PURPOSE: To present a case of leukemic infiltration of the optic nerve head as the initial manifestation of leukemic relapse. CASE SUMMARY: A 65-year-old woman was diagnosed with acute myeloid leukemia. Complete remission was achieved after 4 complete courses of chemotherapy. She complained of a sudden decrease in visual acuity in her left eye. Fundus examination showed severe optic disc edema with peripapillary hemorrhage and serous retinal detachment. Visual acuity and fundus continued to aggravate and high-dose intravenous steroid therapy was instituted. Visual acuity and fundus deteriorated more after treatment. Brain magnetic resonance imaging and CSF study were normal but intrathecal chemotherapy and focal irradiation were performed on account of the suspected CNS involvement of leukemia. Morphologic improvement in the retinal structure was achieved, however, optic atrophy remained and her vision did not recover. CONCLUSIONS: The present case shows the involvement of the optic nerve head as the initial isolated manifestation for the relapse in a patient with complete remission. CNS involvement is rare in acute myeloid leukemia and in particular, the optic nerve is rarely reported as the initial isolated presentation for the relapse. Moreover, the disease progression relatively aggravated after treatment. In the atypical aspects of leukemic relapse, the present case was noticeable.
Aged ; Brain ; Disease Progression ; Edema ; Eye ; Female ; Hemorrhage ; Humans ; Leukemia ; Leukemia, Myeloid, Acute ; Leukemic Infiltration ; Magnetic Resonance Imaging ; Optic Atrophy ; Optic Disk ; Optic Nerve ; Recurrence ; Retinal Detachment ; Retinaldehyde ; Vision, Ocular ; Visual Acuity

PURPOSE: To report a rare case of choroidal metastasis from renal cell carcinoma. CASE SUMMARY: A 61-year-old man visited our clinic with the chief complaint of decreased vision in the right eye. Three years earlier, he underwent right nephrectomy due to renal cell carcinoma. Best corrected visual acuity was 0.2 in the right eye and 1.0 in the left eye. Fundoscopic examination of the right eye revealed a yellowish, dome-shaped elevated choroidal mass with serous retinal detachment, measuring 5.0 x 4.0 disc diameter and located lateral to the macular area. Systemic evaluations showed multiple lung and brain metastases. A diagnosis of choroidal metastasis from renal cell carcinoma was made for the right eye, and the patient received local treatments with oral sorafenib therapy, composed of subtenon triamcinolone injection and intravitreal ranibizumab injection. Tumor progression continued, and visual acuity declined to hand motion. Enucleation was recommended, but the patient refused and is on a regular follow-up after transpupillary thermotherapy. CONCLUSIONS: Ocular manifestation may be the initial presenting sign of a recurrent tumor, and an extensive systemic evaluation for metastatic malignancy should be performed. The present example showed a rare case of choroidal metastasis from renal cell carcinoma.
Antibodies, Monoclonal, Humanized ; Brain ; Carcinoma, Renal Cell ; Choroid ; Eye ; Follow-Up Studies ; Hand ; Humans ; Lung ; Middle Aged ; Neoplasm Metastasis ; Nephrectomy ; Niacinamide ; Phenylurea Compounds ; Retinal Detachment ; Triamcinolone ; Vision, Ocular ; Visual Acuity ; Ranibizumab

We report two cases of progressive localized retinal nerve fiber layer (RNFL) defect following cotton wool spots and a dot hemorrhage, 63-year-old woman with RNFL defect in inferior temporal retina of the right eye and 43-year-old man with RNFL defect in superior temporal retina of the right eye. Case 1 showed a dot hemorrhage adjacent to RNFL defect in the right eye and a new slit-like localized RNFL defect in the left eye 5 months later. The visual field in the left eye demonstrated corresponding field defect 9 months later. Case 2 showed a slit-like RNFL defect starting from cotton wool spots in the right eye and corresponding visual field defect. Cotton wool spots and dot hemorrhages represented ischemic damages at the nerve fiber layer. Two noticeable cases showed the progression of RNFL defect caused by repetitive localized microvascular ischemic events, resulting in visual field loss.
Adult ; Eye ; Female ; Hemorrhage ; Humans ; Middle Aged ; Nerve Fibers ; Retina ; Retinaldehyde ; Visual Fields ; Wool

