bluuebunny/crossref_metadata_2025_split huggingface dataset:
Gamma Knife radiosurgery for vestibular schwannomas: evaluation of tumor control and its predictors in a large patient cohort in The Netherlands
Authors: Klijn Stijn , Verheul Jeroen B. , Beute Guus N. , Leenstra Sieger , Mulder Jef J. S. , Kunst Henricus P. M. , Hanssens Patrick E. J. 
Published: October 2015

OBJECT
The authors of this study sought to assess tumor control and complication rates in a large cohort of patients who underwent Gamma Knife radiosurgery (GKRS) for vestibular schwannoma (VS) and to identify predictors of tumor control.

METHODS
The records of 420 patients treated with GKRS for VS with a median marginal dose of 11 Gy were retrospectively analyzed. Patients with neurofibromatosis Type 2 or who had undergone treatment for VS previously were excluded. The authors assessed tumor control and complication rates with chart review and used the Cox proportional hazards model to identify predictors of tumor control. Preservation of serviceable hearing, defined as Gardner-Robertson Class I–II, was evaluated in a subgroup of 71 patients with serviceable hearing at baseline and with available follow-up audiograms.

RESULTS
The median VS tumor volume was 1.4 cm3, and the median length of follow-up was 5.1 years. Actuarial 5-and 10-year tumor control rates were 91.3% and 84.8%, respectively. Only tumor volume was a statistically significant predictor of tumor control rate. The tumor control rate decreased from 94.1% for tumors smaller than 0.5 cm3 to 80.7% for tumors larger than 6 cm3. Thirteen patients (3.1%) had new or increased permanent trigeminal nerve neuropathy, 4 (1.0%) had new or increased permanent facial weakness, and 5 (1.2%) exhibited new or increased hydrocephalus requiring a shunting procedure. Actuarial 3-year and 5-year hearing preservation rates were 65% and 42%, respectively.

CONCLUSIONS
The 5-year actuarial tumor control rate of 91.3% in this cohort of patients with VS compared slightly unfavorably with the rates reported in other large studies, but the complication and hearing preservation rates in this study were similar to those reported previously. Various factors may contribute to the observed differences in reported outcomes. These factors include variations in treatment indication and in the definition of treatment failure, as well as a lack of standardization of terminology and of evaluation of complications. Last, differences in dosimetric variables may also be an explanatory factor.
Importance of RNF213 polymorphism on clinical features and long-term outcome in moyamoya disease
Authors: Kim Eun-Hee , Yum Mi-Sun , Ra Young-Shin , Park Jun Bum , Ahn Jae Sung , Kim Gu-Hwan , Goo Hyun Woo , Ko Tae-Sung , Yoo Han-Wook 
Published: October 2015

OBJECT
Moyamoya disease (MMD) is an idiopathic cerebrovascular occlusive disorder prevalent in East Asia. In the pathogenesis of MMD, the important role of genetic factors is being elucidated, and RNF213 has recently been identified as a susceptibility gene for MMD. The aim of this retrospective study was to investigate the RNF213 genotype in patients with MMD and to determine their genotype-phenotype associations.

METHODS
The study involved 165 Korean MMD patients from 155 unrelated families who were diagnosed with MMD at a single center from 1995 to 2013. Their demographic, radiological, and clinical findings were evaluated. Direct sequencing of the major RNF213 single nucleotide polymorphisms was performed. The association of the common RNF213 variant with MMD risk was evaluated using historical controls for comparison. Correlations between RNF213 genotype and phenotype were statistically analyzed.

RESULTS
The c.14429G>A (p.R4810K) variant was identified in 125 (75.8%) of 165 MMD patients. Most patients (112) were heterozygous, and 13 patients had 2 copies of the c.14429G>A variant. A novel heterozygous variant, c.12086A>G (p.Q4029R), was found in 1 additional patient. The minor allele frequency of the c.14429G>A variant was significantly higher in the MMD group (138 [41.8%] of 330 patients) than in the control group (8 [1.36%] of 588 subjects; p < 0.001). The c.14429G>A (p.R4810K) variant significantly increased the risk of MMD in Korean patients, with an OR of 52.11 (p < 0.001) compared with controls. Moreover, c.14429G>A (p.R4810K) genotypes occurred more frequently in patients with a family history of MMD. The homozygous variant was highly associated with early-onset MMD (age at onset < 5 years), cerebral infarction at diagnosis, and cognitive impairment in long-term outcome.

