We herein report a case of intractable epilepsy that occurred in a 7-year-old girl, which is consistent with radiological and clinicopathological hallmarks of Rasmussen's encephalitis. The patient showed characteristic primary unilateral involvement with secondary bilateral propagation. Microscopically, the cortical atrophy due to neuronal loss, intense GFAP-immunoreactive astrogliosis, neuronophagia, perivascular lymphocytic infiltration and microglial nodules was seen throughout the cortex and white matter. No viral inclusions were noted; no cytomegalovirus, herpes simplex virus or Epstein-Barr virus was found by in situ hybridization. Granular immunofluorescence for C4, C1q and IgG within the blood vessel walls was noted, and ultrastructurally, only nonspecific vascular injury was found. Rasmussen's encephalitis is a diagnosis of exclusion; it can be diagnosed by the combination of clinical manifestation, neuroimaging and characteristic pathologic features.
Atrophy ; Blood Vessels ; Child ; Cytomegalovirus ; Diagnosis ; Encephalitis* ; Epilepsy ; Female ; Fluorescent Antibody Technique ; Herpesvirus 4, Human ; Humans ; Immunoglobulin G ; Immunohistochemistry ; In Situ Hybridization ; Microscopy, Electron ; Neuroimaging ; Neurons ; Simplexvirus ; Vascular System Injuries

PURPOSE: To report a case of a patient with cytomegalovirus (CMV) retinitis who was treated with oral valganciclovir. CASE SUMMARY: A 34-year-old man who had undergone anti-cancer chemotherapy for Non-Hodgkin lymphoma was referred to the ophthalmologic oncology clinic because of decreased vision in both eyes. Fundus examination showed white, opaque, and granular retinal lesions in both eyes, and a serologic test showed a positive response to CMV antibody IgG and a negative response to CMV antibody IgM. The patient received induction therapy with intravenous ganciclovir and maintenance therapy with oral valganciclovir 900 mg once daily. CMV retinitis reactivated 4 weeks after maintenance therapy was discontinued. At that point, the patient received induction therapy with oral valganciclovir 900 mg twice daily for 3 weeks and maintenance therapy with 900 mg once daily for 5 weeks. The retinal lesion disappeared and did not recur after oral administration of valganciclovir. The patient discontinued valganciclovir after 5 weeks of maintenance therapy, and CMV retinitis did not reactivate during 6 months of follow-up. CONCLUSIONS: Oral valganciclovir was clinically effective in the treatment of CMV retinitis in a patient who was treated with anti-cancer chemotherapy for non-Hodgkin lymphoma.
Administration, Oral ; Adult ; Cytomegalovirus ; Cytomegalovirus Retinitis ; Eye ; Follow-Up Studies ; Ganciclovir ; Humans ; Immunoglobulin G ; Immunoglobulin M ; Lymphoma, Non-Hodgkin ; Retinaldehyde ; Retinitis ; Serologic Tests ; Vision, Ocular

The Kniest syndrome is characterized by disproportionate dwarfism and Kyphoscoliosis which may be associated with flat facies with prominent eyes, cleft palate, hearing loss, myopia and limited joint motion. The skeletal abnormalities are recognizable at birth with shortening and deformity of the extremities and stiff joints. Marked lumbar lordosis and kyphoscoliosis develop in childhood, resulting in disproportionate shortening of the trunk. We experienced a case of kniest syndrome, confirmed by clinical features, radiological features, and histological examination of cartilage. A brief review of the related literature is presented.
Animals ; Cartilage ; Cleft Palate ; Congenital Abnormalities ; Dwarfism ; Extremities ; Facies ; Hearing Loss ; Joints ; Lordosis ; Myopia ; Parturition

Drug-induced toxic epidermal necrolysis (TEN) is a bullous erythematous disease that is characterized by the appearance of scaled lesions and large sheets of pilling on the skin. A caseof TEN occured in a 10 month old female patient. The characteristic skin lesions of TEN developed after oral administration of carbamazepine for a partial seizure. Thig case consists of prodrome of malaise, fever, anorexia, and conjunctivity followed by erythema & flaccid bullae formation. Diagnosis was confirmed by histologic findings. This patient was treated with a massive systemic corticosteroid, antibiotics, fluid and electrolytes, and topical measures. Authors experienced an extremely rare case of TEN dur to carbamazepine, So report it with a brief review of literature.
Administration, Oral ; Anorexia ; Anti-Bacterial Agents ; Carbamazepine ; Diagnosis ; Electrolytes ; Erythema ; Female ; Fever ; Humans ; Infant ; Seizures ; Skin ; Stevens-Johnson Syndrome*

