Serious Game with Electromyography Feedback and Physical Therapy in Young Children with Unilateral Spastic Cerebral Palsy and Equinus Gait: A Prospective Open-Label Study.

The clinical effects of a serious game with electromyography feedback (EMGs_SG) and physical therapy (PT) was investigated prospectively in children with unilateral spastic cerebral palsy (USCP). An additional aim was to better understand the influence of muscle shortening on function. Thirty children with USCP (age 7.6 ± 2.1 years) received four weeks of EMGs_SG sessions 2×/week including repetitive, active alternating training of dorsi- and plantar flexors in a seated position. In addition, each child received usual PT treatment ≤ 2×/week, involving plantar flexor stretching and command strengthening on dorsi- and plantar flexors. Five-Step Assessment parameters, including preferred gait velocity (normalized by height); plantar flexor extensibility (XV1); angle of catch (XV3); maximal active ankle dorsiflexion (XA); and derived coefficients of shortening, spasticity, and weakness for both soleus and gastrosoleus complex (GSC) were compared pre and post treatment (t-tests). Correlations were explored between the various coefficients and gait velocities at baseline. After four weeks of EMGs_SG + PT, there was an increase in normalized gait velocity from 0.72 ± 0.13 to 0.77 ± 0.13 m/s (p = 0.025, d = 0.43), a decrease in coefficients of shortening (soleus, 0.10 ± 0.07 pre vs. 0.07 ± 0.08 post, p = 0.004, d = 0.57; GSC 0.16 ± 0.08 vs. 0.13 ± 0.08, p = 0.003, d = 0.58), spasticity (soleus 0.14 ± 0.06 vs. 0.12 ± 0.07, p = 0.02, d = 0.46), and weakness (soleus 0.14 ± 0.07 vs. 0.11 ± 0.07, p = 0.005, d = 0.55). At baseline, normalized gait velocity correlated with the coefficient of GSC shortening (R = -0.43, p = 0.02). Four weeks of EMGs_SG and PT were associated with improved gait velocity and decreased plantar flexor shortening. A randomized controlled trial comparing EMGs_SG and conventional PT is needed.

The effect of selective dorsal rhizotomy on the improvement of the quality of life of children with stage GMFCS IV and V cerebral palsy: Pain, nursing, positioning, and dressing.

AIM: The objective of this study is to evaluate the benefit of selective dorsal rhizotomy on the quality of life of patients with severe spasticity with significant impairment of gross motor functions (GMFCS stages IV and V) according to 4 items: pain, nursing care, positioning, and dressing. MATERIALS AND METHODS: We conducted a monocentric retrospective cohort study including patients who underwent selective dorsal rhizotomy between March 2008 and May 2022 at the University Hospital of Marseille. RESULTS: Seventy percent of patients showed an improvement in quality of life criteria: dressing, nursing, positioning, and pain at the last follow-up. A small proportion of patients still showed a worsening between the first 2 follow-ups and the last follow-up. Postoperatively, 27.3% of patients were free of joint spasticity treatment, and we have shown that there was a significant decrease in the number of children who received botulinum toxin postoperatively. However, there was no significant reduction in the number of drug treatments or orthopaedic procedures following RDS. For the CPCHILD© scores, an overall gain is reported for GMFCS IV and V patients in postoperative care. The gain of points is more important for GMFCS IV patients. Improvement was mainly observed in 2 domains, "comfort and emotions" and "hygiene and dressing". For the "quality of life" item, only 3 parents out of the 8 noted a positive change. CONCLUSION: Our study shows an improvement in nursing care, positioning, and dressing which are associated with a reduction in pain in children with a major polyhandicap GMFCS IV and V who have benefited from a selective dorsal rhizotomy.

Improved Gait and Radiological Measurements After injection of Botulinum Toxin Into Peroneus Longus in Young Children With USCP and Equinovalgus Gait.

