Purpose: We investigated the ophthalmic manifestations observed in patients diagnosed with ocular myasthenia gravis. Methods: In total, 58 patients diagnosed with ocular myasthenia gravis visited the ophthalmology and neurology departments between January 2011 and August 2022. Patients were followed for > 6 months; their ophthalmic manifestations were analyzed retrospectively. Results: The study included 29 males and 29 females with a mean age of 55.52 ± 15.60 years. Among the patients, 31 tested positive for anti-acetylcholine receptor antibodies, resulting in a positive rate of 53.45%. Abnormalities in repeated nerve stimulation tests were observed in 33.33% of the patients. The antibody-positive group exhibited a higher frequency of progression to generalized myasthenia gravis (p = 0.011) and a higher incidence of chest abnormalities (p < 0.001) compared to the antibody-negative group. Horizontal and vertical diplopia were commonly observed in a complex group of patients with diplopia and blepharoptosis (p < 0.001); there was a difference in the pattern of strabismus between the diplopia single group with esotropia and the complex group with exotropia (p = 0.029). In addition, the combination group demonstrated a higher antibody titer (p = 0.034), a higher frequency of abnormalities in repeated nerve stimulation tests (p = 0.022), and a higher incidence of chest abnormalities (p = 0.022). Conclusions The anti-acetylcholine receptor antibody-positive group had a higher incidence of progression to generalized myasthenia gravis; moreover, the complex group of patients with diplopia and ptosis exhibited elevated levels of anti-acetylcholine receptor antibodies and frequently accompanied both horizontal and vertical strabismus.

Purpose: We report a case of Tolosa-Hunt syndrome with multiple orbital myositis identified via orbital magnetic resonance imaging in a patient with Crohn's disease who developed right eye pain and binocular horizontal diplopia.Case summary: A 46-year-old woman visited our clinic with a 2-month history of right eye pain and migraine, as well as a 3-day history of acute horizontal diplopia. She had previously been diagnosed with Crohn's disease and was taking immunosuppressive drugs. In the eye movement test, esotropia and an abduction limitation of -3.0 in the right eye were observed on the Krimsky test. There were no specific findings in anterior segment and fundus examinations. Orbital magnetic resonance imaging showed multiple extraocular muscle enhancement in the right eye and multiple extraocular muscle hypertrophy in the left eye. The patient was diagnosed with binocular multiple orbital myositis and right Tolosa-Hunt syndrome; she was treated with high-dose steroids for 3 days followed by lower dose oral medications. During the first week of treatment, the right eye pain disappeared and the right eye abduction limitation showed slight improvement. After 3 months of treatment, the right eye abduction limitation and esotropia completely disappeared. Conclusions Orbital myositis and Tolosa-Hunt syndrome are idiopathic, nonspecific chronic granulomatous diseases with painful ophthalmoplegia. We describe a rare case in which the two diseases appear together.

Purpose: The purpose of our study was to investigate the clinical features and course of acquired third cranial nerve (CN3) palsy. Methods: We retrospectively reviewed the medical records of 40 consecutive patients who underwent at least 3 months of follow-up clinical evaluation from March 2016 to December 2019 who were admitted to the ophthalmologic department or referred from other departments of Samsung Changwon Hospital and diagnosed with acquired CN3 palsy. Results: The average age of patients with acquired CN3 palsy was about 64.6 ± 15.9 years and the mean follow-up period was 4.4 ± 8.4 months. Microvasculopathy (twelve patients, 30.0%) was the most common etiology followed by brain vascular lesions (eight patients, 20.0%), and tumors (eight patients, 20.0%). The complete recovery rate was 67.5% and non-isolated CN3 palsy was recorded in 30.0% cases of which six cranial nerve palsy (in eight patients, 66.7%) was the most common. Microvasculopathy (42.9%) and tumors (66.7%) were the most common features in the recovery and persistent groups, respectively. Extraocular movement limitation at the first visit was smaller in the recovery group (−2.4 ± 1.1) than in the persistent group (−3.2 ± 0.6); the difference was statistically significant (p = 0.039). Pupil involvement was found in one (8.3%) patient from the microvascular group and in eight (61.5%) patients from the compressive lesion group. Conclusions The microvascular group or those with a low degree of extraocular movement limitation at the first visit had the highest recovery rate in acquired CN3 palsy. Although compressive lesions showed high pupillary involvement, imaging study should be considered for confirmation rather than attempting to discriminate the causative disease based solely on pupil involvement.

