Hereditary Motor and Sensory Neuropathy Type VI with Bilateral Middle Cerebellar Peduncle Involvement.

Charcot-Marie-Tooth disease (CMT) 2A with optic atrophy is referred to as hereditary motor and sensory neuropathy type VI (HMSN VI) and is caused by mitofusin 2 gene (MFN2) mutation. In patients with MFN2 related CMT, central nervous system is known to be also involved and cerebral white matter is mostly involved. We report a patient confirmed as HMSN VI who had isolated bilateral middle cerebellar peduncular lesions in brain MRI.

Comparison of 1-year therapeutic effect of ranibizumab and bevacizumab for myopic choroidal neovascularization: a retrospective, multicenter, comparative study.

BACKGROUND: To compare the long-term efficacy of ranibizumab versus bevacizumab for myopic choroidal neovascularization (CNV). METHODS: This was a retrospective, multicenter, comparative, non-randomized study of 64 consecutive patients with myopic CNV treated with ranibizumab (22 patients) or bevacizumab (42 patients). Best-corrected visual acuity (BCVA) and central foveal thickness (CFT) on optical coherence tomography were evaluated before and after treatment. All the patients were followed for at least 12 months. RESULTS: BCVA (logarithm of the minimal angle of resolution) improved from 0.63 ± 0.30 to 0.43 ± 0.27, 0.41 ± 0.37, 0.40 ± 0.39, 0.39 ± 0.43, and 0.39 ± 0.42 at 1, 2, 3, 6, and 12 months after treatment in the ranibizumab group, and from 0.67 ± 0.28 to 0.52 ± 0.31, 0.49 ± 0.31, 0.47 ± 0.31, 0.42 ± 0.32, and 0.46 ± 0.43 in the bevacizumab group (all P < 0.05 compared with baseline BCVA in each group). CFT decreased by 20.21%, 19.58%, and 22.43% from the baseline 304 ± 76 µm at 3, 6, and 12 months after treatment in the former group, and by 15.20%, 15.67%, and 15.56% from the baseline 297 ± 62 µm in the latter group (all P < 0.05 compared with baseline CFT in each group). BCVA improvement and CFT reduction did not statistically differ when compared at the same periods from treatment between 2 groups. Neither ocular nor systemic safety problems appeared during follow up. CONCLUSIONS: This study showed a similar functional and anatomical improvement after treatment of ranibizumab and bevacizumab for myopic CNV over a 12-month follow-up period.

Comparative analysis of aqueous humor cytokine levels between patients with exudative age-related macular degeneration and normal controls.

PURPOSE: To investigate the cytokine markers associated with exudative AMD present in aqueous humor. This goal was achieved by comparing the concentrations of more than 500 molecules in aqueous humor, between exudative AMD patients and controls. METHODS: Aqueous humor samples were acquired from 20 patients with exudative AMD and 20 control subjects. Raybio human antibody array technology was used to simultaneously screen for any difference in the expression of any of 507 molecules. To validate the antibody array result, concentrations of insulin-like growth factor binding protein 2 (IGFBP-2), insulin-like growth factor-1 (IGF-1), and VEGF were measured by ELISA. RESULTS: Twenty molecules studied exhibited intergroup differences. Twelve molecules including IGFBP-2, IGFBP-6, IGFBP-7, and glucocorticoid-induced tumor necrosis factor receptor family related gene (GITR) ligand, were detected in high densities in exudative AMD patients. Eight other molecules were present at higher concentrations in control patients. ELISA confirmed that IGFBP-2 levels were higher in patients with exudative AMD (7.47 ± 6.19 ng/mL) in comparison with control subjects (3.07 ± 3.34 ng/mL, P = 0.008). IGF-1 and VEGF levels were also increased in the former group (2.20 ± 0.26 vs. 1.99 ± 0.35 ng/mL, P = 0.040; 122.25 ± 63.24 vs. 86.98 ± 44.41 pg/mL, P = 0.048, respectively). CONCLUSIONS: The pattern of cytokine expression in the aqueous humor of exudative AMD patients varies from that of normal control subjects. The increased levels of IGFBP-2 and IGF-1 in exudative AMD eyes indicate that the altered expression of IGF-related molecules may be involved in disease pathogenesis and suggests potential biomarkers for exudative AMD.

Simple keratectomy and corneal tattooing for limbal dermoids: results of a 3-year study.

AIM: To evaluate and report the efficacy of combined surgical excision and corneal tattooing in patients with limbal dermoids. METHODS: In a retrospective interventional case series, 9 eyes of 8 patients were treated with combined surgery of simple keratectomy and corneal tattooing for limbal dermoids. Medical records, including best-corrected visual acuity, anterior segment photography, demographic, clinical data, and follow-up information were reviewed. RESULTS: The mean follow up period in this study was 50±15(range 36-77) months. There was no evidence of infection or recurrent limbal dermoids in any of the eyes during the follow-up period. All patients achieved good cosmetic outcomes with no complications. CONCLUSION: Simple keratectomy and corneal tattooing of limbal dermoids could be an alternative option for surgery, especially when a donor cornea is not available.

Silent giant cell arteritis in an elderly Korean woman.