PURPOSE: To introduce the clinical utility of the absolute value of the reconstructed waveform method in the analysis of multifocal visual evoked potential (mfVEP). METHODS: The mfVEP with 4-channel recording was performed using RETIscan(R) on 10 eyes of 10 normal subjects. Amplitudes were obtained from ring-shaped 6 areas and 4 sectors. The best visual evoked potential (VEP) response method and the absolute value of the reconstructed waveform method were compared in terms of analysis of the amplitudes. In order to assess the false positive rate of the examination, stimuli were administered with one-half of the cathode ray tube (CRT) monitor completely covered and the results were compared using 2 methods. RESULTS: The amplitudes in 6 areas and 4 sectors analyzed with the best VEP response method and the absolute value of the reconstructed waveform method showed no statistical difference (p > 0.05). The amplitude in the stimuli-blocked area of the absolute value of the reconstructed waveform method was smaller than that of the best VEP response method (p < 0.05) and the amplitude of the stimuli area showed no substantial difference between two methods (p > 0.05). CONCLUSIONS: The absolute value of the reconstructed waveform method has similar reproducibility and lower level of false positives relative to the best VEP response method. Therefore, it can be considered as a useful method in the analysis of the mfVEP.
Adult ; Automatic Data Processing ; Evoked Potentials, Visual/*physiology ; Female ; Follow-Up Studies ; Humans ; Male ; Reference Values ; Reproducibility of Results ; Retina/*physiology ; Retrospective Studies ; *Visual Fields

PURPOSE: To introduce the clinical utility of the absolute value of the reconstructed waveform method in the analysis of multifocal visual evoked potential (mfVEP). METHODS: The mfVEP with 4-channel recording was performed using RETIscan(R) on 10 eyes of 10 normal subjects. Amplitudes were obtained from ring-shaped 6 areas and 4 sectors. The best visual evoked potential (VEP) response method and the absolute value of the reconstructed waveform method were compared in terms of analysis of the amplitudes. In order to assess the false positive rate of the examination, stimuli were administered with one-half of the cathode ray tube (CRT) monitor completely covered and the results were compared using 2 methods. RESULTS: The amplitudes in 6 areas and 4 sectors analyzed with the best VEP response method and the absolute value of the reconstructed waveform method showed no statistical difference (p > 0.05). The amplitude in the stimuli-blocked area of the absolute value of the reconstructed waveform method was smaller than that of the best VEP response method (p < 0.05) and the amplitude of the stimuli area showed no substantial difference between two methods (p > 0.05). CONCLUSIONS: The absolute value of the reconstructed waveform method has similar reproducibility and lower level of false positives relative to the best VEP response method. Therefore, it can be considered as a useful method in the analysis of the mfVEP.
Adult ; Automatic Data Processing ; Evoked Potentials, Visual/*physiology ; Female ; Follow-Up Studies ; Humans ; Male ; Reference Values ; Reproducibility of Results ; Retina/*physiology ; Retrospective Studies ; *Visual Fields

Guillain-Barre syndrome (GBS) is an immune-mediated acute polyradiculoneuropathy and commonly occurs after a preceding infection or immunization sequalae. Following the severe acute respiratory syndrome-coronavirus-2 virus pandemic with co-introduction of massive vaccinations, several GBS cases associated with coronavirus disease 2019 (COVID-19) infection per se or after vaccination for COVID-19 were reported internationally. Herein, we report two cases of Korean GBS presenting with tetraplegia after two different COVID-19 vaccinations (42-year old man by AstraZeneca and 48-year woman by Pfizer vaccines) within four weeks after vaccination. The patients were diagnosed with clinical examination, serial electromyography, and compatible laboratory results and improved after comprehensive rehabilitative treatment and intravenous immunoglobulin therapy. Furthermore, we performed an electrodiagnostic follow-up study of each case to examine their unique characteristics.

