AIM To describe fatigue in Duchenne muscular dystrophy (DMD) from patients' and parents' perspectives and to explore risk factors for fatigue in children and adolescents with DMD.METHOD A multicentre, cross-sectional study design was used. Seventy-one patients (all males; median age 12y, age range 5-17y) identified via the Canadian Neuromuscular Disease Registry, and their parents completed questionnaires. Subjective fatigue was assessed using the Pediatric Quality of Life Inventory Multidimensional Fatigue Scale by child self-report and parent proxy-report. RESULTSPatients with DMD across ages and disease stages experienced greater fatigue compared to typically developing controls from published data. Sleep disturbance symptoms were associated with greater fatigue by child self-report (q=-0.42; p=0.003) and parent proxyreport (q=-0.51; p<0.001). Depressive symptoms were associated with greater fatigue by child self-report (q=-0.46; p<0.001) and parent proxy-report (q=-0.45; p<0.001). Lower functional ability was associated with greater fatigue by parent proxy-report (q=0.26; p=0.03). Physical activity level, and musculoskeletal, respiratory, and cardiac function were not associated with fatigue.

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Background: To attain the most comprehensive view of the quality of life (QoL) of a child with Duchenne Muscular Dystrophy (DMD), the completion of a pediatric QoL measure by the child and his/her parent and the assessment of QoL and health-related quality of life (HRQoL) as separate constructs is crucial. Previous QoL research has not assessed HRQoL as a separate construct. By using the Quality of My Life (QoML) questionnaire, our objective was to describe QoL and HRQoL in boys with DMD based on child-and parent-reports. Methods: Parent and child dyads identified via the Canadian Neuromuscular Disease Registry received QoML questionnaires (2013-2016). Children and parent-proxy each completed the QoL and HRQoL Visual Analog Scales. Responses were marked on a 10-cm line, with higher scores (max=10) reflecting higher QoL and HRQoL. Descriptive statistics were computed for child- and parent-reports of QoL and HRQoL at three time-points. Results: Mean(SD) QoL and HRQoL scores for child- and parent-reports were: 1) Baseline (n=20 dyads), 8.32(1.72) vs. 6.73(2.23) and 7.63(2.51) vs. 6.73(2.19); 2)18-months (n=10 dyads, n=9 dyads), 7.83(2.05) vs 7.66(1.66) and 7.62(2.41) vs 7.41(2.16); 3) 36-months (n=15 dyads) 7.38(2.00) vs. 6.99(1.77) and 7.19(2.70) vs. 6.76(2.26). Conclusions: Boys with DMD report higher QoL and HRQoL compared to their parents.

LE JOURNAL CANADIEN DES SCIENCES NEUROLOGIQUES Suppl. 2 -S31 opinion from public health nurses and infectious disease specialists. Results: There is currently little consensus about vaccination protocols for patients initiating immunosuppressive therapy. We integrated information from all of our sources to create a preliminary protocol for the vaccination of MS patients prior to initiation of immunosuppressive therapy. Conclusions: More work needs to be done to create standardized vaccination protocols for MS patients who will be undergoing immunosuppressive therapy. We have created a preliminary protocol in conjunction with public health to standardize the vaccinations that MS patients receive. We hope that this will streamline immunization of patients immediately after diagnosis of MS so that initiation of immunosuppressive therapy will not be delayed in the future.

