• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.12 no.1
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• pp.149-156
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• 2001

After the fact that obsessive-compulsive symptoms or tic symptoms are common in Sydenham's chorea which is a sequale of rheumatic fever produced by group A beta-hemolytic streptococcus was reported, the association between group A beta-hemolytic streptococcus and a subgroup of obsessivecompulsive disorder(OCD) or tic disorder has been attentioned. This subgroup shared a unique clinical course, characterized by an abrupt onset of symptoms and/or dramatic exacerbations. And this subgroup was distinguished by pre-pubertal onset of symptoms, neurological abnormalities(choreiform movements and a unique pattern of motoric hyperactivity), as well as by relapsing and remitting symptom course. Acronym PANDAS(pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection) was used to denote a subgroup of OCD or tic disorder patients with these clinical characteristics. Then, there was a report suggesting that PANDAS category include some of attention-deficit/hyperactivity disorder(ADHD) and were two case reports of anorexia nervosa and body dysmorphic disorder with characteristics of PANDAS. This case is a patient who developed normally until age of 7, but after pharyngeal inflammation with high fever, he showed disturbance of cognition, social relationship, and language and communication, as well as tic symtoms and abnormal movement on face, hand, and foot. We report this case with review of literatures, because we think that this case belongs to the PANDAS category. Based on this observation, we suggest that PANDAS category include some of childhood disintegrative disorder as well as OCD, tic disorder, and ADHD.

• The Journal of the Korean bone and joint tumor society
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• v.6 no.1
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• pp.1-9
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• 2000

Purpose : Spine is one of the most common sites of the metastatic bone tumors. Conservative management such as chemotherapy or radiotherapy has been preferred in most cases. However, the neurologic deficit has not been usually improved, and the quality of life was poor. The purpose of this study was to show the efficacy of the surgical treatments for metastatic spinal tumors in terms of postoperative improvements of neurologic deficit and survival time. Materials and Methods : Authors have reviewed the literatures and analyzed 14 patients of metastatic spinal tumors who were received operative treatments between August 1991 and June 1999 at the our department of orthopaedic surgery. Results : The most frequent primary tumor was breast cancer in women and lung cancer in men. The thoracic region was the most common site. There was a preponderance of females over males, and the age ranged between 4th and 8th decade. The indications of surgical treatment comprised instability, progressive neurologic findings, resistance to radiotherapy, pathological fracture, long life expectancy( >6weeks), isolated metastasis and need for pathological diagnosis. The degree of preoperative neurological deficit might influence on the improvement of clinical symptoms after surgery. Conclusion : The surgical treatment reduced the neurological deficit and pain regardless of the operative methods. In the early stage of neurological deficit, the surgical treatment was important for better prognosis. Recently early active operative treatment was performed for metastatic spinal tumor to minimize the intractable pain and to prevent the progression of the neurologic deficit. Early intervention can improve the quality of life and long term survival.

• Journal of Genetic Medicine
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• v.7 no.2
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• pp.145-150
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• 2010

Acrodysostosis is an extremely rare disorder characterized by short fingers and toes with peripheral dysostosis, nasal hypoplasia, and mental retardation. We report a 16-year-old Korean boy with acrodysostosis who had characteristic clinical features and cervical spine stenosis manifested by neurologic symptoms. On presentation, he complained of difficulty in raising his arms, and suffered from intermittent pain and weakness in both upper extremities. He had short stature and dysmorphic facial features, including a broad, depressed nasal bridge, small, upturned nose, bilateral epicanthal folds, and mild hypertelorism. Moderate mental retardation and sensorineural hearing loss in both ears were also present. Radiological findings included broad, short metacarpals and phalanges with cone-shaped epiphyses, bilateral Madelung deformities, hypertrophied first metatarsals, and thickening of the calvarium. Magnetic resonance imaging findings included stenosis of the cervical spine, platybasia with compression into the cervicomedullary junction, and downward displacement of the cerebellar tonsils. Here, we report a case of acrodysostosis with symptoms and signs of cervical spinal stenosis first in Korea. If it is diagnosed in the early stages, possible life-threatening complications, including spinal canal stenosis, can be managed properly and permanent neurologic sequelae might be avoided. Therefore, it is important to consider acrodysostosis in the differential diagnosis of peripheral dysostosis.

