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Introduction Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) is a rare variation of internuclear ophthalmoplegia associated with bilateral lesions of the medial longitudinal fasciculus. Case report In this case, the edematous lesions of the ipsilateral midbrain infarction appeared to compress the contralateral medial longitudinal fasciculus, resulting in WEBINO. Treatment of focal cerebral edema caused by ischemic stroke is still under debate. However, in this case, antiedema treatment was implemented to prevent the sequelae of the edematous lesion caused by a midbrain infarction, and a relatively rapid improvement of ocular symptoms was observed. Conclusion Herein, we report a rare case of WEBINO caused by a unilateral midbrain infarction in which the patient experienced edema-related neurological deficits. This case provides evidence that early antiedema therapy may benefit patients with similar presentations.

Klebsiella pneumoniae is an unusual cause of brain abscess. Among the few cases of Klebsiella pneumoniae brain abscess that have been reported, most were associated with another underlying primary focus of infection. Endogenous endophthalmitis caused by Klebsiella pneumoniae is an infrequent but often devastating septic metastatic infection. We report a rare case of Klebsiella pneumoniae brain abscess and endophthalmitis after acute epiglottitis.

Background Sex hormones may be associated with a higher incidence of ischemic stroke or stroke-related events. In observational studies, lower testosterone concentrations are associated with infirmity, vascular disease, and adverse cardiovascular risk factors. Currently, female sexual hormones are considered neuroprotective agents. The purpose of this study was to assess the role of sex hormones and the ratio of estradiol/testosterone (E/T) in patients with acute ischemic stroke (AIS). Methods Between January 2011 and December 2016, 146 male patients with AIS and 152 age- and sex-matched control subjects were included in this study. Sex hormones, including estradiol, progesterone, and testosterone, were evaluated in the AIS patient and control groups. We analyzed the clinical and physiological levels of sex hormones and hormone ratios in these patients. Results The E/T ratio was significantly elevated among patients in the stroke group compared to those in the control group (P = 0.001). Categorization of data into tertiles revealed that patients with the highest E/T ratio were more likely to have AIS [odds ratio (OR) 3.084; 95% Confidence interval (CI): 1.616-5.886; P < 0.001) compared with those in the first tertile. The E/T ratio was also an independent unfavorable outcome predictor with an adjusted OR of 1.167 (95% CI: 1.053-1.294; P = 0.003). Conclusions These findings support the hypothesis that increased estradiol and reduced testosterone levels are associated with AIS in men.

Introduction: Increased neutrophils, and stress-induced low lymphocyte levels both indicate changes in the immune system. Blood neutrophil/lymphocyte ratio (NLR) is shown to be a simple, reliable and inexpensive inflammatory indicator that can provide important information on many conditions. Recently, NLR has been proposed as a useful biomarker to predict cardiovascular risk. However, little is known about the role of NLR in patients with intracranial aneurysm (IA). Hypothesis: The purpose of present study was to investigate whether NLR values differ in patients with IA. Methods: The study group consisted of patients with IA who had been admitted to the neurology department from January 2008 to December 2016. A total of 406 patients including 198 patients with unruptured IA, 208 patient with ruptured IA were enrolled in this study. The control group consisted of 420 age, sex-matched healthy adults who had not harbor IA by CTA, MRA, DSA. The baseline NLR was calculated as the ratio of neutrophil count to lymphocyte count. WBC count>12.000 cells per μL or 38 ° are excluded from the study. Results: The Mean NLR was significantly higher among persons with cerebral aneurysm compared with controls (P < 0.001). The level of NLR in ruptured IA group were found higher compared to unruptured IA and control group (6.1 ± 4.3 vs 2.7 ± 1.5 vs 1.8 ± 1.1, P < 0.001). Other comorbid conditions were balanced between these three groups. Conclusions: We demonstrated that the NLR is an independent predictor of IA patients. In addition, higher NLR is associated with ruptured IA. Unlike many other inflammatory markers and bioassays, NLR are inexpensive and readily available biomarkers that may be useful for risk stratification in patients with cerebral aneurysm. The clinical implications of NLR on IA remain to be established by future investigations. A long term prospective study is needed to clarify this matter.

