Epileptic spasms without hypsarrhythmia in infancy and childhood: tonic spasms as a seizure type

2015 ◽  
Vol 17 (2) ◽  
pp. 188-193 ◽  
Author(s):  
Luciana R De Marchi ◽  
Evelyn A Seraphim ◽  
Jeana T Corso ◽  
Pedro VF Naves ◽  
Kelly Cristina de Carvalho ◽  
...  
Keyword(s):  
Seizure Type ◽  
Epileptic Spasms ◽  
Infancy And Childhood ◽  
Tonic Spasms

scholarly journals Ohtahara syndrome: Early infantile epileptic encephalopathy

2008 ◽  
Vol 61 (11-12) ◽  
pp. 581-585 ◽  
Author(s):  
Marija Knezevic-Pogancev
Keyword(s):  
Epileptic Encephalopathy ◽  
Psychomotor Retardation ◽  
Psychomotor Development ◽  
Seizure Type ◽  
Epileptic Spasms ◽  
Ohtahara Syndrome ◽  
Initial Stage ◽  
Interictal Eeg ◽  
Severe Retardation ◽  
Tonic Spasms

DEFINITION Ohtahara syndrome (early infantile epileptic encephalopathy with suppression bursts), is the earliest developing form of epileptic encephalopathy. ETHIOLOGY It considered to be a result of static structural developing brain damage. CLINICAL PICTURE Variable seizures develop mostly within the first 10 days of life, but may occur during the first hour after delivery. The most frequently observed seizure type are epileptic spasms, which may be either generalized and symmetrical or lateralized. The tonic spasms may occur in clusters or singly, while awake and during sleep alike. The duration of spasms is up to 10 seconds, and the interval between spasms within cluster ranges from 9 to 15 seconds. In one third of cases, other seizure types include partial motor seizures or hemiconvulsions The disorder takes a progressively deteriorating course with increasing frequency of seizures and severe retardation of psychomotor development. DIAGNOSTIC WORKUP In the initial stage of Ohtahara syndrome, interictal EEG shows a pattern of suppression-burst with high-voltage paroxysmal discharges separated by prolonged periods of nearly flat tracing that last for up to 18 seconds. PROGNOSIS AND THREATMENT Half of the reported children having Ohtahara syndrome die in infancy. Anticonvulsant helps little in controlling the seizures and halting the deterioration of psychomotor development. Severe psychomotor retardation is the rule. With time, the disorder may evolve into West syndrome or partial epilepsy. Psychomotor development may be slightly better if the infants do not develop West and later Lennox-Gastaut syndrome.

Are Epileptic Spasms a Seizure Type for the Insular Region?

2020 ◽  
Vol 51 (04) ◽  
pp. 295-297 ◽  
Author(s):  
Volodymyr Kharytonov ◽  
Olivier Dulac
Keyword(s):  
Slow Wave ◽  
Late Onset ◽  
Motor Program ◽  
High Amplitude ◽  
The Other ◽  
Seizure Type ◽  
Wave Characteristic ◽  
The Third ◽  
Epileptic Spasms ◽  
Insular Region

AbstractTwo patients with insular and striatal postnatal scar had epileptic spasms (ES) that were asymmetrical and the only seizure type, whereas none of the usual ictal symptoms of insular seizures occurred. Ictal electroencephalography (EEG) showed the high-amplitude slow-wave characteristic of ES. Vigabatrin remained efficient for over 4 years for one patient and right into the third decade for the other one. Such ES are distinct from infantile and late onset spasms. Furthermore, these observations suggest that in ES insular epilepsy triggers paroxysmal activation of the striatum, and that vigabatrin inhibits the striatal startle motor program, thus interrupting the corticostriatal loop.

scholarly journals Symmetry of ictal slow waves may predict the outcomes of corpus callosotomy for epileptic spasms

2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Sotaro Kanai ◽  
Masayoshi Oguri ◽  
Tohru Okanishi ◽  
Shinji Itamura ◽  
Shimpei Baba ◽  
...  
Keyword(s):  
Slow Wave ◽  
High Voltage ◽  
Amplitude Ratio ◽  
Slow Waves ◽  
Corpus Callosotomy ◽  
Factors Associated ◽  
Wave Duration ◽  
Negative Peak ◽  
Epileptic Spasms ◽  
Tonic Spasms