• Radiation Oncology Journal
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• v.19 no.2
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• pp.87-94
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• 2001

Purpose : To evaluate the role of LINAC-based stereotactic radiosurgery (SRS) in the management of meningiomas, we reviewed clinical response, image response, neurological deficits for patients treated at our institution. Methods and materials : Between February 1995 and December 1999, twenty-six patients were treated with SRS. Seven patients had undergone prior resection. Nineteen patients received SRS as the initial treatment. There were 7 male and 19 female patients. The median age was 51 years (range, $14\~67\;years$). At least one clinical symptom presented at the time of SRS in 17 patients and cranial neuropathy was seen in 7 patients. The median tumor volume was $4.7\;cm^3\;(range,\;0.7\~16.5\;m^3)$. The mean marginal dose was 15 Gy (range, $10\~20\;Gy$), delivered to the $80\%$ isodose surface (range, $46\~90\%$). The median clinical and imaging follow-up periods were 27 months (range, 1-71 months) and 25 months (range, $1\~52\;months$), respectively. Results : Of 14 patients who had clinical follow-up of one year or longer, thirteen patients $(93\%)$ were improved clinically at follow-up examination. Clinical symptom worsened in one patient at 4 months after SRS as a result of intratumoral edema, who underwent surgical resection at 7 months. OF 14 patients who had radiologic follow-up of one year or longer, tumor volume decreased in 7 patients $(50\%)$ at a median of 11 months (range, $6\~25\;months$), remained stable in 6 patients $(43\%)$, and increased in one patient $(7\%)$, who underwent surgical resection at 44 months. New radiation-induced neurological deficits developed in six patients $(23\%)$. Five patients $(19\%)$ had transient neurological deficits, completely resolved by conservative treatment including steroid therapy. Radiation-induced brain necrosis developed in one patient $(3.8\%)$ at 9 months after SRS who followed by surgical resection of tumor and necrotic tissue. Conclusions : LINAC-based SRS proves to be an effective and safe management strategy for small to moderate sized meningiomas, inoperable, residual, and recurrent, but long-term follow-up will be necessary to fully evaluate its efficacy. To reduce the radiation-induced neurological deficit for large size meningioma and/or in the proximity of critical and neural structure, more delicate treatment planning and optimal decision of radiation dose will be necessary.

• The Journal of Internal Korean Medicine
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• v.42 no.5
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• pp.727-737
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• 2021

Objectives: This study investigated the effect of Korean medicine on an elderly patient with posterior circulation cerebral infarction, chronic kidney disease (CKD), and early neurological deterioration (END). Methods: The patient, who already had CKD, was treated with Korean medicine, comprising herbal medicine, acupuncture, moxa, and cupping combined with Western medicine (antiplatelet, diabetes) and physical therapy. A manual muscle test (MMT) and a modified Barthel index (MBI) were used to observe the treatment effects, and blood tests were performed to check estimated glomerular filtration rate (eGFR), creatinine and blood urea nitrogen (BUN), which represent renal function. Results: After the treatment, MMT, MBI, and renal function scores had increased. Conclusions: This study suggests that Korean medicine can effectively treat posterior circulation cerebral infarction with END in CKD, but further studies should be conducted.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.1
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• pp.1-12
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• 2018