Highlights • Compression neuropathy caused by a ganglion cyst is a rare entity. • MRI remains an excellent diagnostic modality to evaluate the masses in the foot. • This is a very rare report of isolated medial plantar neuropathy by a ganglion cyst., Introduction Although ganglion cysts are common soft tissue tumors, nerve compression syndrome caused by a ganglion cyst in the lower extremities is very rare. Herein, we report a 57-year-old man who presented with hypoesthesia in the sole of his right foot for 6 months. We believe that reporting this rare case will help clinicians update their knowledge on possible causes of the plantar neuropathy, and avoid diagnostic delay. Presentation of case The patient had pain and numbness in the inner right sole, as well as a tingling and dull sensation. Tenderness around the area of abnormal sensation was not evident. Percussion at the abductor tunnel gave a positive Tinel’s sign in the medial plantar nerve. No mass was palpable in the right foot. Based on the electrophysiological findings, we diagnosed medial plantar nerve entrapment in the right foot. Magnetic resonance imaging (MRI) was conducted to identify a 5.5-cm long elongated cystic lesion as the cause of entrapment. The patient underwent surgical removal of the cystic mass, with histologic examination confirming the diagnosis of a large ganglion cyst. Discussion The feasibility of nerve conduction studies and electromyography for detection of nerve entrapment is still controversial. MRI is considered the best diagnostic modality, if biopsy is not feasible. Conclusion We suggest that foot imaging and electrophysiological studies should be considered for patients with isolated median plantar neuropathy to exclude the presence of space-occupying lesions, especially when conservative treatment is not effective.

Background Posterior reversible encephalopathy syndrome (PRES) is a neurological complication caused by cerebral hyperperfusion. Case Report A 46-year-old male presented with decreased mental status, left facial palsy, and left-sided weakness after video-assisted thoracoscopic surgery for a solitary pulmonary nodule. During the surgery, phenylephrine was infused intravenously for general anesthesia-induced hypotension. High signal intensity at the right parietooccipital lobe was noted on fluid-attenuated inversion recovering imaging and diffusion-weighted imaging. His neurological symptoms improved two days after initial presentation. Follow-up diffusion-weighted imaging showed resolution of the brain lesions 10 days after the surgery. Conclusions We report a patient who presented with PRES after administration of phenylephrine during resection of a solitary pulmonary nodule. PRES should be considered for patients presented with acute neurologic symptoms following surgical procedures.

Balo's concentric sclerosis is regarded as a rare variant of multiple sclerosis. Traditionally, Balo's concentric sclerosis was a post-mortem diagnosis, but the recent introduction of brain magnetic resonance imaging (MRI) scans may allow noninvasive access without biopsy. Brain MRI findings of Balo's concentric sclerosis is characteristic concentric configuration of alternating bands of white matter of different pathology, with relatively preserved myelination alternating with regions of demyelination in the cerebral white matter. We report a case of Balo's concentric sclerosis with recurrent optic neuritis. J Korean Neurol Assoc 33(4):310-314, 2015

Recurrent Guillain-Barré syndrome (GBS) is a rare, immune-mediated disease of the peripheral nervous system. It has been reported to occur at intervals ranging from four months to 10 years; published case studies suggest that 1%–6% of patients who have had GBS will experience recurrent attacks. The most commonly identified infections coinciding with GBS are Campylobacter jejuni, Haemophilus influenzae, Mycoplasma pneumonia, and cytomegalovirus, while an antecedent infection with Escherichia coli is very uncommon. In this case report, we present a rare episode of recurrent GBS, which followed a urinary tract infection (UTI) by E. coli, and an accompanying literature review. A 75-year-old woman with a prior history of acute motor axonal neuropathy (AMAN), a subtype of GBS, presented with subsequent weakness of limbs and areflexia following 10 days of fever, frequency, and dysuria. Base on nerve conduction studies, cerebrospinal fluid analysis and other clinical investigation, we diagnosed the patient with recurrent GBS caused by E. coli. The patient recovered with mild subjective weakness following treatment of intravenous immunoglobulin with ceftriaxone. We suggest that E. coli causes UTI could be one of the diverse trigger factors involved in recurrent GBS., Graphical Abstract

