◽  
Diagnostic Process ◽  
Sharp Waves ◽  
Eeg Abnormalities

Abstract Background Atypical electroencephalogram (EEG) abnormalities emerge in patients with subacute sclerosing panencephalitis (SSPE), especially in cases with an atypical clinical presentation that can lead to diagnostic difficulties. Case Report In this article, we presented a case of SSPE with an atypical onset with epileptic seizures and Parkinson's features. The neurological examination during the initial evaluation of the patient showed extrapyramidal syndrome, hyperreflexia, intention tremor, and dysmetria. Cranial magnetic resonance imaging was normal. Video EEGs were performed in wakefulness and sleep. In wakefulness, multiple brief seizures (1–1.5 seconds were recorded, consisting of gradual bending of the body forward and to the right that lacked the sudden characteristic of myoclonia. During those episodes, we recorded generalized epileptiform activity of 4 or 5 sharp waves, with higher amplitude in the anterior regions, in some of the paroxysms superimposed on a slow wave or followed by a high amplitude slow wave. The paroxysms appeared periodically every 15 to 30 seconds. However, 2 months later, the EEG showed typical periodic generalized activity of biphasic/triphasic slow waves (Radermecker complexes), accompanied by myoclonias. Conclusion We reported a peculiar EEG pattern in a patient with SSPE that consists of periodic generalized activity of sharp waves. Atypical EEG patterns can appear when the disease progresses, but initially too, before typical periodic complexes and can complicate the diagnostic process.

Seizures with Apnea in Children

PEDIATRICS ◽  
1982 ◽  
Vol 70 (1) ◽  
pp. 87-90
Author(s):  
Kazuyoshi Watanabe ◽  
Kimiko Hara ◽  
Susumu Hakamada ◽  
Tamiko Negoro ◽  
Midori Sugiura ◽  
...  
Keyword(s):  
Limbic System ◽  
Epileptic Seizures ◽  
High Amplitude ◽  
The Other ◽  
Partial Seizures ◽  
Complex Partial Seizures ◽  
Sharp Waves ◽  
Fast Waves ◽  
Respiratory Embarrassment ◽  
Epileptic Fits

Five children who had apneic attacks as a manifestation of epileptic seizures are reported. In three children, the apneic attacks were the sole symptom of epileptic fits. The other two children had additional types of seizures. The apneic seizures usually lasted one to two minutes and were characterized by respiratory embarrassment rather than complete arrest. These seizures are usually accompanied by cyanosis which appears early during the course of the attack. Although consciousness seemed to be impaired, this was difficult to assess accurately in infants. The ictal EEGs disclosed paroxysmal discharges of focal onset in all cases, consisting of high amplitude θ-waves, gradually mixed with or replaced by sharp waves or spikes in some and fast waves followed by slower waves in others. These occurred in temporal areas in three patients and in frontal and central areas in one patient each. Interictal EEGs, however, revealed paroxysmal discharges in only one patient. Apneic attacks in these infants are considered to be an integral part of the manifestations of complex partial seizures originating mostly in the limbic system.

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 Continuous spike-and-wave activity during slow-wave sleep in patients with rett syndrome

2021 ◽  
Vol 16 (1-2) ◽  
pp. 63-68
Author(s):  
S. L. Kulikova ◽  
I. V. Kozyreva ◽  
S. A. Likhachev ◽  
M. Yu. Bobylova
Keyword(s):  
Rett Syndrome ◽  
Slow Wave ◽  
Slow Wave Sleep ◽  
Epileptiform Activity ◽  
Epileptic Seizures ◽  
High Amplitude ◽  
Speech Development ◽  
Early Age ◽  
Entire Observation Period ◽  
Observation Period

The article presents a description of a clinical case of a child 3 years 8 months old with Rett syndrome caused by the mutation of p.Val485fs in the MECP2 gene. According to electroencephalography data at the age of 1 year and 6 months, diffuse continued epileptiform activity in the form of high-amplitude (up to 300 μV) acute – slow wave complexes (continuous spike-waves during slow-wave sleep, CSWS) with an index of 90–100 % was revealed. At the control examination at the age of 2 years and 10 months diffuse epileptiform activity was replaced by multifocal activity with an index of up to 70–80 % at certain epochs, in general, not exceeding 50–60 %. During the entire observation period there were no epileptic seizures. It remains unknown whether the presence of CSWS at such an early age is a predictor of a more severe course of Rett syndrome – in our observation the girl did not acquire walking skills and a delay in psychic and speech development was evident already before the 12 month of life. more research is needed on the frequency of the CSWS phenomenon and its role in the development of clinical features in Rett syndrome.

scholarly journals Mild Clinical Presentation of Joubert Syndrome in a Male Adult Carrying Biallelic MKS1 Truncating Variants

