Central nervous system involvement in primary Sjögren's syndrome (pSS) is less common and usually presents as white matter lesions, neuromyelitis optica spectrum disorder (NMOSD), or transverse myelitis. NMOSD is an immune-mediated inflammatory demyelinating disease of the central nervous system with a high rate of relapse and significant disability. Studies have shown that patients with pSS combined with NMOSD have more severe symptoms and poorer prognosis. Here, we present a case of critical illness in pregnancy-associated NMOSD combined with Sjögren's syndrome. The patient was a 30-year-old pregnant woman with a history of Sjögren's syndrome who was diagnosed with NMOSD. She received combination therapy with steroids, intravenous immunoglobulin (IVIG), and hydroxychloroquine during pregnancy, resulting in partial resolution of numbness below the waist. However, due to irregular medication adherence outside the hospital setting, she developed weakness in her right lower limb accompanied by inability to move it, while her left lower limb still had some mobility but occasional numbness along with urinary and fecal incontinence. Ten days later, she was admitted to the emergency department where an emergency cesarean section was performed to deliver a healthy baby boy. However, her condition worsened postpartum as she developed high fever accompanied by bilateral lower limb paralysis and weakness along with loss of voluntary control over urination and defecation. The patient underwent ano-ther course of treatment consisting of steroids and IVIG; however there was limited improvement in symptoms observed after this intervention. Following administration of rituximab for the first time, the patient developed urinary tract infection which was successfully managed before continuing regular infusions. In later stages the patient could walk slightly with a limp and regained control over urination and defecation, allowing her to resume normal activities. This case suggests that combination therapy with steroids, IVIG, and hydroxychloroquine should be considered for the patients with pregnancy-associated NMOSD combined with Sjögren's syndrome. Rituximab can significantly improve symptoms such as postpartum paralysis in patients with NMOSD, however, there may be a risk of infection associated with its use.
Adult ; Female ; Humans ; Pregnancy ; Cesarean Section/adverse effects* ; Critical Illness ; Hydroxychloroquine/therapeutic use* ; Hypesthesia/complications* ; Immunoglobulins, Intravenous/therapeutic use* ; Inflammation/complications* ; Neuromyelitis Optica/diagnosis* ; Paralysis/complications* ; Pregnancy Complications/therapy* ; Rituximab/therapeutic use* ; Sjogren's Syndrome/complications* ; Steroids/therapeutic use* ; Vision Disorders

OBJECTIVE: To observe clinical effect of acupuncture combined with conventional visual stimulation on cerebral visual impairment (CVI) in children aged 3-10 years and influence on the pattern visual evoked potential (P-VEP). METHODS: A total of 60 cases of children aged 3-10 years with CVI were randomly divided into an observation group and a control group, 30 cases in each group. The children in the control group received conventional visual stimulation therapy, 1 month as a course of treatment. On the basis of the control group, the children in the observation group was treated with acupuncture at Baihui (GV 20), Jingming (BL 1), Taiyang (EX-HN 5), Sibai (ST 2), etc. 3 times a week, and the treatment was given 4 weeks continuously as a course. Both groups received 3 courses of treatment. The visual acuity and P-VEP improvement were compared between the two groups before and after treatment. RESULTS: After treatment, the incubation period (P100-L) of the two groups was shorter than before treatment, and the amplitude (P100-A) was higher than before treatment ( CONCLUSION Acupuncture combined with conventional visual stimulation can improve the incubation period (P100-L) and amplitude (P100-A) of P-VEP in children with CVI, and improve the best corrected visual acuity in children, the clinical effect is better than the conventional visual stimulation alone.
Acupuncture Points ; Acupuncture Therapy ; Brain Diseases ; Child ; Child, Preschool ; Evoked Potentials, Visual ; Humans ; Vision Disorders/therapy*

Dengue/therapy* ; Humans ; Platelet Transfusion ; Vision Disorders/etiology*

PURPOSE: Patients with brain damage suffer from limitations in performing the activities of daily living (ADL) because of their motor function and visual perception impairment. The aim of this study was to help improve the motor function and visual perception ability of patients with brain damage by providing them with virtual reality-based contents. The usability results of the patients and specialists group were also evaluated. METHODS: The ADL contents consisted of living room, kitchen, veranda, and convenience store, similar to a real home environment, and these were organized by a rehabilitation specialist (e.g., neurologist, physiotherapist, and occupational therapist). The contents consisted of tasks, such as turning on the living room lights, organizing the drawers, organizing the kitchen, watering the plants on the veranda, and buying products at convenience stores. To evaluate the usability of the virtual reality-based visual cognitive rehabilitation service, general elderly subjects (n=11), stroke patients (n=7), stroke patients with visual impairment (n=4), and rehabilitation specialists (n=11) were selected. The questionnaires were distributed to the subjects who were using the service, and the subjective satisfaction of individual users was obtained as data. The data were analyzed using SPSS 21.0 software. The general characteristics of the users and the evaluation scores of the experts were analyzed using descriptive statistics. RESULTS: The usability test result of this study showed that the mean value of the questionnaire related to content understanding and difficulty was high, between 4–5 points. CONCLUSION: The virtual reality rehabilitation service of this study is an efficient service that can improve the function, interest, and motivation of stroke patients.
Activities of Daily Living ; Aged ; Brain ; Cognitive Therapy ; Humans ; Motivation ; Physical Therapists ; Rehabilitation ; Specialization ; Stroke ; Vision Disorders ; Visual Perception ; Water

