• 대한유전성대사질환학회지
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• 제4권1호
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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.

• 대한임상검사과학회지
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• 제39권1호
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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.

• 대한장애인치과학회지
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• 제13권1호
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• pp.28-32
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• 2017

진정 약제에 저산소증 병력을 보였던 레녹스-가스토 증후군의 7세 4개월 여아를 전신마취 하에서 안정적으로 치과치료를 진행하였다. 레녹스-가스토 환자의 철저한 술전 평가와 의과와의 협진을 통해 전신마취 하 치과 치료가 매우 효과적으로 진행될 수 있다고 생각된다.

• Annals of Clinical Neurophysiology
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• 제20권1호
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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.

• 대한장애인치과학회지
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• 제9권1호
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• pp.51-55
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• 2013

레녹스-가스토 증후군은 소아 난치성 간질 중의 하나로서, 여러 형태의 간질 발작과 정신지체, 뇌파상 각성시 1.5-2 Hz의 극서파복합이 나타는 것을 특징으로 한다. 간질발작은 대개 1-8세경에 나타나며, 대부분의 환아들은 인지기능의 심각한 장애와 정신지체를 일으킨다. 발작과 정신지체를 동반하여 치과치료 시 양호한 협조도를 얻을 수 없고, 본 증례와 같이 다발성 우식증이 있는 경우, 안정적인 생징후를 유지, 감독하며 내원 횟수를 줄일 수 있는 전신마취 하 치과치료가 유리할 것이다. 본 증례에서는 레녹스-가스토 증후군을 가진 14세 3개월 여아를 전신마취 하에 안정적이고 효과적으로 치과 치료할 수 있었다. 레녹스-가스토 환자의 전신 상태에 대한 충분한 술전 평가가 선행되고, 간질발작 증상에 대한 적절한 조절이 선행된다면, 전신마취 하에 치과 치료를 매우 효과적으로 진행할 수 있을 것을 사료된다.

• 대한장애인치과학회지
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• 제14권1호
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• pp.22-25
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• 2018

레녹스-가스토 증후군은 다양한 종류의 발작이 복합적으로 나타나는 질환으로 치료가 어렵고, 예후가 좋지 않다. 복용 중인 약물에 의해 구강 내 치은 부종 등의 증상이 나타날 수 있고, 치태 축적에 의한 우식의 위험성이 높기 때문에 구강 위생 관리가 중요하다. 치과치료를 필요로 하는 경우, 발작의 정도, 복용 중인 약물 및 환아의 협조도를 고려해 치료계획을 세워야 한다. 발작의 정도가 심하고, 협조도가 좋지 않은 경우, 전신마취를 계획할 수 있으며, 이 때 의과적 자문을 반드시 고려해야 한다.

• 대한장애인치과학회지
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• 제14권2호
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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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• 제9권2호
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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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• 제18권4호
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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.

• 대한임상검사과학회지
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• 제38권2호
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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.