• Journal of The Korean Society of Inherited Metabolic disease
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• v.4 no.1
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• pp.46-53
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• 2004

3-Methylcrotonyl-CoA carboxylase (MCC) is a biotin-dependent enzyme involved in leucine metabolism. We describe a patient with MCC deficiency who manifested with Reye syndrome-like illness with status epilepticus, metabolic acidosis, hypoglycemia, hyperammonemia, elevated liver enzymes and neurologic impairments after the viral gastroenteritis and then, has suffered from Lennox-Gastaut syndrome. Urinary organic acid analysis revealed increased excretion of 3-hydroxyisovaleric acid and 3-methylcrotonylglycine. This patient was managed with leucine restriction diet and supplementation of biotin and carnitine but was not so effective. He has suffered from neurologic sequelae such as Lennox-Gastaut syndrome, motor and cognitive impairement.

• Korean Journal of Clinical Laboratory Science
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• v.39 no.1
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• pp.56-62
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• 2007

Lennox-Gastaut syndrome(LGS) is a severe age-specific epilepsy syndrome that causes medication-resistant seizures in childhood. Vagal nerve stimulation (VNS) has been proposed as a possible way to improve the treatment of refractory epilepsy. We treated 9 patients with Lennox-Gastaut syndrome between the ages of 2 and 11 years (mean 5.8 years), by using the vagus nerve stimulation. The mean follow-up duration was 35 months. The mean reduction of seizure frequency compared with baseline before VNS was 52% after 6 months (range, 0% to 89%; P<0.011), and 58% after 1 year (range 0% to 89%; P<0.012). Seven patients showed improvements of quality of life (QOL) such as alertness, mood, and language skills. The most common side effects were transient hoarseness (6 patients) and drooling (1 patient). Our results suggest that the vagus nerve stimulation could be an effective and safe adjunct therapy for the treatment of Lennox-Gastaut syndrome.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.13 no.1
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• pp.28-32
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• 2017

Lennox-Gastaut syndrome (LGS) is a type of childhood epilepsy, and this syndrome can compromise dental treatment. LGS represents variable etiologies, multiple types of intractable seizures, and cognitive impairment in most patients. A 7-year-4-month old girl with LGS was referred to our hospital for dental treatment under general anesthesia. Clinically, the patient presented with multiple dental caries. The patient had been prescribed a ketogenic diet for the management of repetitive seizures. In addition, she experienced severe types of hypoxia during medical examination using chloral hydrate. Therefore, dental treatment was performed under general anesthesia. LGS patients have a high risk of seizure during dental treatment and can induce a lot of complications. Dental treatment under general anesthesia for LGS patients can be carried out careful preoperative evaluation and in cooperation with medical care.

• Annals of Clinical Neurophysiology
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• v.20 no.1
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• pp.36-40
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• 2018

Lennox-Gastaut syndrome (LGS) is an electro-clinical syndrome composed of the triad of mental retardation, multiple seizure types, and the characteristic generalized slow spike-wave complexes in the electroencephalogram. In this article, we report on two patients with LGS whose brain magnetic resonance imaging showed dysgenesis of corpus callosum (CC). We discuss the role of CC in the genesis of secondary bilateral synchrony.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.9 no.1
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• pp.51-55
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• 2013

Lennox-Gastaut syndrome (LGS) is a severe form of childhood epilepsy that is defined by generalized multiple type seizures, slowness of intellectual growth, and a specific EEG disturbance. Children affected might previously have infantile spasms or underlying brain disorder but etiology can be idiopathic. LGS seizures are often treatment resistant and the long term prognosis is poor. A 14-year-3-month old, 20.5 kg girl with LGS was scheduled for dental treatment under general anesthesia. The patient presented with multiple caries and heavy calculus. General anesthesia was induced and maintained with inhalation agent, Sevoflurane. And caries treatment, scaling, subgingival curretage were performed. LGS patients usually have poor oral hygiene due to mental retardation and have high risk of seizure during dental treatment. In dental treatment under general anesthesia for LGS, it must be considered of seizure control during peri-operative period. Also periodic recall check, instruction of oral hygiene must be performed.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.14 no.1
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• pp.22-25
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• 2018

Lennox-Gastaut syndrome (LGS) is one of the most severe epileptic encephalopathies of childhood onset. It is characterized by multiple types of generalized seizures, slowness of intellectual growth, and specific electroencephalogram pattern. It is one of the most difficult epilepsy syndromes to treat and is frequently resistant to treatment. In this case report, we report a case of dental treatment under general anesthesia in a child with LGS. A 4-year-6-month old boy with LGS visited Seoul National University Dental Hospital for dental treatment. Dental treatment was planned under general anesthesia because of his medical condition. Treatment was successfully done without any postoperative complications. If a LGS patient requires dental treatment, a treatment plan should be established to take into account the degree of seizure, the medication being used, and the cooperation of the patient. If the degree of seizure is severe and the patient's cooperation is poor, dental treatment under general anesthesia would be advantageous.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.14 no.2
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• pp.92-96
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• 2018

Drug-induced gingival overgrowth is an abnormal increase of gingival tissues caused as a side effect of systemic medication. This report presents a severe case of valproate-induced gingival overgrowth combined with ulcerative and hemorrhagic lesions in a patient with Lennox-Gastaut syndrome. Considering the patient's limited cooperative ability, gingivectomy and excisional biopsy under general anesthesia were performed. The lesions were successfully treated without recurrence. When gingival enlargement does not subside with nonsurgical treatments, surgical procedure and excisional biopsy are to be performed. Postoperative management of oral hygiene is critical to prevent recurrence.