Instability following total knee arthroplasty is one of the major causes of revision surgery. In most cases, it can be prevented by using an appropriate prosthesis and a good surgical technique. Particular attention should be given to confirmation of diagnosis for which thorough history taking, complete physical examination and radiographic evaluation are needed. With regard to treatment, identification of the etiology of instability is crucial for establishing proper treatment plans; instability would persist without correction of the cause of the initial instability. For successful revision surgery, balanced medio-lateral and flexion-extension gaps should be achieved. Constrained or rotating-hinge total knee prosthesis should also be considered as an alternative option for certain subsets of patients with instability.
Arthroplasty ; Diagnosis ; Humans ; Knee Prosthesis ; Knee ; Physical Examination ; Prostheses and Implants ; Prosthesis Failure

PURPOSE: To analyze the difference of the ganglion cell-inner plexiform layer (GCIPL) thickness in diabetic and normal eyes of patients using spectral domain optical coherence tomography (OCT) (Carl Zeiss Meditec, Dublin, CA, USA). METHODS: The authors compared and analyzed the difference of the GCIPL thickness measured with spectral domain optical coherence tomography (OCT) in 42 diabetic and 92 normal subjects. RESULTS: The study subjects were divided into 3 groups: 92 normal subjects, 22 diabetic patients without diabetic retinopathy, and 26 diabetic patients with diabetic retinopathy. Presence of diabetes mellitus (DM) or diabetic retinopathy did not influence the retinal nerve fiber layer (RNFL) thickness. The GCIPL thickness tended to be thinner especially in the superior sector GCIPL. The GCIPL thickness of normal subjects, diabetes patients without diabetic retinopathy, and diabetic retinopathy patients was 82.24 +/- 7.21 microm, 81.86 +/- 9.53 microm, and 76.77 +/- 14.13 microm, respectively, especially in the superior sector GCIPL (p = 0.029). CONCLUSIONS: Retinal ganglion cell layer thinning was induced by diabetes and diabetic retinopathy, and originated specifically from the superior part of the retina.
Diabetes Mellitus ; Diabetic Retinopathy ; Ganglion Cysts ; Humans ; Nerve Fibers ; Retina ; Retinal Ganglion Cells ; Retinaldehyde ; Tomography, Optical Coherence

PURPOSE: To investigate the effects of vitreomacular traction (VMT) on ranibizumab treatment response for neovascular age-related macular degeneration (AMD). METHODS: A retrospective review of 85 eyes of 85 patients newly diagnosed with neovascular AMD was conducted. Patients were eligible if they had received more than three consecutive monthly ranibizumab (0.50 mg) treatments and ophthalmic evaluations. Patients were classified into a VMT (+) group or VMT (-) group according to optical coherence tomography imaging. Best corrected visual acuity and central retinal thickness (CRT) measurements were obtained at three and six months after initial injection. RESULTS: One month after the third injection, mean visual acuity (VA) increases of 6.36 and 9.87 letters were observed in the VMT (+) and VMT (-) groups, respectively. The corresponding mean CRT values decreased by 70.29 microm and 121.68 microm, respectively. A total 41 eyes were identified as eligible for a subsequent fourth injection; 71.1% of patients (27 eyes) in the VMT (+) group but only 29.8% of patients in the VMT (-) group needed a subsequent fourth injection. Follow-up was extended to six months for 42 of the 85 enrolled patients (49.4%). The trends in VA and optical coherence tomography were found to be maintained at six-month follow-up. CONCLUSIONS: VA and CRT appeared to be more improved after ranibizumab treatment in the VMT (-) group compared to the VMT (+) group. VMT might antagonize the effect of ranibizumab treatment in a subpopulation of AMD patients.
Aged ; Aged, 80 and over ; Angiogenesis Inhibitors/*therapeutic use ; Female ; Follow-Up Studies ; Humans ; Intravitreal Injections ; Male ; Middle Aged ; Ranibizumab/*therapeutic use ; Retina/pathology ; Retinal Diseases/*physiopathology ; Retrospective Studies ; Tissue Adhesions ; Tomography, Optical Coherence ; Vascular Endothelial Growth Factor A/antagonists & inhibitors ; Visual Acuity/drug effects ; Vitreous Body/*pathology ; Wet Macular Degeneration/*drug therapy/physiopathology

