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Antibodies against the myelin oligodendrocyte glycoprotein (MOG-Ab) can be detected in various pediatric acquired demyelinating syndromes (ADS). Here, we analyze the spectrum of neuroradiologic findings in children with MOG-Ab and a first demyelinating event. The cerebral and spinal MRI of 69 children with different ADS was assessed in regard to the distribution and characteristics of lesions. Children with acute disseminated encephalomyelitis (n = 36) or neuromyelitis optica spectrum disorder (n = 5) presented an imaging pattern characterized predominantly by poorly demarcated lesions with a wide supra- and infratentorial distribution. Younger children also tended to have poorly defined and widespread lesions. The majority of patients with an isolated optic neuritis (n = 16) only presented small non-specific brain lesions or none at all. A longitudinally extensive transverse myelitis mainly affecting the cervical, and less often so the thoracic, lumbar, and conus regions, was detected in 31 children. The three children of our cohort who were then finally diagnosed with multiple sclerosis had at onset already demarcated white matter lesions as well as transverse myelitis. In conclusion, children with MOG seropositive ADS present disparate, yet characteristic imaging patterns. These patterns have been seen to correlate to the disease entity as well as to age of symptom onset.

Objective:To assess the prognostic value of MOG antibodies (abs) in the differential diagnosis of acquired demyelinating syndromes (ADS).Methods:Clinical course, MRI, MOG-abs, AQP4-abs, and CSF cells and oligoclonal bands (OCB) in children with ADS and 24 months of follow-up were reviewed in this observational prospective multicenter hospital-based study.Results:Two hundred ten children with ADS were included and diagnosed with acute disseminated encephalomyelitis (ADEM) (n = 60), neuromyelitis optica spectrum disorder (NMOSD) (n = 12), clinically isolated syndrome (CIS) (n = 101), and multiple sclerosis (MS) (n = 37) after the first episode. MOG-abs were predominantly found in ADEM (57%) and less frequently in NMOSD (25%), CIS (25%), or MS (8%). Increased MOG-ab titers were associated with younger age (p = 0.0001), diagnosis of ADEM (p = 0.005), increased CSF cell counts (p = 0.011), and negative OCB (p = 0.012). At 24-month follow-up, 96 children had no further relapses. Thirty-five children developed recurrent non-MS episodes (63% MOG-, 17% AQP4-abs at onset). Seventy-nine children developed MS (4% MOG-abs at onset). Recurrent non-MS episodes were associated with high MOG-ab titers (p = 0.0003) and older age at onset (p = 0.024). MS was predicted by MS-like MRI (p < 0.0001) and OCB (p = 0.007). An MOG-ab cutoff titer ≥1:1,280 predicted a non-MS course with a sensitivity of 47% and a specificity of 100% and a recurrent non-MS course with a sensitivity of 46% and a specificity of 86%.Conclusions:Our results show that the presence of MOG-abs strongly depends on the age at disease onset and that high MOG-ab titers were associated with a recurrent non-MS disease course.

Purpose: To compare therapy-induced reading and distance visual acuity (dVA) increases in neovascular age-related macular degeneration (nAMD) and uveitis-associated cystoid macular edema. Methods: This longitudinal study included 68 treatment-naive eyes: 39 subfoveal nAMD eyes with disrupted photoreceptor layers treated with monthly ranibizumab and 29 uveitis-associated cystoid macular edema eyes with intact photoreceptor layer treated with 1 triamcinolone injection. Patients were examined with high-definition optical coherence tomography, Early Treatment Diabetic Retinopathy Study dVA (logarithm of the minimum angle of resolution), reading acuity (logRADscore), and maximum reading speed (words per minute) over 3 months of therapy. Results: In uveitis-associated cystoid macular edema, logarithm of the minimum angle of resolution and logRADscore improved 1 day post treatment, from 0.49 ± 0.28 to 0.39 ± 0.3 (P = 0.018) and 0.71 ± 0.53 to 0.56 ± 0.49 (P = 0.012), respectively. In nAMD, logarithm of the minimum angle of resolution improved 1 week after anti–vascular endothelial growth factor therapy from 0.59 ± 0.29 to 0.49 ± 0.24 (P = 0.002), with no change in logRADscore. One month after treatment, logRADscore improved from 1.09 ± 0.65 to 0.90 ± 0.60 (P = 0.002). In uveitis-associated cystoid macular edema, the recovery course of reading and dVA was comparable, and in nAMD, reading acuity recovery was delayed. Irrespective of disease, a small reduction in dVA resulted in a larger reading acuity decrease. Conclusion: Cystoid macular edema resolution was associated with rapid synchronous reading and dVA improvement, whereas nAMD was followed by faster recovery of distance than reading acuity. In both conditions, reading acuity expressed by critical angular resolution was more suppressed by active disease and recovered relatively more than distance acuity. These discrepancies indicate that reading acuity might be a more sensitive measure for vision decrease in macular diseases than dVA. Reading acuity seems to be an important adjunct assessing intravitreal therapy efficacy.