BACKGROUND: Children with cerebral palsy develop foot deformities due to a combination of factors including muscle shortening, hypertonia, weakness, and cocontraction of muscles acting at the ankle joint resulting in an altered gait pattern. We hypothesized these factors affect the peroneus longus (PL) and tibialis anterior (TA) muscles couple in children who develop equinovalgus gait first followed by planovalgus foot deformities. Our aim was to evaluate the effects of abobotulinum toxin A injection to the PL muscle, in a cohort of children with unilateral spastic cerebral palsy and equinovalgus gait. METHODS: This was a prospective cohort study. The children were examined within 12 months before and after injection to their PL muscle. Twenty-five children of mean age 3.4 (S.D.: 1.1) years were recruited. RESULTS: We found significant improvement in foot radiology measures. Passive extensibility of the triceps surae did not change, whereas active dorsiflexion increased significantly. Nondimensional walking speed increased by 0.1 (95% confidence interval [CI], [0.07, 0.16]; P < 0.001), and the Edinburgh visual gait score improved by 2.8 (95% CI, [-4.06, -1.46]; P < 0.001). Electromyography showed increased recruitment for gastrocnemius medialis (GM) and TA but not for PL during the reference exercises (standing on tip toes for GM/PL, active dorsiflexion for TA) and decreased activation percentages for PL/GM and TA across sub-phases of gait. CONCLUSIONS: One key advantage of treating the PL muscle only might be to address foot deformities without interfering with the main plantar flexors that are instrumental to support body weight during gait.

Reduced plantar-flexors extensibility but improved selective motor control associated with age in young children with unilateral cerebral palsy and equinovalgus gait.

BACKGROUND: Children with spastic cerebral palsy gradually lose muscle extensibility but the interplay between the muscular and neurological components of the condition is unclear especially in the pathophysiology of equinovalgus gait. AIM: This study aimed to quantify the muscular and neurological disorders in young children with unilateral cerebral palsy, and to investigate the role of the peroneus longus (PL) in equinovalgus gait. DESIGN, SETTING AND POPULATION: This was an observational study with prospective assessments of 31 children (median age: 2.9 years, range: 2-6) from outpatient clinic in a tertiary teaching hospital. METHODS: Clinical measures of plantar flexor extensibility (XV1), stretch response (XV3), and active ankle dorsiflexion angle (XA) were obtained as well as walking velocity and electromyography of tibialis anterior (TA), gastrocnemius medialis (GM) and PL during walking. RESULTS: We found reduced extensibility of the triceps surae on the paretic side (effect size r = 0.73, p < 0.001 for soleus and r = 0.68, p < 0.001 for gastrocnemius) and a correlation between reduced triceps surae extensibility and earlier stretch response (ρ = 0.5, p = 0.004). During the swing phase, there was major co-contraction between TA and GM/PL, and significantly larger activation of PL compared to GM (r = 0.46, p = 0.011). Both GM and PL activation decreased with age. CONCLUSIONS: Our results suggest gradual deterioration of the muscular disorder and a link between the muscular and neurological disorders, although plantar flexor co-contraction improved with age. The PL was more activated than the GM and may be considered an intervention target to treat equinovalgus gait.

Validity, reliability and responsiveness to change of the French version of the drooling impact scale.

BACKGROUND: work still needs to be done to measure the impact of sialorrhea on quality of life and define the efficacy of different therapies. The Drooling Impact Scale showed good validity and sensitivity to change, especially after botulinum toxin injection. The aim of this study is to present its French translation and to explore its validity, reliability and responsiveness to change in a group of children with Cerebral Palsy. METHODS: multicentre study at six rehabilitation centres in France. Children with Cerebral Palsy aged 4-18 years with sialorrhea problems were included (n = 55), either in a control group (n = 33) or in the intervention group (n = 22, with 3 drug treatment and 19 botulinum toxin injections). The French Drooling Impact Scale was administered twice, 1 month apart. RESULTS: The French Drooling Impact Scale total score at inclusion was meanly 53.9 (Standard Deviation 11.9) in the stable control group and 66.0 (16.1) in the intervention group (p = 0.0058). The validity of the scale was established, as well as an adequate internal consistency (Cronbach's α = 0.71); correlations between each item and the total score were found between 0.5 and 0.71 except for item 5 (r = 0.38) and item 7 (0.41). The test-retest reliability in stable children was good (Lin coefficient = 0.83, bias correction factor = 0.92 and Pearson correlation coefficient = 0.89). There was a high responsiveness to change, mean change was -40.0 in the intervention group and -3.6 in the stable group (p < 0.0001), with Standard Error of Measurement = 2.6. CONCLUSION: the French Drooling Impact Scale has shown sufficient clinometric properties to be used now by clinicians or researchers.IMPLICATIONS FOR REHABILITATIONThe Drooling Impact scale has now its French version.The French version of the Drooling Impact Scale has shown its validity and a good test-retest reliability.The responsiveness to change was explored in a group of children undergoing saliva-control interventions and the scale was able to show a big change.The authors recommend to use this questionnaire in a semi-directed interview conducted by a health professional.

