Sleep-onset Central Sleep Apnea

Sleep-onset central sleep apnea is not uncommon phenomenon, and is usually regarded as a normal sleep pattern. Dysrhytymic breathing is frequently seen at sleep onset. Any process that leads to frequent sleep-wake transitions over the course of the night (such as insomnia) may increase the number of central apneas. Although the exact mechanism is still unclear, rapid loss of the wakefulness drive to breathe and unstable carbon dioxide set point results in central apnea/hypopnea leading to hypercapnia which induces subsequent hyperventilation secondary to arousals. If the following hypocapnia cross the apnea threshold, then central sleep apnea resumes. In this brief review, I will address the underlying physiology influencing sleep-onset central sleep apnea and its clinical implications.ope

간질중첩증 후 해마 내 선택적 세포사멸현상에 미치는 superoxide dismutase 1의 역할

Dept. of Medicine/박사Selected vulnerability of hippocampal neuron after epileptic insults is a well-known phenomenon. Pyramidal neurons in the Cornus Ammonis (CA) 1 and 3 are known as particularly vulnerable; however, the granule cells in the dentate gyrus (DG) as well as neurons in the CA2 are known to be resistant to the various insults including status epilepticus (SE) in the animal models or even in the human temporal lobe epilepsy. The molecular basis for this different susceptibility remains controversial, but different defense mechanisms against exogenous insults have been suggested. Excessive reactive oxygen species (ROS) production has been reported as a responsible process for neural cell death in the various neurological disorders such as ischemic stroke, Alzheimer’s disease, or Huntington disease. In addition, the pathological overproduction of ROS has been suggested as a crucial process in the neuronal death by SE. However, the role of superoxide anion (O2？-), one of the most potent ROS, in the seizure-induced neuronal death has remained unclear. I hypothesized that the pathological excessive production of O2？- may contribute to hippocampal neuronal death after SE, and different activation of superoxide dismutase 1 (SOD1), the selective inhibitor of O2？-, in the hippocampal subfields might be responsible for this selected vulnerability after SE. Adult male C57BL/6J mice were given injections of pilocarpine to induce SE, which was confirmed by visual inspection and electroencephalography. Hippocampal neuronal death was assessed by both cresyl-violet and TUNEL staining. The temporal and spatial production of O2？- in each hippocampal subfield was investigated using in situ detection of oxidized hydroethidine (HEt). Western blot, activity assay, and immunohistochemical staining of SOD1 in the each hippocampal subfield were performed to investigate the role of specific defense system of O2？-. Lamotrigine (LTG), a neuroprotectant in the various animal models by modulating ROS, was treated to see its effect on SOD1. Inhibitor of SOD1, diethyldithiocarbamate (DDC) was treated to identify the effect of SOD1 inhibition. Neuronal cell death and TUNEL-positive cells increased significantly in the hippocampal CA1 and CA3 compared to DG after SE. O2？- measured by oxidized HEt significantly increased after pilocarpine-induced SE, especially in the CA1 and CA3 compared to DG. In the normal control, SOD1 expression in the DG was significantly higher than those of CA1 and CA3. The expression and activity of SOD1 significantly increased in the DG compared to CA1 and CA3 as early as 12 hours after SE. Treatment of LTG significantly increased SOD1 activity, expression, and decreased O2？- production, thus ameliorated the selective neuronal death in the CA1 and CA3. DDC treatment resulted in marked decrease of SOD1 activity, increased production of O2？-, and subsequent extensive neuronal cell death in the DG after SE. This study confirmed that the excessive production of O2？- after SE resulted in different neuronal cell death according to the hippocampal subfields, to less degree in the DG compared to CA1 and CA3. Increased expression and activity of SOD1 in the DG and marked neuronal death by SOD1 inhibition were confirmed. The difference defense mechanism against O2？- by SOD1 in each hippocampal subfield may have a pivotal role in the selected vulnerability of hippocampal subfields after SE.ope

