http://www.archives-pmr.org/article/PIIS0003999310002352/abstract?rss=yes

Exercise Capacity and Idebenone Intervention in Children and Adolescents With Friedreich Ataxia
Presented in part to the American College of Sports Medicine, Seattle, WA, May 28, 2009.

Bart E. Drinkard, MSPTa, Randall E. Keyser, PhDae, Scott M. Paul, MDa, Ross Arena, PhD, PTf, Jonathan F. Plehn, MDb, Jack A. Yanovski, MD, PhDc, Nicholas A. Di Prospero, MD, PhDd+

Abstract
Drinkard BE, Keyser RE, Paul SM, Arena R, Plehn JF, Yanovski JA, Di Prospero NA. Exercise capacity and idebenone intervention in children and adolescents with Friedreich ataxia.

Objective
To determine the exercise capacity of children and adolescents with Friedreich's Ataxia (FA) and to evaluate the effects of 6 months of idebenone treatment on exercise capacity.

Design
Exploratory endpoint in a randomized double-blind, placebo-controlled, phase II clinical trial designed to investigate the effects of idebenone on a biomarker of oxidative stress.

Setting
Exercise physiology laboratory in a single clinical research center.

Participants
Ambulatory subjects (N=48; age range, 9–17y) with genetically confirmed FA.

Intervention
Idebenone administered orally 3 times a day for a total daily dose of approximately 5, 15, and 45mg/kg or matching placebo for 6 months.

Main Outcome Measures
Peak oxygen consumption per unit time (peak VO2) and peak work rate (WR) were measured during incremental exercise testing at baseline and after treatment. Echocardiography and neurologic assessments were also completed before and after treatment.

Results
Baseline mean peak VO2 ± SD was 746±246mL/min (16.2±5.8mL/kg/min), and WR was 40±23W for all subjects. Peak VO2 and WR were correlated with short guanine-adenine-adenine allele length and neurologic function. Relative left ventricular wall thickness was increased but left ventricular ejection fraction was normal in most subjects; there was no relationship between any exercise and echocardiographic measures. There were no significant changes in mean peak VO2 or WR after idebenone treatment at any dose level relative to placebo.

Conclusions
Exercise capacity in children and adolescents with FA was significantly impaired. The basis for the impairment appears to be multifactorial and correlated to the degree of neurologic impairment. Although idebenone has previously been shown potentially to improve features of FA, idebenone treatment did not increase exercise capacity relative to placebo.

a Rehabilitation Medicine Department, Hatfield Clinical Research Center, National Institutes of Health, Bethesda, MD

b Translational Medicine Branch, National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD

c Unit on Growth and Obesity, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD

d Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD

e Center for the Study of Chronic Illness and Disability, George Mason University, Fairfax, VA

f Departments of Internal Medicine, Physiology and Physical Therapy, Virginia Commonwealth University, Richmond, VA

Reprint requests to Bart E. Drinkard, MSPT, National Institutes of Health, Building 10 Room 1-1469, 10 Center Dr, Bethesda, MD 20892-1604

Supported by the National Institutes of Health/National Institute of Neurological Disorders and Stroke/National Heart, Lung, and Blood Institute intramural research funds.

No commercial party having a direct financial interest in the results of the research supporting this article has or will confer a benefit on the authors or on any organization with which the authors are associated.

This trial was registered with ClinicalTrials.gov (NCT00229632).

The opinions and information contained in this article are those of the authors and do not necessarily reflect those of the National Institutes of Health, the Department of Health and Human Services, or the United States Public Health Service.

PII: S0003-9993(10)00235-2

doi:10.1016/j.apmr.2010.04.007

© 2010 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.

---

http://www.jneuroengrehab.com/content/7/1/30

Innovative gait robot for the repetitive practice of floor walking and stair climbing up and down in stroke patients
Stefan Hesse , Andreas Waldner and Christopher Tomelleri

Journal of NeuroEngineering and Rehabilitation 2010, 7:30doi:10.1186/1743-0003-7-30


Published: 28 June 2010

Abstract (provisional)

Background
Stair climbing up and down is an essential part of everyday's mobility. To enable wheelchair-dependent patients the repetitive practice of this task, a novel gait robot, G-EO-Systems (EO, Lat: I walk), based on the end-effector principle, has been designed. The trajectories of the foot plates are freely programmable enabling not only the practice of simulated floor walking but also stair climbing up and down. The article intended to compare lower limb muscle activation patterns of hemiparetic subjects during real floor walking and stairs climbing up, and during the corresponding simulated conditions on the machine, and secondly to demonstrate gait improvement on single case after training on the machine.

Methods
The muscle activation pattern of seven lower limb muscles of six hemiparetic patients during free and simulated walking on the floor and stair climbing was measured via dynamic electromyography. A non-ambulatory, sub-acute stroke patient additionally trained on the G-EO-Systems every workday for five weeks.