Early dynamic changes within the spine following posterior fusion using hybrid instrumentation in adolescents with idiopathic scoliosis: a gait analysis study.

INTRODUCTION: In adolescent idiopathic scoliosis (AIS) patients, mechanical consequences of posterior spinal fusion within the spine remain unclear. Through dynamic assessment, gait analysis could help elucidating this particular point. The aim of this study was to describe early changes within the spine following fusion with hybrid instrumentation in adolescents with idiopathic scoliosis, using gait analysis MATERIALS AND METHODS: We conducted a single-centre prospective study including AIS patients scheduled for posterior spinal fusion (PSF) using hybrid instrumentation with sublaminar bands. Patients underwent radiographic and gait analyses preoperatively and during early postoperative period. Among gait parameters, motion of cervicothoracic, thoracolumbar and lumbosacral junctions was measured in the three planes. RESULTS: We included 55 patients (mean age 15 years, 84% girls). Fusion was performed on 12 levels and mean follow-up was 8 months. There was a moderately strong correlation between thoracolumbar sagittal motion and lumbosacral junction pre- and postoperatively (R = - 0.6413 and R = - 0.7040, respectively, all p < 0.001), meaning that the more thoracolumbar junction was in extension, the more lumbosacral extension movements decreased. There was a trend to significance between postoperative SVA change and thoracolumbar sagittal motion change (R = - 0.2550, p = 0.059). DISCUSSION: This is the first series reporting dynamic changes within the spine following PSF using hybrid instrumentation in AIS patients. PSF led to symmetrization of gait pattern. In the sagittal plane, we found that thoracolumbar extension within the fused area led to decreased extension at cervicothoracic and lumbosacral junctions. Even though consequences of such phenomenon are unclear, attention must be paid not to give a too posterior alignment when performing PSF for AIS patients.

Description of spine motion during gait in normal adolescents and young adults.

INTRODUCTION: Standing radiographs allow analysis of spinal segments and their relative positioning. However, it requires a specific positioning influencing spinal alignment. Knowledge of trunk movements when walking is therefore an essential step to evaluate dynamic sagittal balance. Our objective was to define spinal junction kinematics and their correlations during gait in a healthy population. METHOD: This is a prospective, single-center study. Between 2015 and 2017, 25 healthy volunteers were included. The measurements were taken in a motion analysis laboratory. Several kinematic parameters were studied, including spinal junction movements in the three planes and dynamic sagittal vertical axis (Dyn-SVA). Pearson correlation coefficients were calculated to determine the interactions between the spinal junctions. RESULTS: In the sagittal plane, the average amplitude of variation of the dyn-SVA was 25.5 cm (SD = 8.9). The average range of motion of the lumbosacral and thoracolumbar junction was approximately 3°, they operated in anti-phase during the gait cycle and were strongly correlated (r = -0.5069, p = 0.01). In the transverse plane, the anti-rotation of the upper body relative to the pelvis was mainly ensured by the opposite movements of the lumbosacral and thoracolumbar junction (r = 0.5689, p = 0.003). In the frontal plane, the lateral inclination in the lumbar region was made in the opposite direction from the pelvis toward the carrying member. CONCLUSION: Although there is substantial inter-subject variability, our study characterized the angular movements in the three planes of the different spinal junctions, of the pelvis and the lower limbs during a gait cycle in a healthy population.

