• Asian Pacific Journal of Cancer Prevention
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• v.17 no.3
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• pp.1565-1570
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• 2016

Background: Central neurocytomas are rare neuronal neoplasms with a favorable prognosis. They are typically located in the lateral ventricles of the brain and mostly histologically correspond to WHO grade II with a Mib 1 labelling index of <2%. Similar tumors located in the cerebral hemispheres and spinal cord, for example, are called "extraventricular neurocytomas". A few tumors histologically show atypia, mitoses, vascular proliferation and/or necrosis and a Mib 1 index >2 % and are designated as "atypical neurocytomas. Aim: The aim of our study was to describe the common as well as unusual morphologic features and the role of various immunohistochemical stains in the diagnosis of these rare tumors. Materials and Methods: We retrieved and reviewed 35 cases diagnosed between 2001 and 2015. Results: Sixty percent of patients were males, and the mean age was 26 years. 31 cases (88.6%) were intraventricular and 4(11.4%) were extraventricular. Histologically, 6 cases (17.1%) were compatible with "atypical neurocytomas". All cases showed the classic morphology comprising nests and sheets of uniform, round cells with uniform round to oval nuclei with finely speckled chromatin and perinuclear cytoplasmic clearing (halos). All cases also showed delicate, fibrillary, neuropil-like matrices. Other common histologic features included capillary-sized blood vessels in a branching pattern in 57.1%, foci of calcification in 34.3% and perivascular pseudorosettes in 20%. Rare findings included Homer-Wright or true rosettes in 8.6% and ganglioid cells in 2.9%. Synaptophysin was the most consistent and valuable marker, being positive in almost all cases. GFAP positivity in tumor cells was seen in 25.7% of cases. Follow up was available in 13 patients. Of these 9 had histologically typical and 4 had atypical tumors. Only 1 (with an atypical neurocytoma) died, probably due to complications of surgery within one month, while 12 (including 3 with atypical neurocytomas) remained alive. Recurrence developed in 1 of these 12 patients (histologically consistent with typical morphology) almost 9 years after surgery. Only 4 patients, including 2 with atypical tumors, received postoperative radiotherapy, all with surgery in 2010 or later. Overall, prognosis was excellent with prolonged, recurrence free survival and most patients, even without receiving radiation therapy, were alive and well for many years, even a decade or more after surgery, without developing any recurrence, indicating the benign nature of these neoplasms.

• Journal of Microbiology and Biotechnology
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• v.27 no.11
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• pp.2044-2051
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• 2017

The main pathological hallmark of Alzheimer's disease is the deposition of amyloid-beta ($A{\beta}$) peptides in the brain. $A{\beta}$ has been widely used to mimic several aspects of Alzheimer's disease. However, several characteristics of amyloid-induced Alzheimer's disease pathology are not well established, especially in mice. The present study aimed to develop a new Alzheimer's disease model by investigating how $A{\beta}$ can be effectively aggregated using prokaryotes and eukaryotes. To express the $A{\beta}42$ complex in HEK293 cells, we cloned the $A{\beta}42$ region in a tandem repeat and incorporated the resulting construct into a eukaryotic expression vector. Following transfection into HEK293 cells via lipofection, cell viability assay and western blotting analysis revealed that exogenous $A{\beta}42$ can induce cell death and apoptosis. In addition, recombinant His-tagged $A{\beta}42$ was successfully expressed in Escherichia coli BL21 (DE3) and not only readily formed $A{\beta}$ complexes, but also inhibited the proliferation of SH-SY5Y cells and E. coli. For in vivo testing, recombinant His-tagged $A{\beta}42$ solution ($3{\mu}g/{\mu}l$ in $1{\times}PBS$ containing $1mM\;Ni^{2+}$) was injected stereotaxically into the left and right lateral ventricles of the brains of C57BL/6J mice (n = 8). Control mice were injected with $1{\times}PBS$ containing $1mM\;Ni^{2+}$ following the same procedure. Ten days after the sample injection, the Morris water maze test confirmed that exogenous $A{\beta}$ caused an increase in memory loss. These findings demonstrated that $Ni^{2+}$ is capable of complexing the 50-kDa amyloid and that intracerebroventricular injection of $A{\beta}42$ can lead to cognitive impairment, thereby providing improved Alzheimer's disease models.

• Journal of Biomedical Engineering Research
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• v.25 no.5
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• pp.323-328
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• 2004

The development of group-specific tissue probability maps (TPM) provides a priori knowledge for better result of cerebral tissue classification with regard to the inter-ethnic differences of inter-subject variability. We present sequential procedures of group-specific TPM and evaluate the age effects in the structural differences of TPM. We investigated 100 healthy volunteers with high resolution MRI scalming. The subjects were classified into young (60, 25.92+4.58) and old groups (40, 58.83${\pm}$8.10) according to the age. To avoid any bias from random selected single subject and improve registration robustness, average atlas as target for TPM was constructed from skull-stripped whole data using linear and nonlinear registration of AIR. Each subject was segmented into binary images of gray matter, white matter, and cerebrospinal fluid using fuzzy clustering and normalized into the space of average atlas. The probability images were the means of these binary images, and contained values in the range of zero to one. A TPM of a given tissue is a spatial probability distribution representing a certain subject population. In the spatial distribution of tissue probability according to the threshold of probability, the old group exhibited enlarged ventricles and overall GM atrophy as age-specific changes, compared to the young group. Our results are generally consistent with the few published studies on age differences in the brain morphology. The more similar the morphology of the subject is to the average of the population represented by the TPM, the better the entire classification procedure should work. Therefore, we suggest that group-specific TPM should be used as a priori information for the cerebral tissue classification.

