Objective: The North Star Ambulatory Assessment (NSAA) is a validated 17-item functional rating scale and widely used to assess motor function in boys with Duchenne muscular dystrophy (DMD). The SARS-CoV-2 pandemic and subsequent Government ‘lockdown’ resulted in no face-to-face clinic visits hence the motor abilities were not monitored. The aim was to investigate whether the NSAA was feasible and reliable by video assessment. Method: Ten ambulant DMD boys were selected from the electronic hospital records. Two physiotherapists scored the boys’ NSAA independently and the intraclass correlation coefficient was used to assess agreement. The video scores were compared to two previous NSAA in-clinic scores. Results: Mean scores (SD) for clinic visit one were 22.6 (4.19) and clinic visit two 21.8 (5.3). The two physiotherapists video mean scores were 20.6 (5.66) for physiotherapist 1 and 20.6 (6.53) for physiotherapist 2. The intraclass correlation coefficient was 0.98 (95% CI 0.93–1.00) for the total NSAA and 1.00 (95% CI 1.00 to 1.00) for the rise time. The mean decline in score from clinic visit one (−12 months) to video assessment was 2.0 (2.8SD). Conclusion: The results from the study suggest that video NSAA is partially feasible and reliable.
Spinal cord injuries in new born infants following a traumatic delivery or umbilical cord catheterisation due to thromboembolism are well known. Cases with atraumatic acute onset of neonatal paraplegia have also been described in preterm babies or babies born small for gestational age with a stormy postnatal course related to ischaemic aetiology. We describe a rare case of infarction of the spinal cord from a predominant haemorrhagic aetiology. A term female baby, first child of unrelated parents, was born by normal vaginal delivery. She had meconium aspiration at birth, leading to severe respiratory distress, requiring neonatal intensive care admission. At 2 weeks, she developed new flaccid paraplegia. MRI scan of the spine showed haemorrhagic infarction of the spinal cord from the level of thoracic inlet, vertebral level C7–T1. A follow-up MRI scan at 11 months revealed severe atrophy of the cord distal to C6. At 3 years of age, she had good upper-limb function, diaphragmatic breathing and flaccid paralysis of lower limbs. In an acutely unwell term infant with symptoms of paralysis or spinal cord damage, haemorrhagic infarction needs to be considered in the differential diagnosis. To our knowledge, this is the first reported case of spinal cord injury in a term infant with a haemorrhagic lesion, and it helps to understand the pathogenesis of nontraumatic insult.
Aim: To test sensitivity of the Segmental Assessment of Trunk Control (SATCo) to changes in neutral vertical (NV) head and trunk control in children with neuromuscular disorders (NMD) and compared with typically developing (TD) children. Methods: In this observational cross-sectional study, SATCo was applied in 19 children with NMD (10y6m(3y7m), 2 female) and 19 TD children (7y10m(4y12m), 6 female). Statistical differences between condition (NMD and TD), segment (seven trunk segments), and control type (static, active, and reactive control), and relationship between age and SATCo outcomes were analysed with linear mixed-effects models (LME). Results: SATCo scores were measurably different between conditions (F (1,39.6) =151.0,p<.001), segments (F (6,138.3) =23.3,p<.001), control type (F (2,33.0) =6.3,p=.005), and interactions condition segment (F (6,138.3) =23.3,p<.001) and condition control type (F (2,33.0) =6.3,p=.005). A significant relationship between decreasing active control and increasing age in 10 children with Duchenne muscular dystrophy confirmed measurable decline in trunk control following diagnosis. Interpretation: The results showed that SATCo is sensitive to differences in trunk control status in NMD. While still ambulatory, children with NMD presented already a measurable trunk control deficit at varying segments. SATCo has potential as an outcome measure for therapeutic interventions or effectiveness of treatments promoting NV trunk control in children with NMD and needs further study. What this paper adds: Neutral vertical head/trunk control was different between NMD and TD children SATCo is sensitive to head/trunk control differences between NMD and TD children Ambulant children with NMD showed measurable head/trunk control deficit at various segments SATCo detected subtle head/trunk control changes in early disease stages of NMD Active segmental trunk control declines with increasing age in Duchenne muscular dystrophy
About Me
Neuromuscular consultant based at centre of excellence at Robert Jones and Agnes Hunt Orthopaedic Hospital.
Neuromuscular consultant based at centre of excellence at Robert Jones and Agnes Hunt Orthopaedic Hospital.