Clinical and Radiological Features of Korean Patients With Anti-HMGCR Myopathy

Background@#and Purpose To understand the characteristics of Korean patients with anti-3-hydroxy-3-methylglutaryl-coenxyme A reductase (HMGCR) myopathy, we measured antiHMGCR antibodies and analyzed the clinical, radiological, and pathological features of patients with anti-HMGCR myopathy. @*Methods@#We measured titers of anti-HMGCR antibodies in the sera of 99 patients with inflammatory myopathy, 36 patients with genetic myopathy, and 63 healthy subjects using an enzyme-linked immunosorbent assay. We tested 16 myositis-specific autoantibodies (MSAs) in all patients with anti-HMGCR myopathy. @*Results@#Positivity for the anti-HMGCR antibody was observed in 17 (4 males and 13 females) of 99 patients with inflammatory myopathy. The median age at symptom onset was 60 years.Ten (59%) of the patients with anti-HMGCR positivity had taken statins. The titer of antiHMGCR antibodies was significantly higher in the statin-naïve group (median=230 U/mL, interquartile range=170–443 U/mL) than in the statin-exposed group (median=178 U/mL, interquartile range=105–210 U/mL, p=0.045). The most common symptom was proximal muscle weakness in 15 patients (88%), followed by myalgia in 9 (53%), neck weakness in 4 (24%), dysphagia in 3 (18%), and skin lesions in 2 (12%). The median titer of anti-HMGCR antibody was 202 U/mL. We found eight different MSAs in nine (53%) patients. The median disease duration from symptom onset to diagnosis was significantly shorter in the MSA-positive group than in the MSA-negative group (p=0.027). @*Conclusions@#Our study was the first to measure anti-HMGCR antibodies in inflammatory myopathy. It has provided new findings, including the suggestion of the coexistence of other MSAs in Korean patients.

The Investigation on the Burden of Neurology Residents to Manage the Patient who Received Thrombolytic Treatment in the Emergency Department with Hyperacute Stroke

Background@#Because Korea is the fastest aging country, the stroke incidence is increasing rapidly. We investigate the trend of the number of patients with acute stroke in recent years and estimate the burden of the neurologist to treat the acute stroke patient visited the emergency department. @*Methods@#We requested a questionnaire survey to all teaching hospitals on the number of hospital beds, the number of stroke patients who visited the emergency department, the number of stroke patients in charge of the neurologist, and the number of days on duty of residents from 2016 to 2019. @*Results@#Of 69 teaching hospitals, 41 hospitals answered the survey. The average hospital beds per hospital were increased annually from 909 to 916. The average patients who visited the emergency department with stroke and were in charged to neurologists were rapidly increased from 799 to 867 per hospital. In particular, the number of patients with hyperacute cerebral infarction requiring the thrombolytic administration or mechanical thrombectomy were rapidly increased from 105 to 131. On the other hand, the average number of residents per hospital was decreased from 5.1 to 4.8. Therefore, the days on duty per resident were increased from 74 to 77. @*Conclusions@#The number of acute stroke patients, especially, hyperacute stroke required the rapid cooperation and high labor were increasing rapidly in recent years. However, because the number of residents were decreased, the burden was increasing. To improve the quality of acute stroke treatment, it is necessary to increase the number of residents.

Anti-LGI1 Antibody Limbic Encephalitis Associated with Hepatocellular Carcinoma

Anti-leucine-rich glioma inactivated-1 (LGI1) antibody has been known as the most common antibody in autoimmune limbic encephalitis. We report a case of a 63-year-old woman who presented with repetitive memory impairment. She was diagnosed with anti-LGI1 limbic encephalitis by clinical symptoms, magnetic resonance imaging, electroencephalography, and antibody test. Hepatocellular carcinoma (HCC) was discovered incidentally. Clinical seizures were completely controlled after hepatic segmentectomy without immunotherapy. This is the first case report showing the relationship between HCC and anti-LGI1 limbic encephalitis.

