Diagnostic du syndrome du canal carpien (évaluation comparative de critères électroneurographiques sensitifs du nerf médian et de l échographie du poignet)

Il s agit d une étude comparant électroneuromyogramme (ENMG) et échographie pour le diagnostic du syndrome du canal carpien (SCC), dans une population de 125 mains, représentative de celle consultant habituellement pour paresthésies de la main. Les paramètres sensitifs de l ENMG (interlatence médio-cubitale à l annulaire et vitesse de conduction sensitive transcarpienne) ont, dans cette population, une sensibilité de 79% et une spécificité de 80 %, alors que ces dernières sont de 72 et 56% pour la mesure échographique de l aire de section du nerf médian au niveau de l os pisiforme. L échographie s avère donc être un examen sensible, mais également reproductible et bien corrélé à l ENMG. Cependant, sa faible spécificité dans notre groupe hétérogène de patients incite à réserver son utilisation aux tableaux cliniques typiques de SCC.GRENOBLE1-BU Médecine pharm. (385162101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

Estimation of finger muscle tendon tensions and pulley forces during specific sport climbing grip techniques

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Using EMG data to constrain optimization procedure improves finger tendon tension estimations during static fingertip force production.

International audienceDetermining tendon tensions of the finger muscles is crucial for the understanding and the rehabilitation of hand pathologies. Since no direct measurement is possible for a large number of finger muscle tendons, biomechanical modelling presents an alternative solution to indirectly evaluate these forces. However, the main problem is that the number of muscles spanning a joint exceeds the number of degrees of freedom of the joint resulting in mathematical under-determinate problems. In the current study, a method using both numerical optimization and the intra-muscular electromyography (EMG) data was developed to estimate the middle finger tendon tensions during static fingertip force production. The method used a numerical optimization procedure with the muscle stress squared criterion to determine a solution while the EMG data of three extrinsic hand muscles serve to enforce additional inequality constraints. The results were compared with those obtained with a classical numerical optimization and a method based on EMG only. The proposed method provides satisfactory results since the tendon tension estimations respected the mechanical equilibrium of the musculoskeletal system and were concordant with the EMG distribution pattern of the subjects. These results were not observed neither with the classical numerical optimization nor with the EMG-based method. This study demonstrates that including the EMG data of the three extrinsic muscles of the middle finger as inequality constraints in an optimization process can yield relevant tendon tensions with regard to individual muscle activation patterns, particularly concerning the antagonist muscles

Absence of beta-tropomyosin is a new cause of Escobar syndrome associated with nemaline myopathy.

International audienceWhile TPM2 mutations identified so far in muscular diseases were all associated with a dominant inheritance pattern, we report the identification of a homozygous null allele mutation in the TPM2 gene in a patient who presented with a recessive form of nemaline myopathy associated with a non-lethal multiple pterygium syndrome (Escobar-MPS MIM# 265000). The TPM2 mutation led to a complete absence of the skeletal muscle isoform of beta-tropomyosin not compensated by expression of other beta-tropomyosin isoforms. Escobar syndrome has been recently described as a prenatal form of myasthenia associated with recessive mutations in genes of the neuromuscular junction (CHRNG, CHRNA1, CHRND, RAPSN). This observation expands the cause of Escobar variant-MPS to a component of the contractile apparatus. This first report of the clinical expression of the complete absence of TPM2 in human indicated that TPM2 expression at the early period of prenatal life plays a major role for normal fetal movements

Evidence for a dominant negative disease mechanism in cap myopathy due to TPM3.

International audienceWe report a third patient with typical cap myopathy due to a heterozygous TPM3 mutation, confirming the importance of this causal association. The p.R168C TPM3 mutation we identified has been reported in two previous patients. The histological changes associated with this mutation vary widely from typical cap myopathy with near complete type 1 predominance (two patients), to typical congenital fibre-type disproportion without protein inclusions (one patient). We performed 2D-gel electrophoresis using muscle biopsies from two patients with the p.R168C mutation and show that mutant protein accounts for around 50% of alpha-tropomyosin(slow) in sarcomeres, consistent with a dominant negative mechanism of disease pathogenesis

A prospective multicentre study on sural nerve action potentials in ALS.

International audienceOBJECTIVE: To evaluate sensory nerve conduction studies in ALS in a prospective multicentre study involving 7 neurophysiologists from 6 European countries. METHODS: Bilateral sural potentials were obtained in 35 ALS patients and 35 age-matched controls according to a standardised examination protocol using antidromic surface technique. The recordings from the right sural nerve of the controls were used for reference values. A reduction from the mean of controls greater than 2 SDs was considered abnormal. RESULTS: Reduced sensory nerve action potential (SNAP) amplitude or reduced conduction velocity (CV), or both, was found in 6 ALS patients (17%). Decrease in CV was the most frequent finding, and was observed in 8 nerves from 5 patients. Reduced SNAP amplitude was found in 2 nerves from 2 patients. All changes were minor ranging from -2.1 to -3.2 SDs. CONCLUSIONS: This is the first standardised multicentre study on sensory potentials in ALS. It confirms that although normal sensory findings should be expected in the majority of ALS patients, minor abnormalities are not uncommon. SIGNIFICANCE: Mild sensory abnormalities do not necessarily exclude a diagnosis of ALS