Extensive Leukoencephalopathy in Spastic Paraplegia Type 4: Possible Role of Cerebral Autosomal Arteriopathy With Subcortical Infarcts and Leukoencephelopathy

Despite recent advances in next-generation sequencing, the underlying etiology of adult-onset leukoencephalopathy has been difficult to elucidate. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a representative hereditary adult-onset leukoencephalopathy associated with vasculopathy. Leukoencephalopathy in spastic paraplegia type 4 (SPG4) is known to be rare, but it might be underestimated because most spastic paraplegia with leukoencephalopathy is rarely considered. We report a case of co-occurring SPG4 and CADASIL. A 61-year-old male presented with sudden visual impairment after a headache. He showed a spastic gait and had a family history with similar symptoms. An SPG4 gene mutation and a pathogenic variant in the NOTCH3 gene were found. This case shows that the diverse and complex clinical manifestations of patients with extensive leukoencephalopathy are related to more than one gene mutation. We also suggest the necessity for relevant genetic tests in the diagnosis of adult-onset leukoencephalopathy.

Ischemic Stroke Caused by a Hyaluronic Acid Gel Embolism Treated with Tissue Plasminogen Activator

No abstract available.

Tsutsugamushi Meningitis with Parotitis Mimicking Mumps Meningitis

No abstract available.

Massive REM Rebound on Continuous Positive Airway Pressure in Obstructive Sleep Apnea / 대한임상신경생리학회지

No abstract available.

DNA Microarray-Based Gene Expression Profiling in Porcine Keratocytes and Corneal Endothelial Cells and Comparative Analysis Associated with Xeno-related Rejection

Porcine to rat corneal xenotransplantation resulted in severe inflammation and rejection of the corneal stroma, whereas an allograft showed mainly endothelial cell-associated rejection. We, therefore, investigated and compared the gene expression between porcine keratocytes and corneal endothelial cells. RNA was isolated from primary cultured porcine or human keratocytes and porcine corneal endothelial cells. Gene expression was comparatively analyzed after normalization with microarray method using Platinum pig 13 K oligo chip (GenoCheck Co., Ltd., Ansan, Korea). Real-time polymerase chain reaction (PCR) was performed for C1R, CCL2, CXCL6, and HLA-A in porcine keratocytes and corneal endothelial cells. As a result, upregulated expression more than 2 folds was observed in 1,162 genes of porcine keratocytes versus porcine endothelial cells. Among the immune-regulatory genes, SEMA3C, CCL2, CXCL6, F3, HLA-A, CD97, IFI30, C1R, and G1P3 were highly expressed in porcine keratocytes, compared to porcine corneal endothelial cells or human keratocytes. When measured by real-time PCR, the expression of C1R, CCL2, and HLA-A was higher in porcine keratocytes compared to that in porcine corneal endothelial cells. In conclusion, the increased expression of C1R, CCL2, and HLA-A genes in porcine keratocytes might be responsible for the stromal rejection observed in a porcine to rat corneal xenotransplantation.

Clinical Analysis of Elderly Patients with Acute Abdominal Pain in Emergency Department

BACKGROUND: As the elderly population increases, elderly patients visiting emergency departments (ED) with acute abdominal pain will also likely increase. The underlying causative diseases in these older patients are different from younger patients, usually more serious. Thus, correct diagnosis is very important, but difficult. There needs to be a better understanding of the characteristics of the elderly patient with acute abdominal pain seen in the ED. METHODS: We retrospectively reviewed the medical records of 98 elderly patients (>or=65 years) and 109 adult controls (<65 years) visiting an ED at one general hospital looking at the final diagnoses, characteristics of abdominal pain, admissions, and surgeries. RESULTS: Hepatobiliary tract diseases and bowel obstruction were more common in the elderly patients. The elderly had more admissions and surgeries than younger patients. In addition, they were more likely to have a fatal disease such as myocardial infarction. CONCLUSION: There is a difference in the underlying diseases causing acute abdominal pain in the elderly patients versus younger patients. In terms of frequency of admission and surgery, the elderly present with more serious and fatal diseases. We must consider these features when seeing an elderly patient with acute abdominal pain in an ED.