Motor unit number index: A potential electrophysiological biomarker for pediatric spinal muscular atrophy.

INTRODUCTION/AIMS: In adult spinal muscular atrophy (SMA), the motor unit number index (MUNIX) has been shown to be an useful electrophysiological biomarker. This study evaluated the feasibility and the clinical relevance of using the MUNIX technique for patients with pediatric SMA (Ped-SMA) and correlated MUNIX results with clinical scores. METHODS: Fourteen patients with type II Ped-SMA (11 females; median age 11 y [interquartile range (IQR), 4.8-17 y]) and 14 controls (nine females; median age 10.75 y [IQR, 6.5-13.4 y]) were enrolled and matched by sex, age, height, weight, and body mass index. Clinical examination included manual muscle testing, dynamometry (grasp and pinch), and motor function measure (MFM). The MUNIX technique was evaluated in the abductor digiti minimi (ADM) and abductor pollicis brevis (APB) on two sides when possible. RESULTS: In the patients with Ped-SMA, the MUNIX and compound muscle action potential (CMAP) amplitudes were significantly decreased and the motor size unit index (MUSIX) was significantly increased in the ADM and APB when compared to controls. The intraclass correlation coefficient was good for the intrarater variability of the CMAP amplitude, MUNIX, and MUSIX in the ADM (0.95, 0.83, and 0.89, respectively) and the APB (0.98, 0.96, and 0.94, respectively). The total CMAP amplitude correlated with the grasp and pinch scores (P < .05), and the MUNIX measurements correlated with the MFM scores. DISCUSSION: The MUNIX technique, which accurately estimated lower motor neuron loss and the number of remaining functional motor units, was shown to be a useful electrophysiological biomarker for disease progression and a potential biomarker for treatment response.

Extension of the phenotypic spectrum of GLE1-related disorders to a mild congenital form resembling congenital myopathy.

BACKGROUND: GLE1 (GLE1, RNA Export Mediator, OMIM#603371) variants are associated with severe autosomal recessive motor neuron diseases, that are lethal congenital contracture syndrome 1 (LCCS1, OMIM#253310) and congenital arthrogryposis with anterior horn cell disease (CAAHD, OMIM#611890). The clinical spectrum of GLE1-related disorders has been expanding these past years, including with adult-onset amyotrophic lateral sclerosis (ALS) GLE1-related forms, especially through the new molecular diagnosis strategies associated with the emergence of next-generation sequencing (NGS) technologies. However, despite this phenotypic variability, reported congenital or ALS adult-onset forms remain severe, leading to premature death. METHODS: Through multidisciplinary interactions between our Neuropediatric and Medical Genetics departments, we were able to diagnose two siblings presenting with congenital disorder, using an NGS approach accordingly to the novel French national recommendations. RESULTS: Two siblings with very similar clinical features, meaning neuromuscular disorder of neonatal onset with progressive improvement, were examined in our Neuropediatrics department. The clinical presentation evoked initially congenital myopathy with autosomal recessive inheritance. However, additional symptoms such as mild dysmorphic features including high anterior hairline, downslanted palpebral fissures, anteverted nares, smooth philtrum with thin upper-lip, narrow mouth and microretrognathia or delayed expressive language and postnatal growth retardation were suggestive of a more complex clinical presentation and molecular diagnosis. Our NGS approach revealed an unexpected molecular diagnosis for these two siblings, meaning the presence of the homozygous c.1808G>T GLE1 variant. CONCLUSIONS: We here report the mildest phenotype ever described, in two siblings carrying the homozygous c.1808G>T GLE1 variant, further widening the clinical spectrum of GLE1-related diseases. Moreover, by reflecting current medical practice, this case report confirms the importance of establishing regular multidisciplinary meetings, essential for discussing such difficult clinical presentations to finally enable molecular diagnosis, especially when NGS technologies are used.

Clinical features and evolution of juvenile myasthenia gravis in a French cohort.

