• Clinical and Experimental Pediatrics
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• v.49 no.12
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• pp.1358-1362
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• 2006

Niemann-Pick disease is a group of autosomal recessive disorders associated with hepatosplenomegaly, variable neurologic deficits, and the storage of sphingomyelin and other lipids. Seven cases have been reported in Korea. We report an additional case presenting with hypotonia, early neurodevelopmental delay, hepatosplenomegaly and death by persistent pneumonia and asphyxia at the age of 23 months. MRI of brain and fundoscopic findings of our case at 4 months of age were normal. However, abnormal intensity of the thalamus and atrophy of the right temporal lobe on the MRI and macular cherry red spots were noticed at the age of 17 months. A bone marrow biopsy showed large foamy cells, while hexosaminidase A and B levels were normal. Although biochemical or molecular workup was not done, these findings led to the diagnosis of infantile onset Niemann-Pick disease, probably type A. A brief review of the related literatures was made.

• Journal of Korean Neurosurgical Society
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• v.29 no.5
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• pp.615-622
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• 2000

Objective : Percutaneous vertebroplasty is an effective and minimally invasive procedure consisting of the injection of a PMMA(polymethyl methacrylate) into the vertebral body compression fracture with osteoporosis. Matherials and Methods : Twenty-eight procedures were performed for vertebral body compression fractures with osteoporosis in 25 patients(22 women, 3 men). The mean age was 65.9 years old. The inclusion criteria for percutaneous vertebroplasty were 1) acute vertebral body compression fracture with osteoporosis, 2) expected high operative morbidity in old age, 3) no neurologic deficits, 4) no or minimal canal enchroachment, 5) patient refusal of invasive surgery. All patients underwent MR images before the procedure. Under local anesthesia, after the percutaneous needle puncture of the involved vertebra via a transpedicular approach and venography using the water soluble contrast material, PMMA injection was introduced into the fractured vertebral body. Results : The procedure was technically successful in all patients. All patients experienced excellent pain relief (complete pain relief ; 10, marked pain relief ; 14). One patient experienced marked pain relief, however, the patient died during the follow-up period due to stomach cancer. There were twelve paravertebral tissue leaks, twelve paravertebral venous plexus leaks, four epidural leaks and one intradiskal leak, but no clinically significant complications occurred in all patients. Conclusion : Percutaneous vertebraoplasty is a valuable procedure in the treatment of vertebral body compression fracture with osteoporosis, providing immediate pain relief and early mobilization. MRI is the most reliable diagnostic tool for identifying painful fractured vertebral body.

• Journal of Korean Neurosurgical Society
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• v.41 no.1
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• pp.7-10
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• 2007

Objective : The aim of this study was to determine the efficacy of percutaneous radiofrequency neurotomy[RFN] of ramus communicans nerve in patients suffering from severe low back pain due to osteoporotic compression fracture. Methods : Twenty two patients with lumbar osteoporotic compression fracture who had intractable back pain for less than two weeks and were performed with RFN at L1-L4 from May 2004 to December 2005 were retrospectively analyzed. Clinical outcome using visual analogue scale[VAS] pain scores and modified MacNab's grade was tabulated. Complications related to the procedure were assessed. Results : Twenty-two female patients [age from 63 to 81 years old] were included in this study. The mean VAS score prior to RFN was 7.8, it improved to 2.6 within postoperative time of 48 hours, and the mean VAS score after 3 months was 2.8, which was significantly decreased. Eighteen of 22 patients were graded as excellent and good according to modified MacNab's criteria at final follow up. All patients recovered uneventfully, and the neurologic examination revealed no deficits. Two patients showing poor results worsened in symptom. Percutaneous was performed eventually resulting in symptom improvement. There were no significant complications related to the procedure such as sensory dysesthesia, numbness or permanent motor weakness. Conclusion : RFN is safe and effective in treating the painful osteoporotic compression fracture. in patients with intractable back pain due to lumbar osteoporotic compression fracture, RFN of gray ramus communicans nerve should be considered as a treatment option prior to vertebroplasty.