Inflammatory Myofibroblastic Tumor of the Nasal Cavity / 대한이비인후과학회지

Inflammatory myofibroblastic tumor is an uncommon tumor composed of myofibroblasts and various types of inflammatory infiltrates that rarely undergoes malignant transformation. It commonly involves the lung, liver, mesentery but rarely affects the head and neck region. Although inflammatory myofibroblastic tumor is generally considered a benign reactive inflammatory process, it may present clinically and radiologically as an invasive mass destructing the surrounding structures. Therefore, it should be considered as one of several differential diagnoses from malignant tumors. We present a case of inflammatory myofibroblastic tumor involving the nasal cavity, maxillary, ethmoid, and sphenoid sinus with a fatal outcome.

Recent Advances in Nuclear Medicine Imaging Instrumentation / 핵의학분자영상

This review introduces advances in clinical and pre-clinical single photon emission computed tomography (SPECT) and positron emission tomography (PET) providing noninvasive functional images of biological processes. Development of new collimation techniques such as multi-pinhole and slit-slat collimators permits the improvement of system spatial resolution and sensitivity of SPECT. Application specific SPECT systems using smaller and compact solid-state detector have been customized for myocardial perfusion imaging with higher performance. Combined SPECT/CT providing improved diagnostic and functional capabilities has been introduced. Advances in PET and CT instrumentation have been incorporated in the PET/CT design that provide the metabolic information from PET superimposed on the anatomic information from CT. Improvements in the sensitivity of PET have achieved by the fully 3D acquisition with no septa and the extension of axial field-of-view. With the development of faster scintillation crystals and electronics, time-of-flight (TOF) PET is now commercially available allowing the increase in the signal-to-noise ratio by incorporation of TOF information into the PET reconstruction process. Hybrid PET/SPECT/CT systems has become commercially available for molecular imaging in small animal models. The pre-clinical systems have improved spatial resolution using depth-of-interaction measurement and new collimators. The recent works on solid state detector and dual modality nuclear medicine instrumentations incorporating MRI and optical imagers will also be discussed.

Clinical Analysis on Patients with Altered Mental Status in the Emergency Department: Elderly versus Adult Patients

BACKGROUND: We compared elderly and adult patients and tried to find a way to make an early diagnosis and proper management for elderly patients with altered mental status in the emergency department(ED). METHODS: During one year, two groups -123 elderly patients over 65 years and 127 adult patients from 20 to 64 years who visited ED in National Police Hospital(NPH)-were selected. Sex, age, arrival time after symptom onset, means of transportation, underlying diseases, causative disease, time of notification to other departments, and pattern of discharge of two groups were analyzed. RESULTS: The average age of the elderly and the adults were 76.43+/-9.51 and 42.12+/-15.0(yrs), respectively. As for the means of transportation, 84% of the elderly used a 911 ambulance service, and 11% used other emergency services. The average times from symptom onset to arrival for two groups were 124 minutes and 69 minutes, respectively. 86.99% of the elderly and 68.38% of the adults had underlying diseases. As for final diagnosis, cerebrovascular disease for the elderly and cardiovascular disease for the adults were the main causes. When patients left the hospital, rate of transfer to other hospital was higher in the elderly(60.2%), and rate of discharge was higher in the adults(15.8%). CONCLUSION: The elderly patients had more intracranial causes and needed longer time for diagnosis than the adult patients. In the case of the patients with intracranial cause who needed an emergency care, they were usually diagnosed at the secondary medical facility and then transferred to the other hospitals for proper treatment causing bad effect on the prognosis of the treatment due to time delay.

A Case of Spontaneous Dissection of the Superior Mesenteric Artery Treated by Percutaneous Stent Placement / 대한소화기학회지

Acute mesenteric ischemia can result from emboli, arterial and venous thrombi or vasoconstriction secondary to low-flow states. Isolated spontaneous dissection of the superior mesenteric artery is a rare cause of acute mesenteric ischemia. The mortality rates of acute mesenteric ischemia averages 71% with a range of 59-93%. Diagnosis before the occurrence of intestinal infarction is the most important factor in improving survival rate for patients with acute mesenteric ischemia. A 68-year-old female presented with postprandial epigastric pain, and a dissection of the superior mesenteric artery and a gallbladder polyp were shown in abdominal computed tomographic scan. After the percutaneous metalic stent placement and laparoscopic cholecystectomy, her symptoms improved. We report a case of spontaneous dissection of main trunk of the superior mesenteric artery which was successfully treated by percutaneous stent placement with a review of literature.

