• 대한유전성대사질환학회지
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• 제22권1호
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• pp.15-20
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• 2022

The most common urea cycle disorder is ornithine transcarbamylase deficiency. More than 80 percent of patients with symptomatic ornithine transcarbamylase deficiency are late-onset, which can present various phenotypes from infancy to adulthood. With no regards to the severity of the disease, characteristic fluctuating courses due to hyperammonemia may develop unexpectedly, and can be precipitated by various metabolic stressors. Late-onset ornithine transcarbamylase deficiency is not merely related to a type of genetic variation, but also to the complex relationship between genetic and environmental factors that result in hyperammonemia; therefore, it is difficult to predict the prevalence of neurological symptoms in late-onset ornithine transcarbamylase deficiency. Most common acute neurological manifestations include psychological changes, seizures, cerebral edema, and death; subacute neurological manifestations include developmental delays, learning disabilities, intellectual disabilities, attention-deficit/hyperactivity disorder, executive function deficits, and emotional and behavioral problems. This review aims to increase awareness of late-onset ornithine transcarbamylase deficiency, allowing for an efficient use of biochemical and genetic tests available for diagnosis, ultimately leading to earlier treatment of patients.

• Journal of Korean Neurosurgical Society
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• 제29권8호
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• pp.1126-1135
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• 2000

Objectives : The goal of surgical management of cerebral arteriovenous malformation(AVM) is elimination of the lesion without development of new neurological deficits. To improve the management results of cerebral AVMs in the future, this article discusses about surgical complications of the AVM and their management. Material and Methods : During the past 18 years, 116 patients with cerebral AVMs were managed by surgery. Among these cases, 7 cases died, 7 cases developed new neurological deficits, 11 cases residual AVM and 5 cases intracerebral hematoma(ICH) after surgery. The author analyzes the causes of those complications and investigates the methods to minimized those complications based on the review of the literatures. Results : One stage removal of AVM and ICH in the poor neurological state were performed in 5 of 7 death cases. Subtotal removal of ICH followed by delayed AVM surgery after recovery is regard as one method to improve the outcome of patient with large ICH. Postoperative new neurological deficits developed owing to normal perfusion pressure breakthrough(NPPB) in 3, judgement error in 2, preoperative embolization in 1 and cortical injury in 1 case(s). Proper management of NPPB, accurate anatomical knowledge and physiological monitoring during operation, and well trained skill for embolization are regard as methods to minimize those complications. Residual AVMs after surgery were noticed in 11 cases, in which unintended 6 cases due to inaccurate dissection of peripheral margin of AVM, and intended 3 cases due to massive brain swelling during operation, 1 cases due to diffuse type and 1 case due to multiple type of AVM. Accurate dissection of peripheral margin of AVM and mild hypotension during operation may help to avoid this complication. Postoperative hemorrhage occurred in 3 cases due to rupture of the residual AVM and in 2 cases due to oozing from the AVM bed. Complete resection of AVM, complete control of bleeding points at AVM bed and mild hypotension during early postoperative period are the methods to avoid this complication. Conclusion : A precise but flexible therapeutic strategy and refined skill for endovascular, radiosurgical and microsurgical techniques are required to successful treatment of cerebral AVM. Adequate timing of AVM resection, accurate anatomical knowledge, proper management of NPPB and accurate dissection of peripheral margin of AVM are the key points for avoiding complications of the AVM surgery.

• Journal of Korean Neurosurgical Society
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• 제61권5호
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• pp.548-558
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• 2018

