• Clinical and Experimental Pediatrics
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• v.49 no.10
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• pp.1086-1092
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• 2006

Purpose : Refractory status epilepticus(RSE) is a serious neurological emergency in children. The mortality is high and the neurological outcome is not good. This study aimed to evaluate the clinical significance of cerebrospinal fluid(CSF) pleocytosis in refractory status epilepticus in children. Methods : From January 1999 to January 2006, 25 out of 37 children with refractory status epilepticus had spinal tapping. We retrospectively analyzed the data from these children's medical records. We compared the results between groups with and without CSF pleocytosis, and between a group with first seizure and a group with epilepsy. Result : Six out of 25 children had CSF pleocytosis. The group without CSF pleocytosis had a higher mortality rate and required higher doses of antiepileptic drugs as compared with the group with CSF pleocytosis. The group with CSF pleocytosis had much worse neurologic segualae. However, except for the children with CNS infection, the overall prognosis between the group with and without CSF pleocytosis was not significantly different. All children with CSF pleocytosis came in with first seizures. Conclusion : In children with RSE, a CSF study must be perfomed as soon as possible to exclude the possibility of CNS infection. A CSF study is even more important in cases of first seizure or CNS infection suspected. Mild CSF pleocytosis without evidence of infection does not seem to affect the prognosis, so physicians should therefore be more cautious in selecting antibacterial or antiviral agents for it.

• Journal of The Korean Society of Clinical Toxicology
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• v.6 no.2
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• pp.142-145
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• 2008

Lamotrigine is a newer anti-epileptic drug for adjunctive treatment of refractory epilepsy, partial seizures, generalized tonic-clonic seizures, and bipolar disorder. Lamotrigine overdose causes serious central nervous and cardiovascular problems, but reports are uncommon. Few lamotrigine overdoses have been described because anti-epileptic drug use is limited and usually used with combination of other anti-epileptic drugs. In addition, most patients visit emergency departments with multi-drug overdoses, so few cases of lamotrigine poisoning alone exist. We had a female patient visit our emergency department a couple of hours after a lamotrigine overdose treated with intravenous hydration and urine alkalization by NaHCO3. She recovered successfully without any evidence of renal injury. However, she developed profound rhabdomyolysis, a previously unreported complication of this medication. We suggest that serial creatine kinase levels should be measured after lamotrigine poisoning.

• The Korean Journal of Physiology and Pharmacology
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• v.15 no.2
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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.

• Korean Journal of Biological Psychiatry
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• v.3 no.2
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• pp.302-308
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• 1996

A case of rapid cycling mania secondary to gamma-knife radiosurgery for the treatment of refractory epilepsy was reported. A 21-year old woman who had a gamma-knife radiosurgical operation for the treatment of refractory seizure two years ago was admitted because of manic episodes. Although seizure was relieved, manic symptoms like decreased need for sleep, elated mood, unprovoked laughing, grandiose delusion and bizarre behaviors were developed 11 months after the operation. These symptoms recurred lour rimes for eight months. There were no past personal and family history of mood disorders. Laboratory examinations including electroencephalogram ana endocrinological study did not show any abnormal findings. The rapid cycling secondary manic was relieved by lithium. She was then discharged after 5 months. Mood change was not significant during follow-up while hypomania emerged by dose reduction. The secondary mania seemed to be caused or triggered by the right temporal lobe damage induced by gamma-knife radiosurgery.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.16 no.2
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• pp.131-134
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• 2013

Cecal volvulus is uncommon in pediatric patients and there are few reports of cecal volvulus with cerebral palsy. Here, we report the case of a 19-year-old male patient who presented with abdominal distension, a history of cerebral palsy, refractory epilepsy due to lissencephaly, and chronic constipation. An abdominal x-ray and computed tomography without contrast enhancement showed fixed dilated bowel intensity in the right lower abdomen. Despite decompression with gastric and rectal tube insertion, symptoms did not improve. The patient underwent an exploratory laparotomy that revealed cecal volvulus. Cecal volvulus usually occurs following intestinal malrotation or previous surgery. In this patient, however, intestinal distension accompanying mental disability and chronic constipation resulted in the development of cecal volvulus. We suggest that cecal and proximal large bowel volvulus should be considered in patients presenting with progressive abdominal distension combined with a history of neuro-developmental delay and constipation.