Diagnostics ◽  
2021 ◽  
Vol 11 (7) ◽  
pp. 1218
Author(s):  
Raffaella Brunetti-Pierri ◽  
Marianthi Karali ◽  
Francesco Testa ◽  
Gerarda Cappuccio ◽  
Maria Elena Onore ◽  
...  
Keyword(s):  
Wide Spectrum ◽  
Clinical Manifestations ◽  
Brain Magnetic Resonance Imaging ◽  
Joubert Syndrome ◽  
Male Adult ◽  
Full Field ◽  
Pathogenic Variants ◽  
Disease Spectrum ◽  
Male Individual ◽  
The Central Nervous System

Pathogenic variants in the MKS1 gene are responsible for a ciliopathy with a wide spectrum of clinical manifestations ranging from Meckel and Joubert syndrome (JBTS) to Bardet-Biedl syndrome, and involving the central nervous system, liver, kidney, skeleton, and retina. We report a 39-year-old male individual presenting with isolated Retinitis Pigmentosa (RP), as assessed by full ophthalmological evaluation including Best-Corrected Visual Acuity measurements, fundus examination, Goldmann Visual Field test, and full-field Electroretinography. A clinical exome identified biallelic nonsense variants in MKS1 that prompted post-genotyping investigations for systemic abnormalities of ciliopathy. Brain magnetic resonance imaging revealed malformations of the posterior cranial fossa with the ‘molar tooth sign’ and cerebellar folia dysplasia, which are both distinctive features of JBTS. No other organ or skeletal abnormalities were detected. This case illustrates the power of clinical exome for the identification of the mildest forms of a disease spectrum, such as a mild JBTS with RP in the presented case of an individual carrying biallelic truncating variants in MKS1.

WDR45 Gene and Its Role in Pediatric Epilepsies

2021 ◽  
Author(s):  
Federica Filosco ◽  
Sebastiano Billone ◽  
Ausilia Collotta ◽  
Tiziana Timpanaro ◽  
Monica Tosto ◽  
...  
Keyword(s):  
Language Impairment ◽  
Pediatric Patients ◽  
De Novo ◽  
Somatic Mosaicism ◽  
Brain Magnetic Resonance Imaging ◽  
Iron Accumulation ◽  
Psychomotor Development ◽  
Specific Gene ◽  
Brain Iron ◽  
De Novo Mutations

AbstractWD repeat domain 45 (WDR45) gene has been increasingly found in patients with developmental delay (DD) and epilepsy. Previously, WDR45 de novo mutations were reported in sporadic adult and pediatric patients presenting iron accumulation, while heterozygous mutations were associated with β-propeller protein-associated neurodegeneration (BPAN), a subtype of neurodegeneration with brain iron accumulation disorders, characterized by extrapyramidal movement disorders and abnormal accumulation of iron in the basal ganglia. Overall, people harboring WDR45 mutations have moderate to severe DD and different types of seizures. The phenotype of adult patients is characterized by extrapyramidal movement, dystonia, parkinsonism, language impairment, and involvement of the substantia nigra and in the globus pallidus at brain magnetic resonance imaging. Importantly, there are no findings of brain iron accumulation in brain in BPAN patients in the first decade of life, thus suggesting a progressive course of the disease. Comparatively, the main phenotype of pediatric patients is epilepsy with early onset, most of which present infantile spasms and arrest or regression of psychomotor development. The phenotype of patients with WDR45 mutations is variable, being different if caused by somatic mosaicism or germline mutations, and presenting with a different spectrum of manifestations in males and females. The treatment of affected individuals is symptomatic. Regarding the seizures, specific, gene-based approaches with specific antiepileptic drugs are not currently available. The early diagnosis of BPAN could be useful in some aspects, such as providing families a supportive treatment to their affected children.

scholarly journals Clinical and electroencephalographic characteristics of benign occipital epilepsy of childhood in two tertiary Brazilian hospitals

2011 ◽  
Vol 69 (4) ◽  
pp. 648-653 ◽  
Author(s):  
Soniza Vieira Alves-Leon ◽  
Renata Gomes Nunes ◽  
Maria Emilia Cosenza Andraus ◽  
José Carlos Biagini Junior ◽  
Marta Hemb ◽  
...  
Keyword(s):  
Slow Wave ◽  
Late Onset ◽  
Population Sample ◽  
Occipital Region ◽  
Visual Hallucinations ◽  
Sharp Waves ◽  
Late Form

This study intended to investigate the clinical and electroencephalographic benign occipital epilepsy of childhood (BOEC) characteristics in a population sample of patients from two tertiary Brazilian hospitals. We analyzed retrospectively 4912 electroencephalograms (EEGs) records, and the included patients were submitted to a new clinical and EEG evaluation. Were included 12 (0.92%) patients; 4 (33.3%) with criteria for early BOEC; 6 (50%) for late form and 2 (16.7%) with superimposed early and late onset forms. After new investigation, 2 (16.7%) had normal EEG; 4 (33.3%) had paroxysms over the occipital region; 3 (25%) over the temporal posterior regions and 3 (25%) over the posterior regions. Sharp waves were the predominant change, occurring in 8 (66.6%); spike and slow wave complexes in 1 (8.3%) and sharp and slow wave complexes in 1 (8.3%). Vomiting, headache and visual hallucinations were the most common ictal manifestations, presented in 100% of patients with superimposed forms. Vomiting were absent in the late form and headache was present in all forms of BOEC.