Results
The muscle activation patterns were comparable during the real and simulated conditions, both on the floor and during stair climbing up. Minor differences, concerning the real and simulated floor walking conditions, were a delayed (prolonged) onset (duration) of the thigh muscle activation on the machine across all subjects. Concerning stair climbing conditions, the shank muscle activation was more phasic and timely correct in selected patients on the device. The severely affected subject regained walking and stair climbing ability.

Conclusions
The G-EO-Systems is an interesting new option in gait rehabilitation after stroke. The lower limb muscle activation patterns were comparable, a training thus feasible, and the positive case report warrants further clinical studies.

---

tp://www3.interscience.wiley.com/journal/123513804/abstract?CRETRY=1&SRETRY=0

Longitudinal tracking of gait and balance impairments in cerebellar disease
Susanne M. Morton, PhD, PT 1, Ya-Weng Tseng, PhD, PT 2, Kathleen M. Zackowski, PhD, OTR 3 4 5, Jaclyn R. Daline, PT, DPT 6, Amy J. Bastian, PhD, PT 3 4 5 *
1Graduate Program in Physical Therapy and Rehabilitation Science, University of Iowa Carver College of Medicine, Iowa City, Iowa, USA
2Department of Physical Therapy, Temple University, Philadelphia, Pennsylvania, USA
3Kennedy Krieger Institute, Baltimore, Maryland, USA
4Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
5Department of Physical Medicine and Rehabilitation, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
6Program in Physical Therapy, Washington University School of Medicine, St. Louis, Missouri, USA

email: Amy J. Bastian (bastian@kennedykrieger.org)

*Correspondence to Amy J. Bastian, Kennedy Krieger Institute, 707 N. Broadway, Room G-04, Baltimore, MD 21205

Potential conflict of interest: Nothing to report.
Funded by:
NIH
(NICHD/NCMRR); Grant Number: grants K01 HD050369, K01 HD049476, K12 HD055931, R01 HD040289
Foundation for Physical Therapy

---

http://www.plosgenetics.org/article/fetchObjectAttachment.action;jsessionid=6A548BAFE7F2A0DEABA1E6AAFD928738.ambra01?uri=info%3Adoi%2F10.1371%2Fjournal.pgen.1000984&representation=PDF

---

http://www.orthosupersite.com/view.aspx?rid=65219

Italian orthopaedists reported a low rate of complications when treating patients with high-grade neurological scoliosis using a hybrid construct that combined lumbar pedicle screws, Universal Clamps (Zimmer) and thoracic hooks.

“The hybrid construct … appears safe and effective in the treatment of patients affected by high-grade scoliosis, providing a good correction of deformity and reducing operative time, radiation exposure and blood loss with respect to all-screws constructs,” Guido La Rosa, MD, Chief of Orthopedic Unit, Research Institute Pediatric Hospital “Bambino Gesu,” Rome, Italy, told Orthopaedics Today Europe in an interview prior to the presentation of the study at the EFORT Congress 2010.

Study design

A prospective series of 15 neurological scoliosis patients were treated by La Rosa and colleagues between 2002 and 2008 with the hybrid construct. Patients ranged in age from 10 to 17 years, and etiologies were as follows: cerebral palsy in 12 patients, Friedreich’s ataxia in two and Aicardi Syndrome in one. All patients had a preoperative Cobb angle greater than 100°, according to the study abstract.

Story continues below↓

Neurological scoliosis with a marked hypokyphosis treated by means of bilateral all level lumbar screws plus all level Universal Clamps thoracic instrumentation. Excellent coronal correction with an optimal sagittal contouring. Images: La Rosa G

All patients were treated by posterior access using the hybrid construct to stabilize each affected level. A secondary posterior access was used in three patients to strengthen the effect of the Universal Clamps, adding a concave costotomy. Skull traction by sling and pelvic countertraction to control obliquity were performed in all cases. Pelvic instrumentation provided iliosacral screw fixation, and two concave rods and one convex were used in all patients, the investigators wrote in their abstract.

Passing the tape under the lamina and under the concave corrective rod.

Assembling each titanium clamp to the rod.

Tensioning of the tape by means of tightening-gun and tightening of the blocker at the end of correction.

Deformity correction

Final assembly of the hybrid construct.

The average percentage of correction was 70% (32°±7°), with an average loss of correction of 7° occurring during the mean 36-month follow-up period (range, 12–84 months). Mean operative time was 4 hours with a mean blood loss of 1800 mL. On average, six transpedicular screws (range, 4–11), seven Universal Clamps (range, 5–9) and five thoracic hooks (range, 4–6) were used in the hybrid constructs, according to the abstract.

“The amount of coronal correction is excellent and the control of the sagittal profile seems better than with all-screws assembly,” La Rosa said. “In conclusion, the overall incidence of complications has been quite similar to other corrective procedures — in particular, no neural complications have been recorded.”
 

• Reference:

La Rosa G, Giglio G, Oggiano L. Treatment of neurological scoliosis of more than 1008 by using a hybrid contstruct (pedicle screws plus universal clamps). Paper #F484. To be presented at the EFORT Congress 2010. June 2-5, 2010. Madrid.

 

---

http://www.citeulike.org/user/giovanni/article/7255387