Purpose: Quantification of short-term progression of active neovascular age-related macular degeneration and correlation with 1-year outcome. Methods: Sixty-five patients with newly diagnosed treatment-naive active subfoveal choroidal neovascularization (CNV), who had participated in clinical trials testing anti-vascular endothelial growth factor therapy, were retrospectively assessed. Early Treatment Diabetic Retinopathy Study best-corrected visual acuity (BCVA), Spectral Domain Optical Coherence Tomography (SD-OCT) and fluorescein angiography (FA) were performed twice during the pretreatment period. Changes in BCVA, central retinal thickness (CRT), average macular thickness (AMT) and leakage area were documented within this pretreatment period for all patients and for lesion type I (occult CNV, n = 42) and type II (classic CNV, n = 23). Three-month and 1-year BCVA were then correlated with the pretreatment period. Results: The pretreatment period was 19 ± 3 days (range: 2–108). Neither type I nor type II lesions showed a significant BCVA decrease or CRT/AMT increase during this period. On FA, mean leakage area increased significantly during the pretreatment period: in the pooled group from 5.50 ± 0.62 (screening) to 7.60 ± 0.86 mm2 (baseline) (p

Background Myelin oligodendrocyte glycoprotein (MOG) antibodies have been recently described in children with acute disseminating encephalomyelitis (ADEM), but the clinical and neuroradiological characterisation of this subgroup is lacking. Objective To compare the clinical and neuroradiological features of paediatric ADEM with and without MOG antibodies. Methods Clinical course, cerebrospinal fluid (CSF)-, MRI studies, outcome and MOG status of 33 paediatric ADEM prospectively studied were reviewed. Results MOG antibodies (median 1:2560; range 1:160–1:20 480) were detected in 19 children with ADEM. The majority of children showed a decline of serum MOG-IgG titres over time. Children with MOG antibodies did not differ in their age at presentation, sex ratio, the presence of oligoclonal bands, clinical symptoms or initial severity, apart from a higher CSF cell count (p=0.038), compared with children without MOG antibodies. In addition, further relapsing demyelinating episodes associated with MOG antibodies were observed only in children with MOG antibodies. All 19 children with MOG antibodies had a uniform MRI pattern, characterised by large, hazy and bilateral lesions and the absence of atypical MRI features (eg, mainly small lesions, well-defined lesions), which was significantly different compared to that of children without MOG antibodies (p=0.003; and p=0.032, respectively). In addition, children with MOG antibodies had involvement of more anatomical areas (p=0.035) including the myelon characterised by a longitudinally extensive transverse myelitis (p=0.003), more often a complete resolution of lesions (p=0.036) and a better outcome (p=0.038). Conclusions Patients with ADEM with MOG antibodies in our cohort had a uniform MRI characterised by large, bilateral and widespread lesions with an increased frequency of longitudinal extensive transverse myelitis and a favourable clinical outcome in contrast to children lacking MOG antibodies.

Objective: The aim of the study was to investigate whether there is a difference between body fat mass percentage measured by BIA and DXA method. Design: Transversal study, randomized. Setting: Lipid and Obesity Outpatient Clinic, Department of Pediatrics, University of Vienna, Austria. Subjects: Twenty-seven children and adolescents from the Lipid and Obesity Outpatient Clinic, Department of Pediatrics, University of Vienna, were included in the study (14 boys and 13 girls between 6 and 18 y; mean age 12.6 and 13.1 y). Methods: The body fat percentage was measured with BIA (bioelectrical impedance analyzer BIA 2000-M) and DXA (dual energy X-ray absorptiometry) methods on the same day. Results: The mean difference between the body fat mass percentage measured by BIA and DXA was 4.48 with a standard deviation of 2.93. The results measured by BIA were almost always lower than that by DXA by about 12%. The lower and upper limit of the difference in 95% confidence interval was −5.64 and −3.32. By paired t-test, these results were significantly different (P

Facial nerve paralysis is a common disease in children. Most of the patients show complete recovery. This single-center cohort study exclusively included pediatric patients to investigate the outcome of all patients with facial nerve palsy.Hospital records of all the patients admitted to the Children's Hospital in Linz between January 2005 and December 2010 with facial nerve paralysis were reviewed. Patients with peripheral facial nerve palsy were invited for clinical reevaluation between July 2011 and October 2011. The House-Brackmann score was used for reassessment.Fifty-six patients agreed to return for an additional clinical reevaluation. Study participants were divided in two groups according to their House-Brackmann scores: group 1 (n = 44), with a score2 were considered good outcomes, and group 2 (n = 12), with a score ≥ 2 showed persistent mild to moderate dysfunction of the facial nerve and were considered moderate outcomes. The most important finding was the difference of the reported time to remission (P = 0.003) between the groups.The results of this study indicate that facial paralysis in children is not as benign as supposed. It is suggested that patients and their guardians be informed that a slight face asymmetry may persist, but functional recovery in general is excellent.