Inertial Load Compensation by a Model Spinal Circuit During Single Joint Movement

Office of Naval Research (N00014-92-J-1309); CONACYT (Mexico) (63462

Next generation of growth-sparing techniques: preliminary clinical results of a magnetically controlled growing rod in 14 patients

Session 3A - Early Onset Scoliosis: Paper no. 33SUMMARY: Growth-sparing techniques are commonly used for the treatment of progressive EOS. The standard growing rod (GR) technique requires multiple surgeries for lengthening. The preliminary results of MCGR has shown the comparable outcomes to standard GR without the need for repeated surgery which can be expected to reduce the overall complication rate in GR surgery. INTRODUCTION: The growing rod (GR) technique for management of progressive Early-Onset Scoliosis (EOS) is a viable alternative but with a high complication rate attributed to frequent surgical lengthenings. The safety and efficacy of a non-invasive Magnetically Controlled Growing Rod (MCGR) has been previously reported in a porcine model. We are reporting the preliminary results of this technique in EOS. METHODS: Retrospective review of prospectively collected multi-center data. Only patients who underwent MCGR surgery and at least 3 subsequent spinal distractions were included in this preliminary review. Distractions were performed in clinic without anesthesia or analgesics. T1-T12 and T1-S1 height and the distraction distance inside the actuator were analyzed in addition to conventional clinical and radiographic data. RESULTS: Patients (N=14; 7 F and 7 M) had a mean age of 8y+10m (3y+6m to 12y+7m) and underwent a total of 14 index surgeries (SR: index single rod in 5 and DR: dual rod in 9) and 91 distractions. There were 5 idiopathic, 4 neuromuscular, 2 congenital, 2 syndromic and one NF. Mean follow-up (FU) was 10 months (5.8-18.2). Mean Cobb changed from 57° pre-op to 35° post-op and correction was maintained (35°) at latest FU. T1-T12 increased by 4 mm for SR and 10 mm for DR with mean monthly gain of 0.5 and 1.39, respectively. T1-S1 gain was 4 mm for SR and 17 mm for DR with mean monthly gain of 0.5 mm for SR and 2.35 mm for DR. The mean interval between index surgery and the first distraction was 66 days and thereafter was 43 days. Complications included one superficial infection in (SR), one prominent implant (DR) and minimal loss of initial distraction in three after index MCGR (all SR). Overall, partial loss of distraction was observed following 14 of the 91 distractions (one DR and 13 SR). This loss was regained in subsequent distractions. There was no neurologic deficit or implant failure. CONCLUSION: MCGR appears to be safe and provided adequate distraction similar to the standard GR technique without the need for repeated surgeries. DR patients had better initial curve correction and greater spinal height. No major complications were observed during the short follow-up period. The FDA has not cleared the drug and/or medical device for the use described in this presentation (i.e., the drug or medical device is being discussed for an ‘off label’ use).postprin

Demyelination Disorders

Demyelination disorders are among the most frequent neurological conditions. Types of these disorders include multiple sclerosis, Guillain Barré syndrome, diabetic peripheral neuropathy, entrapment neuropathies, and others, all of which can result in serious physical incapacity and diminished quality of life. This book examines various aspects of demyelination from clinical, diagnostic, and therapeutic points of view. Chapters address different types of demyelination diseases, their associated mechanisms, and pharmacologic and nonpharmacologic treatment approaches, among other topics

EGFR signaling regulates synaptic connectivity via Gurken

The synapse is the essential unit of neural function. It is critical to understand how synapses form during development, how they are maintained throughout the life of an organism, and how their structure and function are affected by neural activity. An understanding of these aspects of synapses will likely provide insight into the etiology of neurodevelopmental disorders such as autism, mental retardation or epilepsy. To identify novel regulators of synaptic development, I screened for mutants with defects in synaptic morphology and growth at the Drosophila larval neuromuscular junction: NMJ). My screen identified several mutants with defects in various aspects of synaptic development. I pursued a more in depth analysis of an identified mutant, happyhour, with defects in synaptic target selection. Synaptic target selection is critical for establishing functional neuronal circuits. The mechanisms regulating target selection remain incompletely understood. I describe a role for the EGF receptor and its ligand Gurken in target selection of octopaminergic Type II neurons in the Drosophila neuromuscular system. Mutants in happyhour, a regulator of EGFR signaling, form ectopic Type II neuromuscular junctions. These ectopic innervations are due to inappropriate target selection. I demonstrate that EGFR signaling is necessary and sufficient to inhibit synaptic target selection by these octopaminergic Type II neurons, and that the EGFR ligand Gurken is the post-synaptic, muscle-derived repulsive cue. These results identify a new pathway mediating cell-type and branch-specific synaptic repulsion, a novel role for EGFR signaling in synaptic target selection, and an unexpected role for Gurken as a muscle-secreted repulsive ligand

EGFR signaling regulates synaptic connectivity via Gurken

The synapse is the essential unit of neural function. It is critical to understand how synapses form during development, how they are maintained throughout the life of an organism, and how their structure and function are affected by neural activity. An understanding of these aspects of synapses will likely provide insight into the etiology of neurodevelopmental disorders such as autism, mental retardation or epilepsy. To identify novel regulators of synaptic development, I screened for mutants with defects in synaptic morphology and growth at the Drosophila larval neuromuscular junction: NMJ). My screen identified several mutants with defects in various aspects of synaptic development. I pursued a more in depth analysis of an identified mutant, happyhour, with defects in synaptic target selection. Synaptic target selection is critical for establishing functional neuronal circuits. The mechanisms regulating target selection remain incompletely understood. I describe a role for the EGF receptor and its ligand Gurken in target selection of octopaminergic Type II neurons in the Drosophila neuromuscular system. Mutants in happyhour, a regulator of EGFR signaling, form ectopic Type II neuromuscular junctions. These ectopic innervations are due to inappropriate target selection. I demonstrate that EGFR signaling is necessary and sufficient to inhibit synaptic target selection by these octopaminergic Type II neurons, and that the EGFR ligand Gurken is the post-synaptic, muscle-derived repulsive cue. These results identify a new pathway mediating cell-type and branch-specific synaptic repulsion, a novel role for EGFR signaling in synaptic target selection, and an unexpected role for Gurken as a muscle-secreted repulsive ligand

'Clinical Triad' findings in Klippel-feil patients

E-Poster - Congenital Deformity: no. 530It has been propagated that Klippel-Feil Syndrome (KFS) is associated with the clinical triad findings (CTF) of short neck, low posterior hairline, and limited range of motion. This study noted that CTFs are not consistently noted in KFS patients. KFS patients with extensive congenitally fused cervical segments were more likely to exhibit one of the components of CTF.postprin