• Nutrition Research and Practice
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• v.4 no.3
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• pp.196-202
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• 2010

To investigate the neuroprotective effects of bovine colostrums (BC), we evaluate the ability of consuming BC after focal brain ischemia/reperfusion injury rat model to reduce serum cytokine levels and infarct volume, and improve neurological outcome. Sprague-Dawley rats were randomly divided into 4 groups; one sham operation and three experimental groups. In the experimental groups, MCA occlusion (2 h) and subsequent reperfusion (O/R) were induced with regional cerebral blood flow monitoring. One hour after MCAO/R and once daily during the experiment, the experimental group received BC while the other groups received 0.9% saline or low fat milk (LFM) orally. Seven days later, serum pro-inflammatory cytokine (IL-$1{\beta}$, IL-6, and TNF-${\alpha}$) and anti-inflammatory cytokine (IL-10) levels were assessed. Also, the infarct volume was assessed by using a computerized image analysis system. Behavioral function was also assessed using a modified neurologic severity score and corner turn test during the experiment. Rats receiving BC after focal brain I/R showed a significant reduction (-26%/-22%) in infarct volume compared to LFM/saline rats, respectively (P < 0.05). Serum IL-$1{\beta}$, IL-6, and TNF-${\alpha}$ levels were decreased significantly in rats receiving BC compared to LFM/saline rats (P < 0.05). In behavioral tests, daily BC intake showed consistent and significant improvement of neurological deficits for 7 days after MCAO/R. BC ingestion after focal brain ischemia/reperfusion injury may prevent brain injury by reducing serum pro-inflammatory cytokine levels and brain infarct volume in a rat model.

• Journal of Korean Neurosurgical Society
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• v.63 no.2
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• pp.248-260
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• 2020

Objective : To investigate the efficiency of diffusion tensor imaging (DTI) fiber-tracking based neuronavigation and assess its usefulness in the preoperative surgical planning, prognostic prediction, intraoperative course and outcome improvement. Methods : Seventeen patients with cerebral masses adjacent to corticospinal tract (CST) were given standard magnetic resonance imaging and DTI examination. By incorporation of DTI data, the relation between tumor and adjacent white matter tracts was reconstructed and assessed in the neuronavigation system. Distance from tumor border to CST was measured. Results : The sub-portion of CST in closest proximity to tumor was found displaced in all patients. The chief disruptive changes were classified as follows : complete interruption, partial interruption, or simple displacement. Partial interruption was evident in seven patients (41.2%) whose lesions were close to cortex. In the other 10 patients (58.8%), delineated CSTs were intact but distorted. No complete CST interruption was identified. Overall, the mean distance from resection border to CST was 6.12 mm (range, 0-21), as opposed to 8.18 mm (range, 2-21) with simple displacement and 2.33 mm (range, 0-5) with partial interruption. The clinical outcomes were analyzed in groups stratified by intervening distances (close, <5 mm; moderated, 5-10 mm; far, >10 mm). For the primary brain tumor patients, the proportion of completely resected tumors increased progressively from close to far grouping (42.9%, 50%, and 100%, respectively). Five patients out of seven (71.4%) experienced new neurologic deficits postoperatively in the close group. At meantime, motor deterioration was found in six cases in the close group. All patients in the far and moderate groups received excellent (modified Rankin Scale [mRS] score, 0-1) or good (mRS score, 2-3) rankings, but only 57.1% of patients in the close group earned good outcome scores. Conclusion : DTI fiber tracking based neuronavigation has merit in assessing the relation between lesions and adjacent white matter tracts, allowing prediction of patient outcomes based on lesion-CST distance. It has also proven beneficial in formulating surgical strategies.

