Glutaric aciduria type 1 is an inborn error of lysine, hydroxylysine, and tryptophan metabolism caused by deficiency of glutaryl-coenzyme A dehydrogenase. The disease often appears in infancy with encephalopathy episode that results in acute basal ganglia and white matter degeneration. The majority of patients develop a dystonic-dyskinetic syndrome. This reports 6year-old boy who had been done previous gastrostomy due to swallowing difficulty underwent bilateral pallidotomy with intraoperative electromyography(EMG) monitoring for disabling dystonia. Intraoperative EMG was used to assess stimulation thresholds required for capsular responses and muscle tone. Surface EMG electrodes were placed on the face and cricopharyngeal muscles. Exact target were directly modified according to MRI-visualized anatomy. EMG response was consistently seen prior to visual observation of muscle activity. The surgery improved dystonic symptoms without swallowing difficulty.
Basal Ganglia ; Deglutition ; Dystonia* ; Electrodes ; Gastrostomy ; Glutaryl-CoA Dehydrogenase ; Humans ; Hydroxylysine ; Lysine ; Male ; Metabolism ; Muscles ; Pallidotomy* ; Tryptophan

Generally, the posterolateral approach had been recommended in case of extraforaminal disc herniation or lateral stenosis but it has been speculated that this procedure may be feasible to posterolateral disc herniation at the high lumbar levels of the L1-L2 interspace. Topographically, a posterolateral approach should be able to access anteromedial side of the spinal canal without the risk of the bony instability and damage to the neural structures. During the past one year period, three patients with high lumbar discs at the L1-L2 level were treated by the modified posterolateral approach. This article describeds the details of this approach with pertinent literature review.
Constriction, Pathologic ; Humans ; Spinal Canal

Verrucous czrcinoma is a well accepted clinicopathologic entity that is a lowgrade variant of squamous cell carcinoma. This tumor develops typically in moist areas which are frequent site of chronic inflammation. We herein report a case of verrucous carcinoma developed in a burn scar on. the posterolateral aspect of left popliteal fossa.
Burns* ; Carcinoma, Squamous Cell ; Carcinoma, Verrucous* ; Cicatrix* ; Inflammation

We present a case of nucleus caudalis DREZ operation for medically refractory facial pain due to invasive sarcoma in skull base. The patient showed excellent pain relief immediately after the operation. Until 5 month later, the pain was less than before surgery. At the point of view of pain characteristics and distribution, it was considered the trigemino-vago-glossopharyngeal neuralgia. It is suggested that the nucleus caudalis DREZ operation is effective in treating medically refractory facial pain due to invasive sarcoma in skull base.
Facial Pain ; Humans ; Neuralgia ; Pain, Intractable* ; Sarcoma* ; Skull Base* ; Skull*

Intraoral Vertical Ramus Osteotomy,along with Sagittal Split Ramus Osteotomy,is an popular surgical technique performed on mandibular prognathism. However Intraoral Vertical Ramus Osteotomy has been suspected for an initial mobilization at the healing phase of segment because it does not employ the rigid fixation between segments. To execute a study on the healing phase of seg-ment after Intraoral Vertical Ramus Osteotomy on the horizontal plane, 102 patients (204 parts) who were diagnosed mandibular prog-nathism and took Intraoral Vertical Ramus Osteotomy at the Yonsei University dental hospital were observed during the period of before operation, immediately postoperation, 1 month, 3 months, 6 months, and 12 months. The change in the width of segment and horizontal angle of proximal segment and condylar head on the Submentovertex Cephalogram taken from those patients represented following results. 1. The width of proximal and distal segment decreased with the lapse of time. It decreased into 84.5% between immediate postoperative and 6M and even continued to decrease till 12M. 2. The horizontal angle of the proximal segment did medial rotation according as the lapse of time and rigorously continued till 3M.The rotation angle of condylar head indicated its tendency of recurrence to the original position but the entire recurrence was not allowed. The bigger an initial angle was, the higher was the tendency of recurrence after the operation while the rotation angle remained still bigger. 3. After grouping into group 1, group 2,and group 3 based on the extent of the variation of rotation angle of condylar head at immediate postoperative, the variation of rotation angle was measures in each group. The result presented that the initial rotation angle of condylar head had correlation with that of proximal segment but had no relation with the extent of setback of the mandible. However a quantitative analysis alone is not a sufficient method for analyzing the healing phase of segment on the horizontal plane.Therefore a multilateral analysis using 3 dimensional data such as CT is recommendable for the future study.
Head ; Humans ; Mandible ; Osteotomy* ; Prognathism* ; Recurrence

