Single-Epileptic Spasms with or without Hypsarrhythmia: A Study of 16 Patients

Objective We evaluated the electroclinical features, etiology, treatment, and outcome of 16 patients with single-epileptic spasms (ESs) with or without hypsarrhythmia (WoH). Methods Nine boys and seven girls had single-ESs. ESs were considered as single epileptic spasm variants when no other spasm occurred for 1 minute before and after each spasm. Age at the onset of ESs was between 2 and 84 months, with a mean age of 11 months. Results We recognized a group of 15 patients with single-ESs as the main type of seizure; 6 patients with WoH and 9 patients with hypsarrhythmia, respectively. Nine of these 15 patients had other types of seizures before the onset of single-ESs, and 12 patients had other types of seizures during the period in which the ESs occurred. Nine of 15 patients had a structural and seven had an unknown etiology. In 10 cases, the ESs were refractory to antiepileptic drugs, while 4 patients responded well to adrenocorticotropic hormone (ACTH), 1 to pyridoxine, and 2 to the ketogenic diet (KD). The remaining patient (patient.16) had single-ESs and electroclinical features of Lennox–Gastaut syndrome (LGS). Conclusion In this article, we present a series of infants who had daily single-ESs with or WoH. Those with single-ESs with hypsarrhythmia evolved to an epileptic encephalopathy. Video-electroencephalogram (EEG) and polygraphic-EEG recordings are crucial to identify the single-ESs.

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Use of Perampanel and a Ketogenic Diet in Nonketotic Hyperglycinemia: A Case Report

Neuropediatrics ◽

10.1055/s-0040-1708536 ◽

2020 ◽

Vol 51 (06) ◽

pp. 417-420

Author(s):

Atsuro Daida ◽

Shin-ichiro Hamano ◽

Satoru Ikemoto ◽

Yuko Hirata ◽

Ryuki Matsuura ◽

...

Keyword(s):

Ketogenic Diet ◽

Adrenocorticotropic Hormone ◽

Seizure Control ◽

Epileptic Encephalopathy ◽

Severe Form ◽

Focal Seizures ◽

Aspartate Receptor ◽

Epileptic Spasms ◽

Glycine Cleavage ◽

Nonketotic Hyperglycinemia

Abstract Background Nonketotic hyperglycinemia is a severe form of early onset epileptic encephalopathy caused by disturbances in the glycine cleavage system; the neurological damage is mainly attributed to overstimulation of the N-methyl-D-aspartate receptor. Case The patient presented with a severe form of nonketotic hyperglycinemia and experienced frequent epileptic spasms and focal seizures, which were resistant to vigabatrin, adrenocorticotropic hormone therapy, and combined dextromethorphan and sodium benzoate treatments. By 9 months of age, perampanel reduced epileptic spasms by >50%. At 14 months of age, the ketogenic diet markedly reduced focal seizures and glycine levels in the cerebrospinal fluid. Conclusion Perampanel reduced fast excitatory neuronal activity, which was induced by an α-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid receptor, followed by prolonged electrical depolarizations due to an N-methyl-D-aspartate receptor. Furthermore, the ketogenic diet may have modulated the excessive neurotoxic cascade through the N-methyl-D-aspartate receptor. Perampanel and ketogenic diet were effective for seizure control in our patient.

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The Direct Costs of Dravet's Syndrome before and after Diagnosis Assessment

Neuropediatrics ◽

10.1055/s-0040-1718518 ◽

2020 ◽

Author(s):

Katarína Česká ◽

Lukáš Český ◽

Hana Ošlejšková ◽

Štefánia Aulická

Keyword(s):