A Case of Pseudo-Meigs' Syndrome / 대한산부인과학회지

Pseudo-Meigs' syndrome is a rare syndrome associated with struma ovarii, yolk sac tumor, ovarian carcinoma, leiomyoma and tuberculosis, which is combined with ascites and pleural effusion. The cause and pathophysiology of Pseudo-Meigs' syndrome are uncertain. The diagnosis is done by characteristic clinical finding. We have experienced a case of Pseudo-Meigs' syndrome associated with uterine smooth muscle tumor of uncertain malignant potential and elevated CA-125, so report this case.

A Case of Significant Arrhythmia Caused by Thioridazine Poisoning

A 51-year-old woman with schizophrenia and depression brought to our emergency room after thioridazine overdose. Her mental state was semicomatous. The initial electrocardiogram showed bradycardia, atrial premature contractions, prolonged PR interval, wide QRS complexes and U waves. She was admitted to the intensive care unit. Continuous electrocardiographic monitoring and artificial ventilation was performed. The treatment included fluids hydration, administration of inotropic agents, alkalization and replacement of electrolytes. On day 2, torsades de pointes on the electrocardiogram was occurred. The rhythms were resolved with isoproterenol infusion. Her hemodynamic state became stable. On day 6, electrocardiographic finding was normalized. She was recovered without any neurologic or cardiac complications. Herein, a rare case is reported, with the review of the literature.

The Value of Radionuclide Imaging as a Screening Test for the Diagnosis of an Acute Thoracic Spinal Fractures

PURPOSE: The diagnosis of acute thoracic spinal fractures is often missed by plain radiography, alone. The purpose of this study was to compare the accuracy of radionuclide imaging with radiography, thoracic for detection of acute thoracic spinal fractures and to propose radionuclide imaging as screening tests for the diagnosis of acute thoracic spinal fractures before thoracic spine CT or MRI. METHODS: Out of the patients who were admitted to our medical center, Emergency Medicine Department for trauma from June 1, 2001, to February 28, 2003. We are retrospectively studied 67 patients who had undergone radionuclide imaging and thoracic MRI to detect acute thoracic spinal fractures. Because of their symptoms those 67 patients were suspected of having thoracic compression fractures, but those fractures had not been detected on plain thoracic spine radiographics. RESULTS: In 19 patients, 43 acute thoracic spinal fractures were detected by thoracic MRI, in 17 patients, 29 acute thoracic spinal fractures were detected by radionuclide imaging (67.4%) and in 11 patients, 14 acute thoracic spinal fractures were detected by radiography, thoracic (32.6%). The diagnostic agreement of radionuclide imaging with thoracic MRI (Kappa=0.771) was higher than that of plain radiography, thoracic with thoracic MRI (Kappa=0.439). The sensitivity, specificity, and accuracy of radionuclide imaging were 67.4%, 100%, and 93.9%, respectively, and those of plain radiography, thoracic were 32.6%, 100%, and 87.3%. The sensitivity and accuracy of radionuclide imaging for the diagnosis of acute thoracic spinal fractures were significantly higher than those of plain radiography, thoracic (p<0.05). CONCLUSION: Radionuclide imaging is more sensitive and accurate in detecting acute thoracic spinal fractures than plain thoracic spine radiography. Further study is required to prove whether radionuclide imaging is a useful screening test for acute thoracic spinal fractures instead of performing expensive thoracic spine CT or MRI.

