Clinical Features of Children with +4.00 Diopters or More Hyperopia Weaning with Age

PURPOSE: To evaluate the clinical features of children with high hyperopia weaning with age. METHODS: The medical records of 203 children wearing spectacles due to hyperopia of +4.00 diopters (D) or greater in at least one eye based on the cycloplegic refraction and with follow-up for 3 years or more were reviewed. The patients were divided into those who showed a decrease in the spherical equivalent (SE) of 1.50 D or greater and those who maintained. The age of wearing spectacles, the magnitude of hyperopia, the angle of deviation, the ratio of accommodative-convergence to accommodation (AC/A), and the frequency of amblyopia and anisometropia were compared. RESULTS: Forty seven patients with decreased hyperopia and 156 patients with sustained hyperopia were included. The decreased-group started to wear spectacles later than the sustained-group (5.0 ± 2.3 years vs. 4.1 ± 2.4 years). The mean SE of the hyperopic eye in the decreased-group was significantly greater at the initial visit than in the sustained-group (6.29 ± 2.18 D vs. 5.47 ± 1.38 D); was identical at the 1 year follow-up (4.83 ± 1.72 D vs. 4.89 ± 1.55 D); and significantly lower at the last follow-up (3.15 ± 1.72 D vs. 4.65 ± 1.56 D). In the decreased-group, the mean hyperopia of 3.14 ± 2.02 D decreased during a mean period of 3.9 years, especially during the first year after spectacle correction. At baseline, the frequency and angle of esotropia at both distant and near with/without hyperopic correction was significantly larger in the sustained-group. The frequency of amblyopia and anisometropia and the AC/A were identical between the two groups, while the frequency of amblyopia at the last follow-up was significantly lower in the decreased-group. CONCLUSIONS: Some patients with hyperopia of +4.00 D or greater who had none or a small angle of esotropia and improved amblyopia showed a decrease in hyperopia with age.

Comparison of Manifest Refraction and Cycloplegic Refraction Using Retinoscopy or an Autorefractor in Children

PURPOSE: To compare the measurements between manifest refraction and cycloplegic refraction using retinoscopy or an autorefractor in children and to investigate factors affecting the difference. METHODS: A total of 388 children with a mean age of 7.4 ± 3.6 years were examined using retinoscopy and a Grand Seiko GR-3500KA autorefractor before and after cycloplegia. We compared the difference in spherical and cylindrical components between refractions and analyzed the results according to gender, age, type of refractive error, amblyopia, strabismus, and neuro-developmental disorder. A difference in refractions of ±0.50 D or more was considered as a significant discrepancy. RESULTS: Before cycloplegia, the spherical portion of the refractive error via autorefractor measurement was more myopic than for the retinoscopic measurement in 47.2% of patients, and the cylindrical portion was greater in 37.1%. The spherical discrepancies were more common in children aged < 7 years, with hyperopia, or amblyopia (respectively, p = 0.002, p < 0.001, and p = 0.033). After cycloplegia, the spherical component of the refractive error by auto-refraction differed from retinoscopic measurement in 29.4% of patients, and the cylindrical portion differed in 30.7%. However, the difference was not significant and there was no difference according to clinical features. More than half of the children with discrepancies in the spherical component between retinoscopic refractions before and after cycloplegia had a discrepancy between auto-refraction and retinoscopic refraction before cycloplegia, and the two discrepancies had a significant correlation. CONCLUSIONS: Auto-refraction after cycloplegia can estimate retinoscopic values partially. Nevertheless, 30% of the children still showed a discrepancy. The discrepancy of manifest refraction or auto-refraction compared to retinoscopic refraction with cycloplegia should be considered in younger children, cases with hyperopia or amblyopia, and cases with a difference in auto-refraction and retinoscopic refraction before cycloplegia.

Comparison of Intraocular Lens Calculation Formulas Measured by Immersion-Type A-Scan Ultrasound and Partial Coherence Interferometry

PURPOSE: To report the accuracy of intraocular lens (IOL) formulas according to axial length, anterior chamber depth, and mean corneal curvature when performing biometry with an immersion type A-scan with mannual keratomery and an IOL Master®. METHODS: Retrospective medical chart reviews were carried out for 82 eyes of 65 patients who underwent cataract surgery performed by a single surgeon. Biometry was performed using IOL Master®, mannual keratometry, and immersion type A-scan ultrasound in sequence. Prediction diopter was obtained using Sanders-Retzlaff-Kraff/Theoretical (SRK-T) and Holladay 1 formulas calculated with the biometric value measured by mannual keratomery and A-scan, and using SRK-T and, Holladay 2 formulas with IOL Master®. The final refractive outcome was determined as manifested refraction at least 7 weeks after the surgery, and it was compared with the preoperative prediction dipoter (D) of the IOL formulas. RESULTS: Mean axial length and mean keratomtric measurements as determined by A-scan with mannual keratomery showed significant statistical differences from those of IOL Master®. However, there was no difference in postoperative mean absolute error between biometric measurements, or among formulas according to axial length, anterior chamber depth, or mean corneal curvature. However, the percentage of actual refraction within ±0.50 D of the intended refraction was dirrerent among the four formalas according to axial length, anterior chamber dept, mean corneal curvature. CONCLUSIONS: Biometry measurement using the immersion-type A-scan with mannual keratomery is as accurate as that using IOL Master® for predicting the postoperative refractive state of cataract surgery. However, it is suggested that the best IOL formula be chosen according to axial length, anterior chamber depth, and mean corneal curvature.