The advantages of a printed circuit heat exchanger (PCHE) are the compactness and efficiency derived from its heat-transfer characteristics; furthermore, a PCHE for which a diffusion bonding method was used during production can be applied to extreme environments such as a cryogenic condition. In this study, a micro-channel PCHE fabricated by diffusion bonding was investigated in a cryogenic environment regarding its thermal performance and the pressure drop. The test rig consists of an LN2 storage tank, vaporizers, heaters, and a cold box, whereby the vaporized cryogenic nitrogen flows in hot and cold streams. The overall heat-transfer coefficients were evaluated and compared with traditional correlations. Lastly, we suggested the modified heat-transfer correlations for a PCHE in a cryogenic condition.

Received July 10, 2015 Revised September 10, 2015 Accepted September 10, 2015 Address for correspondence: Sang-Jun Na, MD, PhD Department of Neurology, Konyang University Hospital, Konyang University College of Medicine, 158 Gwanjeodong-ro, Seo-gu, Daejeon 35365, Korea Tel: +82-42-600-8814 Fax: +82-42-545-0050 E-mail: nukedoc@hanmail.net 거미막낭종은 거미막층 안에 위치한 양성 병변으로 대부분 소아 연령에서 진단된다. 성인에서 진단되는 거미막낭종은 흔

PURPOSE The underlying cause of myasthenia gravis (MG) is unknown, although it likely involves a genetic component. However, no common genetic variants have been unequivocally linked to autoimmune MG. We sought to identify the genetic variants associated with an increased or decreased risk of developing MG in samples from a Korean Multicenter MG Cohort. MATERIALS AND METHODS To determine new genetic targets related to autoimmune MG, a whole genome-based single nucleotide polymorphisms (SNP) analysis was conducted using an Axiom™ Genome-Wide ASI 1 Array, comprising 598375 SNPs and samples from 109 MG patients and 150 neurologically normal controls. RESULTS In total, 641 SNPs from five case-control associations showed p-values of less than 10⁻⁵. From regional analysis, we selected seven candidate genes (RYR3, CACNA1S, SLAMF1, SOX5, FHOD3, GABRB1, and SACS) for further analysis. CONCLUSION The present study suggests that a few genetic polymorphisms, such as in RYR3, CACNA1S, and SLAMF1, might be related to autoimmune MG. Our findings also encourage further studies, particularly confirmatory studies with larger samples, to validate and analyze the association between these SNPs and autoimmune MG.

Most postoperative maxillary cysts develop more than 10 years after a Caldwell-Luc operation. They can manifest with cheek pain, swelling and dental and visual symptoms. Brain imaging should be performed to distinguish trigeminal nerve compression from various other possible causes. It should be treated by surgical intervention to relieve the above-mentioned symptoms. We report a patient who presented with compressive trigeminal neuropathy caused by a postoperative maxillary cyst. J Korean Neurol Assoc 33(3):206-208, 2015

Background: The purpose of this study is to compare the patterns of voiding dysfunction according to the locations of brainstem lesions. Methods: Between November 2008 and December 2011, a total of 30 patients participated in this study. All 30 subjects, consisting of 16 men and 14 women, aged between 41 and 82 years (mean age, 63.0 ± 11.0 years) underwent a urodynamic study within 7 days after the onset of a stroke. Results: Twenty-one (70%) patients had a pontine lesion and 9 (30%) had a medullary lesion. Fourteen of these patients (46.7%) had bladder storage disorder, 7 patients (23.3%) had bladder emptying disorder, and 9 patients (30%) had a normal report. Five of the patients who had a medullary lesion (55.6%) had bladder emptying disorder, whereas only 2 patients who had a pontine lesion (9.5%) had bladder emptying disorder. Thirteen patients who had a pontine lesion (61.9%) showed bladder storage disorder. Discussion: The descending pathway from the midbrain tegmentum is inhibitory, and the pathway from the pontine tegmentum is stimulatory. Because of their location pontine lesions could disrupt the descending fibers of the midbrain tegmentum and medullary lesions could disrupt the descending fibers of the pontine tegmentum.