OBJECTIVE: The study investigates the extent of brain shift and its effect on the accuracy of the stereotaxic procedure. METHODS: Thirty-five patients underwent 40stereotactic procedures between June 2002 and March 2004. There were 26 males, mean age 59years old. There were 34procedures for Parkinson's disease, 2 for essential tremor, 3 for cerebral palsy, 1 for dystonia. Patients were divided in four groups based on postoperative pneumocephalus: under 5cc (9 procedures), between 5~10cc (13procedures), between 10~15cc (11procedures) and more than 15cc (7procedures). The coordinates of the anterior commissure(AC), posterior commissure(PC), and target were defined in pre-and intraoperative magnetic resonance image scans and the amount of air volume was measured with @Target (BrainLab, Heimstetten, Germany). RESULTS: The mean AC-PC was 26.5mm for patients with less than 5cc, 26.9mm for 5~10cc, 25.8mm for 10~15cc and 26.2mm for more than 15cc. The length of AC-PC line and coordinates of AC, PC was also not statistically different, Euclidean distance as well as delta x, delta y, delta z of AC, PC, and target were also not statistically different among the groups (p<0.1). There was a variance in target of 0.7~7.6mm, Euclidean distance of 2.5mm, related to electrophysiology but not to brain-shift. CONCLUSION: The amount of air accumulated in the intracranial space and compressing the cortical surface has no effect on the localization of subcortical stereotactic target and landmarks.
Brain* ; Cerebral Palsy ; Dystonia ; Electrophysiology ; Essential Tremor ; Humans ; Male ; Neurosurgery* ; Parkinson Disease ; Pneumocephalus

PURPOSE: PCNA has been widely used because it is highly accurate, relatively simple and safe to administer. Regardless of its high diagnostic rate, PCNA has the difficulty in excluding malignancy in the cases of, negative for malignancy" results in PCNA cytologic reports. So, we analysed PCNA cases to evaluate the clinical outcome of "negative for malignancy" results in PCNA cytologic reports. MATERIALS AND METHODS: PCNA in 170 cases were done between January, 1991 and December, 1993 and the diagnosis was malignancy in 86 cases, specific benign disease in 30 cases, "negative for malignancy" in 45 cases and inadequate sample in 9 cases. We analysed 36 cases among 45 cases of "negative for malignancy" results in cytologic examinations. The final diagnosis was made on the basis of histopathologic examinations, radiologic findings and clinical courses. we compared the final diagnosis to the radiologic diagnosis. RESULTS: Of the 36 cases with initial "negative for malignancy" results, the final diagnosis was benign in 30 cases and malignant in 6 cases(17%). In radiologic diagnosis of the malignancy, "positive predictive value" was 42%, "negative predictive value" was 96%, and accuracy was 78%. CONCLUSION: In the cases of PCNA results of "negative for malignancy", malignancy can not be excluded because 17% of the cases are proved to be malignant. And if malignancy is highly suggested at radiologic examination, the possibility of malignancy is 42%, and so intensive follow-up examination is needed.
Biopsy, Fine-Needle* ; Diagnosis ; Follow-Up Studies ; Proliferating Cell Nuclear Antigen ; Thorax*

OBJECTIVE: During the follow-up period of extrahepatic malignancy, one may encounter a solitary hepatic metastasis on CT scan which may be difficult to differentiate from hepatic abscess in an ambiguous clinical setting. It was our intention to copmare the radiological similarities and differences between two disease entities from which differentiation can be attempted. MATERIALS AND METHODS: Thirty-six cases of solitary heaptic meastesis and 23 cases of liver abscess were included in this study. Two radiologists interpreted the CT without knowledge of the clinical informations. CT pattern was categorized and the frequency of various findings were compared between the two groups. CT findings of the mass were analysed in misinterpreted cases. RESULTS: Without the clinical informations, the diagnostic accuracy of the mass was 72-76% without pattern analysis. Homogeneous masses were seen in both groups, but all masses larger than 4cm were metastases. In heterogeneous masses, metatases more frequently accompanied high attenuation in central or peripheral portion of the mass and showed thick intermediate zone, Irregular trabecular pattern or septations were more frequently observed in abscesses. Biliary dilatation or stone, pleural effusion, air in mass or biliary tree were more frequently seen in abscesses. The false diagnosis was encountered most frequently when the mass possessed any of the followings; homogeneous attenuation, mosaic pattern in the mass with inhomogeneous attenuation and thin intermediate attenuation area. CONCLUSION: Pattern analysis of the various CT character will be helpful to differentiate hepatic abscess and solitary hepatic metastasis in the equivocal clinical settings. However, similar pattern can be seen in both entities ;in this cases, corrdination of CT pattern and secondary findings is needed for better differentiation.
Abscess ; Biliary Tract ; Diagnosis ; Dilatation ; Follow-Up Studies ; Intention ; Liver Abscess* ; Neoplasm Metastasis ; Pleural Effusion ; Tomography, X-Ray Computed