CONCLUSIONS
The findings indicate that the c.14429G>A (p.R4810K) allele of RNF213 is strongly associated with Korean patients with MMD. The homozygous c.14429G>A (p.R4810K) variant is particularly related to early-onset MMD, severe symptomatic manifestations at diagnosis, and poor prognosis. This genotypic variant may be a useful biomarker for early-onset MMD or unstable MMD with cerebral infarction, which requires early diagnosis and revascularization treatment.
Quality of survival the 1st year with glioblastoma: a longitudinal study of patient-reported quality of life
Authors: Sagberg Lisa Millgård , Solheim Ole , Jakola Asgeir S. 
Published: October 2015

OBJECT
By exploring longitudinal patient-reported health-related quality of life (HRQoL), the authors sought to assess the quality of survival for patients in the 1st year after diagnosis of glioblastoma.

METHODS
Thirty unselected patients ≥ 18 years who underwent primary surgery for glioblastoma in the period 2011–2013 were included. Using the generic HRQoL questionnaire EQ-5D 3L, baseline HRQoL was assessed before surgery and at postoperative follow-up after 1, 2, 4, 6, 8, 10, and 12 months.

RESULTS
There was an apparent correlation between deterioration in HRQoL scores and tumor progression. Patients with permanent deterioration in HRQoL early after surgery represented a subgroup with rapid progression and short survival. Both positive and negative changes in HRQoL were more often seen after surgery than after radio- or chemotherapy. Patients with gross-total resection (GTR) reported better and more stable HRQoL. In a multivariable analysis preoperative cognitive symptoms (p = 0.02), preoperative functional status (p = 0.03), and GTR (p = 0.01) were independent predictors of quality of survival (area under the curve for EQ-5D 3L index values).

CONCLUSIONS
The results indicate that progression-free survival is not only a surrogate marker for survival, but also for quality of survival. Quality of survival seems to be associated with GTR, which adds further support for opting for extensive resections in glioblastoma patients with good preoperative functional levels.
Health-related quality of life in pediatric Chiari Type I malformation: the Chiari Health Index for Pediatrics
Authors: Ladner Travis R. , Westrick Ashly C. , Wellons John C. , Shannon Chevis N. 
Published: October 2015