Gastric cancer with double metastasis to the orbit and duodenum is extremely rare. We report the case of a patient with gastric adenocarcinoma who presented with synchronous orbital and duodenal metastases at the time of initial diagnosis. A 60-year-old man presented with a 1-month history of visual disorder and pain in his right eye. He underwent ophthalmological examinations. The biopsy results suggested intraocular metastatic carcinoma. We conducted a systemic evaluation to identify primary malignancy. Finally, a diagnosis of advanced gastric adenocarcinoma with multi-organ metastasis was made. He planned to be treated with systemic chemotherapy.
Adenocarcinoma ; Biopsy ; Choroid ; Diagnosis ; Drug Therapy ; Duodenum ; Humans ; Middle Aged ; Neoplasm Metastasis ; Orbit ; Stomach Neoplasms ; Vision Disorders

PURPOSE: Crizotinib has demonstrated superior progression-free survival (PFS) and objective response rates (ORRs) versus chemotherapy in previously treated and untreated patients with anaplastic lymphoma kinase (ALK)-positive advanced non-small cell lung cancer (NSCLC). We report the safety and efficacy of crizotinib in Asian subpopulations of two global phase III trials. MATERIALS AND METHODS: This analysis evaluated previously treated and untreated patients in two randomized, open-label phase III trials of crizotinib versus chemotherapy in ALK-positive advanced NSCLC in second-line (PROFILE 1007) and first-line settings (PROFILE 1014). Efficacy and safety were analyzed by race in the intention-to-treat and “as-treated” populations for efficacy and safety endpoints, respectively. RESULTS: In previously treated (n=157) and untreated (n=157) Asian patients, PFS was statistically significantly longer with crizotinib versus chemotherapy (hazard ratio for PFS, 0.526; 95% confidence interval, 0.363 to 0.762; p < 0.001 and hazard ratio, 0.442; 95% confidence interval, 0.302 to 0.648; p < 0.001, respectively). Similar antitumor activity was seen in the non-Asian and overall populations. ORRs were statistically significantly higher with crizotinib versus chemotherapy in both Asian and non-Asian previously treated and untreated patients (p < 0.05). The most common treatment-emergent adverse events (any grade)with crizotinib were vision disorder, diarrhea, and nausea, which were observed at a comparable incidence across Asian and non-Asian populations, irrespective of previous treatment status. Most adverse events were mild to moderate in severity. CONCLUSION: These data, currently the only analysis showing Asian and non-Asian populations in the same study, support the efficacy and safety of crizotinib in Asian patients with previously treated or untreated ALK-positive advanced NSCLC.
Asia ; Asian Continental Ancestry Group* ; Carboplatin ; Carcinoma, Non-Small-Cell Lung* ; Cisplatin ; Continental Population Groups ; Diarrhea ; Disease-Free Survival ; Drug Therapy* ; Humans ; Incidence ; Lymphoma ; Nausea ; Pemetrexed ; Phosphotransferases ; Vision Disorders

The visual system plays an important role in our daily life. In this study, we found that loss of dendritic cell factor 1 (DCF1) in the primary visual cortex (V1) caused a sight deficit in mice and induced an abnormal increase in glutamic acid decarboxylase 67, an enzyme that catalyzes the decarboxylation of glutamate to gamma aminobutyric acid and CO, particularly in layer 5. In vivo electrophysiological recordings confirmed a decrease in delta, theta, and beta oscillation power in DCF1-knockout mice. This study presents a previously unknown function of DCF1 in V1, suggests an unknown contact between DCF1 and GABA systems, and provides insight into the mechanism and treatment of visual deficits.
Animals ; Brain Waves ; genetics ; Disease Models, Animal ; Electroencephalography ; Gene Expression Regulation ; drug effects ; genetics ; Geniculate Bodies ; drug effects ; metabolism ; Ginkgolides ; therapeutic use ; Glutamate Decarboxylase ; metabolism ; Lactones ; therapeutic use ; Membrane Proteins ; deficiency ; genetics ; Mice ; Mice, Inbred C57BL ; Mice, Knockout ; Nerve Tissue Proteins ; deficiency ; genetics ; Photic Stimulation ; Proto-Oncogene Proteins c-fos ; metabolism ; Vision Disorders ; drug therapy ; genetics ; pathology ; physiopathology ; Visual Cortex ; metabolism ; pathology ; gamma-Aminobutyric Acid ; metabolism