INTRODUCTION: 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. METHODS: We conducted a retrospective study of 40 patients with JMG at 2 French pediatric neurology departments from April 2004 to April 2014. RESULTS: Among the patients, 70% had generalized JMG, 52% had positive acetylcholine receptor antibodies, 8% had muscle-specific kinase antibodies, and 40% were seronegative. Treatment with acetylcholinesterase inhibitors was effective and sufficient in 47% of patients. The 6 patients with generalized JMG treated with rituximab and/or immunoadsorption showed improvement. Thirty percent of the patients required hospitalization in an intensive care unit during follow-up (mean 4.7 years). Remission without treatment occurred in 18% of patients. DISCUSSION: As with adults, JMG has high morbidity, particularly among children with generalized symptoms, and rituximab should be considered early in the course of the disease as a second-line treatment. Muscle Nerve 57: 603-609, 2018.

Clinical and allelic heterogeneity in a pediatric cohort of 11 patients carrying MFN2 mutation.

INTRODUCTION: The Mitofusin 2 gene (MFN2), which encodes a mitochondrial membrane protein, is known to be the first cause of autosomal dominant Charcot-Marie-Tooth disease type 2 (CMT2) with early onset. This gene is involved in typical CMT2A and in more atypical phenotypes as optic atrophy or spastic paraplegia. CMT2 refers to inherited axonal polyneuropathy, which associates progressive peripheral motor and sensory neuropathy, a family history consistent mainly with autosomal dominant inheritance, and normal nerve conduction velocities. SUBJECTS: Between 1999 and 2012, the genetic diagnosis of MFN2 mutation was made in 11 children who were treated in our department for different neurological symptoms. All data including family and personal history data, results of standardized clinical and electrophysiology testing, brain magnetic resonance imaging (MRI), neuro-ophthalmic evaluation, muscle biopsy histopathology and molecular diagnosis were retrospectively analyzed. RESULTS: Five different mutations were found in 6 unrelated families. Three of them have previously been described; the two remaining are new mutations: one of them related a new phenotype. Clinical signs appeared before the age of 6 years in more than half of the patients (54%). The motor deficit was predominant in 8 patients (72%). Two children presented an acute onset of disease that stabilized afterwards; the other children showed a more progressive deterioration that was managed symptomatically. CONCLUSION: This large pediatric study describes a great interfamilial and intrafamilial phenotypic variability. We recommend screening this gene in pediatric patient with chronic neurologic symptoms such as motor deficit or optic atrophy but also in acute neurologic deficiencies such as subacute polyradiculoneuritis.

Childhood CIDP: Study of 31 patients and comparison between slow and rapid-onset groups.

OBJECTIVES: To describe 31 children presenting a CIDP; to compare patients with rapid-onset disease vs. patients with slow-onset disease, a rapid-onset disease being defined by a time to peak impairment of less than 8 weeks. STUDY DESIGN: A retrospective chart review identified 31 patients completing criteria for childhood CIDP, with 24 "confirmed CIDP" and 7 "possible CIDP". Data collected were time to peak impairment, clinical presentation, cerebrospinal fluid analysis, nerve conduction study, nerve biopsy, treatments. Evaluation at the end of follow-up was reported according to modified Rankin scale. RESULTS: Thirteen patients (42%) exhibited symptoms in less than 2 months with more often cranial nerve abnormalities (38% vs. 6%, p = 0.059), and sensitive symptoms (62% vs. 11%, p = 0.0057). They evolved predominantly in a relapsing way (69% vs. 22%, p = 0.0047). Length of the disease was also longer in the rapid-onset group (5.5 years vs. 3.83 years) but without statistical difference. The slow-onset group exhibited more frequently ataxia at onset (28% vs. 8%, p > 0.05), and evolved predominantly in a progressive manner (61% vs. 15%, p > 0.05). Outcome was similar and good in the two groups. At least 3 out of the 4 major electrophysiological criteria were positive for 27/31 children (87%). Axonal involvement could be present very early. Immunoglobulins were given in 29 cases and corticosteroids in 22. A partial or complete recovery 1 month after first treatment was reported in 30 cases. Among second-line treatments, only azathioprine seemed effective in two out of three intractable children. CONCLUSIONS: The differences noted between the two groups should be tested in wider populations. Electrophysiological criteria are restrictive and axonal involvement should be studied. Prospective trials are required to find out the best first and second line treatments.