OBJECTThe purpose of this study was to design and validate a patient-reported health-related quality of life (HRQOL) instrument for pediatric Chiari Type I malformation (CM-I), the Chiari Health Index for Pediatrics (CHIP).METHODSThe CHIP has 45 items with 4 components making up 2 domain scores, physical (pain frequency, pain severity, nonpain symptoms) and psychosocial; physical and psychosocial scores are combined to create an overall HRQOL score. Increasing scores (0 to 1) represent increasing HRQOL. Fifty-five patients with CM-I (mean age 12 ± 4 years, 53% male) were enrolled and completed the CHIP and Health Utilities Index Mark 3 (HUI3). Twenty-five healthy controls (mean age 11.9 ± 4 years, 40% male) also completed the CHIP. CHIP scores were compared between these groups via the Mann-Whitney U-test. For CHIP discriminative function, subscore versus presence of CM-I was compared via receiver operating characteristic curve analysis. CHIP scores in the CM-I group were stratified by symptomatology (asymptomatic, headaches, and paresthesias) and compared via Kruskal-Wallis test with Mann-Whitney U-test with Bonferroni correction (p < 0.0167). CHIP was compared with HUI3 (Health Utilities Index Mark 3) via univariate and multivariate linear regression.RESULTSCHIP physical and psychosocial subscores were, respectively, 24% and 18% lower in CM-I patients than in controls (p < 0.001); the overall HRQOL score was 23% lower as well (p < 0.001). The area under the curve (AUC) for CHIP physical subscore versus presence of CM-I was 0.809. CHIP physical subscore varied significantly with symptomatology (p = 0.001) and HUI3 pain-related quality of life (R2 = 0.311, p < 0.001). The AUC for CHIP psychosocial subscore versus presence of CM-I was 0.754. CHIP psychosocial subscore varied significantly with HUI3 cognitive- (R2 = 0.324, p < 0.001) and emotion-related (R2 = 0.155, p = 0.003) quality of life. The AUC for CHIP HRQOL versus presence of CM-I was 0.820. Overall CHIP HRQOL score varied significantly with symptomatology (p = 0.001) and HUI3 multiattribute composite HRQOL score (R2 = 0.440, p < 0.001).CONCLUSIONSThe CHIP is a patient-reported, CM-I-specific HRQOL instrument, with construct validity in assessing pain-, cognitive-, and emotion-related quality of life, as well as symptomatic features unique to CM-I. It holds promise as a discriminative HRQOL index in CM-I outcomes assessment.
Rapid-onset paraparesis and quadriparesis in patients with intramedullary spinal dermoid cysts: report of 10 cases
Authors: Girishan Shabari , Rajshekhar Vedantam 
Published: October 2015

OBJECTIntramedullary dermoid cysts are rare tumors of the spinal cord. Presentation with rapid onset of paraparesis or quadriparesis (onset within 2 weeks) is rarer still. The authors present their experience in the management and outcome of patients with such a presentation.METHODSPatient records between 2000 and 2014 were retrospectively reviewed to identify those with intraspinal dermoid cysts who presented with rapid-onset paraparesis or quadriparesis. Their clinical, radiological, operative, and follow-up data were analyzed.RESULTSOf a total of 50 patients with intraspinal dermoid cysts managed during the study period, 10 (20%) presented with rapid-onset paraparesis or quadriparesis; 9 patients ranged in age from 8 months to 2 years, and 1 patient was 25 years old. A dermal sinus was seen in the lumbar region of 4 patients, the sacral region of 3, and the thoracic region of 1, and in 1 patient no sinus was found. All except 1 patient presented with rapid-onset paraparesis secondary to infection of the intramedullary dermoid cyst. One patient presented with rupture of a dermoid cyst with extension into the central canal up to the medulla. Early surgery was done soon after presentation in all except 2 patients. Among the 9 patients who underwent surgery (1 patient did not undergo surgery), total excision of the intramedullary dermoid cyst was done in 3 patients, near-total excision in 4 patients, and partial excision in 2 patients. Of the 9 patients who underwent surgery, 8 showed significant improvement in their neurological status, and 1 patient remained stable. The 1 patient who did not undergo surgery died as a result of an uncontrolled infection after being discharged to a local facility for management of wound infection.CONCLUSIONSEarly recognition of a dermal sinus and the associated intraspinal dermoid cyst and timely surgical intervention can eliminate the chances of acute deterioration of neurological function. Even after an acute onset of paraparesis or quadriparesis, appropriate antibiotic therapy and prompt surgery can provide reasonably good outcomes in these patients.
Atypical pediatric ganglioglioma is common and associated with a less favorable clinical course
Authors: Patibandla Mohana Rao , Ridder Thomas , Dorris Kathleen , Torok Michelle R. , Liu Arthur K. , Handler Michael H. , Stence Nicholas V. , Fenton Laura Z. , Hankinson Todd C. 
Published: October 2015