Objective : Diagnosing acute cerebral infarction is crucial in determining prognosis of stroke patients. Although many serologic tests for prompt diagnosis are available, the clinical application of serologic tests is currently limited. We investigated whether $S100{\beta}$, matrix metalloproteinase-9 (MMP-9), D-dimer, and heat shock protein 70 (HSP70) can be used as biomarkers for acute cerebral infarction. Methods : Focal cerebral ischemia was induced using the modified intraluminal filament technique. Mice were randomly assigned to 30-minute occlusion (n=10), 60-minute occlusion (n=10), or sham (n=5) groups. Four hours later, neurological deficits were evaluated and blood samples were obtained. Infarction volumes were calculated and plasma $S100{\beta}$, MMP-9, D-dimer, and HSP70 levels were measured using enzyme-linked immunosorbent assay. Results : The average infarction volume was $12.32{\pm}2.31mm^3$ and $46.9{\pm}7.43mm^3$ in the 30- and 60-minute groups, respectively. The mean neurological score in the two ischemic groups was $1.6{\pm}0.55$ and $3.2{\pm}0.70$, respectively. $S100{\beta}$, MMP-9, and HSP70 expressions significantly increased after 4 hours of ischemia (p=0.001). Furthermore, $S100{\beta}$ and MMP-9 expressions correlated with infarction volumes (p<0.001) and neurological deficits (p<0.001). There was no significant difference in D-dimer expression between groups (p=0.843). The area under the receiver operating characteristic curve (AUC) showed high sensitivity and specificity for MMP-9, HSP70 (AUC=1), and $S100{\beta}$ (AUC=0.98). Conclusion : $S100{\beta}$, MMP-9, and HSP70 can complement current diagnostic tools to assess cerebral infarction, suggesting their use as potential biomarkers for acute cerebral infarction.

• Biomolecules & Therapeutics
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• 제25권3호
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• pp.272-278
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• 2017

Fucoidan has been reported to exhibit various beneficial activities ranging from to antivirus and anticancer properties. However, little information is available about the effects of fucoidan on cerebral ischemia-reperfusion injury (IRI). Our study aimed to explore the effects of fucoidan on cerebral IRI, as well as the underlying mechanisms. Sprague-Dawley (SD) rats were randomly subjected to four groups: Sham, IRI+saline (IRI+S), IRI+80 mg/kg fucoidan (IRI+F80), and IRI+160 mg/kg fucoidan (IRI+F160). Fucoidan (80 mg/kg or 160 mg/kg) was intraperitoneally injected from 7 days before the rats were induced to cerebral IRI model with middle cerebral artery occlusion (MCAO) method. At 24 h after reperfusion, neurological deficits and the total infarct volume were determined. The levels of inflammation-associated cytokines (interleukin (IL)-$1{\beta}$, IL-6, myeloperoxidase (MPO), and tumor necrosis factor (TNF)-${\alpha}$), oxidative stress-related proteins (malondialdehyde (MDA) and superoxide dismutase (SOD)) in the ischemic brain were measured by enzyme-linked immunosorbent assay (ELISA). Besides, the levels of apoptosis-related proteins (p-53, Bax, and B-cell lymphoma (Bcl)-2) and mitogen-activated protein kinase (MAPK) pathway (phosphorylation-extracellular signal-regulated kinase (p-ERK), p-c-Jun N-terminal kinase (JNK), and p-p38) were measured. Results showed that administration of fucoidan significantly reduced the neurological deficits and infarct volume compared to the IRI+S group in a dose-dependent manner. Also, fucoidan statistically decreased the levels of inflammation-associated cytokines, and oxidative stress-related proteins, inhibited apoptosis, and suppressed the MAPK pathway. So, Fucoidan plays a protective role in cerebral IRI might be by inhibition of MAPK pathway.

• Journal of Korean Neurosurgical Society
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• 제49권6호
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• pp.329-333
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• 2011

Objective : As medical advances have increased life expectancy, it has become imperative to develop specific treatment strategies for intracranial aneurysms in the elderly. We therefore analyzed the clinical characteristics and outcomes of the treatment of unruptured intracranial aneurysms in patients older than 70 years. Methods : We retrospectively reviewed the medical records and results of neuroimaging modalities on 54 aneurysms of 48 consecutive patients with un ruptured intracranial aneurysms. ($mean{\pm}SD$ age, $72.11{\pm}1.96$ years; range, 70-78 years) who underwent surgical clipping over 10 years (May 1999 to June 2010). Results : Of the 54 aneurysms, 22 were located in the internal carotid artery, 19 in the middle cerebral artery, 12 in the anterior cerebral artery, and 1 in the superior cerebellar artery. Six patients had multiple aneurysms. Aneurysm size ranged from 3 mm to 17 mm ($mean{\pm}SD$, $6.82{\pm}3.07$ mm). Fifty of the 54 aneurysms (92.6%) were completely clipped. Three-month outcomes were excellent in 50 (92.6%) aneurysms and good and poor in 2 each (3.7%), with 1 death (2.0%). Procedure-related complications occurred in 7 aneurysms (13.0%), with 2 (3.7%) resulting in permanent neurological deficits, including death. No postoperative subarachnoid hemorrhage occurred during follow-up. The cumulative rates of stroke- or death-free survival at 5 and 10 years were 100% and 78%, respectively. Conclusion : Surgical clipping of unruptured intracranial aneurysms in elderly group could get it as a favorable outcome in well selected cases.