• Journal of Life Science
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• v.15 no.4 s.71
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• pp.664-667
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• 2005

Hydrosyringomyelia is a dilation of the spinal cord central canal. In human it may be caused by congenital malformations such as Dandy-Walker syndrome and Chiari malformations or may be acquired as a result of infection, trauma or neoplasia. Hydrocephalus is an excessive accumulation of cerebrospinal fluid within the ventricles and occipital dysplasia is the dorsal extension of the foramen magnum. Hydrosyringomyelia and hydrocephalus can be confirmed by computed tomography or magnetic resonance imaging (MRI). A 3-year-old male maltese was presented with a history of long-term seizure. Blood examination was all unremarkable. On rostrodorsal-caudoventral oblique radiograph of the skull showed severe occipital dysplasia. On brain sonography through the persistent fontanelle, severe lateral ventriculomegaly was revealed. MRI examination revealed hydrocephalus and hydrosyringomyelia. Diuretic therapy didn't reduce clinical symptoms and surgical decompression was conducted. The dog responded well with ventriculo-peritoneal shunting. MRI is the most superior modality to diagnose hydrocephalus and hydrosyringomyelia, to plan therapy and to determine the prognosis.

• Korean Journal of Psychosomatic Medicine
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• v.23 no.1
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• pp.66-69
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• 2015

In addition to classical triad such as gait disturbance, urinary incontinence and dementia, parkinsonian extrapyramidal motor signs and neuropsychiatric symptoms can be observed in patients with normal pressure hydrocephalus (NPH). In our case, a 46 year old female patient showed extrapyramidal symptoms such as bradykinesia, rigidity and neuropsychiatric symptoms such as agitation, anxiety, restlessness and regressed behavior beside two(gait disturbance & urinary incontinence) symptoms of three classical triad. It was difficult to diagnose this patient as NPH from the beginning because of her relatively young age and previous psychiatric mediation history for controlling advanced anxiety and affective disorder. Antiparkinsonian agents and discontinuation of psychiatric medications did not work for this patient. Patient's brain computed tomographic finding showed enlarged ventricles. We suspected NPH and did empirical drainage of 30mL CSF. Finally, patient's pyramidal and neuropsychiatric symptoms as well as two of three classical triad of NPH were improved dramatically within several days. It is important to consider NPH as one of the differential diagnosis in patient with parkinsonian symptoms and various neuropsychiatric symptoms who did not respond to usual clinical management especially in case of ventricular enlargement in neuroimaging because of its treatable property by CSF shunt operation.

• Pediatric Infection and Vaccine
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• v.25 no.3
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• pp.113-122
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• 2018

Purpose: The aim of this study was to evaluate the clinical characteristics of children diagnosed as cryopyrin-associated periodic syndrome (CAPS) in Korea. Methods: Diagnosis was made based on clinical features and confirmed by a mutation in the cold-induced autoinflammatory syndrome 1 (CIAS1) gene. Especially, osteocartilaginous overgrowth in the patella or distal femur was so characteristic that its presence warranted a diagnosis of chronic infantile neurologic cutaneous and articular/NOMID. Results: We observed the clinical features of 9 Korean CAPS patients. All the patients suffered from an urticarial rash with recurrent fever. Among the 9 patients, 6 presented with rash and 4 with fever on the 1st or 2nd days of birth. Eight patients showed myalgia, and 7 patients showed arthralgia in the joints, and 6 patients showed radiologic findings of arthropathy including cupping of the metaphysis, excessive growth of the epiphysis, osteopenia or overgrowth of the cartilage. Four patients showed brain atrophy, enlarged ventricles or leptomeningeal enhancement on magnetic resonance imaging. Intellectual disability was observed in 1 patient. Five patients had eye involvement as conjunctivitis, uveitis, chorioretinitis, avascular area or papillary edema, and 3 patients showed progressive hearing loss. All 9 patients showed increased C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR). Conclusions: All the patients carried a mutation on exon 3 of the CIAS1 gene. After the anakinra (interleukin-1 receptor antagonist) therapy, the fever and rash immediately disappeared, and CRP and ESR were improved.