OBJECTGanglioglioma (GG) is commonly recognized as a low-grade tumor located in the temporal lobe, often presenting with seizures. Most are amenable to complete resection and are associated with excellent oncological outcome. The authors encountered several GGs in various locations, which seem to have a less favorable clinical course than GGs in the temporal lobe.METHODSThe authors performed a single-center retrospective review of all children with a histological diagnosis of GG who were treated at Children’s Hospital Colorado between 1997 and 2013. Each tumor was categorized by 2 pediatric neuroradiologists as typical or atypical based on preoperative MRI appearance. Typical lesions were cortically based, within a single cerebral lobe, well-circumscribed, and solid or mixed solid/cystic. The treatment and clinical course of each patient was analyzed.RESULTSThirty-seven children were identified, with a median age at presentation of 8.2 years and median follow-up of 38.0 months. Eighteen tumors (48.6%) were typical and 19 (51.4%) were atypical. All typical lesions presented with seizures, whereas no atypical lesions did so. Sixteen (88.9%) typical lesions were located in the temporal lobe. In the atypical group, tumor location was variable, including 11 (57.9%) in the brainstem. Death during follow-up was statistically more common in the atypical group (31.6% vs 0%, p = 0.02). Gross-total resection (GTR) was achieved for 15 of 16 typical tumors (93.8%), compared with 3 atypical tumors (15.8%, p < 0.0001). Presentation with seizure or non-brainstem location were each associated with survival (p = 0.02 and 0.004, respectively). The presence of mutation in BRAF exon 15 did not differ between the 2 groups.CONCLUSIONSPediatric GG with typical imaging features is associated with excellent rates of GTR and overall survival. Atypical GG is commonly encountered, less amenable to GTR, and associated with a worse outcome. This may relate to anatomical or biological characteristics and merits further investigation.
Fragility Fractures in Patients with Rheumatoid Arthritis and Osteoarthritis Compared with the General Population
Authors: Yamamoto Yuri , Turkiewicz Aleksandra , Wingstrand Hans , Englund Martin 
Published: October 2015

Objective.To determine the rate ratios of hip and distal radius fractures in patients with rheumatoid arthritis (RA), hip osteoarthritis (OA), and knee OA.Methods.Cohort study using healthcare data (1998–2012) covering the entire population of the Skåne region of Sweden.Results.We found an increased rate of hip fracture in both female [standardized fracture rate ratio (SFR) 1.54, 95% CI 1.40–1.70] and male patients with RA (SFR 1.81, 95% CI 1.51–2.17). The hip fracture rate in female OA was reduced by 10–20%, and trochanteric fracture tended to have a higher rate ratio compared with the cervical.Conclusion.The 50–80% increased rate of hip fracture adds to the total burden of RA while the shifted distribution of cervical/trochanteric fractures in OA is in support of subchondral bone alterations.
Choroidal Thickness in Eyes With Central Geographic Atrophy Secondary to Stargardt Disease and Age-Related Macular Degeneration
Authors: Nunes Renata Portella , Rosa Potyra R. , Giani Andrea , Goldhardt Raquel , Thomas Benjamin , Filho Carlos Alexandre Garcia , Gregori Giovanni , Feuer William , Lam Byron L. , Staurenghi Giovanni , Rosenfeld Philip J. 
Published: October 2015

BACKGROUND AND OBJECTIVE:
Choroidal thickness (CT) measurements from eyes with similar areas of macular geographic atrophy (GA) secondary to age-related macular degeneration (AMD) and Stargardt disease (STGD) were compared to determine whether GA from different diseases had a similar or different effect on the underlying subfoveal choroid.

PATIENTS AND METHODS:
Eyes with the diagnosis of central GA secondary to STGD and AMD were matched, with subfoveal CT measurements obtained from the central B-scan using an enhanced depth imaging protocol. The area of GA was measured using fundus autofluorescence (FAF) imaging. AMD eyes were divided into those with and without reticular pseudodrusen.