잘 낫지 않는 두통의 치료와 예방

Headache is one of the most common complaints among patients presenting to an outpatient neurological practice. The evaluation, diagnosis, and treatment of headache can be challenging even for the experienced neurologist. The first step that a physician must keep in mind when seeing the headache patient is to differentiate primary headache and secondary headache, although the latter is relatively small in number, but it needs thoughtful investigation and sometimes emergent management. After ruling out the secondary headache, the physician should make a correct diagnosis of primary headache that can be encountered in most clinical practices according to the “The International Headache Society’s International Classification of Headache Disorders 2nd Edition (ICHD-2)”, and appropriate treatment. In this article, the author review some of the essential factors that are part of headache evaluation, warning signs and investigation plan of secondary headache disorders. Then I will address a brief review on the diagnosis of primary headache disorders and treatment strategies of the more common primary headache disorders focusing on migraine, tension-type headache, and chronic daily headache.ope

A Case of Posterior Cerebral Artery Infarction Induced by Posterior Cerebral Artery Dissection

Background: Artery Dissection should be concerned as a risk factor of stroke in young patients. Case Report: We describe a 26- year-old male patient with posterior cerebral artery infarction resulting from isolated PCA dissection. The brain MRI showed an infarction of the right PCA territory and the DSA (digital subtraction angiography) showed isolated irregular stenosis in PCA P1-P2 junction. Conclusion: PCA dissection can be a cause of strokeope

A Case of Orgasmic Aura Associated with Temporal Lobe Epilepsy from the Nondominant Hemisphere

We experienced a patient with an orgasmic aura originating from the right mesial temporal structure. A 36-year-old right-handed woman suffered from a specific sensation of sexual arousal and orgasm-like euphoria lasting 1~2 minutes for several years. Video EEG monitoring ascertained those sensations as epileptic in nature arising from the right mesial temporal area through a foramen ovale electrode. The findings of 99mTc-ECD-SPECT and 18F-FDG PET were concordant with those of the brain MRI which was pathologically consistent with hippocampal sclerosis.ope

Management of Alcohol Withdrawal Syndrome and Alcohol Withdrawal Seizure

Alcohol withdrawal syndrome (AWS) is a common condition occurring after intentional or unintentional abrupt cessation of alcohol in an alcohol-dependent individual. AWS represents a major problem in our society and alcohol withdrawal seizure is the major cause of seizures encountered by neurology residents in the emergency department. Patients with AWS present with mild symptoms of tremulousness and agitation or more severe symptoms including withdrawal seizures and delirium tremens. Particularly, severe AWS can produce significant rates of the morbidity (complications) and mortality. When diagnosed and managed insufficiently, the morbidity and mortality rates increase. Nevertheless, patients with AWS may be neglected and are often marginalized and the teaching about AWS to neurology residents is usually minimal. Also, attending neurologists are often poorly informed on the topic. Although there is insufficient consensus about the optimal investigation and management, the purpose of this review is to serve as a summary of the appropriate identification and management of this important condition in a neurological setting.ope

Genetic Variations of ABCC2 Gene Associated with Adverse Drug Reactions to Valproic Acid in Korean Epileptic Patients

The multidrug resistance protein 2 (MRP2, ABCC2) gene may determine individual susceptibility to adverse drug reactions (ADRs) in the central nervous system (CNS) by limiting brain access of antiepileptic drugs, especially valproic acid (VPA). Our objective was to investigate the effect of ABCC2 polymorphisms on ADRs caused by VPA in Korean epileptic patients. We examined the association of ABCC2 single-nucleotide polymorphisms and haplotype frequencies with VPA related to adverse reactions. In addition, the association of the polymorphisms with the risk of VPA related to adverse reactions was estimated by logistic regression analysis. A total of 41 (24.4%) patients had shown VPA-related adverse reactions in CNS, and the most frequent symptom was tremor (78.0%). The patients with CNS ADRs were more likely to have the G allele (79.3% vs. 62.7%, p = 0.0057) and the GG genotype (61.0% vs. 39.7%, p = 0.019) at the g.-1774delG locus. The frequency of the haplotype containing g.-1774Gdel was significantly lower in the patients with CNS ADRs than without CNS ADRs (15.8% vs. 32.3%, p = 0.0039). Lastly, in the multivariate logistic regression analysis, the presence of the GG genotype at the g.-1774delG locus was identified as a stronger risk factor for VPA related to ADRs (odds ratio, 8.53; 95% confidence interval, 1.04 to 70.17). We demonstrated that ABCC2 polymorphisms may influence VPA-related ADRs. The results above suggest the possible usefulness of ABCC2 gene polymorphisms as a marker for predicting response to VPA-related ADRs.ope