HSP70 Gene Expression after Mild Transient Global Cerebral Ischemia in Rats

PURPOSE: Cerebral ischemia lead to neuronal damage in a specific area of the brain of both rodents and humans. After ischemia, the pyramidal cells in area CA1 of the hippocampus are particularly sensitive, but death of these pyramidal cells is delayed to at least 3~4 days after the transient cerebral ischemia. Induction of HSP70 has been mainly observed in resistant areas, so a protective effect has been proposed for HSP70. The presence of HSP70 is a good indicator of the general pathophysiologic stress response to ischemia, but not necessarily a marker for neuronal survival. METHODS: Fisher rats (344) were used in the present experiments. Mild Transient ischemia was induced by using two common methods, carotid artery occlusion and reperfusion. For immunostaining, brain tissues were prepared following intracranial perfusion, and frozen sections were cut on a crystal. Section were incubated with mouse monoclonal antibodies by using a free-float method. Hippocampal tissues at the indicated times were prepared for Western blot analysis. For the determination of the protein levels of HSP70, tissue extracts were subjected to ECL Western blot analysis using primary antibodies. The result from Western blotting was expressed numerically through an image analysis. RESULTS: HSP70 expression markedly increased in 2-day group of transient mild cerebral ischemia after reperfusion. HSP70-positive cells were observed in the cerebral cortex, the striata terminalis, and the hippocampus 2 days after the onset of ischemia. In preconditioned ischemia, no ischemic cell death due to necrosis and apoptosis was noted. CONCLUSION: Preconditioning is induced by mild transient cerebral ischemia, and that is a potent stimulator of HSP70 gene expression.

Clinical use of GnRH Antagonist in Minimal Stimulation Protocol for Assisted Reproductive Technologies / 대한산부인과학회잡지

OBJECTIVE: The Aim of this study to compare the clinical outcomes of GnRH antagonist (Cetrorelix) in minimal stimulation protocol for assisted reproductive technologies to that of GnRH agonist combined with long protocol. METHODS: This study considered of 38 cycles was applied to 32 patients from Mar. 2001 to Feb. 2002. They were normoovulation patients and normal sperm. We excluded the poor responder for severe endometriosis women. The study group consisting of 16 patients was applied with 18 cycles of minimal protocol using GnRH antagonist. The control group consisting of 16 patients was applied with 20 cycles of long protocol using GnRH agonist. RESULTS: The age and infertility duration of the study group were 34.2 +/- 2.8 years and 5.2 +/- 0.4 years each, whereas those of the control group were 33.8 +/- 3.2 years and 4.3 +/- 0.5 years respectively, thus showing insignificant difference between the two groups. E2 (pg/ml)/LH (mIU/ml)/FSH (mIU/ml), tested on the 3rd day of menstrual period as a baseline, of the study group were 18.4 +/- 3.4/8.4 +/- 1.2/6.3 +/- 0.8 respectively. Those of the control group were 19.2 +/- 2.5/7.8 +/- 0.7/6.8 +/- 0.6, which also were insignificantly difference from those of the study group. As for the hMG dosage, 24.5 +/- 3.5 amples were injected to the control group and a significant less dosage of 12.3 +/- 2.3 amples were injected to the study group (p<0.05). E2 level of the study on the hCG injection day was 864.3 +/- 34.5 (pg/ml) which was significantly less than 184.0 +/- 89.4 of the control group (p<0.05). The numbers of ovarian follicles on the ovum pick-up day were 4.2 +/- 1.2 and 8.6 +/- 2.4 for the study and control groups respectively. The number of embryo transferred in the study group was 3.8 +/- 0.5, which was less than 5.3 +/- 0.6 in the control group (p<0.05). However, there were no significant differences in the fertility and pregnancy rates. There were no premature LH surges in both groups. One case of abortion in 5 pregnancies of study group and one in 6 pregnancies of control group, whereas none of them were in the study group. CONCLUSION: The minimal stimulation protocol using GnRH antagonist might be a simple, safe and effective method in the controlled ovarian hyperstimulation compared with GnRH agonist combined long protocol. The use of GnRH antagonist ended with less amples of gonadotropin, less complication even with similar clinical outcomes and efficacy, which was caused by long protocol using GnRH agonist.

Development of a Small Animal Positron Emission Tomography Using Dual-Layer Phoswich Detector and Position Sensitive Photomultiplier Tube: Preliminary Results / 대한핵의학회잡지

