Objective To develop and apply an implanted neuroprosthesis to restore arm and hand function to individuals with high level tetraplegia Design Case study. Setting Clinical research laboratory. Participants Two individuals with spinal cord injuries at or above the C4 motor level. Interventions The individuals were each implanted with two stimulators (24 stimulation channels and 4 myoelectric recording channels total). Stimulating electrodes were placed in the shoulder and arm, including the first chronic application of spiral nerve cuff electrodes to activate a human limb. Myoelectric recording electrodes were placed in the head and neck areas. Main Outcome Measures The successful installation and operation of the neuroprosthesis, along with the electrode performance, range of motion, grasp strength, joint moments, and performance in activities of daily living. Results The neuroprosthesis system was successfully implanted in both individuals. Spiral nerve cuff electrodes were placed around upper extremity nerves and activated the intended muscles. In both individuals, the neuroprosthesis has functioned properly for at least 2.5 years post-implant. Hand, wrist, forearm, elbow and shoulder movements were achieved. A mobile arm support was needed to support the mass of the arm during functional activities. One individual was able to perform several activities of daily living with some limitations due to spasticity. The second individual was able to partially complete two activities of daily living. Conclusions Functional electrical stimulation is a feasible intervention for restoring arm and hand functions to individuals with high tetraplegia. Forces and movements were generated at the hand, wrist, elbow and shoulder that allowed the performance of activities of daily living, with some limitations requiring the use of a mobile arm support to assist the stimulated shoulder forces.

Objective-To evaluate the clinical effects of spinal cord stimulation (SCS) to restore cough in subjects with cervical spinal cord injury.Design-Clinical trial assessing the clinical outcomes and side effects associated with the cough system. Setting-Out-patient hospital or residenceParticipants-Subjects (N = 9; 8 men, 1 woman) with cervical spinal cord injury Disclosure: We certify that we have affiliations with or financial involvement (eg, employment, consultancies, honoraria, stock ownership or options, expert testimony, grants and patents received or pending, royalties) with an organization or entity with a financial interest in, or financial conflict with, the subject matter or materials discussed in the manuscript and all such affiliations and involvements are disclosed on the title page of the manuscript. Intervention(s)-SCS was performed at home by either the subjects themselves or caregivers on a chronic basis and as needed for secretion management. Explanation ofMain Outcome Measure(s)-Ease in raising secretions, requirement for trained caregiver support related to secretion management and incidence of acute respiratory tract infections.Results-The degree of difficulty in raising secretions improved markedly, and the need for alternative methods of secretion removal was virtually eliminated. Subject life quality related to respiratory care improved with subjects reporting greater control of breathing problems and enhanced mobility. The incidence of acute respiratory tract infections fell from 2.0 ± 0.5 to 0.7 ± 0.4 events/ subject year (p < 0.01), and mean level of trained caregiver support related to secretion management measured over a 2 week period decreased from 16.9 ± 7.9 to 2.1 ± 1.6 and 0.4 ± 0.3 times/week (p < 0.01) at 28 and 40 weeks following implantation of the device, respectively. Three subjects developed mild hemodynamic effects which abated completely with continued SCS. Subjects experienced mild leg jerks during SCS, which were well tolerated. There were no instances of bowel or bladder leakage.Conclusion(s)-Restoration of cough via SCS is safe and efficacious. This method improves life quality and has the potential to reduce the morbidity and mortality associated with recurrent respiratory tract infections in this patient population.

Inadequate or severely deficient levels of VitD-25(OH) were highly prevalent in patients with SCI admitted to an acute inpatient rehabilitation service. Evaluation of serum VitD-25(OH) levels are recommended in patients with SCI because low levels may contribute to osteoporosis.

