Vigabatrin and epilepsy: lessons learned.

PURPOSE: The risk factors for visual field loss attributable to vigabatrin (VAVFL) are equivocal. This multinational, prospective, observational study aimed to clarify the principal/major factors for VAVFL. METHODS: Interim analysis of three groups with refractory partial epilepsy, stratified by age (8-12 years; >12 years) and exposure to vigabatrin (VGB). Group I comprised participants treated with VGB for >or=6 months, Group II participants previously treated with VGB for >or=6 months who had discontinued the drug for >or=6 months and Group III those never treated with VGB. Perimetry was undertaken at least every six months, for up to 36 months; results were evaluated masked to drug exposure. RESULTS: Based upon 563 participants in the locked data set, 432 yielded one or more Conclusive visual field examinations. For Group I, the frequency of VAVFL at the last Conclusive examination was 10/32 (31.2%) for those aged 8-12 years and 52/125 (41.6%) for those aged >12 years. For Group II, the proportions were 4/39 (10.3%) and 31/129 (24.0%). No cases resembling VAVFL manifested in Group III. VAVFL was associated with duration of VGB therapy (Odds ratio [OR] 14.2; 95% CI 5.0 to 40.5); mean dose of VGB (OR 8.5; 95% CI 2.2 to 33.2); and male gender (OR 2.1; 95% CI 1.2 to 3.7). VAVFL was more common with static than kinetic perimetry (OR 2.3, 95% CI 1.3 to 4.2). CONCLUSIONS: The therapeutic benefit of VGB is counteracted by the progressive accrual of the risk of VAVFL with continued exposure and with increase in mean dose.

Chlamydia psittaci infection and clinicopathologic analysis of ocular adnexal lymphomas in Korea.

Ocular adnexal lymphoma (OAL) is a mostly extranodal marginal zone lymphoma (EMZL). Recent findings have suggested an association between Chlamydia psittaci (Cp) infection and OAL. We sought to confirm this issue and to analyze the clinicopathologic characteristics of OAL in Korea. Between 1993 and 2004, 33 OAL cases were identified at the Asan Medical Center, Seoul, Korea. DNA was extracted from paraffin-embedded tissues, and touchdown enzyme time release polymerase chain reaction was performed to identify three Chlamydia species (Cp, C. tracomatis, and C. pneumoniae). The same procedures were also performed in 21 samples from patients with non-neoplastic ocular adnexal disease (NNOAD). All OAL cases were EMZL. Cp DNA was detected in 26/33 (79%) OAL samples compared with 5/21 (23%) NNOAD samples (P < 0.001). With a median follow-up of 38.5 months (range: 1-105 months), the 5-year progression-free survival (PFS) and overall survival (OS) rates of OAL patients were 72% and 93%, respectively. Clinicopathologic characteristics, recurrence rate, PFS, and OS were not associated with Cp infection. Our study demonstrates an association between OAL and Cp infection in Korea, suggesting that Cp plays a role as a causative antigen in Korean OAL patients.

New concept in cavernous sinus dural arteriovenous fistula: correlation with presenting symptom and venous drainage patterns.

BACKGROUND AND PURPOSE: An extradurally located cavernous sinus dural arteriovenous fistula (CSDAVF) exhibits different clinical behavior from other dural arteriovenous fistulas (DAVFs) located between 2 dural leaves. The aim of this study is to define angiographic types of CSDAVF associated with presenting symptom (Sx) and venous drainage patterns. METHODS: CSDAVFs during a mean of 23-month follow-up period of 58 patients (17 to 73 years, male:female ratio=8:50) were retrospectively analyzed. The 3 types of CSDAF, ie, proliferative (PT), restrictive (RT), and late restrictive (LRT) types, were categorized by the degrees and patterns of prominent arteriovenous shunt as well as venous flow. The status of the venous connection with CS and the presenting Sx patterns classified as orbital (OrbSxP), ocular (OcuSxP), cavernous (CavSxP), and cerebral (CerSxP) were associated with angiographic types as well as symptom onset, age, and gender. Correlations of discrete and categorical variables were statistically analyzed using the chi2 or Fisher exact test. RESULTS: PT (n=23) and RT (n=23) of CSDAVF were more common than LRT (n=12) (P=0.016) in patients with younger than 65 years and were related to OrbSxP (P=0.015) and CavSxP (P=0.038) in contrast to LRT to OcuSxP (P=0.004). Early onset of Sxs was related to the OrbSxP (P=0.08) and CavSxP (P<0.001). CerSxP (5%) was noted in RT or LRT. OrbSxP was related to the superior ophthalmic venous drainage (P=0.026) and CavSxP to the inferior petrosal sinus (P=0.046) and posterior fossa venous drainages (P=0.014). Seven patients revealed chronological progression of CSDAVF from PT to LRT and even to complete healing. CONCLUSIONS: CSDAVF presents as 3 distinctive angiographic types and is associated with presenting Sxs and venous drainage patterns.