• Journal of Korean Neurosurgical Society
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• v.37 no.4
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• pp.275-281
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• 2005

Objective: The prolactinoma is the most common pituitary tumor and sometimes shows severe invasiveness to the adjacent cavernous sinus, especially in the male patient. The dopamine agonist can be used as an alternative treatment modality to surgery. But, the transsphenoidal or transcranial approach for tumor removal has been more preferred treatment option of neurosurgeons in invasive prolactinoma. Especially rapid decompression of mass effect and resolution of the neurologic deficit is demanded. The prospective study is done in order to identify the therapeutic efficacy of bromocriptine as an initial treatment option for the invasive prolactionomas. Methods: Twenty patients with invasive prolactinoma were studied. Preoperative neurological and endocrinological evaluations were done, and size and invasiveness of the tumor was estimated on MRI. Bromocriptine was administrated by increasing dose planning reaching maximum dose at 1 month of treatment, with close neurological and endocrinological monitoring. At 3months after treatment, MRI was taken and decision was made whether to continue bromocriptine or to have surgical intervention. Results: Thirteen patients showed excellent result with only bromocriptine treatment. These patients showed not only marked reduction of tumor volume and prolactin level, but also, improving clinical symptoms and other hormonal deficits. 13patients who had visual field defect and decreased visual acuity had all improved visual symptoms. But, the remaining 4patients required surgical treatment due to insufficient reduction of tumor size inspite of normalized prolactin level within 3months. Remaining 2patients had $20{\sim}30%$ of tumor size reduction, but prolactin level was not normalized. One patient required radiation therapy. Conclusion: Bromociptine can be used as initial treatment for the invasive prolactinomas with careful monitoring of the neurological and endocrinological status. It should be carefully followed up for tumor size reduction within 3 months after initiation of treatment.

• Annals of Clinical Neurophysiology
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• v.4 no.1
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• pp.70-73
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• 2002

Acute autonomic neuropathy is a rare disease. Since the first case was reported by Young et.al., in 1969, a number of similar cases have been described, with some variation of the accompanied neurologic deficits. Acute autonomic and sensory neuropathy(AASN) is characterized by the acute onset of autonomic dysfunction and sensory disturbances. A 16-year-old girl experienced high fever($40^{\circ}C$) and erythematous rash on whole trunk and face followed by pain and sensory loss over the whole body, dysphagia, ataxia, urinary retention, and postural hypotension. There was no evidence of limb weakness. The electrophysiologic studies of this patient revealed sensory polyneuropathy and the various autonomic function test showed autonomic dysfunction. The recovery of her autonomic and sensory symptoms is incomplete, three months after the onset of the symptoms. The etiology of the acute autonomic and sensory neuropathy is not known. Most previous authors have suggested the dysautonomia may be an acute immunological damage to peripheral fibers of the autonomic nervous system. We report a case of acute autonomic and sensory neuropathy.

• Journal of Korean Neurosurgical Society
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• v.53 no.5
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• pp.281-287
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• 2013

Objective : Pleomorphic xanthoastrocytoma (PXA) is a rare primary low-grade astrocytic tumor classified as WHO II. It is generally benign, but disease progression and malignant transformation have been reported. Prognostic factors for PXA and optimal therapies are not well known. Methods : The study period was January 2000 to March 2012. Data on MR findings, histology, surgical extents and adjuvant therapies were reviewed in twenty-two patients diagnosed with PXA. Results : The frequent symptoms of PXA included seizures, headaches and neurologic deficits. Tumors were most common in the temporal lobe followed by frontal, parietal and occipital lobes. One patient who died from immediate post-operative complications was excluded from the statistical analysis. Of the remaining 21 patients, 3 (14%) died and 7 (33%) showed disease progression. Atypical tumor location (p<0.001), peritumoral edema (p=0.022) and large tumor size (p=0.048) were correlated with disease progression, however, Ki-67 index and necrosis were not statistically significant. Disease progression occurred in three (21%) of 14 patients who underwent GTR, compared with 4 (57%) of 7 patients who did not undergo GTR, however, it was not statistically significant. Ten patients received adjuvant radiotherapy and the tumors were controlled in 5 of these patients. Conclusion : The prognosis for PXA is good; in our patients overall survival was 84%, and event-free survival was 59% at 3 years. Atypical tumor location, peritumoral edema and large tumor size are significantly correlated with disease progression. GTR may provide prolonged disease control, and adjuvant radiotherapy may be beneficial, but further study is needed.

