• Clinical and Experimental Pediatrics
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• 제56권12호
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• pp.540-544
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• 2013

Down syndrome, the most common chromosomal abnormality, may be associated with various neurologic complications such as moyamoya syndrome, cervical spinal cord compression due to atlantoaxial subluxation, and basal ganglia damage, as well as epileptic seizures and stroke. Many cases of Down syndrome accompanied by isolated neurologic manifestations have been reported in children; however, Down syndrome with multiple neurologic conditions is rare. Here, we have reported a case of Down syndrome in a 10-year-old girl who presented with asymptomatic moyamoya syndrome, atlantoaxial subluxation with spinal cord compression, and basal ganglia calcification. To the best of our knowledge, this is the first report of Down syndrome, in a child, which was accompanied by these 3 neurologic complications simultaneously. As seen in this case, patients with Down syndrome may have neurologic conditions without any obvious neurologic symptoms; hence, patients with Down syndrome should be carefully examined for the presence of neurologic conditions.

• Journal of Korean Neurosurgical Society
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• 제62권3호
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• pp.344-352
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• 2019

Epilepsy is one of the major chronic neurological diseases affecting many patients. Resection surgery is the most effective therapy for medically intractable epilepsy, but it is not feasible in all patients. Vagus nerve stimulation (VNS) is an adjunctive neuromodulation therapy that was approved in 1997 for the alleviation of seizures; however, efforts to control epilepsy by stimulating the vagus nerve have been studied for over 100 years. Although its exact mechanism is still under investigation, VNS is thought to affect various brain areas. Hence, VNS has a wide indication for various intractable epileptic syndromes and epilepsy-related comorbidities. Moreover, recent studies have shown anti-inflammatory effects of VNS, and the indication is expanding beyond epilepsy to rheumatoid arthritis, chronic headaches, and depression. VNS yields a more than 50% reduction in seizures in approximately 60% of recipients, with an increase in reduction rates as the follow-up duration increases. The complication rate of VNS is 3-6%, and infection is the most important complication to consider. However, revision surgery was reported to be feasible and safe with appropriate measures. Recently, noninvasive VNS (nVNS) has been introduced, which can be performed transcutaneously without implantation surgery. Although more clinical trials are being conducted, nVNS can reduce the risk of infection and subsequent device failure. In conclusion, VNS has been demonstrated to be beneficial and effective in the treatment of epilepsy and various diseases, and more development is expected in the future.

• Annals of Clinical Neurophysiology
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• 제2권1호
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• pp.27-30
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• 2000

Reflex epilepsies are distinct but not clearly understood clinical entity. Various cerebral activities induced by simple stimulation including visual, auditory, somatosensory stimulation, as well as diverse functional tasks such as reading, calculation, complex thinking are believed to be seizure-inducing factors. We experienced two patients whose seizures were readily precipitated by complex, strenuous thinking. Both patients was teen-aged boy at the onset of seizure(13, and 15 years of age each) with normal physical and mental growth. Although first seizure was precipitated by watching TV and playing puzzles in each patient, initial diagnosis was idiopathic generalized epilepsy, possibly juvenile myoclonic epilepsy( JME). For the first few years, seizures were infrequent but mostly precipitated by the tasks needs concentration such as playing computer games, decision-making, mathematics, reading, or during the examination. EEG revealed various thinking process including reading hard books, drawing complex figure, complex calculation induced epileptic discharges even if it usually needs certain period of concentration. Phenytoin, valproic acid, clonazepam, vigabatrin, and lamotrigine sometimes abated their seizures but none of these made them seizure-free. Complex reflex epilepsy induced by thinking was proposed to be a separate type of epilepsy or a variant of JME. Age, sex, stereotypic seizure-inducing factors, clinical course, and refractory epilepsies in these patients highly suggested this type of epilepsy as a variant of JME but its refractoriness and unique provocation still needs more speculation.

• 한국임상약학회지
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• 제33권3호
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• pp.195-201
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• 2023

Background: Hematocrit is usually measured from venous blood collected by invasive venipuncture. This study was performed to determine hematocrit accurately and precisely using minimally invasive volumetric absorptive microsampling (VAMS) technique. Such technique is to be applied to determining hematocrit in various clinical settings for the care, including therapeutic drug monitoring, of neonatal or epileptic patients, or patients with high risk of infection or bleeding. Methods: The study was performed using 31 VAMS samples obtained from 21 pancreatic cancer patients. Hematocrit was determined using the values of potassium concentrations obtained from blood in VAMS tips (Hct_VAMS). Hct_VAMS was compared with hematocrit measured from blood collected by venipuncture (Hct_VP). The accuracy and precision of Hct_VAMS in comparison to Hct_VP were evaluated using Bland-Altman plot, Deming regression and mountain plot. Results: Bland-Altman plot displayed a random scattering pattern of the differences between Hct_VAMS and Hct_VP with the mean bias of -0.010 and the 95% limit of agreement ranging from -0.063 to 0.044. Deming regression for Hct_VAMS and Hct_VP line demonstrated very small proportional and constant biases of 1.04 and -0.003, respectively. Mountain plot exhibited a narrow and symmetrical distribution of the differences with their median of -0.011 and central 95% range from -0.049 to 0.033. Conclusion: Hematocrit was accurately and precisely determined using less invasive VAMS technique. Such technique appears to be applicable to determining hematocrit in situations that venipuncture is not favorable or possible.