Introduction: Blood neutrophil/lymphocyte ratio (NLR) is a simple marker of subclinical inflammation that can be easily obtained. The NLR has recently emerged as a prognostic marker in patients with cancer and coronary artery disease. However, little is known about the role of NLR in patients with intracranial aneurysm (IA). Hypothesis: The purpose of this study was to determine the clinical significance of NLR in patients with IA. Methods: The study group consisted of patients with IA who had been admitted to the neurology department from January 2008 to December 2014. A total of 362 patients including 176 patients with unruptured IA, 186 patient with ruptured IA were enrolled in this study. The control group consisted of 178 age, sex-matched healthy adults who had not harbor IA by CTA, MRA, DSA. The baseline NLR was calculated as the ratio of neutrophil count to lymphocyte count. WBC count>12.000 cells per μL or 38 ° are excluded from the study. Results: The Mean NLR was significantly higher among persons with cerebral aneurysm compared with controls (P < 0.001). The level of NLR in ruptured IA group were found higher compared to unruptured IA and control group (7.0 ± 6.6 vs 2.5 ± 1.5 vs 1.6 ± 0.5, P < 0.001). Other comorbid conditions were balanced between these three groups. Conclusions: Higher NLR is associated with cerebral aneurysm. Unlike many other inflammatory markers and bioassays, NLR are inexpensive and readily available biomarkers that may be useful for risk stratification in patients with cerebral aneurysm.

Although some studies have supported the association between white matter hyperintensities (WMH) and cognitive impairment, whether WMH are associated with the impairments in instrumental activities of daily living (IADLs) remains unknown. This cross-sectional study investigated differences in basic ADLs and IADLs among different severity of WMH in a large, well-defined registry of patients with amnestic mild cognitive impairment (aMCI). 1,514 patients with aMCI were divided into three groups according to the degree of WMH (1,026 mild, 393 moderate, and 95 severe). We compared the total IADL scores and analyzed the prevalence of the impairment for each IADL item for each group. The severity of WMH was associated with the impairments in IADLs. Among 15 Seoul IADL items, "using public transportation", "going out (short distance)", "grooming" and "participating in leisure activities/hobbies" showed greater positive association with the severity of WMH. WMH in patients with aMCI were associated with the impairments in IADLs but not in basic ADLs. These findings are likely to be more obvious with respect to using public transportation, going out (short distances), shopping, grooming and participating in leisure activities/hobbies.

A new cytokine, interleukin-32 (IL-32), has been implicated in the pro-inflammatory immune responses in several autoimmune disorders, such as rheumatoid arthritis and inflammatory bowel diseases. Myasthenia gravis (MG) is a well-characterized autoimmune disease directed at the postsynaptic acetylcholine receptor (AChR) or end plate of the neuromuscular junction. IL-32 is a cytokine that induces tumor necrosis factor (TNF)-α, IL-6, IL-1β, and chemokine. IL-6, TNF-α, and IL-2 are related to the pathogenesis and immunoregulation of MG. The gene expression of IL-32 is increased in human natural killer (NK) cells and T lymphocytes when stimulated by IL-2 or mitogen. NK cells influence the development of experimental autoimmune MG (EAMG) and possibly MG. The aim of this study was to examine whether IL-32α levels are increased in patients with MG and to investigate the relationship between IL-32α levels and disease activity in human MG. Serum IL-32α levels were significantly higher in the MG patients (p = 0.03): 460.07 ± 192.30 pg/mL in MG patients and 248.45 ± 188.42 pg/mL in the healthy control group. Although there was no significant statistical difference, serum IL-32α levels of patients with both anti-AChR binding and blocking antibodies trended to be higher than those without either antibodies (521.56 ± 212.92 pg/mL vs. 339.52 ± 182.78 pg/mL, p = 0.16). IL-32α serum levels tended to decrease with clinical improvement in generalized MG. This study suggests the possibility that IL-32 might contribute to MG pathogenesis or immunoregulation.