Chronic Recurrent Cholangitis Induced by Carbamazepine

Carbamazepine (CBZ) is one of the most commonly used antiepileptic agents. With its potent effects against seizure or neuropathic pain, it also has several undesirable adverse events. CBZ has been known to induce hepatotoxicity because the drug is mainly metabolized through hepatic system, and asymptomatic liver enzyme elevation occurs in 5~10% of patients receiving CBZ. There are several cases of symptomatic hepatitis or hepatic necrosis by CBZ, however, reports of chronic cholangitis associated with CBZ medication are rare. Here, we present a case of chronic recurrent cholangitis by CBZ with pathological evidence.ope

Clinical outcome following medical treatment of cavernous malformation related epilepsy

PURPOSE: The study was conducted to assess the long-term outcome of antiepileptic drug (AED) treatment in drug-naïve patients with cavernous malformation (CM) related epilepsy (CRE). METHOD: This is a retrospective, single-center, long-term observational study of 34 patients with previously untreated seizures related to CM. All patients were followed-up for at least two years. Drug resistant epilepsy (DRE) was defined as two or more seizures per year after trial of two appropriate AEDs. Patients who had only one seizure during the previous one year were assigned as "epilepsy with rare seizures (ERSs)". RESULTS: Terminal 1-year seizure remission (1-YTR) was achieved in 22 (64.7%) patients, nine (26.5%) patients were diagnosed as DRE, and three (8.8%) patients were as ERSs. 1-YTR was achieved in 18 (52.9%) patients by the first drug regimen and in additional four (11.8%) patients by the second drug regimen. None of nine patients who failed to first two drug regimens did achieve 1-YTR. The location of CM in the temporal lobe was the only prognostic factor predicting a poor seizure outcome (p=0.012). CONCLUSION: The outcome of AEDs therapy in patients who were presented with new onset of CRE was quite comparable with that of patients with newly diagnosed epilepsy. Failure to achieve seizure-free after adequate trials of two AEDs seems appropriate as the criteria for their referral to surgical treatment. For patients with temporal lobe CRE, earlier presurgical evaluation may be considered justifiable once they failed to an adequate trial of the first drug.ope

Development of epilepsy after posterior reversible encephalopathy syndrome

PURPOSE: This study was intended to describe the risk of epilepsy subsequent to posterior reversible encephalopathy syndrome (PRES) and the clinical features of post-PRES epilepsy. METHOD: We retrospectively identified all patients with PRES who were admitted to Severance Hospital and consulted with the Department of Neurology between 2001 and 2013 and the subgroup of these patients who subsequently developed epilepsy. We also describe clinical features of patients who were not treated with PRES as inpatients at our center but who presented later with post-PRES epilepsy during the study period. We studied clinical characteristics during the acute symptomatic phase of PRES and after the development of epilepsy. RESULTS: During the study period 102 patients were treated at our center during the acute phase of PRES. Four of these patients (3.9%) subsequently developed epilepsy. Two additional patients with a history of PRES presented to our hospital after the acute phase of their illness with post-PRES epilepsy. During the acute phase, five of six patients had acute symptomatic seizures and four had convulsive or nonconvulsive status epilepticus (SE). Acute phase MRI showed cytotoxic edema in five patients, and follow-up MRI showed focal atrophic changes including hippocampal sclerosis in four. Presumptive epileptogenic foci were located in the left-side temporal, parietal and occipital lobes, corresponding to the regions that showed cytotoxic edema or severe vasogenic edema as well as with the location or lateralization of EEG abnormalities during the acute phase. CONCLUSION: Our findings indicate a small but not insignificant risk for the development of epilepsy after PRES. The presence of cytotoxic edema and severe, acute symptomatic seizures, such as SE suggests irreversible brain damage and may predict the development of epilepsy.ope