Pelvic incidence: a predictive factor for three-dimensional acetabular orientation-a preliminary study.

Acetabular cup orientation (inclination and anteversion) is a fundamental topic in orthopaedics and depends on pelvis tilt (positional parameter) emphasising the notion of a safe range of pelvis tilt. The hypothesis was that pelvic incidence (morphologic parameter) could yield a more accurate and reliable assessment than pelvis tilt. The aim was to find out a predictive equation of acetabular 3D orientation parameters which were determined by pelvic incidence to include in the model. The second aim was to consider the asymmetry between the right and left acetabulae. Twelve pelvic anatomic specimens were measured with an electromagnetic Fastrak system (Polhemus Society) providing 3D position of anatomical landmarks to allow measurement of acetabular and pelvic parameters. Acetabulum and pelvis data were correlated by a Spearman matrix. A robust linear regression analysis provided prediction of acetabulum axes. The orientation of each acetabulum could be predicted by the incidence. The incidence is correlated with the morphology of acetabula. The asymmetry of the acetabular roof was correlated with pelvic incidence. This study allowed analysis of relationships of acetabular orientation and pelvic incidence. Pelvic incidence (morphologic parameter) could determine the safe range of pelvis tilt (positional parameter) for an individual and not a group.

Relevance of intraglandular injections of Botulinum toxin for the treatment of sialorrhea in children with cerebral palsy: a review.

BACKGROUND: After the age of 4 years, drooling becomes pathological and impacts the quality of life of children with cerebral palsy. Intraglandular injection of Botulinum toxin is one of the treatments available to limit this phenomenon. AIMS: The objectives of this review were to validate the efficacy of Botulinum toxin injections for drooling in children with cerebral palsy, determine recommendations and identify potential side effects. METHODS: We conducted a literature review from 2001 in the following databases: Embase, Pubmed and Cochrane using the keywords: sialorrhea, drooling, hypersalivation, Botulinum toxin, cerebral palsy and children. Only the articles evaluating the efficacy of Botulinum toxin in children with cerebral palsy over the age of 4 were researched. RESULTS: Eight studies were found: 2 case studies, 3 open and non-controlled studies and 3 randomized controlled trials. Efficacy results in this indication are quite encouraging and the use of BTX injections is safe but the overall level of evidence of these studies was quite low. CONCLUSION: However, intraglandular injection of Botulinum toxin has a place among the therapeutic array available for the management of sialorrhea in this population even if no standardized protocol is available yet.

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).

How is sagittal balance acquired during bipedal gait acquisition? Comparison of neonatal and adult pelves in three dimensions. Evolutionary implications.

We compare adult and intact neonatal pelves, using a pelvic sagittal variable, the angle of sacral incidence, which presents significant correlations with vertebral curvature in adults and plays an important role in sagittal balance of the trunk on the lower limbs. Since the lumbar curvature develops in the child in association with gait acquisition, we expect a change in this angle during growth which could contribute to the acquisition of sagittal balance. To understand the mechanisms underlying the sagittal balance in the evolution of human bipedalism, we also measure the angle of incidence of hominid fossils. Fourty-seven landmarks were digitized on 50 adult and 19 intact neonatal pelves. We used a three-dimensional model of the pelvis (DE-VISU program) which calculates the angle of sacral incidence and related functional variables. Cross-sectional data from newborns and adults show that the angle of sacral incidence increases and becomes negatively correlated with the sacro-acetabular distance. During ontogeny the sacrum becomes curved, tends to sink down between the iliac blades as a wedge and moves backward in the sagittal plane relative to the acetabula, thus contributing to the backwards displacement of the center of gravity of the trunk. A chain of correlations links the degree of the sacral slope and of the angle of incidence, which is tightly linked with the lumbar lordosis. We sketch a model showing the coordinated changes occurring in the pelvis and vertebral column during the acquisition of bipedalism in infancy. In the australopithecine pelves, Sts 14 and AL 288-1, and in the Homo erectus Gona pelvis the angle of sacral incidence reaches the mean values of humans. Discussing the incomplete pelves of Ardipithecus ramidus, Australopithecus sediba and the Nariokotome Boy, we suggest how the functional linkage between pelvis and spine, observed in humans, could have emerged during hominid evolution.