BACKGROUND: We assessed the effects of customized in-hospital, in-person education provided by an education-specialized nurse in ischemic stroke patients. METHODS: All ischemic stroke patients who were hospitalized between April 2015 and December 2015 were included. They were provided with education about stroke by an education-specialized nurse during their hospital stay. The knowledge of stroke warning signs and appropriate responses was examined both before the in-hospital education and 3 months after discharge in 127 patients. RESULTS: The awareness of the following stroke warning signs increased significantly at 3 months after discharge compared to before receiving the education (all p values <0.001): sudden difficulty in speaking or in understanding speech (74.0→93.7%), sudden numbness or weakness (72.4→92.1%), sudden dizziness (71.7→89.8%), sudden severe headache (44.9→82.7%), and sudden visual impairment (38.6→69.3%). The proportion of patients with a good knowledge of stroke warning signs (defined as providing at least five correct answers) increased significantly, from 38.6% to 81.9%. Almost half of them (46.5%) correctly answered that they should call an ambulance first when someone shows stroke symptoms before receiving the education, with this proportion increasing to 68.5% at 3 months after discharge (p<0.001). The proportions of patients who understood the need for prompt treatment of stroke and the golden time window increased from 80.3% to 96.9% and from 66.1% to 86.6%, respectively (both p<0.001). The proportion of patients with knowledge of thrombolytic therapy for stroke also increased significantly after the in-hospital education, from 11.0% to 76.4% (p<0.001). CONCLUSIONS: In-hospital, in-person education was effective at increasing the understanding that patients have of stroke, even at 3 months after discharge. In-hospital education provided by an education-specialized nurse would be an effective intervention for increasing the likelihood of stroke patients reacting appropriately to stroke recurrence.
Ambulances ; Dizziness ; Education* ; Headache ; Humans ; Hypesthesia ; Length of Stay ; Recurrence ; Stroke* ; Thrombolytic Therapy ; Vision Disorders

Methanol poisoning is a medical emergency that requires rapid elimination of the toxin and its metabolites for recovery. The danger of methanol results from the accumulation of its toxic metabolite formic acid. This accumulation may result in the development of metabolic acidosis, visual impairment, and damage to the basal ganglia. Extracorporeal treatment is recommended in severe cases of methanol poisoning with coma, seizure, new vision deficits, metabolic acidosis, high serum anion gap, elevated methanol concentrations or impaired kidney function. Although the serum methanol concentration is helpful in determining the use of extracorporeal treatment, methanol assays are not standard laboratory tests in Korea. Herein, we report a case of methanol poisoning in which the patient's clinical improvement was confirmed using serum and urine methanol levels.
Acid-Base Equilibrium ; Acidosis ; Basal Ganglia ; Coma ; Emergencies ; Extracorporeal Circulation ; Kidney ; Korea ; Methanol* ; Osmolar Concentration ; Poisoning* ; Renal Replacement Therapy* ; Seizures ; Vision Disorders

Anaphylactic transfusion reaction is caused by deficiency of certain protein(s) in the recipient. We report on the experience of platelet count recovery using washed platelets for transfusion in a patient who developed an anaphylactic transfusion reaction. A 50-year old male diagnosed with angioimmunoblastic T-cell lymphoma was treated with chemotherapy followed by autologous hematopoietic stem cell transplantation. Immediately after starting transfusion of apheresis platelets, he began sweating and complained of visual impairment, chest discomfort, and abdominal pain. Both systolic and diastolic blood pressures and oxygen saturation monitored by pulse oximetry were decreased. Platelet transfusion was discontinued immediately and hydrocortisone was administered, and the symptoms and signs were resolved within two hours. Laboratory test using post-transfusion blood showed no apparent evidence of hemolysis. Platelet washing procedure using normal saline three times was newly set to prevent anaphylactic reaction in the patient. Transfusions of washed platelets were performed 20 times for 60 days, and the patient showed no anaphylactic reaction during this period. He showed no evidence of immunoglobulin A, haptoglobin, C3, or C4 deficiencies. We confirmed that washed platelet transfusion is highly effective for prevention of anaphylactic transfusion reaction.
Abdominal Pain ; Anaphylaxis ; Blood Component Removal ; Blood Group Incompatibility* ; Blood Platelets* ; Drug Therapy ; Haptoglobins ; Hematopoietic Stem Cell Transplantation ; Hemolysis ; Humans ; Hydrocortisone ; Immunoglobulin A ; Lymphoma, T-Cell ; Male ; Oximetry ; Oxygen ; Platelet Count ; Platelet Transfusion* ; Sweat ; Sweating ; Thorax ; Vision Disorders