OBJECTIVE: The purpose of this study is to evaluate the clinicopathologic characteristics of the torsion of the ovarian tumor in postmenopausal women. METHODS: We reviewed the medical records of 20 postmenopausal patients with a postoperative diagnosis of torsion of the ovarian tumor in Department of Obstetrics and Gynecology, Hanyang University Hospital from January 1989 to December 1998. RESULTS: The postmenopausal patients with torsion of the ovarian tumor constitute 20/94 (21.3%) of all adnexal torsion patients encountered during this period. The mean (+/-SD) age of the 20 patients was 63.5+/-9.0 years, with a range of 52-90 years. The mean time since menopause was 16.5+/-10.3 years, with a range of 1-39 years. The mean parity was 5.4+/-2.0 (range of 3-10). Lower abdominal pain (65%) was the most frequently presenting symptom, palpable mass(20%) or diagnosed mass(15%) being the second. But, in 20% of cases there was no lower abdominal pain. The torsion occurred at the right side in 11cases (55%) and at the left side in 9 cases. The most frequent degree of torsion was those cases that was rotated twice (720 degrees). The neoplasms undergoing torsion ranged in diameter between 5cm and 30cm and the most prevalent size was 6 to 10 cm (8 cases). Most of the patients (17cases) were treated by total abdominal hysterectomy and bilateral salpingo-oophorectomy. The tumors that produce torsion varied histologically and the most common type was cystic teratoma (in 5 cases), simple cyst(in 3 cases), serous cystadenoma(in 2 cases), mucinous cystadenoma(in 2 cases). CONCLUSIONS: In our study, ovarian tumor torsion occurring in postmenopausal patients constituted approximately 20% of all torsion patients, therefore adnexal torsion should be considered when a postmenopausal woman presents with lower abdominal pain. When torsion is diagnosed, total abdominal hysterectomy and bilateral salpingo-oophorectomy is the usual treatment for postmenopausal patients. Histologically, in more than 90% of cases, the tumors that produce torsion were benign. If cancer is evident, more extensive surgery is required.
Abdominal Pain ; Diagnosis ; Female ; Gynecology ; Humans ; Hysterectomy ; Medical Records ; Menopause ; Mucins ; Obstetrics ; Parity ; Teratoma

PURPOSE: To evaluate the role of the MRI after the stereotactic radiofrequency ventralis intermedius nucleus (VIM) thalamotomy for the treatment of tremor. MATERIALS AND METHODS: 156 cases of the postthalamotomy MR findings were analized retrospectively. The sagittal T1 weighted image(WI), axial and coronal Proton and T2WI were obtained by using 0.38 T(Resonex Sunnyvale, U. S. A) machine. The interval between thalamotomy and MR examination was from 3 days to 2 months. The MR characteristics and complications related to thalamotomy were reviewed. In 16 cases, a follow-up MR was done 3 to 13 months after the initial MR study. We also reviewed the follow-up MR findings. RESULTS: The mean size of the thalamus lesion was 16 mm. The thalamus lesions were noted as a single layer in 23 cases and as layers of different signal intensity in 100 cases;(2 layers in 84 cases, and 3 or more layers in 16 cases). In 74 cases of the 84 cases with 2 layers, the inner layer was isointense with gray matter on T1WI, hypointense on T2WI, and the outer layer was hypointense on T1WI, hyperintense on T2WI. There were extrathalamic lesions that were related to mistargetting of stereotactic radiofrequency. The locations of the extrathalamic lesions were the posterior limb of the internal capsule(119 cases), the posterior limb of the internal capsule and the midbrain(39 cases), the posterior limb of the internal capsule and the basal ganglia(11 cases), and the midbrain(9 cases). In 5 cases of the mistargetting, double radiofrequency lesions were visualized because of the repeated coagulation. The other complications were intracerebral hemorrhage(2 cases), subdural hemorrhage(2 cases), epidural hemorrhage(1 case), and intraventricular hemorrhage(1 case). On the follow up MR studies(16 cases), 2 cases showed the hemosiderin deposition in periphery of the lesion. CONCLUSION: The MRI was useful for the evaluation of the thalamic lesions and complications after the stereotactic radiofrequency VIM-thalamotomy for the treatment of tremor.
Extremities ; Follow-Up Studies ; Hemosiderin ; Internal Capsule ; Magnetic Resonance Imaging ; Protons ; Retrospective Studies ; Thalamus ; Tremor