BACKGROUND: Children with Down syndrome (DS) have a 10- to 20-fold increased risk of developing leukemia. However, in some patients, leukemia does not become apparent despite significant number of blast cells in the peripheral blood. This condition is called Transient myeloproliferative disorder (TMD), and is a disease entity unique to DS newborns and defined as the morphologic detection of blasts in DS less than three months of age. The present study investigated whether there was a difference between leukemia and TMD, and determined prognostic and risk factors.METHODS: We collected blood samples from 317 patients of 433 DS confirmed patients. We found 18 patients who had blast cells in their peripheral blood.RESULTS: Twelve patients were positive for blasts during the neonate period, and only one patient progressed to leukemia. The other 11 patients were later diagnosed with TMD. Six more patients were later diagnosed with leukemia, therefore, 7 patients were diagnosed with leukemia in total. All patients diagnosed with leukemia had anemia at the time of diagnosis, which was not found in TMD patients. All leukemia patients developed their disease after three months of life. Acute Myeloid Leukemia (AML) patients had additional chromosome mutation to trisomy 21 when they were diagnosed.CONCLUSION: In patients with Down Syndrome, anemia at diagnosis and age of onset could be helpful in distinguishing TMD from acute leukemia. Cancerous mutations in the chromosomes of peripheral and marrow blast cells of Down syndrome patients may foreshadow acute leukemia.
Age of Onset ; Anemia ; Bone Marrow ; Child ; Diagnosis ; Down Syndrome ; Humans ; Infant, Newborn ; Leukemia ; Leukemia, Myeloid, Acute ; Myeloproliferative Disorders ; Risk Factors

OBJECTIVES: Nicotine has various adverse effects including negative impacts associated with maternal exposure. In the current study, we examined nicotine-induced damage of hair cells and embryotoxicity during zebrafish development. METHODS: Zebrafish embryos were exposed to nicotine at several concentrations (5, 10, 20, and 40 μM) and embryotoxicity were evaluated at 72 hours, including hatching rate, mortality, teratogenicity rate, and heart rate. Hair cells within the supraorbital (SO1 and SO2), otic (O1), and occipital (OC1) neuromasts were identified at 120 hours. Apoptosis and mitochondrial damage of hair cells were analyzed using TUNEL (terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick end labeling) and DASPEI (2-[4-(dimethylamino)styryl]-N-ethylpyridinium iodide) assays, respectively, and changes of ultrastructure were observed by scanning electron microscopy. RESULTS: The control group without nicotine appeared normal with overall mortality and teratogenicity rate < 5%. The hatching rate and mortality rate was not significantly different according to nicotine concentration (n=400 each). The abnormal morphology rate (n=400) increased and heart rate (n=150) decreased with increasing nicotine concentration (P < 0.05). Nicotine-induced hair cell damage significantly increased as nicotine concentration increased. A significantly greater number of TUNEL-positive cells (P < 0.01) and markedly smaller DASPEI area (P < 0.01) were shown as nicotine concentration increased. CONCLUSION: The current results suggest that nicotine induces dose-dependent hair cell toxicity in embryos by promoting apoptosis and mitochondrial and structural damage.
Apoptosis ; Embryonic Structures ; Female ; Hair* ; Heart Rate ; In Situ Nick-End Labeling ; Maternal Exposure ; Microscopy, Electron, Scanning ; Mortality ; Nicotine* ; Tobacco ; Zebrafish*

Background and Objectives: Human mesenchymal stem cells (MSCs) are emerging as a treatment for atopic dermatitis (AD), a chronic inflammatory skin disorder that affects a large number of people across the world. Treatment of AD using human umbilical cord blood-derived MSCs (hUCB-MSCs) has recently been studied. However, the mechanism underlying their effect needs to be studied continuously. Thus, the objective of this study was to investigate the immunomodulatory effect of epidermal growth factor (EGF) secreted by hUCB-MSCs on AD. Methods: and Results: To explore the mechanism involved in the therapeutic effect of MSCs for AD, a secretome array was performed using culture medium of hUCB-MSCs. Among the list of genes common for epithelium development and skin diseases, we focused on the function of EGF. To elucidate the effect of EGF secreted by hUCB-MSCs, EGF was downregulated in hUCB-MSCs using EGF-targeting small interfering RNA. These cells were then co-cultured with keratinocytes, Th2 cells, and mast cells. Depletion of EGF disrupted immunomodulatory effects of hUCB-MSCs on these AD-related inflammatory cells. In a Dermatophagoides farinae-induced AD mouse model, subcutaneous injection of hUCB-MSCs ameliorated gross scoring, histopathologic damage, and mast cell infiltration. It also significantly reduced levels of inflammatory cytokines including interleukin (IL)-4, tumor necrosis factor (TNF)-α, thymus and activation-regulated chemokine (TARC), and IL-22, as well as IgE levels. These therapeutic effects were significantly attenuated at all evaluation points in mice injected with EGF-depleted hUCB-MSCs. Conclusions EGF secreted by hUCB-MSCs can improve AD by regulating inflammatory responses of keratinocytes, Th2 cells, and mast cells.