AbstractWe aimed to analyse the ictal electrographic changes on scalp electroencephalography (EEG), focusing on high-voltage slow waves (HVSs) in children with epileptic spasms (ES) and tonic spasms (TS) and then identified factors associated with corpus callosotomy (CC) outcomes. We enrolled 17 patients with ES/TS who underwent CC before 20 years of age. Post-CC Engel’s classification was as follows: I in 7 patients, II in 2, III in 4, and IV in 4. Welch’s t-test was used to analyse the correlation between ictal HVSs and CC outcomes based on the following three symmetrical indices: (1) negative peak delay: interhemispheric delay between negative peaks; (2) amplitude ratio: interhemispheric ratio of amplitude values for the highest positive peaks; and (3) duration ratio: interhemispheric ratio of slow wave duration. Ages at CC ranged from 17–237 months. Four to 15 ictal EEGs were analysed for each patient. The negative peak delay, amplitude ratio and duration ratio ranged from 0–530 ms, 1.00–7.40 and 1.00–2.74, respectively. The negative peak delay, amplitude ratio and duration ratio were significantly higher in the seizure residual group (p = 0.017, <0.001, <0.001, respectively). Symmetry of ictal HVSs may predict favourable outcomes following CC for ES/TS.

A distinctive seizure type in patients with CDKL5 mutations: Hypermotor-tonic-spasms sequence

Neurology ◽  
2011 ◽  
Vol 76 (16) ◽  
pp. 1436-1438 ◽  
Author(s):  
K. M. Klein ◽  
S. C. Yendle ◽  
A. S. Harvey ◽  
J. H. Antony ◽  
G. Wallace ◽  
...  
Keyword(s):  
Seizure Type ◽  
Tonic Spasms

Insufficient efficacy of vagus nerve stimulation for epileptic spasms and tonic spasms in children with refractory epilepsy

2018 ◽  
Vol 140 ◽  
pp. 66-71 ◽  
Author(s):  
Tohru Okanishi ◽  
Ayataka Fujimoto ◽  
Mitsuyo Nishimura ◽  
Sotaro Kanai ◽  
Hirotaka Motoi ◽  
...  
Keyword(s):  
Vagus Nerve ◽  
Nerve Stimulation ◽  
Vagus Nerve Stimulation ◽  
Refractory Epilepsy ◽  
Epileptic Spasms ◽  
Tonic Spasms

Analysis for the Association Between Corpus Callosum Thickness and Corpus Callosotomy Outcomes for Patients With Epileptic Spasms or Tonic Spasms

2019 ◽  
Vol 95 ◽  
pp. 79-83 ◽  
Author(s):  
Shinji Itamura ◽  
Tohru Okanishi ◽  
Mitsuyo Nishimura ◽  
Sotaro Kanai ◽  
Shimpei Baba ◽  
...  
Keyword(s):  
Corpus Callosum ◽  
Corpus Callosotomy ◽  
Epileptic Spasms ◽  
Tonic Spasms

The Evolution of Subclinical Seizures in Children With Tuberous Sclerosis Complex

2019 ◽  
Vol 34 (12) ◽  
pp. 770-777 ◽  
Author(s):  
Anne-Elise C. de Groen ◽  
Jeffrey Bolton ◽  
Ann Marie Bergin ◽  
Mustafa Sahin ◽  
Jurriaan M. Peters
Keyword(s):  
Status Epilepticus ◽  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Clinical Symptoms ◽  
Seizure Type ◽  
Epileptic Spasms ◽  
Clinical Seizure ◽  
Eeg Data ◽  
Electrographic Seizures ◽  
Region Match

Background:Subclinical seizures are electrographic seizures that present without subjective or objective clinical symptoms. In tuberous sclerosis complex, it is not known whether subclinical seizures occur alone, forewarn, or coexist with clinical seizures. To address this knowledge gap, we studied the prevalence and evolution of subclinical seizures in tuberous sclerosis complex.Methods:We retrospectively reviewed electroencephalography (EEG) data from our tuberous sclerosis complex clinic with subclinical seizures and clinical seizures in a blinded fashion. Based on EEG location and ictal pattern, subclinical seizures were classified as having a clinical counterpart from the same epileptogenic region (match) or not (no match).Results:Of 208 children with tuberous sclerosis complex, 138 had epilepsy and available EEG data. Subclinical seizures were detected in 26 of 138 (19%) children. Twenty-four children had both subclinical seizures and clinical seizures captured on EEG. In 13 of 24, subclinical seizures were detected as a novel, not previously recorded seizure type. In these children, subclinical seizures preceded matching clinical seizures in 4 (31%) within a median time of 4.5 months (range 2-14), whereas 9 (69%) never had any matching clinical seizure. In 11 of 24 children, subclinical seizures were not novel and could be matched to a previously recorded clinical seizure. Matching seizure types were focal (n = 10, 67%), tonic (n = 2), epileptic spasms (n = 2), and status epilepticus (n = 1).Conclusions:Subclinical seizures occur in one-fifth of children with tuberous sclerosis complex and epilepsy, and match with clinical seizures in a small majority. In a third of patients presenting with a novel subclinical seizure, matching clinical seizures follow.