Isolated superior rectus palsy due to contralateral midbrain infarction.

BACKGROUND: Isolated superior rectus palsy due to a contralateral midbrain lesion has not been reported. CASE DESCRIPTION: A 71-year-old woman suddenly developed diplopia. Examination showed that she had isolated superior rectus paresis. Magnetic resonance imaging showed a tiny infarct at the area of the oculomotor nucleus on the contralateral side. CONCLUSION: Isolated superior rectus palsy may be caused by a contralateral midbrain lesion that selectively involves crossing superior rectus nerve fibers.

Clinicopathologic analysis of ocular adnexal lymphomas: extranodal marginal zone b-cell lymphoma constitutes the vast majority of ocular lymphomas among Koreans and affects younger patients.

The majority of ocular adnexal lymphomas (OAL) are primary marginal zone B-cell lymphomas (MALT lymphomas). The present study correlated the clinicopathological variables with the histologic subtypes by World Health Organization (WHO) classification with emphasis on MALT lymphomas in OALs of Koreans. There were 68 cases (31 males and 37 females), with a mean age of 45.9 years (range 7-89 years). Histologically, 61 MALT-type, 2 diffuse large B-cell (DLBCL), 2 mantle cell type (MCL), 1 anaplastic large-cell (ALCL), and 2 NK/T-cell lymphomas (NK/T-L) were counted among them. Fifty-seven were primary cases (P-OAL), and 11 were secondary cases (S-OAL). Nearly all P-OALs were MALT lymphomas (n = 56, 98%), with an exception of 1 MCL. Eleven S-OALs included 5 MALT type, 2 DLBCL, 1 ALCL, 1 MCL, and 2 NK/T-L. All MALT lymphoma patients were alive (n = 59) except for 2 after a mean duration of follow-up of 27.6 months (range: 0-108 months): one died of an unrelated cause and one died of recurrence. One non-MALT type P-OAL was alive with no evidence of disease (42 months). Of the 11 S-OAL, 4 had marrow involvement and 5 had progression or relapse outside the orbit. Compared with the other subtypes, MALT lymphoma was more likely to present with local disease (P = 0.001), achieve complete remission (CR) (0.022), and be alive at last follow-up (0.197), and less likely to experience recurrence (P = 0.06). In conclusion, OALs in Koreans are characterized by a preponderance of primary lymphomas over systemic lymphomas, striking predominance of MALT type lymphomas, and young age of occurrence. Histologic subtype by WHO classification has a significant correlation with the final outcome, with the most favorable outcome associated with OALs of the MALT type.

Nonsurgically placed nasolacrimal stents for epiphora: long-term results and factors favoring stent patency.

PURPOSE: To evaluate long-term effectiveness of a polyurethane stent in lacrimal system treatment. MATERIALS AND METHODS: Fluoroscopically guided placement of a polyurethane lacrimal stent was evaluated in 727 obstructed lacrimal systems in 588 patients with a mean follow-up period of 77 weeks (range, 1-277 wk), from which the technical and initial success rates and long-term patency were calculated. The impact of factors affecting long-term patency, including symptom duration, previous probing, presence of pus or eye discharge, lesion multiplicity, and site of obstruction were evaluated. Changes in 295 patients' lacrimal sac configuration after stent removal were analyzed and classified into three categories: contraction, widening, and no change. RESULTS: The overall technical success rate in this study was 95.9%, with a 90.8% initial clinical success rate. The median primary patency duration was 504.33 days plus minus 23.17; secondary median patency duration was 642.87 days plus minus 24.68. Most favorable factors such as symptom duration, previous probing, presence of pus, or eye discharge showed no significant difference; however, site of obstruction and lesion multiplicity influenced mean patency. Lesions below the junction and single lesions showed longer patency periods. Common canalicular obstruction after stent removal occurred in 50 (17%) lacrimal systems. Irregular sac configuration appeared in 171 (58%) lacrimal systems after stent removal. Sac configuration was the same in 185 (62.7%) lacrimal systems, contracted in 105 (35.6%), and widened in five (1.7%). In 151 eyes of 295, epiphora recurred 121.6 days (range, 1-1,182 d) after stent removal. CONCLUSION: Fluoroscopic lacrimal stent placement is a simple and safe outpatient procedure, but high recurrence and sac irregularities after stent removal are not encouraging.