• Pediatric Infection and Vaccine
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• v.3 no.2
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• pp.207-213
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• 1996

Herpes simplex virus(HSV) infections of the CNS are associated with significant morbidity and mortality even when appropriate antiviral therapy is administered. HSV infections of the brain can be subdivided into two categories : neonatal HSV infections, which usually are caused by HSV type 2, and herpes simplex encephalitis(HSE), which occur in patients over 3 months old and is nearly uniformly caused by HSV type 1. The clinical presentation of HSE is one of the focal encephalopathic process associated with altered levels of consciousness, fever, focal seizures and hemiparesis. But because of the lack of pathognomic clinical presentation and diagnostic procedure, the efforts to develop alternative diagnostic procedure have led to the use of new diagnostic technique such as polymerase chain reaction(PCR). We report a case of HSV type 1 encephalitis in 13 month old male infant who presented with altered level of consciousness, fever and focal seizures. With the use of the PCR, HSV-1 DNA was detected in cerebrospinal fluid from the patient. The symptoms and signs of encephalitis subsided by treatment with acyclovir in 14 days.

• Journal of The Korean Society of Clinical Toxicology
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• v.11 no.2
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• pp.127-129
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• 2013

The imidazopyridine, zolpidem, a non-benzodiazepine hypnotic drug, is widely-prescribed for insomnia. It is regarded as a good alternative to benzodiazepine because of the reduced possibility for abuse and development of dependence. However, more recently, due to the reduced possibility for abuse and development of dependence, it is regarded as a good alternative to benzodiazepine. adverse effects of zolpidem have been recognized. The objective of this report is to provide information on the potential for occurrence of benzodiazepine-like withdrawal seizure in patients who chronically take zolpidem continually. We present and discuss a case of seizure after sudden interruption of the protracted use of an abusively high dose of zolpidem. Zolpidem may not be the ideal drug for longterm pharmacotherapeutic management of insomnia. Clinicians should administer zolpidem at a low-dose for a short period of time for prevention of drug abuse and dependence and the potential for occurrence of benzodiazepine- like withdrawal seizure.

• Korean Journal of Veterinary Service
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• v.40 no.2
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• pp.143-147
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• 2017

A Korean domestic short hair (1-year-old, male) presented with 2 to 3 weeks of seizures, aggressive behavior, vomiting, anorexia, and lethargy. The frequency of seizure had gradually increased from once a week to once every 3 hours. Physical and neurologic examination, diagnostic screening tests, including complete blood count (CBC), serum chemistry, electrolyte, coagulation test, X-ray, ultrasonography, and urinalysis were performed. Feline Leukemia Virus (FeLV), Feline Immunodeficiency Virus (FIV) and Toxoplasma spp. All tested negative, but the Feline Corona Virus (FCoV) kit revealed a positive result. To determine the exact diagnosis, magnetic resonance imaging (MRI) was performed but yielded no specific findings. The patient was then diagnosed with idiopathic epilepsy and treatment of phenobarbital was initiated. A month's treatment with phenobarbital proved ineffective as symptoms worsened. Zonisamide was then selected as an additional anticonvulsant. After adding zonisamide, symptoms improved, and seizures abated for 15 months. This is the first case report in South Korea describing the use of phenobarbital and zonisamide in the treatment of a cat with idiopathic epilepsy.

• The Journal of Korean Medicine
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• v.36 no.2
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• pp.50-55
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• 2015

Infants with tuberous sclerosis complex (TSC) have a higher chance of experiencing seizures before the age of 1 year; in particular, they commonly accompany infantile spasms. In cases where infantile spasms resulting from TSC are drug-resistant, more severe neuro-developmental and cognitive impairments occur. This particular case dealt with an infant with TSC who continued to experience partial seizures and infantile spasms despite using two different kinds of antiepileptic drugs (AEDs). His spasms ceased on the seventh day of taking modified Yukmijihwang-tang (YMJ), at which point he stopped the use of all AEDs. He became seizure-free after a month of the treatment and modified hypsarrythmia was found to have been resolved in the electroencephalogram test. Until now, the infant has been taking YMJ for 16 months and is maintaining the seizure-free state without side effects. Moreover, his developmental status is continually improving, with a significant progress in language and cognitive-adaptive abilities. Such results suggest that YMJ can serve as an alternative treatment option for refractory epilepsy.

• Clinical and Experimental Pediatrics
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• v.45 no.10
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• pp.1278-1282
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• 2002

Glutaric aciduria(type 1) is characterized clinically by progressive dystonia and dyskinesia in childhood, pathologically by degeneration of caudate and putamen, biochemically by tissue deficiency of glutaryl-CoA dehydrogenase(GCDH), and is transmitted as an autosomal recessive traits. Mutations of the GCDH gene on chromosome 19 have been implicated in the causation of glutaric aciduria(type 1). Macrocephaly in infancy and crossing of percentiles for head circumference are real clues to early diagnosis. Acute neuroregression of dystonia following an initial phase of normal or almost normal development is a common mode of presentation, at times preceded by seizures. We experienced a case of glutaric aciduria(type 1) in a 13-month old girl. She was admitted due to development delay and choreoasthetoid movememt that developed after generalized tonic-clonic type seizures. She was diagnosed as having glutaric aciduria(type 1) based on brain MRI and urine organic acid analysis finding.

