• Journal of The Korean Dental Society of Anesthesiology
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• v.8 no.1
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• pp.35-39
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• 2008

Patients with seizures tend to demonstrate a poor oral condition and gingival hyperplasia due to some antiepileptic drugs. Although most patients attain good control of their seizures with anticonvulsant drugs, seizures can occur during dental treatment for various reasons. Sedation can be recommended for anxiety control, and general anesthesia should be considered for uncooperative patient such as patients with intellectual disability or autism. The purpose of this case report was to describe the dental treatment for a patient with seizure history and intellectual disability under general anesthesia.

• Journal of Genetic Medicine
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• v.18 no.1
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• pp.70-74
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• 2021

Cohen-Gibson syndrome (CGS) was first reported by Cohen et al., who identified the mutation of the gene encoding the embryonic ectoderm development (EED) in a patient with phenotypes similar to Weaver syndrome. CGS manifests as an overgrowth and intellectual disability, in addition to the characteristic facial features and organ anomalies. CGS has been reported in only 11 unrelated patients since 2015. A girl aged 6 years and 3 months presented with seizures. She had macrosomia, a dysmorphic face, and intellectual disability. Her mother and younger sister and brother also had macrosomia, intellectual disability, and similar facial features; additionally, her mother experienced seizures and had an arachnoid cyst, while her siblings had valvar pulmonary stenosis. Whole-exome sequencing for the proband revealed a mutation of EED (c.581A>G, p.Asn194Ser), which was also verified in the mother and both siblings using Sanger sequencing. This is the first report of familial CGS.

• Clinical and Experimental Pediatrics
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• v.50 no.11
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• pp.1097-1103
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• 2007

Purpose : Acute symptomatic seizure is defined as a temporary seizure together with acute systemic, metabolic, or toxic insult in association with an acute central nervous system insult. And unprovoked seizure is defined as seizure without provocating factors. We studied the risk factors of unprovoked seizures after acute symptomatic seizure in children. Methods : We retrospectively reviewed the records of one hundred and ten children with acute symptomatic seizures who were admitted to the pediatric department of Chungbuk National University Hospital between January, 1998 and December, 2003. We analyzed overall risk factors of unprovoked seizures after acute symptomatic seizures involving etiology, incidence, type of seizure, duration and neuroimaging. Results : We analyzed records of 110 children with acute symptomatic seizures aged from 1 month to 17 years. 24 children had unprovoked seizures (21.8%) after acute symptomatic seizures. Causes in order of frequency were encephalopathy, central nervous system infection, brain tumor, cerebrovascular disease. The risk of unprovoked seizure was significantly greater for those with status epilepticus (68.4%) than without status epilepticus, with partial seizure (64.7%) than generalized seizure. And the risk of unprovoked seizure was strongly associated with abnormal finding of electroencephalogram (79.1%) and neuroimaging (41.6%). Conclusion : In conclusion, the leading cause of subsequent unprovoked seizure in children with acute symptomatic seizure was encephalopathy and age specific incidence was high in the group aged 24-72 months. The risk for subsequent unprovoked seizure was greater for those with partial seizure, status epilepticus, abnormal finding of neuroimaging and electroencephalography.

• Clinical and Experimental Pediatrics
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• v.48 no.12
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• pp.1342-1347
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• 2005

Purpose : Among perinatal risk factors, neonatal seizures are one of the strongest independent discriminators of adverse outcome, representing high risks of mortality and neurologic morbidity. This study was undertaken to evaluate the neurologic outcome of neonatal status epilepticus according to underlying etiology, seizure pattern, onset time, and duration. Methods : We reviewed retrospectively 36 neonates(19 males, 17 females) with status epilepticus who were admitted to the neonatal intensive care unit, Inha Hospital between July, 1988 and June, 2003. They were evaluated with neurologic examination, laboratory data, EEG findings, and neuroimaging studies etc. Results : The mean gestational period of the patients was $37.0{\pm}3.6$ weeks and birth weight was $2.70{\pm}0.82$ kilogram. Fifty two point eight percent of the neonates were male and 66.7 percent were born at term. The most common cause of neonatal status epilepticus was hypoxic-ischemic encephalopathy. In preterm babies, intracranial hemorrhages showed an especially high frequency(P=0.034). Gestational age and birth weight did not show a correlation with neurologic complications. The incidence of neurological sequelae were significantly related to prolonged seizures lasting more than 1 hour(P=0.002). Neonates with seizures within the first 72 hours tended to be more frequent among those who developed adverse outcomes(P=0.016). Generalized tonic seizures had the worst prognosis, whereas those children who had subtle seizures had better outcomes than any other type(P<0.05). Generalized tonic seizures were primarily represented on EEG by abnormal background, whereas subtle seizure showed a significantly more normal EEG than any other seizures(P<0.05). Conclusion : Our results indicate that neonatal status epilepticus with early onsets, prolonged durations. And generalized tonic types can predict an increased risk for neurologic sequelae. So, those seizures must be perceived as medical emergencies and treated aggressively with antiepileptic drugs.