• Korean Journal of Radiology
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• v.25 no.9
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• pp.807-823
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• 2024

Autoimmune encephalitis (AE) is a category of immune-mediated disorders of the central nervous system (CNS) affecting children and adults. It is characterized by the subacute onset of altered mentation, neurocognitive issues, refractory seizures/drug-resistant epilepsy, movement disorders, and/or autonomic dysfunction. AE is mediated by autoantibodies targeting specific surface components or intracytoplasmic antigens in the CNS, leading to functional or structural alterations. Multiple triggers that induce autoimmunity have been described, which are mainly parainfectious and paraneoplastic. The imaging features of AE often overlap with each other and with other common causes of encephalitis/encephalopathy (infections and toxic-metabolic etiologies). Limbic encephalitis is the most common imaging finding shared by most of these entities. Cortical, basal ganglia, diencephalon, and brainstem involvement may also be present. Cerebellar involvement is rare and is often a part of paraneoplastic degeneration. Owing to an improved understanding of AE, their incidence and detection have increased. Hence, in an appropriate setting, a high degree of suspicion is crucial when reporting clinical MRIs to ensure prompt treatment and better patient outcomes. In this review, we discuss the pathophysiology of AE and common etiologies encountered in clinical practice.

• Journal of the Korean Child Neurology Society
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• v.26 no.4
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• pp.276-279
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• 2018

Klinefelter syndrome a genetic disorder with various clinical manifestations. Neurological symptoms, such as seizures, are rarely reported with Klinefelter syndrome, and it response well to anti-epileptic drugs. A 5-month-old boy visited the Inha university hospital due to jerking movements and hiccups. The patient had been diagnosed with Klinefelter syndrome at birth and had a medical history of admission to the neonatal intensive care unit due to opisthotonus and ocular deviation at 26 days of age. The patient's serum testosterone level was decreased and his anti-$M{\ddot{u}}llerian$ hormone level was increased. The brain image examination was normal and the electoencephalography and other blood test results showed no specific findings. However, after admission, the patient recurred generalized tonic-clonic-seizures recurred intermittently even after the administration of antiepileptic drugs. This paper reports a case of non-febrile seizures in a child with Klinefelter syndrome who presented with a refractory course.

• Journal of Korean Neurosurgical Society
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• v.48 no.3
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• pp.244-250
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• 2010

Objective : Adequate management of increased intracranial pressure (ICP) is critical in patients with traumatic brain injury (TBI), and decompressive craniectomy is widely used to treat refractory increased ICP. The authors reviewed and analyzed complications following decompressive craniectomy for the management of TBI. Methods : A total of 89 consecutive patients who underwent decompressive craniectomy for TBI between February 2004 and February 2009 were reviewed retrospectively. Incidence rates of complications secondary to decompressive craniectomy were determined, and analyses were performed to identify clinical factors associated with the development of complications and the poor outcome. Results : Complications secondary to decompressive craniectomy occurred in 48 of the 89 (53.9%) patients. Furthermore, these complications occurred in a sequential fashion at specific times after surgical intervention; cerebral contusion expansion ($2.2{\pm}1.2$ days), newly appearing subdural or epidural hematoma contralateral to the craniectomy defect ($1.5{\pm}0.9$ days), epilepsy ($2.7{\pm}1.5$ days), cerebrospinal fluid leakage through the scalp incision ($7.0{\pm}4.2$ days), and external cerebral herniation ($5.5{\pm}3.3$ days). Subdural effusion ($10.8{\pm}5.2$ days) and postoperative infection ($9.8{\pm}3.1$ days) developed between one and four weeks postoperatively. Trephined and post-traumatic hydrocephalus syndromes developed after one month postoperatively (at $79.5{\pm}23.6$ and $49.2{\pm}14.1$ days, respectively). Conclusion : A poor GCS score ($\leq$ 8) and an age of $\geq$ 65 were found to be related to the occurrence of one of the above-mentioned complications. These results should help neurosurgeons anticipate these complications, to adopt management strategies that reduce the risks of complications, and to improve clinical outcomes.