Broad Sharp Waves—An Underrecognized EEG Pattern in Patients with Epileptic Seizures

2008 ◽  
Vol 25 (5) ◽  
pp. 250-254 ◽  
Author(s):  
Gerhard Bauer ◽  
Richard Bauer ◽  
Judith Dobesberger ◽  
Iris Unterberger ◽  
Martin Ortler ◽  
...  
Keyword(s):  
Epileptic Seizures ◽  
Sharp Waves ◽  
Eeg Pattern

scholarly journals Surgical treatment of drug resistant epilepsy in Sturge-Weber syndrome: review of the literature and clinical case presentation

2019 ◽  
Vol 11 (1) ◽  
pp. 53-62
Author(s):  
G. S. Ibatova ◽  
S. K. Akshulakov ◽  
S. M. Malyshev ◽  
R. G. Khachatryan ◽  
T. M. Alekseeva ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Medical Records ◽  
Clinical Manifestations ◽  
Epileptic Seizures ◽  
Positive Outcomes ◽  
Class Iii ◽  
Case Presentation ◽  
Weber Syndrome ◽  
Sturge Weber Syndrome ◽  
Drug Resistant Epilepsy

The paper addresses the relatively rare inherited neurodermal disorder – Sturge-Weber syndrome that can manifest in epileptic seizures. We describe updated concepts, epidemiology, etiology, pathogenesis, clinical manifestations, and surgical treatment of the disease. We examined medical records of 21 patients (aged from 1 to 11 years) with Sturge-Weber syndrome treated over the period of 1996-2016. After surgical treatment of 10 patients (five cases with hemispheretomy and five – with multifocal resection), positive outcomes (Engel class I, II) were found in 70% of cases, and negative (Engel class III, IV) – in 20% of cases. Оne child suddenly died during epileptic seizures. In non-operated children (age from 2 to 5 years) under our observation, an improvement was noted in six cases, no changes – in three cases, and a further progression of the disease – in three cases. In this article, we analyze two of these cases in detail.

scholarly journals Absence Seizure Detection Algorithm for Portable EEG Devices

2021 ◽  
Vol 12 ◽  
Author(s):  
Pawel Glaba ◽  
Miroslaw Latka ◽  
Małgorzata J. Krause ◽  
Sławomir Kroczka ◽  
Marta Kuryło ◽  
...  
Keyword(s):  
Detection Rate ◽  
Low Cost ◽  
Clinical Manifestations ◽  
Epileptic Seizures ◽  
Seizure Detection ◽  
Detection Algorithm ◽  
Absence Epilepsy ◽  
Absence Seizure ◽  
Epileptiform Discharges ◽  
Morlet Continuous Wavelet Transform

Absence seizures are generalized nonmotor epileptic seizures with abrupt onset and termination. Transient impairment of consciousness and spike-slow wave discharges (SWDs) in EEG are their characteristic manifestations. This type of seizure is severe in two common pediatric syndromes: childhood (CAE) and juvenile (JAE) absence epilepsy. The appearance of low-cost, portable EEG devices has paved the way for long-term, remote monitoring of CAE and JAE patients. The potential benefits of this kind of monitoring include facilitating diagnosis, personalized drug titration, and determining the duration of pharmacotherapy. Herein, we present a novel absence detection algorithm based on the properties of the complex Morlet continuous wavelet transform of SWDs. We used a dataset containing EEGs from 64 patients (37 h of recordings with almost 400 seizures) and 30 age and sex-matched controls (9 h of recordings) for development and testing. For seizures lasting longer than 2 s, the detector, which analyzed two bipolar EEG channels (Fp1-T3 and Fp2-T4), achieved a sensitivity of 97.6% with 0.7/h detection rate. In the patients, all false detections were associated with epileptiform discharges, which did not yield clinical manifestations. When the duration threshold was raised to 3 s, the false detection rate fell to 0.5/h. The overlap of automatically detected seizures with the actual seizures was equal to ~96%. For EEG recordings sampled at 250 Hz, the one-channel processing speed for midrange smartphones running Android 10 (about 0.2 s per 1 min of EEG) was high enough for real-time seizure detection.

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