scholarly journals Risk pathways to autism in a cohort of children and adolescents with Tuberous Sclerosis Complex

2021 ◽  
Author(s):  
Fiona McEwen ◽  
Charlotte Tye ◽  
Holan Liang ◽  
Emma Woodhouse ◽  
Lisa Underwood ◽  
...  
Keyword(s):  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Structural Equation Modelling ◽  
Structural Equation ◽  
Single Gene ◽  
Autistic Traits ◽  
Autism Spectrum ◽  
Seizure Type ◽  
Epileptic Spasms ◽  
Equation Modelling

Tuberous Sclerosis Complex (TSC) is a single gene disorder – caused by mutation in the TSC1 or TSC2 gene – that carries a high risk of autism spectrum disorder (ASD). Various neurological complications increase the risk of ASD but the way risk factors operate together is unclear. We aimed to explore risk pathways to ASD by modelling the interplay between genetic mutation (TSC1/TSC2), cortical tuber count, seizure type and severity. The Tuberous Sclerosis 2000 Study is a UK population-based, prospective study of the natural history of TSC. We recruited newly diagnosed children (N=125, 49.6% male, median age=2.7 years [range 4 weeks – 18 years]) and collected data on mutation, cortical tuber count (cranial MRI/CT), seizure history, and IQ. ASD and IQ assessments were carried out at 10-year follow up (N=86, 45.0% male, median age=12.5 years [range 7.8 – 26.9 years]). Assessment for ASD included the Autism Diagnostic Interview–Revised (ADI-R) and the Autism Diagnostic Observation Schedule (ADOS-2). Structural equation modelling using full information maximum likelihood estimation was used to explore pathways that mediate between mutation and ASD. Risk of ASD was high: 39.5% met research criteria for ASD and a further 41.9% showed autistic traits. Structural equation modelling resulted in two indirect pathways, with cortical tuber count and occurrence/severity of epileptic spasms in infancy mediating between mutation and ASD (mutation→tubers→spasms→ASD, B=2.08, 95% CI 0.15–8.02; mutation→spasms→ASD, B=2.98, 95% CI 0.04–8.89). Concurrent seizures (B=3.08, 95% CI 0.42–6.18) and IQ (B=-117.10, 95% CI -183.57–-59.16) were also associated with ASD symptoms. There was significantly elevated risk of ASD and subclinical autistic traits. Tuber count and severity of epileptic spasms predicted ASD severity, suggesting that seizures in infancy may push genetically vulnerable individuals over the threshold for an ASD diagnosis. Prevention/control of seizures in infancy may decrease severity of ASD symptoms. However, ASD was occasionally reported in the absence of overt seizures in infancy, so their causal role requires further investigation.

scholarly journals Infantile Spasms: Opportunities to Improve Care

2020 ◽  
Vol 40 (02) ◽  
pp. 236-245 ◽  
Author(s):  
Ricka Messer ◽  
Kelly G. Knupp
Keyword(s):  
Alternative Therapy ◽  
Infantile Spasm ◽  
Primary Care Providers ◽  
Epileptic Encephalopathy ◽  
Seizure Type ◽  
Epilepsy Syndrome ◽  
Care Providers ◽  
Epileptic Spasms ◽  
Challenges And Opportunities ◽  
Oral Corticosteroids

AbstractInfantile spasm (IS) is a distinct epilepsy syndrome characterized by epileptic spasms (the clinical seizure type) and hypsarrhythmia (the electrographic abnormality). IS is frequently accompanied by impaired neurodevelopment and is often associated with structural, genetic, or metabolic etiologies. Prompt treatment of this severe epileptic encephalopathy improves long-term outcomes but remains elusive in many situations. Despite common misconceptions, even patients with identified etiologies or preexisting developmental delay benefit from proven standard therapies, including adrenocorticotropic hormone (ACTH), oral corticosteroids, or vigabatrin. Treatment efficacy should be assessed with electroencephalography at 2 weeks, and an alternative therapy is indicated if epileptic spasms or hypsarrhythmia have not resolved. Collaboration with primary care providers is critical to mitigate the potentially serious adverse effects of standard treatments and also to provide developmental interventions. Although new approaches are on the horizon, addressing current challenges and opportunities now can dramatically improve patient outcomes.

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