• Clinical and Experimental Pediatrics
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• 제59권2호
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• pp.74-79
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• 2016

Purpose: Febrile seizure, the most common type of pediatric convulsive disorder, is a benign seizure syndrome distinct from epilepsy. However, as epilepsy is also common during childhood, we aimed to identify the prognostic factors that can predict epilepsy in children with febrile seizures. Methods: The study comprised 249 children at the Korea University Ansan Hospital who presented with febrile seizures. The relationship between the subsequent occurrence of epilepsy and clinical factors including seizure and fever-related variables were analyzed by multivariate analysis. Results: Twenty-five patients (10.0%) had additional afebrile seizures later and were diagnosed with epilepsy. The subsequent occurrence of epilepsy in patients with a history of febrile seizures was associated with a seizure frequency of more than 10 times during the first 2 years after seizure onset (P<0.001). Factors that were associated with subsequent occurrence of epilepsy were developmental delay (P<0.001), preterm birth (P =0.001), multiple seizures during a febrile seizure attack (P =0.005), and epileptiform discharges on electroencephalography (EEG) (P =0.008). Other factors such as the age at onset of first seizure, seizure duration, and family history of epilepsy were not associated with subsequent occurrence of epilepsy in this study. Conclusion: Febrile seizures are common and mostly benign. However, careful observation is needed, particularly for prediction of subsequent epileptic episodes in patients with frequent febrile seizures with known risk factors, such as developmental delay, history of preterm birth, several attacks during a febrile episode, and epileptiform discharges on EEG.

• Journal of Korean Neurosurgical Society
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• 제37권2호
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• pp.124-128
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• 2005

Objective: Congenital bilateral perisylvian syndrome(CBPS) has been defined as a characteristic malformative perisylvian polymicrogyria(PMG) in patients with clinical symptoms of pseudobulbar palsy and epileptic seizures. For the present study, we investigate clinicopathologic features of CBPS associated with timing of lesion formation. Methods: Clinicopathologic features of CBPS from 6 patients with surgical resection of the cerebral lesions due to medically intractable seizures were studied. Results: Seizure onset ranged from 1 to 10years (average 6.7years) of age, and average duration of seizure was 23years. All had complex partial seizures, and two patients had additional tonic clonic seizures. Magnetic resonance (MR) images showed polymicrogyria, atropic gyri with gliosis. In the histopathologic examination, the cortical lesions revealed features of ulegyria; atrophic and sclerotic gyri, laminar loss of neurons, extensive lobular gliosis throughout the gray and white matter, neuronoglial nodule formation, and many amyloid bodies. Unlayered or four-layered PMG was not identified. Conclusion: Above data suggest that CBPS might be caused by ulegyria resulting from developmental cortical defect during early fetal stage or acquired hypoxic/ischemic injury in prenatal or postnatal life.

• The Korean Journal of Physiology and Pharmacology
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• 제15권2호
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• pp.67-81
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• 2011

Epilepsy is a chronic disease occurring in approximately 1.0% of the world's population. About 30% of the epileptic patients treated with availably antiepileptic drugs (AEDs) continue to have seizures and are considered therapy-resistant or refractory patients. The ultimate goal for the use of AEDs is complete cessation of seizures without side effects. Because of a narrow therapeutic index of AEDs, a complete understanding of its clinical pharmacokinetics is essential for understanding of the pharmacodynamics of these drugs. These drug concentrations in biological fluids serve as surrogate markers and can be used to guide or target drug dosing. Because early studies demonstrated clinical and/or electroencephalographic correlations with serum concentrations of several AEDs, It has been almost 50 years since clinicians started using plasma concentrations of AEDs to optimize pharmacotherapy in patients with epilepsy. Therefore, validated analytical method for concentrations of AEDs in biological fluids is a necessity in order to explore pharmacokinetics, bioequivalence and TDM in various clinical situations. There are hundreds of published articles on the analysis of specific AEDs by a wide variety of analytical methods in biological samples have appears over the past decade. This review intends to provide an updated, concise overview on the modern method development for monitoring AEDs for pharmacokinetic studies, bioequivalence and therapeutic drug monitoring.