• Clinical and Experimental Pediatrics
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• v.55 no.5
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• pp.159-163
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• 2012

Most children with epilepsy are of normal intelligence. However, a significant subset will have temporary or permanent cognitive impairment. Factors that affect cognitive function are myriad and include the underlying neuropathology of the epilepsy, seizures, epileptiform discharges, psychosocial problems, age at seizure onset, duration of epilepsy, and side effects associated with antiepileptic drugs. This review article discusses cognitive function in children with idiopathic epilepsy and the effects of antiepileptic drugs on cognitive function in children.

• Clinical and Experimental Pediatrics
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• v.51 no.4
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• pp.420-425
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• 2008

Purpose : The purpose of this study was to evaluate acute childhood seizures, one of the most important causes of emergency room visits, to provide appropriate medical services. Methods : We reviewed the medical records of 433 (4.6%) pediatric patients with acute seizures that visited the emergency room at Masan Samsung hospital from 2004 to 2006. Results : The male to female ratio was 1.4:1 and the mean age was $40.9{\pm}34.9$ months range. The order of geographical distribution was Masan, Changwon, Haman, and others. Fever was present in 40.6% of patients; December (14.8%) was the most frequent month for visits and generalized tonic clonic seizures (62.7%) were the most common type of seizure. The average frequency and duration of the seizure was $1.5{\pm}1.0$ and $6.7{\pm}13.2$ minutes respectively. Febrile seizures were present in 69.7% of patients and afebrile seizures in 30.3%. The causes of the febrile seizures were acute pharyngotonsillitis (44.6%), acute bronchitis, gastroenteritis, pneumonia, urinary tract infection, and unknown origin, in order of frequency. The most common cause of an afebrile seizure was epilepsy (71.5%) followed by a benign convulsion with mild gastroenteritis (BCwMG), sequela of a perinatal brain injury or brain malformation, and acute CNS infection. Evaluation of the causes of an acute seizure according to age showed that febrile seizures, epilepsy, and the sequela of perinatal brain injuries were more common between 2 and 6 years of age and epilepsy, febrile seizures and acute CNS infection, in order of frequency, were common between 6 and 15 years of age. Many patients, 49.4%, were discharged without admission. Conclusion : The common characteristics of pediatric patients presenting to the emergency room were male gender, an age between 2-6 years, presenting during the month of December, with generalized tonic clonic seizures due to acute pharyngitis. The most common presentation for the group less than 6 years of age was a febrile seizure and in the group more than 6 years of age, it was epilepsy. In many cases, the seizures stopped by the time the family presented to the emergency room.

• Annals of Clinical Neurophysiology
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• v.2 no.2
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• pp.119-124
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• 2000