• 동의생리병리학회지
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• 제20권2호
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• pp.498-502
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• 2006

Neurological impairment produces cognitive, communicational, physical, and social deficits. Music has the power to help stroke patients to regain speech and overcome other deficits. Rhythm and melody help to rehabilitate memory, muscles, breathing, etc. This article introduces how music therapy approaches stroke patients and helps them. It focuses particularly on speech; however, music affects not only one part of the body but the whole body. In cases in which music therapy is used, we can see how music helps with stroke patients and how to achieve these goals.

• Journal of Korean Neurosurgical Society
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• 제47권2호
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• pp.102-106
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• 2010

Objective : To evaluate the clinical results of gross total resection in the surgical approach to spinal ependymoma. Methods : Between June 1995 and May 2009,13 males and 8 females (mean age 34) diagnosed with intramedullary or extramedullary spinal ependymoma were surgically treated at our centre. The neurological and functional state of each patient were evaluated according to the modified McCormick scale. Results : The average follow-up duration was 54 months (ranging from 12 to 168 months). The locations of the lesions were : thoracic region (4, 19%), lumbar region (7, 34%), cervical region (4, 19%), cervicothoracic region (3, 14%) and conus medullaris (3, 14%). Four patients (19%) had deterioration of neurological function in the early postoperative period. The neurological function of three patients was completely recovered at the 6th postoperative month, while that of another patient was recovered at the 14th month. In the last assessment of neurological function, 20 patients (95%) were assessed as McCormick grade 1. No perioperative complications developed in any of our patients. In one patient's 24-month assessment, tumour recurrence was observed. Re-operation was not performed and the patient was taken under observation. Conclusion : Two determinants of good clinical results after spinal ependymoma surgery are a gross total resection of the tumour and a good neurological condition before the operation. Although neurological deficits in the early postoperative period can develop as a result of gross total tumour resection, significant improvement is observed six months after the operation.

• Journal of Korean Neurosurgical Society
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• 제37권3호
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• pp.241-243
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• 2005

We report a case of recurrent dermatofibrosarcoma in a 30-years-old woman who had undergone operations three times during 60 months and had received post-operative radiotherapy. On neurological examination, no neurological deficits were noticed. In brain magnetic resonance image(MRI), there was right parieto-occipital scalp mass with high signal in T2-weighted image, low signal in T1-weighted image with homogeneous enhancement. The removal was done including about 2cm uninvolved margins and pathologic examination of the lesion revealed dermatofibrosarcoma protuberans(DFSP). The prognostic factors of local recurrence may be related to surgical margins for resection; the length from the grossly intact margins, and the microscopically controlled excision in margins.

• Journal of Korean Neurosurgical Society
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• 제51권3호
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• pp.164-166
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• 2012

Penetrating injuries to the upper cervical spine resulting from gunshots are rare in South Korea due to restrictions of gun use. Moreover, gunshot wounds to the upper cervical spine without neurological deficits occur infrequently because of the anatomic location and surrounding essential structures. We present an uncommon case involving the surgical removal of a bullet located in the anterior arch of first cervical vertebra (C1) via a transoral approach without neurological complications or subsequent mechanical instability.

• Journal of Korean Neurosurgical Society
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• 제42권5호
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• pp.406-409
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• 2007

Craniovertebral junction (CVJ) tuberculosis is a rare disease, potentially causing severe instability and neurological deficits. The authors present a case of CVJ tuberculosis with atlantoaxial dislocation and retropharyngeal abscess in a 28-year-old man with neck pain and quadriparesis. Radiological evaluations showed a widespread extradural lesion around the clivus, C1, and C2. Two stage operations with transoral decompression and posterior occipitocervical fusion were performed. The pathological findings confirmed the diagnosis of tuberculosis. Treatment options in CVJ tuberculosis are controversial without well-defined guidelines. But radical operation (anterior decompression and posterior fusion and fixation) is necessary in patient with neurological deficit due to cord compression, extensive bone destruction, and instability or dislocation. The diagnosis and treatment options are discussed.