• Clinical and Experimental Pediatrics
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• v.52 no.4
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• pp.458-463
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• 2009

Purpose : Schizencephaly is a uncommon congenital brain anomaly characterized by congenital clefts spanning the cerebral hemispheres from pial surface to lateral ventricles and lined by gray matter. In this study, we investigated the clinical manifestation and radiologic findings of pediatric schizencephaly. Methods : The data of 13 patients who were diagnosed with schizencephaly in Severance Childrens Hospital and Yongdong Severance Hospital from January 2005 to December 2007 were analyzed retrospectively. Results : The mean age at diagnosis was $9.08{\pm}2.67$ months old and ranged from 1 to 30 months. The ratio of male to female patients was 3.33:1. Five (38.5%) patients had bilateral clefts, while 8 (61.5%) had unilateral clefts. Five (38.5%) patients had closed lip clefts, and 4 (30.8%) had opened lip clefts. Four (30.8%) patients had multiple clefts. Associated anomalies showed in all cases. The clinical features consisted of mild unilateral weakness in 7 (53.8%) cases and a hemiparesis was present in 3 (23.1%) patients. A tetraparesis was in 3 (23.1%) patients. There was no difference in motor deficit between unilateral and bilateral clefts. Delayed development was observed in all cases. Epilepsy was present in 7 (53.8%) patients, 5 patients with unilateral clefts and 2 patients with bilateral clefts. Three (42.8%) patients showed intractable seizures. Conclusion : Schizencephaly showed variable clinical manifestations and radiologic findings in association with the types and locations of the clefts. It is necessary to diagnose schizencephaly early and to detect the development of epilepsy. Intensive and large studies of the correlation of clinical outcomes and radiologic findings should be continued for more effective treatment.

• Clinical and Experimental Pediatrics
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• v.48 no.11
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• pp.1225-1231
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• 2005

Purpose : This study compares the first epileptic seizures between preterm and term-born children with periventricular leukomalacia and epilepsy. Methods : From 108 cases having lesions of high signal intensity around the ventricles in T2 weighted imaging of a brain magnetic resonance study, we selected 37 cases that showed epileptic seizures two times or more and divided them into the group of preterm-born(27 cases) and term-born children(10 cases). A retrospective study was made by comparing the two groups with regard to age, type of the first epileptic seizures, EEG findings and responsiveness to anticonvulsants. Results : The age of the first epileptic seizure was $22.2{\pm}18.3$ months in the preterm-born group and $26.9{\pm}21.1$ months in the term-born group(P=0.505). As for the first epileptic seizure, 11 out of the 27 cases in the preterm-born group had infantile spasms. Out of the 10 cases in the term-born group, 7 had complex partial seizures. In the preterm group, hypsarrhythmias were found in 11 cases, focal epileptiform discharges in 6 cases. In term-born group, focal epileptiform discharges were found in 5 cases but no epileptiform discharge was found in 3 cases. Intractable epilepsies were diagnosed in 6 cases and all of them belonged to the preterm-born group. Conclusion : More severe epilepsies such as infantile spasm and intractable epilepsies seem to be more common in preterm-born epileptic children with PVL as well as more severely abnormal EEG finding compared to term-born epileptic children.

• Tuberculosis and Respiratory Diseases
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• v.59 no.6
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• pp.656-663
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• 2005

Background : The B-type natriuretic peptide (BNP) is a cardiac neurohormone that is specifically secreted from the ventricles in response to volume expansion and a pressure overload. Differentiating congestive heart failure from the pulmonary causes of dyspnea is very important for patients presenting with acute dyspnea. Methods : A retrospective study was carried out on 261 patients who were admitted to the emergency department of Hanyang University Hospital due to acute dyspnea from March to July 2004. The serum BNP levels of the patients were measured using the ELISA method. Results : The BNP levels were 382, 111-1140 pg/ml (median, interquartile range) in the heart failure group (n=119) and 29, 7-81 pg/ml in the non-heart failure group (n=142). The BNP levels according to the subgroups of heart failure were 820, 354-1620 pg/ml, 1650, 239-1990 pg/ml, and 378, 106-1120 pg/ml for the chronic obstructive pulmonary disease (COPD) with combined left heart failure (n=5), cor pulmonale (n=3), and left heart failure groups (n=111), respectively. The BNP levels according to the subgroups of non-heart failure were 39, 21-101 pg/ml, 59, 10-129 pg/ml, and 15, 6-47 pg/ml for the COPD (n=20), other pulmonary diseases (n=56), and other causes groups (n=66), respectively. The BNP levels were significantly different according to the underlying etiology (p<0.001), and were significantly higher in the COPD patients with left heart failure than in those without (p=0.002). When the patients with no cardiovascular risk factor such as diabetes mellitus, hypertension, smoking or renal failure were analyzed, the BNP levels were also significantly higher in the patients with heart failure than in those without(p<0.001). When 133 pg/ml was designated as the BNP cut-off level the sensitivity for predicting heart failure was 73 percent and the specificity was 87 percent. Conclusion : BNP measurements are useful for determining the presence of heart failure in the patients presenting with acute dyspnea. The possibility of heart failure must be seriously considered in patients with high BNP levels even when they have a pre-existing pulmonary disease such as COPD or no risk factors for heart failure.