• The Korean Journal of Physiology and Pharmacology
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• v.26 no.1
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• pp.47-57
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• 2022

Stiripentol is an anti-epileptic drug for the treating of refractory status epilepticus. It has been reported that stiripentol can attenuate seizure severity and reduce seizure-induced neuronal damage in animal models of epilepsy. The objective of the present study was to investigate effects of post-treatment with stiripentol on cognitive deficit and neuronal damage in the cornu ammonis 1 (CA1) region of the hippocampus proper following transient ischemia in the forebrain of gerbils. To evaluate ischemia-induced cognitive impairments, passive avoidance test and 8-arm radial maze test were performed. It was found that post-treatment with stiripentol at 20 mg/kg, but not 10 or 15 mg/kg, reduced ischemia-induced memory impairment. Transient ischemia-induced neuronal death in the CA1 region was also significantly attenuated only by 20 mg/kg stiripentol treatment after transient ischemia. In addition, 20 mg/kg stiripentol treatment significantly decreased ischemia-induced astrocyte damage and immunoglobulin G leakage. In brief, stiripentol treatment after transient ischemia ameliorated transient ischemia-induced cognitive impairment in gerbils, showing that pyramidal neurons were protected and astrocyte damage and blood brain barrier leakage were significantly attenuated in the hippocampus. Results of this study suggest stiripentol can be developed as a candidate of therapeutic drug for ischemic stroke.

• Clinical and Experimental Pediatrics
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• v.48 no.7
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• pp.753-759
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• 2005

Purpose : This study was performed to characterize clinical features of benign convulsions with gastroenteritis(CwG) in infants. Methods : We reviewed clinical features of 67 episodes in 64 patients with afebrile seizure accompanied gastroenteritis admitted to Dept. of Pediatrics Bundang CHA hospital from January 2001 to June 2004. Patients with meningitis, encephalitis/encephalopathy or apparent history of epilepsy were excluded. Results : There were 32 boys and 35 girls. The age of onset ranged from 1 to 42 months($18.5{\pm}6.1$ months). The number of children admitted to the hospital with acute gastroenteritis was 2,887 in the same period. The percentage of patients with CwG was 2.3. Seizure type was exclusively generalized tonic or tonic-clonic seizure. The average number of seizures during a single episode was 3.1 (range, 1-13). Two or more seizures occurred in 53(79.1%) of the 67 episodes. Antiepileptic drugs were administered for 42 episodes. Seizure did not cease after the administration of one kind of antiepileptic drug in 23 episodes(54.7%). The seizures were rather refractory to initial antiepileptic treatment. There were no abnormalities in serum biochemistry test including glucose and electrolytes. Cerebrospinal fluid was normal in all 54 episodes. Stool cultures were negative in 49 episodes. Rotavirus was positive in stools in 51(82.3%) of 62 episodes. Norovirus was positive in stools in 2 episodes and astrovirus in 1 of 18 episodes. CT and/or MRI were performed in 15 cases and demonstrated no neuroradiologic abnormalities. Of 73 Interictal EEG, initial 24 cases showed occasional spike or sharp wave discharges from the mid-line area during stage I-II sleep, which were apparently differentiated from vertex sharp transient or K-complexes. The mean follow-up period was 5.7 months(1-36 months). Three patients experienced a recurrence of CwG, but all patients exhibited normal psychomotor development at the last follow-up. Conclusion : Afebrile infantile convulsions with gastroenteritis are brief generalized seizure in cluster with normal laboratory findings and good prognosis. Therefore CwG is likely to be categorized as situation-related seizure of special syndrome. Recognition of this entity should lead to assurance of the parents and long-term anticonvulsant therapy is not usually warranted.