Ophthalmologic Manifestations of Ocular Myasthenia Gravis

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.

Tolosa-Hunt Syndrome with Multiple Orbital Myositis in a Patient with Crohn’s Disease

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.

Clinical Features and Course of Acquired Third Cranial Nerve Palsy

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.

Clinical Features and Risk Factors of Facial Herpes Zoster: Ocular Complications and Postherpetic Neuralgia / 대한피부과학회지

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.

Clinical Features and Course of Acquired Third Cranial Nerve Palsy

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.

Metastatic Small Cell Neuroendocrine Carcinoma to the Orbit Manifesting as Total Ophthalmoplegia

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.

A Case of Idiopathic Unilateral Abducens Nerve Palsy in an Adolescent Patient

PURPOSE: To report a case of idiopathic unilateral abducens nerve palsy in an adolescent patient. CASE SUMMARY: A healthy 14-year-old boy presented with binocular horizontal diplopia that started that same day. He did not have any history of trauma, vaccination or infectious disease and showed no other neurological signs. The best corrected visual acuity of both eyes was 20/20. Slit lamp and fundus examinations revealed no other abnormalities. On the alternative prism cover test, he had 6 prism diopter (PD) esotropia with −1 degree abduction limitation in the left eye. After 1 week, the abduction limitation in the left eye was progressed to −3.5 degrees, so we performed brain imaging. The brain magnetic resonance imaging (MRI) and systemic evaluation (serologic test, cerebrospinal fluid examination) were normal therefore, we suspected idiopathic unilateral abducens nerve palsy. Thus, intravenous steroid injection was started, and the patient was partially recovered after 5 days of treatment. At 5 weeks after presentation, diplopia and abduction limitation in the left eye were completely resolved, and recurrence was not found during the 1 year of follow-up. CONCLUSIONS: We report a case of progressive idiopathic unilateral abducens nerve palsy in an adolescent which has resolved completely after intravenous steroid treatment in a short period.

Keratitis Caused by Paecilomyces lilacinus after Cataract Surgery in a Patient with Systemic and Autoimmune Disease

PURPOSE: To report a case of Paecilomyces lilacinus fungal keratitis after cataract surgery in a patient with chronic systemic and autoimmune disease who was treated with medical therapy and penetrating keratoplasty. CASE SUMMARY: A 72-year-old female was referred for decreased visual acuity and ocular pain in the left eye. She underwent cataract surgery in the left eye 1 month earlier and was treated for 2 weeks for corneal edema and stromal infiltration around the corneal suture. She had a chronic systemic disease with hypertension, hyperlipidemia, hepatitis C and rheumatoid arthritis. Suspecting infectious keratitis, the patient was instructed to stop applying topical and systemic steroids and use topical amphotericin B (0.15%) and moxifloxacin (0.5%). However, without improvement, amphotericin B (0.15%) and moxifloxacin (0.5%) were changed to natamycin (5%) and topical voriconazole (2%) and systemic voriconazole was added. However, her systemic status deteriorated and corneal melting developed, scleral graft implantation and amniotic membrane implantation were performed to prevent corneal perforation 6 weeks after the initial visit. Paecilomyces lilacinus was identified in culture at 7 weeks and penetrating keratoplasty was performed 12 weeks after the initial visit. After penetrating keratoplasty, corneal status was stable for 6 months and no signs of recurrence were observed. CONCLUSIONS: In a patient with Paecilomyces lilacinus fungal keratitis and chronic systemic and autoimmune disease, penetrating keratoplasty showed good prognosis when the disease was refractory to topical and systemic antifungal agents.

Astigmatic Changes and Clinical Outcomes after Scleral Fixation of IOL

PURPOSE: To evaluate the clinical outcomes, complications and surgically induced astigmatism (SIA) after scleral fixation in patients with intraocular lens (IOL) or crystalline lens dislocation. METHODS: The present study retrospectively investigated the postoperative best corrected visual acuity (BCVA), refractory change, corneal astigmatism, clinical outcomes, and influencing factors of SIA in 57 eyes of 55 patients with a follow-up of 6 months after the IOL scleral fixation. RESULTS: In comparison of preoperative and postoperative 6 months, BCVA, spherical equivalent and astigmatism were significantly improved but corneal astigmatism was not and SIA (diopter, D) improved from 2.10 +/- 1.88 D to 0.86 +/- 0.73 D (p = 0.002). 4 eyes having redislocation were repositioned and 4 eyes having tilted IOL, 6 eyes having elevated intraocular pressure, 3 eyes having exposure scleral knots, 1 eye having endophthalmitis, and 1 eye showing macular edema were observed. At postoperative 3 months, the SIA of a large incision size (>3 mm) and small incision size (< or =3 mm) was significantly differented (p = 0.041). According to the location of scleral fixation, SIA at postoperative 1 month was significantly different but, was not different at postoperative 6 months. CONCLUSIONS: Surgical management of dislocated IOLs or crystalline lens resulted in significant improvement of visual acuity and absence of influencing SIA factors. However, location of scleral fixation and small incision size influenced corneal astigmatism.