• Neonatal Medicine
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• v.28 no.2
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• pp.94-98
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• 2021

Neonatal diabetes mellitus (NDM) is a rare disease that occurs at less than 6 months of age and is presumably caused by a mutation in the gene that affects pancreatic beta-cell function. Approximately 80% of NDM cases reveal a known genetic mutation, and mutations in potassium inwardly rectifying channel subfamily J member 11 (KCNJ11) and ABCC8 affecting the pancreatic beta-cell adenosine triphosphate-sensitive potassium channel may be treated with oral sulfonylurea. Early recognition of mutations in KCNJ11 and ABCC8 is important because early administration of sulfonylurea can not only control blood glucose levels but also improve neurodevelopmental outcomes. In the present study, we report a case of NDM that initially presented as diabetic ketoacidosis at the age of 1 month, accompanied by seizures during hospitalization. After confirmation of the KCNJ11 gene mutation (c.989A>C), we started administering oral sulfonylurea (glimepiride) at the age of 2 months. After gradually increasing the dosage of glimepiride, insulin was discontinued at the age of 3 months. To date, the infant's blood glucose levels have been well controlled without significant hypoglycemic events. No further episodes of seizures have occurred, and his developmental status is favorable.

• Journal of Korean Neurosurgical Society
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• v.62 no.3
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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.

• Journal of Korean Neurosurgical Society
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• v.62 no.3
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• pp.296-301
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• 2019

An epileptic seizure is defined as the transient occurrence of signs and/or symptoms due to abnormally excessive or synchronous neuronal activity in the brain. The type of seizure is defined by the mode of onset and termination, clinical manifestation, and by the abnormal enhanced synchrony. If seizures recur, that state is defined as epilepsy. Antiepileptic drugs (AEDs) are the mainstay of treatment. Knowledge about initiating and maintaining adequate AEDs is beneficial for the clinician who treats children with epilepsy. This article will delineate the general principles for selecting, introducing, and discontinuing AEDs and outline guidelines for monitoring adverse effects. In general, AED therapy following a first unprovoked seizure in children is not recommended. However, treatment should be considered after a second seizure. In children and adolescents, if they are seizure-free for at least 2 years, attempts to withdraw medication/s should be made, taking into account the risks vs. benefits for the individual patient. The decision on when and what AED to use should be tailored according to the patient. For optimal treatment, the selection of adequate AEDs can be achieved by considering the precise definition of the patient's seizure and epilepsy syndrome. Continuous monitoring of both therapeutic and adverse effects is critical for successful treatment with AEDs.

• Childhood Kidney Diseases
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• v.28 no.1
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• pp.44-50
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• 2024

Systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory disease that affects multiple organs. More than half of the patients with SLE have kidney involvement, and up to 10% of patients with lupus nephritis develop end-stage renal disease (ESRD). Central nervous system (CNS) involvement in SLE occurs in 21% to 95% of patients. Severe neurological manifestations such as seizures, cerebrovascular disease, meningitis, and cerebrovascular accidents can develop in childhood-onset SLE, but cerebral infections, such as brain abscess and hemorrhage, are seldom reported in lupus nephritis, even in adults. Here, we report a rare case of childhood-onset SLE with ESRD, cerebral abscess, and hemorrhage. A 9-year-old girl diagnosed with lupus nephritis was administered high-dose steroids and immunosuppressant therapy to treat acute kidney injury (AKI) and massive proteinuria. The AKI deteriorated, and after 3 months, she developed ESRD. She received hemodialysis three times a week along with daily peritoneal dialysis to control edema. She developed seizures, and imaging showed a brain abscess. This was complicated by spontaneous cerebral hemorrhage, and she became unstable. She died shortly after the hemorrhage was discovered. In conclusion, CNS complications should always be considered in clinical practice because they increase mortality, especially in those with risk factors for infection.

• Journal of Genetic Medicine
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• v.21 no.1
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• pp.36-40
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• 2024

Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episode (MELAS) syndrome is a maternally inherited mitochondrial disorder that usually affects the cerebral cortex and prevents high-energy demands from being met. Herein, we present the case of a male patient who rapidly developed multiple seizures, headaches, and altered mentality accompanied by severe metabolic acidosis and lactic acidosis. Initially, a brain imaging study confirmed stroke-like lesions (SLLs) only in the cerebellum. During follow-up, newly developed SLLs with lactic acidosis were observed in the basal ganglia (BG), cerebellum, and occipital lobe. The m.3243A>G variant had been found in the patient and MELAS was diagnosed, despite the BG and cerebellum being atypical locations for SLLs in MELAS. Since most cases of m.3243A>G variant MELAS show SLLs in the cerebral cortex, this case is unusual considering the location of the lesion. We emphasize that in the case of lactic acidosis accompanied by neurological symptoms, such as seizures, as in this case, MELAS should be included in the differential diagnosis, even if SLLs are observed in areas other than the cerebral cortex.