Health Care ◽

Financial Burden ◽

Correct Diagnosis ◽

Diagnostic Testing ◽

Epileptic Encephalopathy ◽

Care Utilization ◽

Unknown Etiology ◽

Early Assessment ◽

Before And After ◽

Care Costs

AbstractThe objective of this study was to estimate the direct cost before and after diagnosis assessment in patients with Dravet's syndrome (DS). The basis of the economic study was to calculate the costs of health care before and after diagnosis of DS. We retrospectively evaluated all SCN1A positive patients with phenotype of DS treated in our hospital. Statistical analyses were performed by IBM SPSS Statistics 24.0 software. After the diagnosis of DS, there was a significant decline of health care costs (−85.6%) an average of €29.4 ± 26.1 monthly per patient. We estimated the monthly costs at €204.5 ± 167 (median: €193.9, range: €35.5–534.4) per patient before DS diagnosis. The major cost was for hospitalization in neurological department: €43.3 ± 52 (median: €21.9, range: €9.5–179.4) per patient. Minimal cost per patient per months before DS diagnosis was cost of psychological testing/care and complementary rehabilitation (0.13 and 0.6% of total cost). After DS diagnosis, the major cost was focused on nonhospitalization care of patients (64.8%), minimal (€0) for genetic testing and major for outpatient care (18%, mean: €5.3, median: €7). DS results in essential health care utilization and high financial burden before diagnosis elucidation caused by repeated hospitalization and extensive diagnostics tests of “epileptic encephalopathy of unknown etiology.” The results of this study point out that early assessment of the diagnosis leads to significant decrease of the financial costs because of adequate therapeutic management and exclusion of redundant diagnostic testing after elucidation of correct diagnosis.

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Reiterating the role of corpus callosum in generalization of interictal and ictal epileptiform discharges: a case report with post-callosotomy intracranial electroencephalography in Lennox–Gastaut syndrome

Epilepsia and paroxyzmal conditions ◽

10.17749/2077-8333/epi.par.con.2021.086 ◽

2021 ◽

Vol 13 (3) ◽

pp. 249-253

Author(s):

S. Gopinath ◽

A. Pillai ◽

A. G. Diwan ◽

J. V. Pattisapu ◽

K. Radhakrishnan

Keyword(s):

Corpus Callosum ◽

Epileptic Encephalopathy ◽

Drop Attacks ◽

Epileptiform Discharges ◽

Interictal Epileptiform Discharges ◽

Eeg Abnormalities ◽

Before And After ◽

Intracranial Electroencephalography ◽

Electroencephalogram Eeg

Lennox–Gastaut syndrome (LGS) is an epileptic encephalopathy characterized by delayed mental development and intractable multiple seizure types, predominantly tonic. Drop attacks are the commonest and the most disabling type of seizures. Resective surgery is often not possible in LGS as the electroencephalogram (EEG) abnormalities are usually multifocal and generalized, and magnetic resonance image is often either normal or multilesional. We report a case of LGS with bilateral parieto-occipital gliosis where EEG before and after callosotomy demonstrated synchronized bilateral interictal epileptiform discharges and ictal discharges becoming desynchronized and running down. This phenomenon emphasizes the role of the corpus callosum in secondary bilateral synchrony.

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Decreased abundance of Akkermansia after adrenocorticotropic hormone therapy in patients with West syndrome

BMC Microbiology ◽

10.1186/s12866-021-02189-z ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Lu Xu ◽

Dandan Chen ◽

Congying Zhao ◽

Lihua Jiang ◽

Shanshan Mao ◽

...

Keyword(s):