Objective(s)-To explore and quantify the physical and functional effects of stabilizing the torso with electrical stimulation of the paralyzed hip and trunk musculature after motor complete tetraplegia.Design-Single-subject case study with repeated measures and concurrent controls. Setting-Academic outpatient rehabilitation center.Participants-Forty-four year old male with C4 ASIA A tetraplegia 20 years post spinal cord injury.Intervention-A surgically implanted multichannel pulse generator and intramuscular stimulating electrodes to activate lumbar erector spinae, quadratus lumborum, and gluteus maximus muscles bilaterally.Main Outcome Measure(s)-Outcomes assessed with and without stimulation included a) spinal alignment and pelvic orientation, b) pulmonary function and ventilatory volumes, c) forward bimanual reaching distance, d) seated stability and resistance to externally applied disturbances, e) maximal force and speed of rowing-like movements, and the ability to f) independently return to an erect seated position from full forward or lateral flexion, and g) roll in bed without assistance. SUPPLIERSThe custom multichannel implanted pulse generator, stimulating electrodes, external controller and associated software utilized in this study were designed, fabricated and supplied by the Technical Development Laboratory of the Cleveland FES and Advanced Platform Technology Centers of Excellence of the US Department of Veterans Affairs, 3 rd Floor Bingham Building, Case Western Reserve University, 10900 Euclid Avenue, Cleveland OH 44106. These custom devices are for investigational use only and are unavailable for sale. The VICON 370 motion capture system is a product of VICON (London, UK), the business entity formed by the combination of Vicon Motion Systems and Peak Performance Inc. Vicon is a subsidiary of Oxford Metrics Group (OMG), a public company trading on the London stock exchange. The Biodex System 3 robotic dynamometer is a product of Biodex Medical Systems, 20 Ramsay Road, Shirley, New York, 11967-4704.We certify that no party having a direct interest in the results of the research supporting this article has or will confer a benefit on us or on any organization with which we are associated AND, if applicable, we certify that all financial and material support for this research (eg, NIH or NHS grants) and work are clearly identified in the title page of the manuscript. NIH Public Access Author ManuscriptArch Phys Med Rehabil. Author manuscript; available in PMC 2010 February 1. Results-Stimulation improved spinal convexity and kyphosis by 26° and 21°, reduced posterior pelvic tilt by 11°; increased forced expiratory volume and vital capacity by 10% and 22%, and improved forward reach by > 7 cm. Average resistance to sagittal disturbances increased by > 40% (p<0.002) and mean force exerted during underhanded pulling more than doubled (p=0.014) with stimulation. Restoration of upright sitting in both sagittal and coronal planes and bed turning were made possible through appropriately timed ac...

Objective The aims of this study were to report the current incidence of wheelchair breakdowns, repairs, and consequences and to compare current data with historical data. Design A convenience sample survey of 723 participants with spinal cord injury who use a wheelchair for more than 40 hrs/wk treated at a Spinal Cord Injury Model Systems center was conducted. Results Significant increases were found in the number of participants reporting repairs (7.8%) and adverse consequences (23.5%) in a 6-mo period (2006Y2011) compared with historical data (2004Y2006) (P G 0.001). When examining current data, minorities experienced a greater frequency and higher number of reported consequences (P = 0.03). Power wheelchair users reported a higher number of repairs and consequences than did manual wheelchair users (P G 0.001). Wheelchairs equipped with seat functions were associated with a greater frequency of adverse consequences (P = 0.01). Repairs did not vary across funding source, but individuals with wheelchairs provided by Medicare and Medicaid reported a higher frequency of consequences than did the combined group of the Department of Vocational Rehabilitation, Worker’s Compensation, and the Veterans Administration (P = 0.034 and P = 0.013, respectively). Conclusions The incidence and consequences of repairs are increasing from what was already a very high statistic in this United States population. Further investigation into causality is required, and intervention is needed to reverse this potential trend.

Hereditary inclusion body myopathy (HIBM) is an autosomal recessive adult-onset myopathy due to mutations in the GNE (UDP-N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase) gene. Affected patients have no therapeutic options. We have previously demonstrated in preclinical testing the ability to safely correct GNE gene function through liposomal delivery of the wild-type GNE gene. Results were verified in a single patient treated by intravenous infusion of GNE gene lipoplex. A single patient (patient 001) with severe HIBM treated with a compassionate investigational new drug received seven doses of GNE gene lipoplex via intravenous infusion at the following doses: 0.4, 0.4, 1.0, 4.0, 5.0, 6.0, and 7.0 mg of DNA. GNE transgene expression, downstream induction of sialic acid, safety, and muscle function were evaluated. Transient low-grade fever, myalgia, tachycardia, transaminase elevation, hyponatremia, and hypotension were observed after infusion of each dose of GNE gene lipoplex. Quadriceps muscle expression of the delivered GNE, plasmid, and RNA was observed 24 hr after the 5.0-mg dose and at significantly greater levels 72 hr after the 7.0-mg infusion in comparison with expression in quadriceps muscle immediately before infusion. Sialic acid-related proteins were increased and stabilization in the decline of muscle strength was observed. We conclude that clinical safety and activity have been demonstrated with intravenous infusion of GNE gene lipoplex. Further assessment will involve a phase I trial of intravenous administration of GNE gene lipoplex in individuals with less advanced HIBM with more muscle function.

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Epidemiologic study, level III.