RESULTS:

A total of 22 eyes of 22 patients were included in the STGD and AMD groups and were matched with respect to the area of GA. The mean age of the STGD patients was 48.9 years (standard deviation [SD] = 17.1), and the mean age was 81.8 years (SD = 6.2) for the AMD patients. Mean area measurements of GA for the STGD and AMD groups were 5.4 mm
2
(SD = 4.1) and 5.1 mm
2
(SD = 4.0), respectively (
P
= .83). After adjusting for age and axial length, eyes with STGD had a mean CT measurement greater than the AMD eyes (336.1 µm vs. 198.1 µm, respectively;
P
= .039). However, this difference was driven by AMD eyes with reticular pseudodrusen (RPD) and by a single Stargardt case with a very thick choroid. Eyes with RPD had statistically thinner subfoveal CT measurements when compared with all other groups.

CONCLUSION:
A small but statistically significant increase in the CT of STGD eyes was observed when compared with normal controls and AMD eyes without RPD. However, this small increase in CT was driven by a single case with a markedly thicker choroid within the STGD group, so it is unlikely that a clinically significant difference exists. However, AMD eyes with GA and RPD had significantly thinner subfoveal CT measurements.

[
Ophthalmic Surg Lasers Imaging Retina
. 2015;46:814–822.]
A Central Hyporeflective Subretinal Lucency Correlates With a Region of Focal Leakage on Fluorescein Angiography in Eyes With Central Serous Chorioretinopathy
Authors: Yannuzzi Nicolas A. , Mrejen Sarah , Capuano Vittorio , Bhavsar Kavita V. , Querques Giuseppe , Freund K. Bailey 
Published: October 2015

BACKGROUND AND OBJECTIVE:
To correlate the appearance of a hyporeflective lucency on spectral-domain optical coherence tomography (SD-OCT) with a focal leak on fluorescein angiography (FA) in eyes with central serous chorioretinopathy (CSC).

PATIENTS AND METHODS:
Multimodal imaging of 18 patients with CSC who had hyperreflective fibrin surrounding a hyporeflective lucency on SD-OCT was analyzed to investigate any potential correlation with an active leak on FA. The lucent area was evaluated using en face imaging and followed for resolution of the active leak.

RESULTS:
High-resolution SD-OCT images of the lucency were found to correlate with the active leak. In certain cases, the lucent area could be visualized as communicating with a defect in a pigment epithelial detachment. En face imaging of the lucency revealed a smoke-stack appearance, and resolution of the leak correlated with the disappearance of the lucency on SD-OCT.

CONCLUSION:
Visualization of a lucency within surrounding fibrin may suggest an active leak. En face imaging of the lucency may provide insight into the pathophysiology of the smoke-stack leak on FA.

[
Ophthalmic Surg Lasers Imaging Retina
. 2015;46:832–836.]
Randomized Clinical Trial to Compare Micropulse Photocoagulation Versus Half-dose Verteporfin Photodynamic Therapy in the Treatment of Central Serous Chorioretinopathy
Authors: Kretz Florian T.A. , Beger Isabell , Koch Frank , Nowomiejska Katarrzyna , Auffarth Gerd U. , Koss Michael J. 
Published: October 2015

BACKGROUND AND OBJECTIVE:
To evaluate subthreshold diode-laser micropulse (SDM) versus half-dose verteporfin photodynamic therapy (hd-PDT) in central serous chorioretinopathy (CSC).

PATIENTS AND METHODS:
62 eyes of 62 patients were prospectively followed for changes in fluorescein angiography (FA), fundus autofluorescence (FAF), central macular thickness (CMT), best-corrected visual acuity (BCVA), and contrast visual acuity (CVA) after SDM (n = 20) or hdPDT (n = 24). CSC observation served as control group (n = 18).

RESULTS:
Both treatment groups (60% SDM vs. 66.7% hdPDT) showed significant improvement in reduction of leakage activity compared to the control group (37.5%) at 16 weeks. CMT decreased by 69.7 µm (SDM), 109.8 µm (hdPDT), and 89 µm (control). BCVA improved by +6.7 (SDM group), +8.5 (hdPDT), and +1.5 ETDRS letters (control). CVA was best improved in the hdPDT group. No secondary RPE alterations could be detected by FAF after any intervention.

CONCLUSION:
In comparison to the control group, hdPDT and SDM resulted in reduced leakage activity in FA and enhanced photopic and scotopic visual acuity in patients with CSC.