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.

Relationship between sacral pelvic incidence and acetabular orientation.

The importance of the sacral pelvic incidence (SPI) in relation to individual variations of sagittal spinal curvature has become well-recognised. We attempted to determine the relationship between SPI and acetabular orientation. The three-dimensional coordinates of 47 homologous points were observed on 51 adult anatomical pelvises (26 female and 25 male). The reference vertical plane was Lewinnek's anterior pelvic plane. 10 angular parameters and 11 linear parameters were defined and calculated. These were expressed both in absolute value (in millimetres) and in "acetabular unit" (relative to the mean value of the right and left acetabular rays). Mean values of the parameters were calculated for all pelvises and according to gender. There were two dominant parameters: the "sacral slope" and the "V pubic angle". "Acetabular tilting" was primarily dependent on the "sacral slope" and its intermediary on the "SPI", while "acetabular anteversion" dependent on the "V pubic angle" via the "angle of prow". It is recommended that positioning of the acetabular cup in total hip arthroplasty relates to anatomical parameters, and to the global sagittal balance of the pelvi-spinal unit.

Perineal descent predicted by a pelvic bone factor: the pelvic incidence angle.

PURPOSE: By measuring the pelvic incidence angle, we assessed the relationship between pelvic floor disorders and pelvic morphology, which allowed us to document for the first time the hypothesis that pelvic incidence may be a predictive factor of perineal descent. METHODS: In a retrospective study of 197 women, the perineal descent at rest and during straining was assessed by defecography. The pelvic incidence angle (53 degrees +/- 9 degrees , independent of the subject position) was defined as the angle between the line perpendicular to the sacral plate at its midpoint and the line connecting this point to the middle of the femoral heads axis. The pelvic incidence angle was correlated with the incidence of pelvic floor descent. RESULTS: In those with pelvic floor descent at rest compared with those without, pelvic incidence angle was significantly larger (64 degrees vs. 53 degrees , P < 10E-06). As a predictive factor of perineal descent at rest, a great pelvic incidence (>62 degrees ) had a sensitivity (73 percent), specificity (82 percent), positive predictive value (81 percent), and negative predictive value (75 percent). CONCLUSIONS: A large pelvic incidence (>62 degrees ) may be a predictive factor of perineal descent at rest before the apparition of other acquired factors. With pelvic incidence >62 degrees , a large overhang between the insertions increases the strains on the perineum, which is rather horizontal.

Three-dimensional study of pelvic asymmetry on anatomical specimens and its clinical perspectives.

The aim of this study was to assess pelvic asymmetry (i.e. to determine whether the right iliac bone and the right part of the sacrum are mirror images of the left), both quantitatively and qualitatively, using three-dimensional measurements. Pelvic symmetry was described osteologically using a common reference coordinate system for a large sample of pelvises. Landmarks were established on 12 anatomical specimens with an electromagnetic Fastrak system. Seventy-one paired variables were tested with a paired t-test and a non-parametric test (Wilcoxon). A Pearson correlation matrix between the right and left values of the same variable was applied exclusively to values that were significantly asymmetric in order to calculate a dimensionless asymmetry index, ABGi, for each variable. Fifteen variables were significantly asymmetric and correlated with the right vs. left sides for the following anatomical regions: sacrum, iliac blades, iliac width, acetabulum and the superior lunate surface of the acetabulum. ABGi values above a threshold of +/- 4.8% were considered significantly asymmetric in seven variables of the pelvic area. Total asymmetry involving the right and the left pelvis seems to follow a spiral path in the pelvis; in the upper part, the iliac blades rotate clockwise, and in the lower part, the pubic symphysis rotates anticlockwise. Thus, pelvic asymmetry may be evaluated in clinical examinations by measuring iliac crest orientation.