• Annals of Clinical Neurophysiology
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• v.9 no.2
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• pp.63-68
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• 2007

The EEG plays an important diagnostic role in epilepsy and provides supporting evidence of a seizure disorder as well as assisting with classification of seizures and epilepsy syndromes. There are a variety of electroclinical syndromes that are really defined by the EEG such as Lennox-Gastaut syndrome, benign rolandic epilepsy, childhood absence epilepsy, juvenile myoclonic epilepsy and also for localization purposes, it is vitally important especially for temporal lobe epilepsy. The sensitivity of first routine EEG in diagnosis of epilepsy has been known about 20-50%, but this proportion rises to 80-90% if sleep EEG and repetitive recording should be added. Convincing evidences suggest that the EEG may also provide useful prognostic information regarding seizure recurrence after a single unprovoked attack and following antiepileptic drug (AED) withdrawal. Moreover, patterns in the EEG make it possible to disclose an ictal feature of nonconvulsive status epilepticus, separate epileptic from other non-epileptic episodes and clarify the clues predictive of the cause of the encephalopathy (i.e., triphasic waves in metabolic encephalopathy). Therefore, regardless of its low sensitivity and other pitfalls, EEG should be considered not only in the situation of new onset episode such as a newly developed, unprovoked seizure or a condition manifesting decreased mentality from obscure origin, but also as a barometer of the long-term outcome following AED withdrawal.

• Journal of Dental Anesthesia and Pain Medicine
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• v.18 no.4
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• pp.235-244
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• 2018

Background: Patients with intellectual disability (ID) often require general anesthesia during oral procedures. Anesthetic depth monitoring in these patients can be difficult due to their already altered mental state prior to anesthesia. In this study, the utility of electroencephalographic indexes to reflect anesthetic depth was evaluated in pediatric patients with ID. Methods: Seventeen patients (mean age, $9.6{\pm}2.9years$) scheduled for dental procedures were enrolled in this study. After anesthesia induction with propofol or sevoflurane, a bilateral sensor was placed on the patient's forehead and the bispectral index (BIS) was recorded. Anesthesia was maintained with sevoflurane, which was adjusted according to the clinical signs by an anesthesiologist blinded to the BIS value. The index performance was accessed by correlation (with the end-tidal sevoflurane [EtSevo] concentration) and prediction probability (with a clinical scale of anesthesia). The asymmetry of the electroencephalogram between the left and right sides was also analyzed. Results: The BIS had good correlation and prediction probabilities (above 0.5) in the majority of patients; however, BIS was not correlated with EtSevo or the clinical scale of anesthesia in patients with Lennox-Gastaut, West syndrome, cerebral palsy, and epilepsy. BIS showed better correlations than SEF95 and TP. No significant differences were observed between the left- and right-side indexes. Conclusion: BIS may be able to reflect sevoflurane anesthetic depth in patients with some types of ID; however, more research is required to better define the neurological conditions and/or degrees of disability that may allow anesthesiologists to use the BIS.

• Korean Journal of Clinical Laboratory Science
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• v.38 no.2
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• pp.141-146
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• 2006

Vagal nerve stimulation (VNS) has been proposed as a possible way to improve the control of refractory epilepsy. We report the effects following VNS treatment in patients with refractory epilepsy. Seventeen patients with a mean age of 12.8 years, ranging from 5 to 29 years, underwent the implantation of vagal nerve stimulation (Cyberonics, Houston, TX). We reviewed the clinical findings before and after VNS in seizure frequency, number of antiepileptic drugs (AED), and quality of life (QOL). All of the patients had intractable seizures, eleven of the patients had additional medical complications, three had hippocampus atrophy, one had encephalomalacia, five had encephalitis, one had pachygyria, and one had schizencephaly. Thirteen patients had symptomatic partial epilepsies, three patients had Lennox-Gastaut syndrome and one had cryptogenic partial epilepsy. The mean follow up duration was 35 months. The mean reduction of seizure frequency compared with baseline before VNS was 26.1% after 3 months (p<0.005), 41.9% after 6 months (p<0.001), 46.9% after 1 year (p<0.001), and 53% at the latest follow-up (p<0.001). Twelve patients showed an improvement of QOL such as mood, language, alertness, expression, and motor function. The most common side effects were transient hoarseness or voice change or cough, which was detected in six patients (35%) and wound infection in one patient (5%). This study has shown a good anti-seizure effect of VNS, decrease in seizure frequency and improvements in QOL. We concluded that VNS is a beneficial therapy in refractory epilepsy with a non-resectable epileptic focus. Further studies should be focused on the prediction of unresponsiveness and the adjustment of VNS parameters for maximum efficacy in patients with various medical histories.