A Case of Cavernous Sinus Dural Arteriovenous Fistula Following Tolosa-Hunt Syndrome

PURPOSE: To report a case of cavernous sinus dural arteriovenous fistula following Tolosa-Hunt syndrome. CASE SUMMARY: A 64-year-old female with a history of hypertension, presented with blepharoptosis and periorbital pain in the right eye and diplopia. Her right pupil was dilated. She had right exotropia and right hypertropia with inability to elevate, depress, and adduct the right eye. Magnetic resonance imaging including angiography, revealed hyperintensities in the right cavernous sinus consistent with inflammation and no vascular abnormalities. Three days after oral corticosteroid therapy, the pain disappeared. A presumptive diagnosis was Tolosa-Hunt syndrome presenting as a form of complete oculomotor nerve palsy. Two months later, she experienced severe pain in the right periocular area, even though blepharoptosis was resolved and ocular motility was improved. A rapid response to high-dose intravenous corticosteroids was consistent with recurrence of Tolosa-Hunt syndrome. Three months later, she had normal ocular motility, but developed a conjunctival injection, pulsatile orbital bruits, and exophthalmos in the right eye. Cerebral angiography showed a cavernous sinus dural arteriovenous fistula. She received a transvenous coil embolization and her symptoms markedly improved over 2 months. CONCLUSIONS: Tolosa-Hunt syndrome, a granulomatous inflammation in the cavernous sinus may be followed by cavernous sinus dural arteriovenous fistula and should be considered during follow-up.

Two Cases with Positive Ice Tests Mimicking Ocular Myasthenia Gravis

PURPOSE: To report two patients with positive ice tests who had diplopia and blepharoptosis caused by something other than ocular myasthenia gravis. CASE SUMMARY: A 35-year-old female presented with a one-week history of inability to adduct the left eye and left blepharoptosis. Although the ice test was positive, serum anti-acetylcholine receptor binding antibody and the repetitive nerve stimulation test were negative. Brain angiography showed an aneurysm of the left posterior communicating artery, leading to the diagnosis of left pupil-sparing oculomotor nerve palsy. A 25-year-old female presented with an 8-month history of intermittent horizontal diplopia and blepharoptosis in the right eye. The ice test was positive, however serum anti-acetylcholine receptor binding antibody and the repetitive nerve stimulation test were negative. Anti-nuclear antibody was positive (titer 1:160). CONCLUSIONS: The ice test is a useful screening test for myasthenia gravis. However, a series of differential diagnoses including blepharoptosis and ocular motility disorder should be considered because these conditions also show a positive result in the ice test.

Clinical Features and Prognostic Effects of Behavioral and Psychological Symptoms in Patients with Amyotrophic Lateral Sclerosis

BACKGROUND: The evaluation of behavioral and psychological symptoms (BPS) in ALS is important because its existence may serve as a prognostic factor and suggest a shared pathology with frontotemporal dementia (FTD) in ALS. In this study, we sought to identify the prevalence of the BPS of ALS patients and evaluate its relationship with the clinical profiles and survival of ALS patients. METHODS: One hundred sixty-six patients were enrolled in a cross-sectional cohort analysis from September 2008 to February 2012. All patients had sporadic ALS without a genetic mutation and were collected clinical profiles. The t-test and chi-square test were used to assess differences in the clinical characteristics and caregiver-administered neuropsychiatric inventory (CGA-NPI) scores. The Kaplan-Meier method and Cox proportional hazard model were used for the survival analysis. RESULTS: Forty-two patients had clinically significant BPS (42/166, 25.3%). ALS patients with BPS had worse clinical dementia rating (CDR), ALS Functional Rating Scale-Revised (ALSFRS-R) score, and progression rate of disease than those without BPS. Among CGA-NPI subscales, depression, irritability, apathy, and agitation were higher prevalent than the others. There was a trend for ALS patients with BPS having short survival time than those without BPS in the Kaplan-Meier analysis (p=0.006). However, in the Cox proportional hazard model, BPS in ALS patients were not associated with poor survival. CONCLUSION: These results support the presence of an overlapping spectrum between ALS and FTD and emphasize the importance of neuropsychiatric evaluations in ALS. Although the association between BPS and prognosis are not explained clearly, these results could be used to stratify ALS patients according to neuropsychiatric symptoms and help investigators to evaluate the BPS in ALS patients.