PURPOSE: The purpose of this study was to develop a small animal PET using dual layer phoswich detector to minimize parallax error that degrades spatial resolution at the outer part of field-of-view (FOV). MATERIALS AND METHODS: A simulation tool GATE (Geant4 Application for Tomographic Emission) was used to derive optimal parameters of small PET, and PET was developed employing the parameters. Lutetium Oxyorthosilicate (LSO) and Lutetium-Yttrium Aluminate-Perovskite (LuYAP) was used to construct dual layer phoswitch crystal. 8 X 8 arrays of LSO and LuYAP pixels, 2 mm X 2 mm X 8 mm in size, were coupled to a 64-channel position sensitive photomultiplier tube. The system consisted of 16 detector modules arranged to one ring configuration (ring inner diameter 10 cm, FOV of 8 cm). The data from phoswich detector modules were fed into an ADC board in the data acquisition and preprocessing PC via sockets, decoder block, FPGA board, and bus board. These were linked to the master PC that stored the events data on hard disk. RESULTS: In a preliminary test of the system, reconstructed images were obtained by using a pair of detectors and sensitivity and spatial resolution were measured. Spatial resolution was 2.3 mm FWHM and sensitivity was 10.9 cps/micro Ci at the center of FOV. CONCLUSION: The radioactivity distribution patterns were accurately represented in sinograms and images obtained by PET with a pair of detectors. These preliminary results indicate that it is promising to develop a high performance small animal PET.

A Computer Simulation for Small Animal Iodine-125 SPECT Development / 대한핵의학회잡지

PURPOSE: Since I-125 emits low energy (27-35 keV) radiation, thinner crystal and collimator could be employed and, hence, it is favorable to obtain high quality images. The purpose of this study was to derive the optimized parameters of I-125 SPECT using a new simulation tool, GATE (Geant4 Application for Tomographic Emission). MATERIALS AND METHODS: To validate the simulation method, gamma camera developed by Weisenberger et al. was modeled. NaI (Tl) plate crystal was used and its thickness was determined by calculating detection efficiency. Spatial resolution and sensitivity curves were estimated by changing variable parameters for parallel-hole and pinhole collimator. Performances of I-125 SPECT equipped with the optimal collimator were also estimated. RESULTS: In the validation study, simulations were found to agree well with experimental measurements in spatial resolution (4%) and sensitivity (3%). In order to acquire 98% gamma ray detection efficiency, NaI (Tl) thickness was determined to be 1 mm. Hole diameter (mm), length (mm) and shape were chosen to be 0.2: 5: square and 0.5: 10: hexagonal for high resolution (HR) and general purpose (GP) parallel-hole collimator, respectively. Hole diameter, channel height and acceptance angle of pinhole (PH) collimator were determined to be 0.25 mm, 0.1 mm and 90 degree. The spatial resolutions of reconstructed image of the I-125 SPECT employing HR: GP: PH were 1.2: 1.7: 0.8 mm. The sensitivities of HR: GP: PH were 39.7: 71.9: 5.5 cps/MBq. CONCLUSION: The optimal crystal and collimator parameters for I-125 imaging were derived by simulation using GATE. The results indicate that excellent resolution and sensitivity imaging is feasible using I-125 SPECT.

A Case of Metastatic Angiosarcoma of Ovary / 대한부인종양콜포스코피학회잡지

Angiosarcoma is rare malignant neoplasms, which account for less than 1% of all sarcomas. They have a wide distribution in various body organs and tissues: approximately a third of cases occur in the skin and a quarter in soft tissues. Angiosarcoma rarely involves the female reproductive system. We present a case of a 37-yaer-old woman who had primary angiosarcoma of the left breast; With metastases to the spleen and ovary.

A case of Primary Leiomyosarcoma of the Vagina / 대한산부인과학회잡지

The primary leiomyosarcoma of the vagina is very rare tumor in the female lower genital tract. A case of primary leiomyosarcoma of the vagina in the 44 years old woman was presented with lower abdominal pain and cystic and solid mass in the vagina. The mass was mainly comprised with spindle shaped cells showing moderate degree of cellular atypia and mitosis up to 8/10HPF. The mainstay of treatment is surgical excision of the primary mass. Treatment with chemotherapy or radiation therapy usually reserved for inoperable or recurrent cases. We present this case with a review of literature.

Thrombolith of the Extradural Vertebral Venous Plexus as a Cause of Lumbar Radiculopathy: A case Report / 대한정형외과학회잡지

Lumbar radiculopathy may occur in the course of many diseases, but abnormalities or pathologic change of extradural vertebral venous plexus is a rare cause of the lumbar radiculopathy. We report a case of lumbar radiculopathy due to thrombolith of the extradural vertebral venous plexus diagnosed by CT and MRI and treated by hemilaminectomy and removal of thrombolith.