The Prevalence of Rapid Eye Movement Sleep Behavior Disorder in Amyloid Positron Emission Tomography Positive Alzheimer's Disease

OBJECTIVES@#Rapid eye movement (REM) sleep behavior disorder (RBD) is well known as a prodromal symptom of neurodegenerative disease, especially α-synucleinopathy. In the previous literature, it has been reported the incidence rate of RBD in Alzheimer's disease (AD) is about 10%. This incidence rate is relatively low, if we consider that nearly half of AD patients also have Lewy body pathology. The objective of this study is to determine the incidence rate of RBD among patients with AD, confirmed by amyloid positron emission tomography (PET) image.@*METHODS@#We enrolled 57 patients with clinically probable AD patients who identified AD pathology in amyloid PET scan. RBD was evaluated with REM sleep behavior disorder questionnaire-Korean vesion (RBDQ-KR), which validated the RBD screening questionnaire as Korean version to confirm RBD. Detailed neuropsychological tests were measured using the Seoul Neuropsychological Screening Battery (SNSB).@*RESULTS@#We analyzed RBDQ-KR data collected from 57 AD patients. Dementia due to AD was 36 patients (63.2%) and mild cognitive impairment (MCI) due to AD was 21 (36.8%). AD with RBD was 14 (24.6%). Among these, AD dementia with RBD was 13 (36.1%), and MCI due to AD with RBD was 1 (4.8%). There were no significant differences in cognitive function between the patients with or without RBD, evaluated by detailed neuropsychological test.@*CONCLUSIONS@#In this study, the incidence of RBD in AD was not rare; therefore, if the patient shows symptoms of RBD, we should not rule out AD.

Metastatic Esophageal Squamous Cell Carcinoma to the Orbit and Periorbit Masquerading as Periorbital Abscess

A 61-year-old man with no history of malignancy presented with a rapidly expanding left periorbital mass, first noticed one month prior to presentation. The mass was firm, and a pus-like discharge drained spontaneously from the center of the lesion. A biopsy was performed, and histopathology confirmed squamous cell carcinoma. Systemic evaluation revealed that the patient had a primary esophageal squamous cell carcinoma with multiple metastases. The prognosis of orbital metastasis is generally poor, and this patient expired after one month of conservative treatment.

The Clinical Characteristics of Facial Herpes Zoster in Korean Patients

PURPOSE: To investigate the clinical characteristics of Korean patients with facial herpes zoster. METHODS: The present study enrolled patients with facial involvement among the patients with herpes zoster. The percentage of involved branches of the trigeminal nerve, types and frequency of ocular complications, and changes in visual acuity depending on systemic treatment were analyzed. RESULTS: A total of 146 patients (15.9%) out of 916 patients with herpes zoster were diagnosed with facial herpes zoster. The ophthalmic branch was the most frequently involved (55.5%), and coinfection with the ophthalmic and maxillary branch was also common (31.5%). Ocular complications developed in 82 eyes (56.2%) and were most common in the group of coinfection with the ophthalmic and maxillary branch (Fisher's exact test, p=0.003). Conjunctivitis (72.0%) was the most commonly observed ocular complication, while punctate keratitis and endothelitis were also frequent. After treatment with a systemic antiviral agent, visual acuity was significantly improved (paired t test, p=0.001). CONCLUSIONS: A higher incidence of ophthalmic complications was observed in facial herpes zoster patients with a wider area of involvement, including the ophthalmic branch. There was significant improvement of visual acuity and ocular complications after systemic treatment. Therefore, systemic antiviral treatment should be considered when a broad herpes zoster skin lesion is observed.

The Role of Electroretinography in Assessing the Progression of Diabetic Retinopathy

PURPOSE: To investigate the clinical significance of electroretinographic (ERG) responses, including the photopic negative response parameter, in assessing the progression of diabetic retinopathy. METHODS: Standard flash ERG was tested on 28 normal controls and 143 patients who were diagnosed with diabetes mellitus. Of those, 97 patients had diabetic retinopathy in different stages. Electroretinography was performed according to the International Society for Clinical Electrophysiology of Vision (ISCEV) standards. Amplitudes and implicit times of ERG responses, including the photopic negative response (PhNR), were compared at different stages of diabetic retinopathy. RESULTS: Amplitudes of oscillatory potentials were significantly reduced in mild NPDR. Cone b-wave amplitude, 30--Hz flicker response, and PhNR were significantly reduced in moderate NPDR. CONCLUSIONS: These results suggest that oscillatory potentials are good indicators of retinal function change in the early stage of diabetic retinopathy, and the appropriate results of the cone b-wave, 30-Hz flicker response and PhNR tests are good indicators of moderate NPDR.