Background: Longitudinal data on health-related quality of life (HRQOL) and fatigue in paediatric Duchenne muscular dystrophy (DMD) are limited. Recently, fatigue was reported to be the greatest predictor of poor HRQOL in paediatric DMD. Understanding the trajectory of HRQOL and its relationship with fatigue may facilitate the development of improved therapeutic strategies. Our objective was to describe three-year changes in HRQOL and fatigue in children with DMD. Methods: Patients identified via the Canadian Neuromuscular Disease Registry received mailed questionnaires (2013–2016). HRQOL was assessed using the PedsQLTM GCS and NMM domains, and fatigue was assessed using the MFS domain (patient- and parent-report). Mean three-year change in scores were computed. Pearson correlations were computed between three-year change in HRQOL and fatigue. Results: Mean decline in MFS scores for patient- and parent-reports were 1.03 and 1.19, respectively. Mean decline in GCS scores for patient- and parent-report were 1.75 and 4.13, respectively. Mean change in NMM scores for patient- and parent-report were 0.72 and -8.36, respectively. Change in MFS score was associated with changes in GCS (r=0.72, p<0.001) and NMM scores (r=0.84, p<0.001) by patient-report. Conclusions: Children with DMD experience worse fatigue and HRQOL over time. Parents perceive a greater decline in HRQOL over time compared to patients.

LE JOURNAL CANADIEN DES SCIENCES NEUROLOGIQUESSuppl. 2 -S15 in Canada, with the goal of facilitating discourse that could lead to national standards for EMG training. Methods: An online survey was distributed to senior neurology and physiatry residents (postgraduate years 3-5), at seven tertiary Canadian centres. The study authors, who are trainees and consultants with a broad range of EMG expertise (junior and senior resident, clinical neuromuscular fellows, senior physiatrist and neuromuscular neurologists), developed pertinent demographic and qualitative questions. Results: Thirty-eight residents completed the survey (23 neurology, 15 physiatry). There was inter-program variation in quantity of the training experience, content of the curriculum, access to expertise (including technologists) and goals for future training and practice. Similarly, differences were identifi ed between the training experiences of neurology and physiatry residents. Conclusions: Inter-program variability in EMG training was identifi ed. Additionally, differences were identifi ed between neurology and physiatry resident training. This data provides evidence of training discrepancies across the country and can be used to establish national training standards for EMG in Canada. D.08 ACT DMD (Ataluren Confi rmatory Trial in Duchenne Muscular Dystrophy): effect of Ataluren on timed function tests (TFT) in nonsense mutation (nm) DMD N Goemans (Leuven) C Campbell (London)* CM McDonald (Sacramento) T Voit (London) X Luo (South Plainfi eld) G Elfring (South Plainfi eld) H Kroger (South Plainfi eld) P Riebling (South Plainfi eld) T Ong (South Plainfi eld) R Spiegel (South Plainfi eld) SW Peltz (South Plainfi eld) K Bushby (Newcastle upon Tyne)doi: 10.1017/cjn.2016.81Background: Ataluren is the fi rst drug to treat the underlying cause of nmDMD. Methods: ACT DMD is a Phase 3, randomized, double-blind study. Males 7-16 years with nmDMD and a screening six-minute walk distance (6MWD) ≥150m and <80%-predicted were randomized to ataluren 40 mg/kg/day or placebo for 48 weeks. A pre-specifi ed subgroup included patients with baseline 6MWD 300-400m. A meta-analysis of the overall ACT DMD population and the 'ambulatory decline phase' subgroup of the Phase 2b study (those patients meeting ACT DMD entry criteria) was pre-specifi ed in the statistical plan. Results: In the overall ACT DMD population (N=228), changes in TFTs favored ataluren over placebo: 10-meter walk/run, -1.2s (p=0.117); 4-stair climb, -1.8s (p=0.058); 4-stair descend, -1.8s (p=0.012). In the pre-specifi ed subgroup (n=99), these differences increased to -2.1s, -3.6s, and -4.3s, respectively, and were statistically signifi cant (p<0.01) for 4-stair climb and descend. Results are supported by the meta-analysis (N=291), which demonstrated significant differences (p<0.05) in 10-meter walk/run, 4-stair climb, 4-stair descend. Conclusions: TFT results showed a benefi t for ataluren in ACT DMD, and a larger treatment effect in the pre-specifi ed baseline 6MWD 300-400m subgroup as well as the...