Amyotrophic Lateral Sclerosis in Korea: Clinical Characteristics and Prognostic Factors

BACKGROUND: No prospective analyses of clinical characteristics and prognostic factors on amyotrophic lateral sclerosis (ALS) have been performed in Korea. METHODS: The prognosis of 227 sporadic ALS patient with El-Escorial-compatible criteria were evaluated using the serial Korean Amyotrophic Lateral Sclerosis Functional Rating Scale-revised (K-ALSFRS-R), and the progression rate of K-ALSFRS-R (DeltaFS=48-ALSFRS-R at the time of diagnosis). Their clinical characteristics and survival times were also analyzed. The endpoint was defined as either time to death or the tracheostomy state. The effects of individual prognostic factors on disease progression were assessed with the Kaplan-Meier life-table method and multivariate Cox proportional regression analysis. The declining ALSFRS-R curve, epidemiological data, and neuropsychological tests were evaluated. RESULTS: Among 331 motor neuron disease (MND) patients, 227 were finally diagnosed as sporadic ALS. The mean age at onset in 227 sporadic ALS patients was 50.7 years (range 19-76 years). Analysis of sequential ALSFRS-R data revealed that a mean DeltaFS of 0.73/month and a high DeltaFS score were related with early death or a tracheostomy state. Neuropsychological testing revealed nonmotor symptoms, and especially cognitive impairment, had developed in half of our patients. CONCLUSIONS: Our data suggest that DeltaFS is valuable parameter for evaluating prognosis and clinical course of ALS patients, and clinical characteristics obtained from this study would be informative data for understanding epidemiology of ALS in Korea.

Seoul Neuropsychological Screening Battery-Dementia Version (SNSB-D): A Useful Tool for Assessing and Monitoring Cognitive Impairments in Dementia Patients

The Seoul Neuropsychological Screening Battery (SNSB) is one of the standardized neuropsychological test batteries widely used in Korea. However, it may be a bit too lengthy for patients with decreased attention span; and it does not provide the score of global cognitive function (GCF), which is useful for monitoring patients longitudinally. We sought to validate a dementia version of SNSB (SNSB-D) that was shorter than the original SNSB and contained only scorable tests with a GCF score of 300. We administered SNSB-D to patients with mild cognitive impairment (MCI) (n=43) and Alzheimer's disease (AD) (n=93), and normal controls (NC) (n=77). MCI and AD groups had GCF scores significantly different from NC group, and GCF scores were able to distinguish patients with Clinical Dementia Rating of 0.5 and 1. Test-retest reliability was high, with a correlation coefficient of 0.918 for AD, 0.999 for MCI, and 0.960 for NC. The GCF score significantly correlated with the Mini-Mental State Examination (MMSE). Through ROC-curve analysis, GCF scores were found to yield more accurate diagnoses than the MMSE. The SNSB-D is a valid, reliable tool for assessing the overall cognitive function, and can be used to monitor cognitive changes in patients with dementia.

Comparison of Combined Immune Suppression, Bone Marrow Transplantation and Androgen Treatment in Childhood Severe Aplastic Anemia / 대한소아혈액종양학회지

PURPOSE: The purpose of this study is to compare treatment response and survival according to treatment modalities, such as allogeneic bone marrow transplantation (BMT), combined immunosuppression and supportive care, and according to etiologies in children with severe aplastic anemia. METHODS: Thirty-four patients who had severe aplastic anemia received either BMT, combined immunosuppression, or androgen treatment at Chonnam University Hospital from Jan. 1990 to Mar. 1998. The clinical characteristics and response according to treatment modalities or etiologies were studied retrospectively. RESULTS: 1) Patients in Group 1 received combined immunosuppression of ALG/ATG+methylprednisolone+/-cyclosporin (N=18); Group 2, BMT (N=11); Group 3, supportive treatment of oxymetholone+prednisolone+transfusion (N=5). Clinical characteristics were not different among three groups. 2) In Group 1, the response rate was 50% with complete response (CR) in 4 (22.2%) and partial response (PR) in 5 (27.8%). CR was attained in 91% (10/11) of Group 2 patients, while all 5 in Group 3 were died. Relapse was found in 1 out of 9 (11.1%) in Group 1. Three of 10 in Group 2 who had initial response experienced late graft failure. The Kaplan-Meier 4-year-survival in each group was 75.5%, 81.8% and 20%, respectively (P = 0.019). 3) Among 5 patients were Fanconi's anemia, two survived who underwent HLA- matched sibling transplants. Three patients with secondary aplastic anemia, including posthepatitis (N=2) and post-rubella responded to either BMT or immune suppression. Idiopathic cases (N=26) showed response rate of 43.8% to immunosuppression [CR, 3/16; PR 4/16], 85.7% to BMT, and 0% to supportive care. 4) The relative risk of death was 0.26 (P = 0.052) in Group 1, and 0.15 (P = 0.03) in Group 2 in comparison with Group 3. CONCLUSION: The study showed that BMT and combined immunosuppression resulted in better response and survival than supportive care in the management of childhood severe aplastic anemia. Although BMT seemed to be better than immunosuppression, effective measure to reduce late graft failure and graft versus host disease should be pursued.