An Incidental Finding of Pseudoexfoliation in Patient With Ophthalmic Artery Occlusion

PURPOSE: To report a case of pseudoexfoliation in the unaffected eye in a patient with ophthalmic artery occlusion. CASE SUMMARY: A 49-year-old female presented with sudden vision loss in her right eye. On the initial examination, best corrected visual acuity was 20/500 in the right eye, and 20/20 in the left eye. Intraocular pressure in both eyes was 17 mmHg. Fundus examination showed retinal edema and a cotton wool spot in the right eye. There was a filling delay of choroidal and retinal vessels in the fluorescein angiography. On the slit lamp examination, there was pseudoexfoliation material around the pupil margin and lens capsule in the unaffected eye but no evidence of glaucoma. At the six-month follow-up, best corrected visual acuity in the right eye improved to 20/30 and a retinal nerve fiber layer defect was found at the spot of the previous cotton wool spot. On the visual field examination, visual defects corresponded with a retinal nerve fiber layer defect. CONCLUSIONS: Pseudoexfoliation may be related to a systemic vascular disorder.

Electrophysiological and Morphological Changes After Intravitreal Bevacizumab Injection with Macular Edema or Choroidal Neovascularization

PURPOSE: To evaluate the functional and anatomical statuses of macula with multifocal electroretinogram (mfERG) and optical coherence tomography (OCT) for the treatment of patients with macular edema after intravitreal bevacizumab injection. METHODS: Patients were injected with intravitreal bevacizumab (1.875 mg/0.075 ml) for macular edema with choroidal neovascularization (CNV), diabetic retinopathy and retinal vein occlusion. RESULTS: A total of 120 eyes (96 patients) were diagnosed with diabetic retinopathy, CNV with age-related macular degeneration and retinal vein occlusion. Visual acuity improved from logMAR 0.85+/-0.39 to 0.54+/-0.38 after intravitreal bevacizumab injection. The implicit time of P1 and N2 in mfERG decreased, and the amplitude of P1 showed a statistically significant increase. Central macular thickness decreased from 374.4+/-135.2 micrometer to 249.0+/-72.0 micrometer. CONCLUSIONS: After intravitreal bevacizumab injection, functional and anatomical statuses of mfERG and OCT improved. This study demonstrates a method for utilizing mfERG to assess the effectiveness of treatments such as bevacizumab.

ERRATUM: Citicoline Protects Against Cognitive Impairment in a Rat Model of Chronic Cerebral Hypoperfusion

The publisher wishes to apologise for incorrectly displaying the second author's academic degree. We correct the second author's academic degree from MS to AD.

Acute Transient Myopic Shift in a Patient With Fever of Unknown Origin: A Case Report

PURPOSE: To report a case of acute transient myopic shift in a patient with fever of unknown origin. CASE SUMMARY: A 31-year-old woman without a history of any ophthalmic problems presented with a sudden onset of bilateral blurred vision. She was admitted with the diagnosis of fever of unknown origin (FUO). Ophthalmic examination revealed a visual acuity of 0.06 in the right eye and 0.1 in the left eye. A myopic shift of 3.12 diopters (D) and 2.25D was noted in the right and left eyes, respectively. Retinal folds and choroidal thickening accompanied the myopic shift. On the third day after onset, visual acuity had decreased even more. Myopic shift increased to 4.87D in the right eye and 4.50D in the left eye. At the same time, the depth of the anterior chamber maximally decreased and the thickness of the lens was maximally increased. One week later, the myopic shift was normalized to emmetropia. The depth of the anterior chamber and the thickness of the lens also normalized as the fever subsided.

Citicoline Protects Against Cognitive Impairment in a Rat Model of Chronic Cerebral Hypoperfusion