An acute febrile disease, scrub typhus is caused by rickettsial nfection transferred by a bite of the chigger larvae infected with rientia tsutsugamushi. It is also known as tsutsugamushi disase and is characterized by focal or disseminated vasculitis and erivasculitis, which may involve the lung, heart, liver, spleen nd central nervous system [1–3]. The main pathologic change s focal or disseminated vasculitis caused by the destruction of ndothelial cells and the perivascular infiltration of leukocytes 1]. Scrub typhus occurs in a wide area of eastern Asia and he western pacific region [3]. It occurs in persons who ngage in occupational or recreational behavior that brings hem into contact with mite-infested habitats such as bush and rass. Scrub typhus can present with neurologic signs resembling septic meningitis or encephalitis syndrome. According to a study y Pai et al., twelve (48%) of 25 patientswith scrub typhus hadmild SF pleocytosis [7]. However, focal neurologic signs and abnormal euroradiologic findings in scrub typhus are rarely reported. We eport a case of scrub typhusmeningo-encephalitis with focal neuologic signs and associated neuroradiologic abnormalities along ith a review of the literature.

Acute motor and sensory axonal neuropathy (AMSAN) are recently described subtypes of Guillain-Barre syndrome characterized by acute onset of distal weakness, loss of deep tendon reflexes, and sensory symptoms. A 21-yr-old male was transferred to our hospital due to respiration difficulties and progressive weakness. In laboratory findings, immunoglobulin M antibodies against hepatitis A were detected in blood and cerebrospinal fluid. The findings of motor nerve conduction studies showed markedly reduced amplitudes of compound muscle action potentials in bilateral peroneal, and posterior tibial nerves, without evidence of demyelination. Based on clinical features, laboratory findings, and electrophysiologic investigation, the patient was diagnosed the AMSAN following acute hepatitis A viral infection. The patient was treated with intravenous immunoglobulin and recovered slowly. Clinicians should consider this rare but a serious case of AMSAN following acute hepatitis A infection.

Dermatomyositis (DM) is an idiopathic inflammatory myopathy with bimodal onset age distribution. The age of onset is between 5-18 yr in juvenile DM and 45-64 yr in adult DM. DM has a distinct clinical manifestation characterized by proximal muscle weakness, skin rash, extramuscular manifestations (joint contracture, dysphagia, cardiac disturbances, pulmonary symptoms, subcutaneous calcifications), and associated disorders (connective tissue disease, systemic autoimmune diseases, malignancy). The pathogenesis of juvenile and adult DM is presumably similar but there are important differences in some of the clinical manifestations, associated disorders, and outcomes. In this study, we investigated the clinical characteristics and outcomes of 16 patients with juvenile DM and 48 with adult DM. This study recognizes distinctive characteristics of juvenile DM such as higher frequency of neck muscle involvement, subcutaneous calcifications, and better outcomes.

We report on a 32-year-old woman who developed reversible cortical blindness and right-sided weakness after cesarean section at 36 weeks of gestation, due to preeclampsia. An initial brain MRI demonstrated high signal intensity lesions in the bilateral occipito-parietal and left frontal lobes on T2-weighted and diffusion-weighted imaging. All of the lesions showed low signal intensity on apparent diffusion coefficient (ADC) map, which were compatible with cytotoxic edema, and MR angiography (MRA) showed diffuse vasospasm of the intracranial vessels. A follow-up brain MRI showed that most of the lesions disappeared and the vasospasm also resolved. This case suggests that the cytotoxic edema in preeclampsia may evolve differently from the pattern in cerebral infarction and explains the relatively benign course of the neurological signs in preeclampsia.