Relative Abundance ◽

Adrenocorticotropic Hormone ◽

Illumina Miseq ◽

Epileptic Encephalopathy ◽

Shannon Index ◽

West Syndrome ◽

Healthy Infants ◽

Blood Cytokines ◽

Before And After ◽

Acth Therapy

Abstract Background Infants suffer from a severe epileptic encephalopathy known as West syndrome (WS). Treatment with adrenocorticotropic hormone (ACTH) indicates the involvement of the gut-brain axis in WS. Several pieces of evidence show the communication of the gut microbiota (GM) with the brain via the hypothalamic–pituitary–adrenal axis (HPA axis) and blood cytokines. This study aimed at (1) determining the GM diversity in infants having WS and (2) comparing the results of infants having WS with those of the healthy infants and also in the patients with WS before and after the ACTH therapy. Results In this study, 29 infants with WS and 29 healthy infants aged 3–13 months were recruited. Fecal samples were collected, and DNA was extracted and sequenced on the Illumina MiSeq platform. Kruskal-Wallis rank-sum test was used to analyze the between-group differences in the Chao1 index, Shannon index, and the abundances of GM at different taxonomy levels. R software was used to plot the graphs. The top five dominant GM genera between patients with WS and healthy infants showed no significant differences. However, the relative abundance of genus Akkermansia was observed to be significantly (P = 0.011) higher in the BT group than in the HC group and AT group. After 2 weeks of ACTH therapy, the relative abundance of Akkermansia significantly (P = 0.003) decreased. Conclusion The relative abundance of Akkermansia was observed to be significantly higher in patients with WS than that in healthy infants. However, the relationship between Akkermansia and WS pathogenesis needs to be clarified in further studies.

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P.107 Response to the Ketogenic Diet in refractory epileptic spasms at BC Children’s Hospital

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

10.1017/cjn.2021.384 ◽

2021 ◽

Vol 48 (s3) ◽

pp. S49-S50

Author(s):

PM Webb ◽

A Datta

Keyword(s):

Ketogenic Diet ◽

Neurodevelopmental Outcome ◽

Retrospective Chart Review ◽

Optimal Time ◽

Unknown Etiology ◽

Seizure Type ◽

Early Therapy ◽

Children's Hospital ◽

Epileptic Spasms ◽

Children’S Hospital

Background: Epileptic spasms (ES) are a devastating seizure type with poor neurodevelopmental outcome; 1/3 are resistant to treatment with first line therapies. Recently attention has been drawn to the ketogenic diet (KD) as a potentially effective therapy, though data regarding optimal time of initiation, and its sustained effectiveness, are lacking. Methods: Retrospective chart review of all patients with ES treated with KD at BC Children’s Hospital between 2002 and 2020 (n=28) with comparison of spasm response based on age of initiation of KD in two groups: < 12 months (n=11) and ≥ 12 months (n=17). Results: Comparing the <12 months and ≥ 12 months groups showed: unknown etiology in 9% vs 25%; spasm freedom for 3 months on KD in 18% vs 41%; median time to spasm freedom was 2 vs 6 weeks; relapse after a period of spasm freedom occurred in 66% vs 70%. Conclusions: Although more effective in children ≥ 12 months of age in the first 3 months, spasm freedom in either group was not sustained with KD. KD is recommended as early therapy for refractory ES, but this study suggests clinicians be aware the KD has limited efficacy in long-term control of ES and must be used with other therapies.

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High Adrenocorticotropic Hormone before and after bilateral adrenal surgery

Endocrine Abstracts ◽

10.1530/endoabs.56.p98 ◽

2018 ◽

Author(s):

Ana Valea ◽

Roxana Turturea ◽

Oana Botezan ◽

Mara Carsote ◽

Bogdan Mircea Botezan ◽

...

Keyword(s):

Adrenocorticotropic Hormone ◽

Adrenal Surgery ◽

Before And After

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Faculty Opinions recommendation of Retrospective evaluation of low long-term efficacy of antiepileptic drugs and ketogenic diet in 39 patients with CDKL5-related epilepsy.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.725803195.793509944 ◽

2015 ◽

Author(s):

Sanjay Sisodiya

Keyword(s):

Antiepileptic Drugs ◽

Ketogenic Diet ◽

Retrospective Evaluation ◽

Term Efficacy

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Possible Role of High-Dose Barbiturates and Early Administration of Parenteral Ketogenic Diet for Reducing Development of Chronic Epilepsy in Febrile Infection-Related Epilepsy Syndrome: A Case Report

Neuropediatrics ◽

10.1055/s-0040-1716903 ◽

2020 ◽

Author(s):

Shimpei Baba ◽

Tohru Okanishi ◽

Koichi Ohsugi ◽

Rika Suzumura ◽

Keiko Niimi ◽

...