Purpose: Disorders of organic acid metabolism have various clinical manifestations and it may be life-threatening. The prognoses of affected children are dependent on early diagnosis and treatment. We report this study to find out detection rate of referred samples, clinical manifestations and age distribution after introduction of neonatal screening test using tandem mass spectrometry in Hallym University Chuncheon Sacred Heart Hospital during 8 years and 9 months. Methods: The 2,794 patients referred from Jan. 2007 to Sep. 2015 were divided into four groups according to age. We conducted organic acid analysis of urine samples of patients and analyzed clinical manifestations and distributions of age at the diagnosis. For patients with ambiguous results, reanalysis of urine organic acid after diet restriction, protein loading and restriction, has been done. Results: A total of 626 patients with 20 disorders were diagnosed. Mitochondrial disorders (482 patients) were the most common diagnosis, followed by ketolytic defects (67), 3-hydroxyisobutyric aciduria (32), EPEMA syndrome (8), 3-methylcrotonyl glycinuria (7), glutaric aciduria type II (6) and type I (4), methylmalonic aciduria (3), isovaleric aciduria (3) and medium chain acyl-CoA dehydrogenase deficiency (3). Conclusion: As neonatal screening test using tandem mass spectrometry is increasingly common and medical environment is changed, detection rate of disorders of organic acid metabolism in this study has decreased compared to previous report. Because the deterioration can be prevented by early diagnosis and treatment, many pediatricians have to pay special attention to possibility of the disorders and make an effort for early diagnosis in clinical setting.

• Nuclear Medicine and Molecular Imaging
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• v.42 no.4
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• pp.267-274
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• 2008

Purpose: Transient neurological deterioration (TND) is one of the complications after extracranial-intracranial bypass surgery, and it has been assumed to be caused by postoperative transient hyperperfusion. This study was performed to evaluate the relationship between TND and preoperative and postoperative cerebral perfusion status on brain perfusion SPECT following superficial temporal artery - middle cerebral artery (STA-MCA) anastomosis surgery. Materials and Methods: A total of 60 STA-MCA anastomosis surgeries of 56 patients (mean age: $50{\pm}16$ yrs; M:F=29:27; atherosclerotic disease: 33, moyamoya disease: 27) which were done between September 2003 and July 2006 were enrolled. The resting cerebral perfusion and cerebral vascular reserve (CVR) after acetazolamide challenge were measured before and 10 days after surgery using 99mTc-ethylcysteinate dimer (ECD) SPECT. Moreover, the cerebral perfusion was measured on the third postoperative day. With the use of the statistical parametric mapping and probabilistic brain atlas, the counts for the middle cerebral artery (MCA) territory were calculated for each image, and statistical analyses were performed. Results: In 6 of 60 cases (10%), TND occurred after surgery. In all patients, the preoperative cerebral perfusion of affected MCA territory was significantly lower than that of contralateral side (p=0.002). The cerebral perfusion on the third and tenth day after surgery was significantly higher than preoperative cerebral perfusion (p=0.001, p=0.02). In TND patients, basal cerebral perfusion and CVR on preoperative SPECT were significantly lower than those of non-TND patients (p=0.01, p=0.05). Further, the increases in cerebral perfusion on the third day after surgery were significant higher than those in other patients (p=0.008). In patients with TND, the cerebral perfusion ratio of affected side to contralateral side on third postoperative day was significantly higher than that of other patients (p=0.002). However, there was no significant difference of the cerebral perfusion ratio on preoperative and tenth postoperative day between patients with TND and other patients. Conclusion: In patients with TND, relative and moderate hyperperfusion was observed in affected side after bypass surgery. These finding may help to understand the pathophysiology of TND.

• Radiation Oncology Journal
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• v.19 no.2
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• pp.81-86
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• 2001

Prupose : To evaluate the role of linac based radiosurgery (RS) in the treatment of meningiomas, we retrospectively analyzed the results of clinical and follow up CT/MRI studies. Methods and Materials : From the 1988 July to 1998 April, twenty patients of meningioma had been treated with 6 MV linear accelerator based radiosurgery. Of the 20 patients, four $(20\%)$ were male and 16 $(80\%)$ were female. Mean age was 51 years old ($22\~78$ years old). Majority of intracranial location of tumor for RS were parasagittal and sphenoid wing area. RS was done for primary treatment in 6 $(30\%)$, postoperative residual lesions in 11 $(55\%)$ and regrowth after surgery in 3 $(15\%)$. Mean tumor volume was $5.72\;cm^3\;(0.78\~15.1\;cm^3)$ and secondary collimator size was 2.04 cm $(1\~3\;cm)$. The periphery of tumor margin was prescribed with the mean dose of 19.6 Gy $(9\~30\;Gy)$ which was $40\~90\%$ of the tumor center dose. The follow up duration ranged from 2.5 to 109 months (median 53 months). Annual CT/MRI scan was checked. Results : By the follow up imaging studies, the tumor volume was reduced in 5 cases $(25\%)$, arrested growth in 14 cases $(70\%)$, and increased size in 1 case $(15\%)$. Among these responsive and stable 19 patients by imaging studies, there showed loss of contrast enhancement after CT/MRI in four patients. In clinical response, nine $(45\%)$ patients were considered improved condition, 10 $(50\%)$ patients were stable and one $(5\%)$ was worsened to be operated. This partly resulted in necrosis after surgery. Conclusion : The overall control rate of meningiomas with linac based RS was $95\%$ by both imaging follow-up and clinical evaluation. With this results, linac based RS is considered safe and effective treatment method for meningioma.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.1
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• pp.34-41
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• 2016