Giant cell arteritis (GCA) is a rare disease among Asians. Arteritic anterior ischemic optic neuropathy, which accompanies GCA, has not yet been reported in Koreans. Diagnosis of GCA is difficult if typical symptoms other than visual loss are absent. Here, we report a case of an 83-year-old Korean woman presenting with sudden visual loss in both eyes (oculus uterque, OU). Her visual acuities included perception of light in the right eye (oculus dexter, OD) and perception of hand motion in the left eye (oculus sinister, OS). The results of the Hardy-Rand-Rittler test and Ishihara test showed total dyschromatopsia OU. The Goldmann perimetry test revealed a total field defect OD and paracentral island OS. Fundus examination revealed chalky-white disc swelling OU. Other systemic symptoms and signs were unremarkable. The erythrocyte sedimentation rate, C-reactive protein and platelet count were highly elevated. Temporal artery biopsy revealed multiple lymphocytes and multinucleated giant cells in the arterial media layer. To our knowledge, this is the first report of GCA in a Korean that has been confirmed with temporal artery biopsy. In conclusion, silent GCA can occur in Koreans, and hence, elderly patients presenting with chalky-white disc swelling, and corresponding laboratory findings must be evaluated for GCA.

Intraoperative iatrogenic peripheral retinal break in 23-gauge transconjunctival sutureless vitrectomy versus 20-gauge conventional vitrectomy.

BACKGROUND: To compare the incidence of intraoperative iatrogenic peripheral retinal breaks (IPRBs) during 23-gauge transconjunctival sutureless vitrectomy (TSV) and conventional 20-gauge vitrectomy for various indications. METHODS: This was a single-center, comparative, retrospective, interventional case series of 973 23-gauge TSVs and 402 conventional 20-gauge vitrectomies done by two surgeons between January 2004 and December 2009. The incidence rate of intraoperative IPRBs and risk factors were analyzed in association with various clinical and surgical factors. RESULTS: IPRBs occurred significantly less often during 23-gauge TSV (16 of 973 cases, 1.6 %) than during conventional vitrectomy (25 of 402 cases, 6.2 %, P<0.001). Univariate analysis revealed that conventional vitrectomy and operation time were risk factors for the complication. Multivariate logistic regression analysis also revealed that conventional vitrectomy (P=0.03, OR=2.91), operation time (P<0.01, OR=1.01), and intraoperative induction of posterior vitreous detachment (PVD, P=0.04, OR=1.97) were risk factors for IPRBs. CONCLUSIONS: The 23-gauge TSV procedure with the trocar system has a lower incidence of intraoperative IPRBs than conventional 20-gauge vitrectomy. Longer operation time and induction of PVD are also independent risk factors of the complication.

Myasthenia mimicking monocular elevation deficiency.

Monocular elevation deficiency is the inability to elevate the eye in abduction and adduction. Myasthenia gravis could mimic any pattern of ophthalmoplegia. However, myasthenia gravis mimicking monocular elevation deficiency appears to be very rare. A 12-month-old girl presented with the inability to elevate the left eye and blepharoptosis from 3 months of age. A neostigmine test showed a weakly positive response. Repetitive stimulation of the left orbicularis oculi produced no decremental response. Three assays for acetylcholine receptor antibodies were negative. Placing ice over the lids definitely improved left blepharoptosis. In conclusion, myasthenia gravis could mimic elevation deficiency. An ice test could be very helpful in making the diagnosis. Myasthenia gravis should be included in the differential diagnosis of monocular elevation deficiency even at a young age.

A comparative study of the effect of fibrin glue versus sutures on clinical outcome in patients undergoing pterygium excision and conjunctival autografts.

PURPOSE: To compare the effect of using fibrin glue or 10-0 nylon sutures on the clinical outcome of patients undergoing pterygium excision and conjunctival autografting. METHODS: We retrospectively reviewed the medical records of 52 eyes from 46 patients who underwent pterygium excision and conjunctival autografting and were followed up for more than 3 months. The operation duration, postoperative inflammation, complications, and recurrence rates were compared between groups of 20 patients (22 eyes) for whom fibrin glue was used (fibrin glue group) and 26 patients (30 eyes) for whom suturing was performed with 10-0 nylon (suture group) in pterygium excision and conjunctival autografting. RESULTS: The operation duration was 27.71 (5.22) minutes in the fibrin glue group and 43.30 (8.18) minutes in the suture group (p = 0.000). Seven days after the operation, the fibrin glue group showed milder conjunctival inflammation than the suture group (p = 0.000). Postoperative complications and corneal recurrence rates were not statistically different between the two groups. CONCLUSIONS: The use of fibrin glue in pterygium excision with conjunctival autografting is likely to be a more effective, safer procedure than suturing.

A case of sympathetic ophthalmia presenting with extraocular symptoms and conjunctival pigmentation after repeated 23-gauge vitrectomy.

PURPOSE: To report a case of sympathetic ophthalmia presenting with extraocular symptoms after 23-gauge sutureless vitrectomy. METHODS: Observational case report. RESULTS: A 29-year-old woman underwent 23-gauge sutureless vitrectomy for spontaneous rhegmatogenous retinal detachment in the right eye. After vitrectomy, dense conjunctival pigmentations developed around the sclerotomy sites. One month after the second vitrectomy for a recurred retinal detachment, she presented with decreased visual acuity and metamorphopsia in both eyes, headache, tinnitus, and facial swelling. She was diagnosed with sympathetic ophthamia and treated with oral prednisolone and cyclosporine. Her visual acuity and symptoms improved after treatment. CONCLUSION: Sympathetic ophthalmia can occur after 23-gauge sutureless vitrectomy and conjunctival pigmentation may be the preceding sign.