Keyword(s):

Continuous Infusion ◽

Ketogenic Diet ◽

Epilepsy Syndrome ◽

High Dose ◽

Early Administration ◽

Chronic Epilepsy ◽

Time Period ◽

Irreversible Brain Damage ◽

Electroencephalogram Eeg

AbstractWe describe the efficacy of high-dose barbiturates and early administration of a parenteral ketogenic diet (KD) as initial treatments for acute status epilepticus (SE) in an 8-year-old girl with febrile infection-related epilepsy syndrome (FIRES). The patient was admitted to our hospital with refractory focal SE. Abundant epileptic discharges over the left frontal region were observed on electroencephalogram (EEG). Treatment with continuous infusion of thiamylal for 4 hours, increased incrementally to 40 mg/kg/h, successfully ended the clinical SE, and induced a burst-suppression coma. The infusion rate was then gradually decreased to 4 mg/kg/h over the next 12 hours. Parenteral KD was administered from days 6 to 21 of illness. Continuous infusion of thiamylal was switched to midazolam on day 10 without causing seizures or EEG exacerbations. The patient has remained seizure free in the 15 months since hospital discharge. The effectiveness of KD for the treatment of FIRES has attracted attention amongst clinicians, but KD treatment may need to last for 2 to 4 days before it can stop SE, a time period that could cause irreversible brain damage. Considering the severity of SE in our patient and the dose of barbiturates needed to treat it, we consider this case to have had a good clinical outcome. The results suggest that rapid termination of seizure using high-dose barbiturates in conjunction with early administration of parenteral KD could reduce the development of chronic epilepsy in patients with FIRES.

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Effects of Two Months of Very Low Carbohydrate Ketogenic Diet on Body Composition, Muscle Strength, Muscle Area, and Blood Parameters in Competitive Natural Body Builders

Nutrients ◽

10.3390/nu13020374 ◽

2021 ◽

Vol 13 (2) ◽

pp. 374

Author(s):

Antonio Paoli ◽

Lorenzo Cenci ◽

PierLuigi Pompei ◽

Nese Sahin ◽

Antonino Bianco ◽

...

Keyword(s):

Body Composition ◽

Muscle Strength ◽

Ketogenic Diet ◽

Inflammatory Cytokines ◽

Blood Parameters ◽

Maximal Strength ◽

Muscle Area ◽

Low Carbohydrate ◽

Before And After ◽

Adequate Quantity

Background: Ketogenic diet (KD) is a nutritional approach that restricts daily carbohydrates, replacing most of the reduced energy with fat, while maintaining an adequate quantity of protein. Despite the widespread use of KD in weight loss in athletes, there are still many concerns about its use in sports requiring muscle mass accrual. Thus, the present study sought to investigate the influence of a KD in competitive natural body builders. Methods: Nineteen volunteers (27.4 ± 10.5 years) were randomly assigned to ketogenic diet (KD) or to a western diet (WD). Body composition, muscle strength and basal metabolic rate were measured before and after two months of intervention. Standard blood biochemistry, testosterone, IGF-1, brain-derived neurotrophic factor (BDNF) and inflammatory cytokines (IL6, IL1β, TNFα) were also measured. Results: Body fat significantly decreased in KD (p = 0.030); whilst lean mass increased significantly only in WD (p < 0.001). Maximal strength increased similarly in both groups. KD showed a significant decrease of blood triglycerides (p < 0.001), glucose (p = 0.001), insulin (p < 0.001) and inflammatory cytokines compared to WD whilst BDNF increased in both groups with significant greater changes in KD (p < 0.001). Conclusions: KD may be used during body building preparation for health and leaning purposes but with the caution that hypertrophic muscle response could be blunted.

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