Purpose: The purpose of our study was to investigate the clinical features and course of acquired third cranial nerve (CN3) palsy. Methods: We retrospectively reviewed the medical records of 40 consecutive patients who underwent at least 3 months of follow-up clinical evaluation from March 2016 to December 2019 who were admitted to the ophthalmologic department or referred from other departments of Samsung Changwon Hospital and diagnosed with acquired CN3 palsy. Results: The average age of patients with acquired CN3 palsy was about 64.6 ± 15.9 years and the mean follow-up period was 4.4 ± 8.4 months. Microvasculopathy (twelve patients, 30.0%) was the most common etiology followed by brain vascular lesions (eight patients, 20.0%), and tumors (eight patients, 20.0%). The complete recovery rate was 67.5% and non-isolated CN3 palsy was recorded in 30.0% cases of which six cranial nerve palsy (in eight patients, 66.7%) was the most common. Microvasculopathy (42.9%) and tumors (66.7%) were the most common features in the recovery and persistent groups, respectively. Extraocular movement limitation at the first visit was smaller in the recovery group (−2.4 ± 1.1) than in the persistent group (−3.2 ± 0.6); the difference was statistically significant (p = 0.039). Pupil involvement was found in one (8.3%) patient from the microvascular group and in eight (61.5%) patients from the compressive lesion group. Conclusions The microvascular group or those with a low degree of extraocular movement limitation at the first visit had the highest recovery rate in acquired CN3 palsy. Although compressive lesions showed high pupillary involvement, imaging study should be considered for confirmation rather than attempting to discriminate the causative disease based solely on pupil involvement.

Background/Aims: Myelodysplastic syndrome (MDS) is caused by genetic and epigenetic alteration of hematopoietic precursors and immune dysregulation. Approximately 20% of patients with MDS develop an autoimmune disease (AID). Here, we investigated whether particular genetic mutations are associated with AID in patients with MDS. Methods: Eighty-eight genetic mutations associated with myeloid malignancy were sequenced in 73 MDS patients. The association between these mutations and AID was then analyzed. Results: The median age of the 73 MDS patients was 70 years (interquartile range, 56 to 75), and 49 (67.1%) were male. AID was observed in 16 of 73 patients (21.9%). Mutations were detected in 57 (78.1%) patients. The percentage (68.8% vs. 80.7%, p = 0.32) and the mean number of mutations (1.8 ± 1.6 vs. 2.2 ± 1.8, p = 0.34) in MDS patients with or without AID were similar. However, the ten-eleven translocation- 2 (TET2) mutation rate was significantly higher in patients with AID than in those without (31.3% vs. 5.3%, respectively; p = 0.001). All TET2 mutations were variants of strong clinical significance. Conclusions Mutation of TET2 in patients with MDS may be associated with increased risk of developing AID.

Background: Facial herpes zoster can be accompanied by several complications. In particular, postherpetic neuralgia (PHN) and ocular complications (OCs) are relatively common. Both PHN and OC are of interest because they can lower the quality of life. Objective: This study aimed to evaluate the clinical features of facial herpes zoster and to assess the risk factors of OCs and PHN. Methods: We analyzed the medical records of 146 patients with facial herpes zoster from January 2014 to May 2019. We assessed the proportion of OCs and PHN in patients with facial herpes zoster according to several clinical factors, including age, sex, dermatomal distribution, delayed time to treatment, and associated systemic conditions. OCs were divided into mild and severe ocular complications (SOC) by ophthalmologic diagnosis. Results: The incidence rate of OCs (83.8%) and SOC (37.8%) were highest in patients in their 70s. Herpes zoster involving the ophthalmic and maxillary branches of the trigeminal nerves showed a significantly higher incidence rate of OCs and SOC than those involving only the ophthalmic branch (p=0.031, p=0.025). Patients who received antiviral treatment within 4 days showed lower rates of OCs and SOC than patients who received treatment after 5 days (p＜0.001, p=0.003). The incidence of PHN was significantly higher in those over 60 years old, when both the ophthalmic and maxillary branches were involved, and for those treated more than 4 days after the onset. Conclusion To decrease the risk of OCs and PHN in facial herpes zoster, it is important to provide early antiviral treatment and appropriate ophthalmologic consultation.

Purpose: To understand the effects of datasets with various parameters on pretrained network performance, the generalization capacity of the artificial neural network for myelin water imaging (ANN-MWI) is explored by testing datasets with various scan protocols (i.e., resolution and refocusing RF pulse shape) and types of disorders (i.e., neuromyelitis optica and edema). Materials and Methods: ANN-MWI was trained to generate a T2 distribution, from which the myelin water fraction value was measured. The training and test datasets were acquired from healthy controls and multiple sclerosis patients using a multiecho gradient and spin-echo sequence with the same scan protocols. To test the generalization capacity of ANN-MWI, datasets with different settings were utilized.The datasets were acquired or generated with different resolutions, refocusing pulse shape, and types of disorders. For all datasets, the evaluation was performed in a white matter mask by calculating the normalized root-mean-squared error (NRMSE) between the results from the conventional method and ANN-MWI. Additionally, for the patient datasets, the NRMSE was calculated in each lesion mask. Results: The results of ANN-MWI showed high reliability in generating myelin water fraction maps from the datasets with different resolutions. However, the increased errors were reported for the datasets with different refocusing pulse shapes and disorder types. Specifically, the region of lesions in edema patients reported high NRMSEs. These increased errors indicate the dependency of ANN-MWI on refocusing pulse flip angles and T 2 characteristics. Conclusion This study proposes information about the generalization accuracy of a trained network when applying deep learning to processing myelin water imaging.