Adult-onset type II citrullinemia (CTLN2) is characterized by episodes of neurologic symptoms associated with hyperammonemia leading to disorientation, irritability, seizures, and coma. CTLN2 is distinct from classical citrullinemia, which is caused by a mutation of the argininosuccinic acid synthetase (ASS) gene. The serum citrulline level is elevated, while the activity of ASS in liver tissue is decreased. CTLN2 is known to have a poor prognosis if the proper treatment is not taken. We reported a female aged 37 years who developed recurrent attacks of altered consciousness, aberrant behavior, and vomiting. We initially suspected the patient had CTLN2 because of the signs of hyperammonemic encephalopathy, such as altered mentality, memory disturbance, and aberrant behaviors provoked by exercise-induced stress and excessive intravenous amino acid administration. Through her peculiar diet preferences and laboratory findings that included hyperammonemia and citrullinemia, we diagnosed the patient as CTLN2, and SLC25A13 sequencing revealed known compound heterozygous mutations (IVS11+1G>A, c.674C> A). Her parents were heterozygous carriers, and we identified that her older sister had the same mutations. The older sister had not experienced any episodes of hyperammonemia, but she had peculiar diet preferences. The patient and her sister have been well with conservative management. When considering the clinical course of CTLN2, it was meaningful that the older sister could be diagnosed early in an asymptomatic period and that preemptive treatment was employed. Through this case, CTLN2 should be considered in adults who present symptoms of hyperammonemic encephalopathy without a definite etiology. Because of its rare incidence and similar clinical features, CTLN2 is frequently misdiagnosed as hepatic encephalopathy, and it shows a poor prognosis due to the lack of early diagnosis and proper treatment. A high-carbohydrate diet, which is usually used to treat other urea cycle defects, can also exaggerate the clinical course of CTLN2, so proper metabolic screening tests and genetic studies should be performed.

• The Korean Journal of Pharmacology
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• v.31 no.1 s.57
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• pp.1-9
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• 1995

The slow development of histopathological changes and long period required for stabilization of lesions have suggested that secondary injury processes exacerbate the effect of initial mechanical insult after traumatic spinal cord injury (SCI). The importance of glutamate receptors in the normal functions of spinal cord, in concert with the large body of evidence that points to their involvement in neurotoxicity due to both ischemic and traumatic insults to the CNS, suggested a probable role of glutamate receptors in secondary injury process after traumatic SCI. In order to investigate the involvement of excitatory amino acid in the secondary injury process after SCI, this study examined the effect of dextrorphan, a noncompetitive NMDA receptor antagonist, on the recovery of hindlimb function and the residual tissue at injury site following SCI. Locomotor function was assessed using open field test (21 point scale). At 8 weeks spinal cord tissue was examined using quantitative histopathologic technique. Prior to surgery female Long-Evans rats were adapted to the test environment. Rats received laminectomies (T9/T10), and spinal cord contusions (NYU impactor) were produced by a 10 gm weight dropped 25 mm. DXT (15 or 30 mg/kg, i.p.) or saline was injected 15 min before contusion. Behavioral testing resumed 2 days post-injury and continued twice a week for 8 weeks. No differences between DXT and saline groups were found for hindlimb function and sparing tissue at the lesion site. These results suggest that NMDA receptor might not be involved in secondary injury processes after traumatic SCI.