[
Ophthalmic Surg Lasers Imaging Retina
. 2015;46:837–843.]
Clinical Experience with the WIOL-CF Accommodative Bioanalogic Intraocular Lens: Czech National Observational Registry
Authors: Studeny Pavel , Krizova Deli , Urminsky Juraj 
Published: October 2015

Purpose To assess efficacy and patient satisfaction after cataract surgery and implantation of a new accommodative bioanalogic intraocular lens (IOL). Methods We evaluated the collected data of 48 patients with bilateral cataract surgery and Wichterle IOL (WIOL) implantation included in the Czech national observational registry. Monocular and binocular uncorrected distance visual acuity (UDVA), uncorrected intermediate visual acuity, uncorrected near visual acuity (UNVA), best spectacle-corrected visual acuity for distance (CDVA), best spectacle-corrected visual acuity for near (CNVA), distance-corrected near visual acuity (DCNVA), and distance-corrected intermediate visual acuity were evaluated 6 months after surgery. Subjective patient satisfaction was assessed at 3 months postoperatively. Results The mean monocular UDVA was 0.074 ± 0.108 logMAR, the mean monocular CDVA was 0.047 ± 0.125 logMAR, the mean monocular UNVA was 0.328 ± 0.146 logMAR, the mean monocular DCNVA was 0.339 ± 0.131 logMAR, and the mean monocular CNVA was 0.139 ± 0.107 logMAR. A total of 24 patients (50%) had no problems with any light phenomena. A total of 18 patients were very satisfied (37.5%), 11 were satisfied (22.9%), 15 (31.2%) were rather satisfied, 4 (8.3%) were rather dissatisfied, and 0 were dissatisfied. Conclusions The WIOL–continuous focus polyfocal lens offers very good vision at far and intermediate distance comparable with other types of multifocal IOLs, and relatively good near vision, while the incidence of adverse side phenomena is relatively low.
Intravitreal Anti-VEGF Therapy for Macular Radiation Retinopathy: A 10-Year Study
Authors: Finger Paul T. , Chin Kimberly J. , Semenova Ekaterina A. 
Published: October 2015

Purpose To report long-term experience with intravitreal anti–vascular endothelial growth factor treatment for radiation maculopathy. Methods From 2005-2015, 120 consecutive patients underwent intravitreal anti-VEGF therapy for radiation maculopathy. Inclusion criteria included a diagnosis of uveal melanoma treated with plaque radiotherapy and subsequent macular radiation vasculopathy (exudate, retinal hemorrhage, intraretinal microangiopathy, neovascularization, edema). Anti-VEGF therapy involved continuous injections in 4- to 12-week intervals with doses of 1.25 mg/0.05 mL, 2.0 mg/0.08 mL, 2.5 mg/0.1 mL, or 3.0 mg/0.12 mL of bevacizumab as well as 0.5 mg/0.05 mL or 2.0 mg/0.05 mL of ranibizumab. Goals were maintenance of visual acuity and normative macular anatomy. Safety and tolerability (retinal detachment, hemorrhage, infection), visual acuity, central foveal thickness on optical coherence tomography imaging, and clinical features of radiation maculopathy were analyzed. Results Progressive reductions in macular edema, hemorrhages, exudates, cotton-wool spots, and microangiopathy were noted. At last follow-up, 80% remained within 2 lines of their initial visual acuity or better, with a mean treatment interval of 38 months (range 6-108 months). Kaplan-Meier analysis of the probability of remaining within 2 lines of initial visual acuity was 69% at 5 years and 38% at 8 years of anti-VEGF therapy. Discontinuation of therapy was rare. Relatively few acute or long-term side effects were noted, allowing for good long-term patient accrual. Conclusions Continuous intravitreal anti-VEGF therapy in patients with radiation maculopathy was well-tolerated and preserved vision. In most cases, reductions or resolution of retinal hemorrhages, cotton-wool spots, and retinal edema were noted for up to 10 years.