C. Boulay

Dynamic equinus with hindfoot valgus in children with hemiplegia.

In children with hemiplegia, it is important to distinguish between equinus with hindfoot varus (equinovarus) or valgus (equinovalgus). Premature onset of medial gastrocnemius (GM) EMG in individuals with equinus is well documented. Premature onset of Peroneus longus (PL) EMG has been described in neurologically impaired adults with equinovalgus, but not in children. Our aim was to record the onset of PL and GM activity on the hemiplegic side of children with equinovalgus deformity. Fifteen children GMFCS 1 (3.8 yrs ± 2) with hemiplegia had a goniometric assessment of passive ankle range of motion and assessment of ankle function from video and surface EMG recording during gait. The clinical and video observations were used to determine the equinovalgus, as defined by Wren, at initial contact (IC). The premature onset of muscle activity was normalised as a swing (SW) percentage prior to IC of the following stance (ST). A paired T-test compared the onset of muscle activity between PL and GM. The ankle passive dorsiflexion was 13° ± 12° (hemiplegic side) versus 18° ± 10° (non-involved side) (p<0.05). For the non-involved limb, the onset of GM activity was at 14% of the gait cycle (midstance), the onset of PL activity was at 19% (p<0.05). For the hemiplegic limb with equinovalgus, there was a premature onset activity of PL (-24%) and GM(-8%) (p<0.001). On the non involved side, the onset of PL activity occurred, as in adults, after the onset of GM activity, during ST. On the hemiplegic side, there was no triceps surae contracture and the onset of PL activity occurred prior to the onset of GM activity, during terminal SW. This study confirmed the overactivity of PL in hemiplegic children with equinovalgus.

Diagnosis and Outcome of SCN4A-Related Severe Neonatal Episodic Laryngospasm (SNEL): 2 New Cases

Mutations of SCN4A encoding the skeletal muscle sodium channel Nav 1.4 cause several types of disease, including sodium channel myotonias. The latter may be responsible for neonatal symptoms, including severe neonatal episodic laryngospasm (SNEL). Establishing the diagnosis of SCN4A-related SNEL early in the neonatal period is crucial because treatment is available that can reduce laryngospasm and improve vital and cerebral outcome. We report 2 new unrelated French patients who presented with SNEL. The first patient was initially diagnosed with laryngomalacia and underwent laryngeal surgery in the neonatal period before being diagnosed with myotonia at 14 months of age. The episodes of laryngospasm disappeared spontaneously, although occasional circumstances such as cold exposure could trigger laryngeal reactions; in addition, he developed myotonia corresponding to an adult myotonia permanens phenotype. This patient is now 24 years old and leading a normal life. The second patient was initially diagnosed with gastroesophageal reflux, then SNEL; his condition improved with carbamazepine treatment, and he is now 6 months old. The diagnostic sequence in both patients was the same: first, severe episodic apneic attacks necessitating hospitalization occurring in the first week of life; second, observation of muscle hypertrophy and peripheral hypertonia with a clear myotonic pattern on electromyogram (at 14 and 3 months of age, respectively); third, genetic testing revealing de novo SCN4A G1306E mutation. Both patients have had good therapeutic response to sodium channel blockers (carbamazepine or mexiletine).

Planovalgus foot deformity in cerebral palsy corrected by botulinum toxin injection in the peroneus longus: Clinical and radiological evaluations in young children

BACKGROUND In children with cerebral palsy (CP), overactivity of the peroneus longus (PL) muscle is a major contributor to pes planovalgus. This retrospective study assessed whether abobotulinumtoxinA injections into a PL showing premature activity on electromyography (EMG) clinically improved foot morphology in children with CP. METHODS Study participants were <6 years old, had a diagnosis of CP, good functional abilities (Gross Motor Function Classification System level 1 or 2), equinovalgus (initial contact with the hallux or head of the first metatarsal) and overactive PL on EMG. The fore-, mid- and hindfoot were evaluated clinically and radiologically before and after injection of abobotulinumtoxinA (6-7 U/kg) into the PL. Radiological data were compared with reference values for children without pes planovalgus. RESULTS In total, 16 children (8 males; 10 hemiplegia, 6 diplegia; mean age: 3.2±1.5 years) received treatment. Mean pre-and post-treatment angles in clinical assessment of dorsiflexion of the talocrural articulation did not differ with both knees flexed (24.4±7.5 vs. 22.2±8.0 degrees; P=0.19) or extended (17.2±8.0 vs. 16.6±6.8 degrees; P=0.36). Radiographic data pre-treatment versus reference data revealed forefoot pronation (metatarsal stacking angle 2.1±8.3 vs. 8.0±2.9 degrees; P=0.002), midfoot planus (lateral talo-first metatarsal 28.5±15.0 vs. 13.0±7.5 degrees; P<0.001; talocalcaneal angle 54.6±8.6 vs. 49.0±6.9 degrees; P=0.004) and significantly decreased calcaneus dorsiflexion, without hindfoot equinus (calcaneal pitch angle 7.9±6.0 vs. 17.0±6.0 degrees; P<0.001). After treatment, the metatarsal stacking angle did not differ from reference values (P=0.15). As compared with before treatment, treatment improved mean angles for metatarsal stacking (2.1±8.3 vs. 7.1±3.9 degrees, respectively, P=0.002), lateral talo-first metatarsal and talocalcaneal (both P<0.001), with no change in the hindfoot. CONCLUSION PL may be an early target for abobotulinumtoxinA treatment in pes planovalgus associated with premature PL activity in children with CP.

Dynamic EMG of peroneus longus in hemiplegic children with equinovarus.

OBJECTIVE In hemiplegic children the appearance of equinovarus is correlated with premature electromyography (EMG) activity of the gastrocnemius medialis (GM) prior to initial contact. The goal was to analyze the onset of EMG activation in the GM and, more particularly, the peroneus longus (PL) in cases of equinovarus: is PL activity likewise premature? MATERIAL AND METHODS As 15 hemiplegic children (age 5 years±1.5) with equinovarus walked, their PL and GM EMG activity was being recorded. The latter was normalized in terms of gait cycle percentage (0-100%) and detected through semi-automatic selection with activation threshold set at 20μV. A paired t-test compared activation onset of the PL versus the GM muscles. RESULTS As regards the healthy limb, activity onset of the GM (+14.55%) and the PL (+19.2%) muscles occurred only during the ST. In cases of equinovarus, activation of the GM (-5.2%) and the PL (-6.1%) occurred during the SW and was premature. For each muscle, comparison between the healthy and the hemiplegic side was highly significant (P<0.001). CONCLUSION Premature PL and GM EMG activity preceding initial contact corresponds not to a disorder secondary to imbalance but rather, more probably, to motor command dysfunction. While the PL consequently contributes to equinus deformity, its possible role in varus genesis is less evident. EMG study needs to be completed by comparing PL and tibialis posterior strength while taking foot bone morphology into full account.

Clinical Features and Evolution of Juvenile Myasthenia Gravis In a French Cohort

In this study we determined the clinical, paraclinical, and treatment‐related features of juvenile myasthenia gravis (JMG) as well as the clinical course in a cohort of French children.