• Korean Journal of Biological Psychiatry
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• v.9 no.2
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• pp.103-111
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• 2002

Neurocognitive research focusing on cognitive deficits in Depression has resulted in several important but yet potentially contradictory findings. Much literature documents the presence of significant neurocognitive impairments in depressive patients. Studies have shown that dysthymic disorder patients demonstrate a diffuse pattern of cognitive impairment which is frequently indistinguishable from that of focal braindamaged patients. Some reports have suggested that there is a focal pattern of deficit, such as anterior cingulate dysfunction, frontal lobe impairment, or dysfunction of the temporal-limbic cortex. The aim of this study is to evaluate the neurocognitive functions in dysthymic disorder patients, and to compare the functions with those of major depressive disorder patients. The subjects are 17 dysthymic disorder patients. And their neurocognitive functions are compared with those of 23 major depressive episode patients. Patients with a history of neurologic disease, alcohol dependence, substance abuse and mental retardation are excluded. They are assessed with a part of Vienna Test System which is computerized neurocognitive function tests and can evaluate attention, eductive ability, reproductive ability, visuoperceptual analysis, vigilance, visual immediate memory, the speed of information-processing, judgement, and fine motor coordinations. There are no other specific difference between two groups, except the result of cognitrone test. This study provides information about the neurocognitive functions and some difference between major depressive disorder patients and carefully diagnosed dysthymic disorder patients.

• Clinical and Experimental Pediatrics
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• v.50 no.10
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• pp.931-937
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• 2007

The hemolytic uremic syndrome (HUS) is a rare disease of microangiopathic hemolytic anemia, low platelet count and renal impairment. HUS usually occurs in young children after hemorrhagic colitis by shigatoxin-producing enterohemorrhagic E. coli (D+HUS). HUS is the most common cause of acute renal failure in infants and young children, and is a substantial cause of acute mortality and morbidity; however, renal function recovers in most of them. About 10% of children with HUS do not reveal preceding diarrheal illness, and is referred to as D- HUS or atypical HUS. Atypical HUS comprises a heterogeneous group of thrombomicroangiopathy (TMA) triggered by non-enteric infection, virus, drug, malignancies, transplantation, and other underlying medical condition. Emerging data indicate dysregulation of alternative complement pathway in atypical HUS, and genetic analyses have identified mutations of several regulatory genes; i.e. the fluid phase complement regulator Factor H (CFH), the integral membrane regulator membrane cofactor protein (MCP; CD46) and the serine protease Factor I (IF). The uncontrolled activation of the complement alternative pathway results in the excessive consumption of C3. Plasma exchange or plasma infusion is recommended for treatment of, and has dropped the mortality rate. However, overall prognosis is poor, and many patients succumb to end-stage renal disease. Clinical presentations, response to plasma therapy, and outcome after renal transplantation are influenced by the genotype of the complement regulators. Thrombotic thrombocytopenic purpura (TTP), another type of TMA, occurs mainly in adults as an acquired disease accompanied by fever, neurologic deficits and renal abnormalities. However, less frequent cases of congenital or hereditary TTP associated with ADAMTS-13 (a disintegrin and metalloprotease, with thrombospondin 1-like domains 13) gene mutations have been reported, also. Recent advances in molecular genetics better allow various HUS to be distinguished on the basis of their pathogenesis. The genetic analysis of HUS is important in defining the underlying etiology, predicting the genotype-related outcome and optimizing the management of the patients.