BACKGROUND AND PURPOSE: Cerebral white matter (WM) lesions are frequently observed in human cerebrovascular diseases, and are believed to be responsible for cognitive impairment. Various neuroprotective agents can suppress this type of WM or neuronal damage. In this study, we investigated whether citicoline, a drug used to treat acute ischemic stroke, can attenuate WM lesions and cognitive decline caused by chronic hypoperfusion in the rat. METHODS: Animals were divided into immediate- and delayed-treatment groups. Those in the immediate-treatment group received a sham operation, citicoline (500 mg/kg/day), or phosphate buffered saline (PBS) treatment. Citicoline or PBS was administered intraperitoneally for 21 days after occluding the bilateral common carotid arteries. Rats in the delayed-treatment group were intraperitoneally administered with either 500 mg/kg/day citicoline or PBS for 21 days beginning on the 8th day after the operation. From the 17th day of administration, the rats were placed in an eight-arm radial maze to examine their cognitive abilities. After completing the administration, tissues were isolated for Kluver-Barrera and the terminal deoxynucleotidyl transferase biotin-dUTP nick end labelling (TUNEL) staining. RESULTS: In the immediate-treatment group, cognitive functions were preserved in the citicoline-treated group, and WM damage and TUNEL-positive cells differed significantly between the citicoline- and PBS-treated animals. In the delayed-treatment group, there was no decrease in WM damage and TUNEL-positive cells, but cognitive improvement was evident for citicoline treatment relative to PBS treatment. CONCLUSIONS: These results show that citicoline can prevent WM damage and aid cognitive improvement, even after a certain extent of disease progression. Citicoline might be useful in patients with acute ischemic stroke as well as in chronic stroke accompanied with cognitive impairment.

HBsAg screening test of Korean pregnant women during antenatal period / 대한산부인과학회지

OBJECTIVE: To evaluate and improve the method in management of HBsAg positive mothers in Korea by the screening time and method. METHODS: From January 2003 to March 2003, questionnaires were obtained using mail or telephone from total 848 hospitals or clinics handling more than 100 deliveries in year 2001 and 341 of them (40.2%) returned their answers. 279,734 out of 507,151 (55.2%) were investigated in number of delivery cases. The screening time and method for HBsAg in antenatal period were analyzed. RESULTS: Most of hospitals tested for HBV infectivity more than once during antenatal period. 99.1% of hospitals tested for HBsAg and 96.1% of hospitals tested for HBsAb. 87.1% of pregnant women were tested for HBsAg on the first visit. HBsAb was tested at the same time with HBsAg in 96.2%. 41% of hospitals repeated test in third trimester for those with negative HBsAg and 54% of HBsAg positive mothers were retested in third trimester. For those who admitted only for delivery, 45.9% of hospitals tested for HBsAg irrespective of their history. Clinics used RPHA for test method in 38.1% which was higher in proportion compare to hospitals. CONCLUSION: HBV infectivity must be screened in all pregnant women. Repeating test in third trimester may be unnecessary and a policy should be made for those who admit for delivery alone. EIA method is recommended instead of RPHA method.

A case of low-grade endometrial stromal sarcoma with multiple metastasis / 대한산부인과학회지

Uterine sarcomas are rare tumors of mesodermal origin and constitute 2-6% of uterine malignancies. They are classified into leiomyosarcoma (LMS), malignant mixed mullerian tumors (MMMTs), and endometrial stromal sarcoma (ESS) by histologic types. Endometrial stromal sarcomas account for about 7-15% of uterine sarcomas, about 0.2% of female genital tract malignancies. Endometrial stromal sarcomas are divided into endometrial stromal nodule, low-grade endometrial stromal sarcoma, and high-grade endometrial stromal sarcoma on the basis of mitotic activity and vascular invasion. Clinical outcome of low-grade endometrial sarcoma is better than that of high-grade endometrial stromal sarcoma and has a propensity for slow metastasis. We have experienced a case of low-grade endometrial stromal sarcoma with multiple metastasis in a 41-year-old woman and was treated by surgical resection and combination chemotherapy, which is presented with a review of brief literature.

Acute fatty liver of pregnancy complicating acute respiratory distress syndrome: A case report with survival of mother and child / 대한산부인과학회지

We report our experience with the diagnostic, clinical and biochemical findings, complications and maternal-perinatal outcome in a case of acute fatty liver of pregnancy. A 31-year-old multipara presented herself at 35 weeks' gestation with general malaise, gastroesophageal reflux, jaundice, hepatorenal dysfunction and disseminated intravascular coagulation. An abdominal ultrasound revealed a mild fatty liver. A tentative diagnosis of acute fatty liver of pregnancy was made, and immediately a healthy male infant weighing 2330 gm was delivered by emergency cesarean section. On 1, 2 and 6 days after delivery, oliguria, acute respiratory distress syndrome and pancreatitis developed, respectively. Abnormal levels of AST/ALT, creatinine, fibrinogen, PT/PTT and platelet recovered to normal ranges 7-10 days after delivery. Serum amylase and lipase became normal 24 days after delivery with conservative treatment. Ventilator care for acute respiratory distress syndrome continued up to 49 days after delivery, when total bilirubin, ammonia, alkaline phosphatase, r-GT, and AST/ALT began to normalize. The discharge was recommended on the 88th postpartum day. Our result suggests that acute fatty liver of pregnancy can be diagnosed on the basis of clinical and laboratory findings. Prompt delivery, intensive supportive care and awareness of its complications can markedly improve maternal and perinatal outcome.