The Effect of Internal Limiting Membrane Peeling in Epiretinal Membrane Including Pseudolamellar Macular Hole

PURPOSE: To report the effect and visual improvement of internal limiting membrane (ILM) peeling, and epiretinal membrane (ERM) peeling in symptomatic ERM with pseudolamellar macular hole. METHODS: This study included 27 eyes in 26 consecutive patients with ERM including pseudolamellar macular hole that underwent vitrectomy, ERM peeling, intravitreal gas tamponade, and maintained a face-down position for 7 days. ILM peeling was performed only in 13 eyes of 13 patients. The patients were divided into 2 groups: eyes with or without ILM peeling (14 eyes and 13 eyes, respectively) and the follow-up period was 12 months or more in all cases. The postoperative anatomic results based on optical coherence tomography (OCT) and improvement of best corrected visual acuity (BCVA) were retrospectively compared between the 2 groups. RESULTS: Anatomic closure after surgery was achieved in 11 eyes (78.6%) in the ILM without peeling group and in 12 eyes (92.3%) in the ILM with peeling group (p = 0.596). The BCVA improved from 0.41 +/- 0.31 (log MAR) to 0.33 +/- 0.21 in the ILM without peeling group (p = 0.479) and from 0.46 +/- 0.41 (log MAR) to 0.28 +/- 0.25 in the ILM with peeling group (p = 0.001). CONCLUSIONS: ILM peeling with the addition of ERM peeling is an effective technique for BCVA improvement in the ERM with pseudolamellar macular hole. Vitrectomy, ERM peeling, gas tamponade, and ILM peeling are important for anatomic and functional success in the ERM with pseudolamellar macular hole.

A Case of Endophthalmitis Caused by Shewanella algae after Trauma

PURPOSE: To report a rare case of endophthalmitis caused by Shewanella algae after trauma. CASE SUMMARY: A 27-year-old man was referred for trauma caused by a fishing sinker in his right eye. On initial examination at another hospital, his visual acuity was light perception, and intraocular pressure was 50 mm Hg. On slit lamp examination, corneal edema and severe anterior chamber inflammation were observed. Consequently, the next day total pars plana vitrectomy, lensectomy, intravitreal silicone oil injection, and antibiotics injection were performed. After the operation, intraocular pressure was 15 mm Hg and the patient's pain was temporarily decreased. The presence of Shewanella algae in the vitreous culture was determined but antibiotic sensitivity was not proven. The patient received postoperative topical fortified vancomycin, ceftazidime, and tobramycin hourly and underwent intravenous antibiotic therapy. On postoperative day 25, the patient transferred to our hospital and ocular pain presented continuously. Intraocular inflammation was not severe but visual acuity was light perception because of retinal necrosis in the posterior pole. Therefore, the patient received topical fortified antibiotics and intravenous antibiotics therapy. On postoperative month 2, visual acuity was light perception and the patient's right eye progressed to pthisis bulbi. CONCLUSIONS: Here in the first case reported in Korea or other conturies of primary endophthalmitis by Shewanella algae after traumas. Shewanella algae endophthalmitis has a rapid progression and poor visual prognosis in spite of aggressive therapy.

A Case of Unilateral Electric Cataract with Optic Neuropathy after Electric Injury

PURPOSE: Electric injury may result in cataracts, which usually occur bilaterally. In this report, we present a rare complication of such an injury presenting as a unilateral cataract with optic neuropathy. CASE SUMMARY: A 17-year-old male patient presented with gradual worsening of vision in his right eye 7 weeks after sustaining an injury from 22,900 volts of high-voltage electric current his right hand. On initial eye examination, the best corrected visual acuity (BCVA) was 20/60 in the right eye and 20/20 in the left eye. Relative afferent pupillary defect (RAPD) was noted in the right eye. Slit-lamp examination confirmed the typical anterior subcapsular lenticular opacities and funduscopy showed a slightly pale optic nerve head in the right eye. Pattern reversal visually evoked potential (P-VEP) showed a significant delay of P-100 implicit time in the right eye. After 7 months, phacoaspiration and posterior chamber intraocular lens implantation were performed in the right eye. Postoperatively, the BCVA improved to 20/30 but visual field examination showed a partial defect in the temporal area of the right eye. Cataract did not develop in the left eye during 15 months of follow-up. The electron microscopic findings showed that the number of mitochondria in the cytoplasm increased. The rough endoplasmic reticulum in the cytoplasm and microfilaments were enriched. CONCLUSIONS: Manifestation time of ocular complications after electric injuries is different. Therefore an ophthalmic examination should be performed regularly in the early recovery period of such injuries and in particular, progression of bilateral electric cataract must be checked.