Thunderclap headache (TCH) refers to a sudden-onset, severe headache that features in subarachnoid hemorrhage, unruptured intracranial aneurysm, cerebral venous thrombosis, pituitary apoplexy, cervical artery dissection, and hypertensive reversible posterior leukoencephalopathy. TCH is a rare manifestation in cerebral or cerebellar infarctions. Herein, we report on a 60-year-old woman with a thunderclap headache as the first symptom of cerebellar infarction, in the absence of abnormal findings in the brain computed tomography (CT), CT angiography, and lumbar puncture. An urgent brain MRI showed an acute infarction of the right cerebellar hemisphere. The next day, the patient presented with right side ataxia. In emergency cases presenting with thunderclap headache, one should consider an expanded evaluation and/or close observation, with frequent neurological examinations, even though the findings are normal on the initial neurological examination, cerebrospinal fluid analysis, and brain CT.

Prophylactic cranial irradiation (PCI) should now be considered as a part of the standard treatment of patients with small cell lung cancer (SCLC) in complete remission. The PCI has been offered in SCLC to reduce the incidence of brain metastasis and increase survival. The complications of PCI were reported brain necrosis, seizure or dementia. The complications were more frequent when chemotherapy was given at the time of cranial irradiation, or large radiation fraction size was employed. It is established that the pathophysiological reaction to irradiation in the normal brain tissue is necrosis, demyelinization, and diffuse changes due to wall thickening of the vascular structures. However, central pontine myelinolysis (CPM) of low dose irradiation like PCI is very rare. We report a patient with the classical syndrome of CPM following PCI for SCLC. The diagnosis was supported by typical features on magnetic resonance imaging.

Eosinophilic vasculitis has been described as part of Churg-Strauss syndrome (CSS), but it also affects the central nervous system in

Oculomotor nerve palsy frequently occurs because of external compression by an internal carotid-posterior communicating artery aneurysm and diabetes mellitus. In addition, pontine infarction, cavernous sinus tumors, demyelinating disease, and autoimmune disorder are well-known causes of oculomotor nerve palsy. However, cases of complete oculomotor nerve palsy by neurovascular conflicts presented with a sudden onset of clinical symptoms are extremely rare. We experienced a rare case of complete oculomotor nerve palsy because of direct vascular compression of the oculomotor nerve by the posterior cerebral artery.

Background and Purpose No previous studies have investigated the relationship between various anti-ganglioside antibodies and the clinical characteristics of Guillain-Barré syndrome (GBS) in Korea. The aim of this study was to determine the prevalence and types of anti-ganglioside antibodies in Korean GBS patients, and to identify their clinical significance. Methods Serum was collected from patients during the acute phase of GBS at 20 university-based hospitals in Korea. The clinical and laboratory findings were reviewed and compared with the detected types of anti-ganglioside antibody. Results Among 119 patients, 60 were positive for immunoglobulin G (IgG) or immunoglobulin M antibodies against any type of ganglioside (50%). The most frequent type was IgG anti-GM1 antibody (47%), followed by IgG anti-GT1a (38%), IgG anti-GD1a (25%), and IgG anti-GQ1b (8%) antibodies. Anti-GM1-antibody positivity was strongly correlated with the presence of preceding gastrointestinal infection, absence of sensory symptoms or signs, and absence of cranial nerve involvement. Patients with anti-GD1a antibody were younger, predominantly male, and had more facial nerve involvement than the antibody-negative group. Anti-GT1a-antibody positivity was more frequently associated with bulbar weakness and was highly associated with ophthalmoplegia when coupled with the coexisting anti-GQ1b antibody. Despite the presence of clinical features of acute motor axonal neuropathy (AMAN), 68% of anti-GM1- or anti-GD1a-antibody-positive cases of GBS were diagnosed with acute inflammatory demyelinating polyradiculoneuropathy (AIDP) by a single electrophysiological study. Conclusions Anti-ganglioside antibodies were frequently found in the serum of Korean GBS patients, and each antibody was correlated strongly with the various clinical manifestations. Nevertheless, without an anti-ganglioside antibody assay, in Korea AMAN is frequently misdiagnosed as AIDP by single electrophysiological studies.