A case of successful pregnancy in Addison's disease / 대한산부인과학회지

Primary adrenal insufficiency is a rare disease which can lead to fatal outcomes when complicated in pregnancy. The vague symptoms such as nausea, fatigue and abdominal pain may interfere with its diagnosis. But once the diagnosis is made after a simple ACTH stimulation test, a successful pregnancy and delivery can be accomplished by replacement of steroids. It is to be noted that proper management in crises such as labor, delivery, operation and postpartum is very important. We report a case of successful delivery of a woman with primary adrenal insufficiency with a brief review of literature.

Evaluation of prevention program for neonatal vertical transmission from HBsAg positive mother in Korea / 대한산부인과학회지

OBJECTIVE: For evaluation of prevention program for neonatal vertical transmission from HBsAg positive mother in Korea METHODS: From January 15th to February 15th 2003, the status of neonatal vaccination, immunoglobuline injection and breast feeding of HBsAg positive mother was evaluated by using mailed questionnaire in 848 hostpitals with more than 100 deliveries in the year of 2001. 341 out of 848 (40.2%) hospitals returned questionnaires. RESULTS: 91.9% of total hospital reported that the vaccination of hepatitis B and immunoglobulin injection was done within 12 hours after birth in more than 90% of neonate. The most commonly used vaccine type was 0-1-6 method. In case of unknown result of HBsAg/ HBsAb in mother, 84.4% of hospitals delayed immunoglobulin injection until complete report, however 63% of hospitals gave hepatitis vaccine without delay before complete result. The breast feeding of HBsAg positive mother was recommended according to the result of HBeAg in 66.3% of hospitals while 24.9% of hospitals recommend breast feeding. CONCLUSION: Most hospitals in Korea reported that vaccination of Hepatitis B and immunoglobulin injection was done within 12 hours after in over 90% of neonate whose mother was HBsAg Positive. But continuous education and careful monitoring are required for injection method and vaccination policy in neonates of mothers with unknown result of HBsAg/HBsAb of mother.

A case of primary parovarian cystadenocarcinoma of borderline malignancy / 대한산부인과학회지

Parovarian tumors may be of mesothelial, mesonephric (Wolffian), or paramesonephric (Mullerian) origin. An estimated 10% of adnexal masses are parovarian cysts, most commonly mesothelial or paramesonephric in origin. Benign neoplasms such as cystadenomas may occasionally develop in parovarian cysts. Malignancy has been reported in 2.0% to 2.8% of parovarian cystic masses, but it seems to be even less frequent in masses smaller than 5 cm. The histologic appearance of the tumor is identical with that of tumors of ovarian origin. However, their similarity of biologic behavior is uncertain. The appropriate therapy for this unusual lesion has not been fully defined, since a minimal amount of follow-up data is available on the few cases which have been reported. We had experienced a case of papillary serous cystadenocarcinoma of borderline malignancy arising from a parovarian paramesonephric cyst and report this case with a brief review of literature.

A Case of Intramural Pregnancy / 대한산부인과학회잡지

Intramural pregnancy is among the rarest forms of ectopic pregnancy. Since it has been first reported by Doederlein in 1913, 37 cases were reported in worldwide literatures. The pathologic criteria required for intramural pregnancy is that the product of conception is completely surrounded by uterine musculature and is separated from the uterine cavity and the fallopian tube or round ligament. Because of the difficult early diagnosis of intramural pregnancy, most cases were found after the onset of complications such as uterine rupture, shock and hemoperitoneum. Early detection of intramural pregnancy with the use of transvaginal ultrasonogram is important, and MRI is a useful, noninvasive imaging modality. We report a case of successful conservative chemotherapy for a intramural pregnancy with brief review of literatures.