Background: Predicting epilepsy following a first seizure is difficult. Network abnormalities are observed in patients with epilepsy using resting-state functional MRI (rs-fMRI), which worsen with duration of epilepsy. We use rs-fMRI to identify network abnormalities in patients after a first seizure that can be used as a biomarker to predict development of epilepsy. Methods: Patients after a single, unprovoked seizure and age/sex matched healthy controls underwent 7 Tesla structural and resting-state functional MRI. Data were analyzed using graph theory measures. Patients were followed for development of epilepsy. Results: Nine patients and nine control subjects were analyzed. There were no differences in baseline characteristics. No patients developed epilepsy (average follow-up 3 months). No differences between groups occurred on a whole-brain network level. At a 20% threshold, significant differences occurred in the default mode network (DMN). Patients demonstrated an increased local efficiency (p=0.02) and clustering coefficient (p=0.04), and decreased path length (p=0.02) and betweenness centrality (p=0.02). Conclusions: No whole-brain network changes occur after a single unprovoked seizure. No patient has developed epilepsy suggesting this group does not have network alterations after a single seizure. In the DMN, the alterations noted indicate increased segregation of network function. GP.05CaCN PreSideNt'S Prize Background: Perinatal stroke is the most common cause of hemiparetic cerebral palsy. Post-stroke plasticity is well studied in adults, but mechanisms in children are poorly understood. To better understand the relationship between functional connectivity and disability, we used rsfMRI to compare connectivity with sensorimotor dysfunction. Methods: Subjects with periventricular venous infarction were compared to controls. Resting-state BOLD signal was acquired on 3T MRI and analyzed using SPM12. Functional connectivity was computed between S1 and M1 of the left/non-lesioned and right/ lesioned hemisphere. Primary outcome was connectivity expressed as a Pearson correlation coefficient. Motor function was measured using the Assisting Hand Assessment (AHA), and Melbourne Assessment (MA). Proprioceptive function was measured using a robotic position matching task (VarXY). Results: Subjects included 17 PVI and 21 controls. AHA and MA in patients were negatively correlated with connectivity (increased connectivity=poorer performance). Correlations between AHA and connectivity between non-lesioned M1 to bilateral S1s were significant. VarXY in PVI was inversely correlated with

DESIGN/METHODS: Retrospective review of data collected from infants born < 37 weeks with RDS treated with surfactant replacement therapy between February 1, 2015 and March 1, 2016. Data were analysed to determine the timing of initial surfactant administration, length of time to wean to room air and number of doses required. Infants were sub grouped within gestational age stratum; 23 0 weeks to 27 6 weeks (extreme pretermgroup 1), 28 0 weeks to 31 6 weeks (very preterm-group 2) and 32 0 weeks to 36 6 weeks (group 3). RESULTS: Ninety eight premature infants with RDS were treated with surfactant during the study period. Twenty one infants were excluded due to incomplete data collection (16/21) and ongoing oxygen requirement prior to second dose of surfactant (5/21).Seventy seven infants were analysed; mean gestational age and birth weight were 28 6 weeks (SD 3.5) and 1250 grams (SD 602). Forty infants (52%) were in group 1, 21 (27%) in group 2 and 16 (21%) in group 3.The initial dose of surfactant in group 1 was given at a median time of 29 minutes (IQR=24) after birth compared to 150 minutes (IQR=595) in group 2 and 990 minutes (IQR=1973) in group 3 (c 2 =21.89, p<0.001). Median length of time to wean to room air was 14 minutes (IQR=51) in group 1, 10 minutes (IQR=48) in group 2, and 10 minutes (IQR=33) in group 3 (p=0.88). Only 6% of all infants required repeated doses of BLES, 4 infants in group 1 and 1 infant in group 3. CONCLUSION: Extreme preterm infants received their initial dose of BLES® earlier in their RDS management. Given the rapid response to BLES® in the entire patient population, careful monitoring of ventilator parameters should be made, allowing for rapid weaning and eventual extubation after surfactant administration.