OBJECTIVE: This 2009–2011 nation-wide study of adult Koreans was aimed to provide characteristics, medical utilization states, and survival rates for newly diagnosed patients with primary nonmalignant and malignant spine tumors. METHODS: Data for patients with primary spine tumors were selected from the Korean Health Insurance Review and Assessment Service database. The data included their age, sex, health insurance type, co-morbidities, medical cost, and hospital stay duration. Hospital stay duration and medical costs per person occurring in one calendar year were used. In addition, survival rates of patients with primary malignant spine tumors were evaluated. RESULTS: The incidence rate of a primary spine tumor increased with age, and the year of diagnosis (p≤0.0001). Average annual medical costs ranged from 1627 USD (pelvis & sacrum & coccyx tumors) to 6601 USD (spinal cord tumor) for primary nonmalignant spine tumor and from 12137 USD (spinal meningomas) to 20825 USD (pelvis & sacrum & coccyx tumors) for a primary malignant spine tumor. Overall survival rates for those with a primary malignant spine tumor were 87.0%, 75.3%, and 70.6% at 3, 12, and 24 months, respectively. The Cox regression model results showed that male sex, medicare insurance were significantly positive factors affecting survival after a diagnosis of primary malignant spine tumor. CONCLUSION: Our study provides a detailed view of the characteristics, medical utilization states, and survival rates of patients newly diagnosed with primary spine tumors in Korea.
Adult* ; Coccyx ; Diagnosis ; Epidemiologic Studies* ; Epidemiology ; Humans ; Incidence ; Insurance ; Insurance, Health ; Korea ; Length of Stay ; Male ; Medicare ; Sacrum ; Spine* ; Survival Rate

PURPOSE: This study aims to evaluate early changes in retinal structure and BMP2 expression in the retina and crystalline lens by comparing streptozotocin-induced diabetic pigs and normal control group pigs. METHODS: Five eye samples from five diabetic Micro-pigs (Medikinetics, Pyeongtaek, Korea) and five eye samples from five control pigs bred in a specific pathogen-free area were used. Diabetes was developed through intravenous injection of nicotinamide and streptozotocin, and the average fasting glucose level was maintained at 250 mg/dL or higher for 16 weeks. To evaluate BMP2 expression in the retina and crystalline lens, Western blotting was performed. RESULTS: In Hematoxylin and Eosin staining, most diabetic pigs showed structural abnormalities in the inner plexiform layer. The number of nuclei in the ganglion cell layer within the range of 10⁴µm² was 3.78±0.60 for diabetic pigs and 5.57±1.07 for control group pigs, showing a statistically significant difference. In immunohistochemical staining, diabetic retinas showed an overall increase in BMP2 expression. In Western blotting, the average BMP2/actin level of diabetic retinas was 1.19±0.05, showing a significant increase compared to the 1.06±0.03 of the control group retinas (P=0.016). The BMP2/actin level of diabetic crystalline lenses was similar to the control group crystalline lenses (P=0.730). CONCLUSIONS: Compared to control group pigs, the number of nuclei in the inner nuclear layer of retinas from streptozotocin-induced diabetic pigs decreased, while an increase in BMP2 expression was observed in the retina of diabetic pigs.
Blotting, Western ; Crystallins* ; Diabetes Mellitus ; Eosine Yellowish-(YS) ; Fasting ; Ganglion Cysts ; Glucose ; Gyeonggi-do ; Hematoxylin ; Injections, Intravenous ; Lens, Crystalline* ; Niacinamide ; Retina* ; Retinaldehyde* ; Streptozocin ; Swine*

Purpose: To date, studies of firearm and explosive injuries in the Korean military have been limited compared to its importance. To overcome this, this study examined the characteristics of musculoskeletal damages in soldiers who have suffered firearm and explosive injuries over the past four years. Materials and Methods: From January 2015 to July 2019, military forces who had suffered musculoskeletal injuries from firearms or explosive substances were included. The medical records and radiographs were reviewed retrospectively, and telephone surveys about Short Musculoskeletal Functional Assessment (SMFA) for this group were conducted. To compare the functional outcomes, statistical analysis was performed using a t-test for the types of weapons, and ANOVA for others. Results: Of the 61 patients treated for firearms and explosives injuries, 30 patients (49.2%) were included after undergoing orthopedic treatment due to musculoskeletal injury. The average age at injury was 26.4 years old (21–52 years old). The number of officers and soldiers was similar. Eleven were injured by gunshot and 19 by an explosive device. Sixteen were treated in the Armed Forces Capital Hospital and 10 at private hospitals. More than half of the 16 patients (53.3%) with a fracture had multiple fractures. The most common injury site was the hand (33.3%), followed by the lower leg (30.0%). There were 14 patients (46.7%) with Gustilo-Anderson classification 3B or higher who required a soft tissue reconstruction. Fifteen patients agreed to join the SMFA survey for the functional outcomes. Between officers and soldiers, officers had better scores in the Bother Index compared to soldiers (p=0.0045). Patients treated in the Armed Forces Capital Hospital had better scores in both the Dysfunction and Bother Index compared to private hospitals (p=0.0008, p=0.0149). Conclusion This is the first study to analyze of weapons injuries in the Korean military. As a result of the study, the orthopedic burden was high in the treating patients with military weapon injuries. In addition, it is necessary to build a military trauma registry, including firearm and explosive injuries, for trauma treatment evaluation and development of military trauma system.