• 대한간호학회지
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• 제47권5호
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• pp.624-637
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• 2017

Purpose: This study aimed to identify variables influencing the health-related quality of life (HRQoL) of adults with epilepsy in order to establish a structural model and design an intervention strategy to improve patients' HRQoL. Methods: The selected subjects were 212 patients with epilepsy aged between 18 and 70 years who were currently receiving treatment from hospital, general hospital, and clinic. They were surveyed using a structured questionnaire. Results: The goodness of fit measures of the final hypothetical model were as follows: ${\chi}^2/df=2.51$, GFI=.91, AGFI=.90, CFI=.96, SRMR=.04, NFI=.93, and RMSEA=.08. The major variables influencing the HRQoL of adults with epilepsy were epilepsy self-efficacy, depression, social support, and side effects of anti-epileptic drugs (AEDs), which were significant in the mentioned order, whereas the duration of AEDs use and perceived stigma did not show any effects. Six variables accounted for 75.6% of HRQoL. Variables having a direct and total effect on the HRQoL of adults with epilepsy were the side effects of AEDs, social support, epilepsy self-efficacy, and depression, and those with an indirect effect were the side effects of AEDs and social support. Conclusion: It is necessary to accurately identify the side effects of AEDs in adults with epilepsy and accurately observe the physical changes caused by depression. In addition, it is imperative to establish an active and effective nursing intervention program to strengthen the self-efficacy of the patients and to improve their quality of life through social support provided by family members and medical professionals.

• Journal of Korean Neurosurgical Society
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• 제62권3호
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• pp.353-360
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• 2019

Epilepsy surgery that eliminates the epileptogenic focus or disconnects the epileptic network has the potential to significantly improve seizure control in patients with medically intractable epilepsy. Magnetic resonance-guided laser interstitial thermal therapy (MRgLITT) has been an established option for epilepsy surgery since the US Food and Drug Administration cleared the use of MRgLITT in neurosurgery in 2007. MRgLITT is an ablative stereotactic procedure utilizing heat that is converted from laser energy, and the temperature of the tissue is monitored in real-time by MR thermography. Real-time quantitative thermal monitoring enables titration of laser energy for cellular injury, and it also estimates the extent of tissue damage. MRgLITT is applicable for lesion ablation in cases that the epileptogenic foci are localized and/or deep-seated such as in the mesial temporal lobe epilepsy and hypothalamic hamartoma. Seizure-free outcomes after MRgLITT are comparable to those of open surgery in well-selected patients such as those with mesial temporal sclerosis. Particularly in patients with hypothalamic hamartoma. In addition, MRgLITT can also be applied to ablate multiple discrete lesions of focal cortical dysplasia and tuberous sclerosis complex without the need for multiple craniotomies, as well as disconnection surgery such as corpus callosotomy. Careful planning of the target, the optimal trajectory of the laser probe, and the appropriate parameters for energy delivery are paramount to improve the seizure outcome and to reduce the complication caused by the thermal damage to the surrounding critical structures.

• 대한핵의학회지
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• 제34권3호
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• pp.169-182
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• 2000

목적: Ictal SPECT의 간질 병소를 찾는 민감도와 정확도는 각 연구마다 다르다. 발작 중에 발생할 수 있는 여러 가지 형태의 뇌혈류변화를 알아보고 이와 관련된 임상 요인들을 찾아보기 위하여 본 연구를 수행하였다. 대상 및 방법: 간질 수술을 받을 61명의 부분적 간질 환자들에서 interictal SPECT와 ictal SPECT를 시행한 후 두 영상을 차감하여 subtraction SPECT (sub-SPECT)영상을 구하였다. Sub-SPECT에서 보인 발작 중 뇌혈류변화의 형태는 1) 국소 뇌혈류증가(focal hyperperfusion), 2) 뇌혈류증가 플러스(hyperperfusion-plus), 3) 국소 뇌혈류감소(focal hypoperfusion), 그리고, 4) 혈류증가-감소 복합형(combined hyperperfusion-hypoperfusion)의 4가지로 나누었다. 각 형태 별로 간질초점 (epielptic focus)과의 일치율 (concordance rate)을 산출하였다. 결과: Conventional ictal SPECT 분석법의 간질촛점 진단율은 68.9%이었고, sub-SPECT의 진단율은 뇌혈류증가 만을 분석하였을때는 85.2%이었고, 뇌혈류 증가 및 감소를 모두 평가하였을 때는 91.3%이었다. 발작 중 국소 뇌혈류 감소는 측두엽외 간질에 비하여, 측두엽 간질에서 더 드물게 관찰되었다. 내측 측두엽에서만 발생한 뇌혈류증가는 내측 측두엽간질에서만 관찰되었고, 외측 측두엽에서만 발생한 뇌혈류증가는 신피질 측두엽간질에서만 관찰되었다. 해마 경화를 동반한 측두엽간질은 다른 병리소견에 비하여 발작 중 뇌혈류감소 현상을 매우 적게 보였다. 결론: Ictal SPECT를 분석할 때는 뇌혈류증가 뿐만 아니라 뇌혈류감소 현상도 유심히 보아야 하며, 발작 중 국소 뇌혈류감소의 기전으로 간질초점의 발작 중 초기 에너지고갈 (intra-ictal early exhaustion) 또는 간질초점 주변부로의 발작파 전파에 의한 steal 현상 등을 생각할 수 있지만 앞으로 규명하여야 할 과제이다.