BACKGROUND/AIMS: To evaluate and select microRNAs relevant to acute myeloid leukemia (AML) pathogenesis, we analyzed differential microRNA expression by quantitative small RNA next-generation sequencing using duplicate marrow samples from individual AML patients. METHODS: For this study, we obtained paired marrow samples at two different time points (initial diagnosis and first complete remission status) in patients with AML. Bone marrow microRNAs were profiled by next-generation small RNA sequencing. Quantification of microRNA expression was performed by counting aligned reads to microRNA genes. RESULTS: Among 38 samples (32 paired samples from 16 AML patients and 6 normal marrow controls), 27 were eligible for sequencing. Small RNA sequencing showed that 12 microRNAs were selectively expressed at higher levels in AML patients than in normal controls. Among these 12 microRNAs, mir-181, mir-221, and mir-3154 were more highly expressed at initial AML diagnosis as compared to first complete remission. Significant correlations were found between higher expression levels of mir-221, mir-146, and mir-155 and higher marrow blast counts. CONCLUSIONS: Our results demonstrate that mir-221 and mir-181 are selectively enriched in AML marrow and reflect disease activity. mir-3154 is a novel microRNA that is relevant to AML but needs further validation.
Bone Marrow ; Diagnosis ; Humans ; Leukemia, Myeloid, Acute* ; MicroRNAs* ; RNA* ; Sequence Analysis, RNA*

PURPOSE: To report a case of primary ureteral small cell neuroendocrine carcinoma metastasis in the left orbital wall, with a review of the literature.CASE SUMMARY: A 79-year-old male visited our clinic with a 10 day history of ocular pain, ptosis, and ophthalmoplegia in the left eye. He had been diagnosed with diabetes mellitus and was being treated for prostate adenocarcinoma. The corrected visual acuity was 0.8 in the right eye and 0.5 in the left eye. An extraocular movement test showed total ophthalmoplegia, mild exophthalmos, and ptosis in the left eye. Orbital computed tomography (CT) and magnetic resonance imaging showed an irregular mass-like enhancement in the superolateral orbital wall of the left eye, suggesting infectious and inflammatory orbital disease. After antibiotic treatment, high dose systemic steroids were administered. However, there was no improvement and the orbital CT was again performed, with no changes. A surgical biopsy for differentiating orbital tumors was performed and diagnosed as a metastatic orbital small cell neuroendocrine carcinoma. Using positron emission tomography, he was later diagnosed with a metastatic orbital small cell neuroendocrine carcinoma with multiple metastases. CONCLUSIONS When the patient has a mass-like lesion on imaging with ocular pain, ophthalmoplegia, or ptosis, a surgical biopsy should be performed to make a diagnosis and determine the optimal management.

The present report presents a case of dens invaginatus (DI) in a patient with 4 maxillary incisors. A 24-year-old female complained of swelling of the maxillary left anterior region and discoloration of the maxillary left anterior tooth. The maxillary left lateral incisor (tooth #22) showed pulp necrosis and a chronic apical abscess, and a periapical X-ray demonstrated DI on bilateral maxillary central and lateral incisors. All teeth responded to a vitality test, except tooth #22. The anatomic form of tooth #22 was similar to that of tooth #12, and both teeth had lingual pits. In addition, panoramic and periapical X-rays demonstrated root canal calcification, such as pulp stones, in the maxillary canines, first and second premolars, and the mandibular incisors, canines, and first premolars bilaterally. The patient underwent root canal treatment of tooth #22 and non-vital tooth bleaching. After a temporary filling material was removed, the invaginated mass was removed using ultrasonic tips under an operating microscope. The working length was established, and the root canal was enlarged up to #50 apical size and obturated with gutta-percha and AH 26 sealer using the continuous wave of condensation technique. Finally, non-vital bleaching was performed, and the access cavity was filled with composite resin.
Abscess ; Bicuspid ; Dental Pulp Calcification ; Dental Pulp Cavity ; Dental Pulp Necrosis ; Female ; Gutta-Percha ; Humans ; Incisor ; Tooth ; Tooth Bleaching ; Ultrasonics ; Young Adult