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 MUNIX technique was evaluated in the abductor digiti minimi (ADM) and abductor pollicis brevis (APB) on two sides when possible.
Kairon ritchey manual#
Clinical examination included manual muscle testing, dynamometry (grasp and pinch), and motor function measure (MFM). Methodsįourteen patients with type II Ped-SMA (11 females median age 11 y ) and 14 controls (nine females median age 10.75 y ) were enrolled and matched by sex, age, height, weight, and body mass index. 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. In adult spinal muscular atrophy (SMA), the motor unit number index (MUNIX) has been shown to be an useful electrophysiological biomarker. Nusinersen in later‐onset spinal muscular atrophy. ĭarras BT, Chiriboga CA, Iannaccone ST, et al. Natural history of infantile‐onset spinal muscular atrophy. Motor Unit Number Index (MUNIX) of hand muscles is a disease biomarker for adult spinal muscular atrophy. Günther R, Neuwirth C, Koch JC, et al. Motor Unit Index (MUNIX): a novel neurophysiological marker for neuromuscular disorders test‐retest reliability in healthy volunteers. Neuwirth C, Nandedkar S, Stalberg E, et al. Motor unit number index: guidelines for recording signals and their analysis. Nandedkar S, Barkhaus PE, Stålberg EV, Neuwirth C, Weber M. MUNIX: principle, methods and findings in healthy subjects and patients with motor neuron disease. The Hammersmith functional motor scale for children with spinal muscular atrophy: a scale to test ability and monitor progress in children with limited ambulation. Main M, Kairon H, Mercuri E, Muntoni F. Implementing Motor Unit Number Index (MUNIX) in a large clinical trial: real world experience from 27 centers.
Neuwirth C, Braun N, Claeys KG, et al. Motor unit number index and neurophysiological index as candidate biomarker of pre‐symptomatic motor neuron loss in amyotrophic lateral sclerosis. Įscorcio‐Bezerra ML, Abrahao A, Nunes KF, et al. MUNIX: reproducibility and clinical correlations in Amyotrophic Lateral Sclerosis. Įscorcio‐Bezerra ML, Abrahao A, de Castro I, et al. Motor unit loss estimation by the multipoint incremental MUNE method in children with spinal muscular atrophy‐ a preliminary study. Gawel M, Kostera‐Purszczyk A, Lusakowska A, et al. The motor response to SMN1 deficiency in spinal muscular atrophy. Motor unit number estimation in infants and children with spinal muscular atrophy. Motor nerve conduction studies: measurement principles and interpretation of findings.
Kairon ritchey trial#
209th ENMC International Workshop: outcome measures and clinical trial readiness in spinal muscular atrophy 7‐9 November 2014, Heemskerk, The Netherlands. Finkel R, Bertini E, Muntoni F, Mercuri E.
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