Background : We studied EEG changes during pilocarpine-induced status epilepticus(SE), a widely used model whose EEG characteristics have not been fully described previously. Methods : Male Sprague-Dawley rats weighing 250-350 grams were used as subjects. SE was induced 5-7 days after placement of chronic epidural electrodes, using 360-380 mg/Kg pilocarpine IP. Rats were observed with continuous EEG recording following pilocarpine injection until end of the SE episode. Results : SE occurred in 10/12 rats studied. SE began with a series of discrete seizures $11.1{\pm}3.93$ minutes after pilocarpine injection. $5.2{\pm}2.71$ seizures occurred over $10.9{\pm}4.62$ minutes, until the EEG converted to a waxing and waning pattern, during which the amplitude and frequency of epileptiform activity increased. After $1.4{\pm}1.82$ minutes, a pattern of continuous high amplitude rapid spiking was established. Continuous spiking continued for $3.4{\pm}0.48$ hours with a very gradual decline in amplitude and frequency, until periodic epileptiform discharges(PEDs) began to occur. The EEG consisted primarily of PEDs for another $7.4{\pm}3.09$ hours, until electrographic generalized seizures began to occur. These continued for $5.8{\pm}4.82$ hours until death. Duration of SE was $17.0{\pm}5.88$ hours. Flat periods were a prominent feature during all EEG patterns in this model. Conclusion : EEG features distinctive in pilocarpine SE(but not unique to it) include flat periods during all patterns and resumption of continuous spiking episodes after the onset of PEDs. The sequence of discrete seizures to waxing and waning to continuous spiking to PEDs was identical to that which has been described in humans and other animal models.

• BMB Reports
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• v.53 no.9
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• pp.484-489
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• 2020

Epilepsy is a neurological disorder characterized by unpredictable seizures, which are bursts of electrical activity that temporarily affect the brain. Cereblon (CRBN), a DCAFs (DDB1 and CUL4-associated factors), is a well-established protein associated with human mental retardation. Being a substrate receptor of the cullin-RING E3 ubiquitin ligase (CRL) 4 complex, CRBN mediates ubiquitination of several substrates and conducts multiple biological processes. In the central nervous system, the large-conductance Ca²⁺-activated K⁺ (BK_Ca) channel, which is the substrate of CRBN, is an important regulator of epilepsy. Despite the functional role and importance of CRBN in the brain, direct injection of pentylenetetrazole (PTZ) to induce seizures in CRBN knock-out mice has not been challenged. In this study, we investigated the effect of PTZ in CRBN knock-out mice. Here, we demonstrate that, compared with WT mice, CRBN knock-out mice do not show the intensification of seizures by PTZ induction. Moreover, electroencephalography recordings were also performed in the brains of both WT and CRBN knockout mice to identify the absence of significant differences in the pattern of seizure activities. Consistently, immunoblot analysis for validating the protein level of the CRL4 complex containing CRBN (CRL4^Crbn) in the mouse brain was carried out. Taken together, we found that the deficiency of CRBN does not affect PTZ-induced seizure.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.11 no.1
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• pp.144-149
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• 2000

A psychogenic seizure is a disorder which is occasionally met by neurologists and psychiatrists. The most important thing that clinicians should do is differential diagnosis with true epileptic seizures. Especially psychiatrists are expected to be able to diagnose psychogenic seizures through a thorough history taking and an observation of clinical features to some degree before the confirmatory diagnoses. We experienced a case of 11-year-old girl with psychogenic seizure which was characterized by obvious psychological and environmental stress, clinical features, and good outcome by prompt a loboratoy examination and psychiatric intervention. To diagnose and treat childhood psychogenic seizures, we suggest that it is very important to examine the familial background and take a thorough history, and perform psychoeducational intervention in addition to electroencephalogram.

• Clinical and Experimental Pediatrics
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• v.60 no.2
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• pp.50-54
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• 2017

Purpose: The aims of this study were to evaluate the safety and pharmacokinetics of levetiracetam (LEV) in neonates with seizures and to establish a population pharmacokinetics (PPK) model by using the software NONMEM. Methods: A retrospective analysis of 18 neonatal patients with seizures, who were treated with LEV, including 151 serum samples, was performed. The mean loading dose was 20 mg/kg, followed by a mean maintenance dose of 29 mg/kg/day. Results: Seventeen neonates (94%) had seizure cessation within 1 week and 16 (84%) remained seizure-free at 30 days under the LEV therapy. The mean serum concentration of LEV was $8.7{\mu}g/mL$. Eight samples (5%) were found above the therapeutic range. No serious adverse effects were detected. In the PPK analysis for Korean neonates, the half-life was 9.6 hours; clearance, 0.357 L/hr; and volume of distribution, 4.947 L, showing differences from those in adults. Conclusion: LEV is a safe and effective option for the treatment of neonatal seizures with careful therapeutic drug monitoring.