ABSTRACT
The Covid-19 pandemic has highlighted the diffi- culty in the management of neuromuscular patients and the need for continued implementation of the standard of care. With the new pandemia psychometrically robust but quick outcome measures are needed to monitor patients' clinical status. The slow progressive nature of several muscle disorders and the wide pattern of involvement in muscular dystrophies and myopathies make it difficult to establish the prognosis, predict clinical evolution and perform trials and define the impact of natural history, and use new therapies that are becoming available. We constructed a motor function test that is easy and quick to use. This quick Motor Function test: Gait, Stair, Gower's, Chair (GSGC) was constructed based on the clinical expertise of several physicians involved in the care of DMD;LGMD, and Pompe patients. The GSGC score can be integrated by the use of the motor function of the upper motor limbs with the arm function test (GSGCA). It consists of a simple standardized functional test which grades the ability of the patient to raise their upper arms over the head. Grade 0 corresponds to a full circle of arm abduction, while with grade 6 the patients cannot raise their arms to their mouth and effectively use their hands. The Gardner-Medwin Walton (GMW) scale even modifi ed appears in comparison rather insensitive. The GSGC test includes 4 items. The test provides a detailed picture of motor function by including a quantitative measure of four performances i.e. time to perform four activities: Gait =walking for 10 meters, S=climbing 4 steps on a Stair, G= Gower's maneuver, C= rising from a Chair (Figure) The GSGC final score is obtained by adding the grades of the four functional tests and ranges from a minimum of 4 (normal performance) to a maximum of 27 (worst performance).GSGCA test includes 5 tests (total score from 5 to 32). Validity and test reliability were determined in a cohort of 9 adult Pompe patients (15 to 54 years of age) and then validated in 40 LOPD cases by a collaborative group. The responsiveness of the GSGCA scale to changes in clinical course over time was examined in a subgroup of 13 LGMD 2B/R2 untreated patients. Interrater and intrarater reliabilities were most usually confirmatory. The motor outcomes are different in various myopathies and depend on a correct diagnosis, while exercise in myopathy patients should be moderate, but not necessarily discouraged. The muscle MRI imaging might be helpful for follow-up of the proximal or distal muscle involvement, to detect fat, and connective tissue replacement, which might be usually absent in metabolic myopathies, except for LOPD. Diet and exercise in LOPD might be an additional therapeutic option synergistic to ERT. In this presentation, we examine the use of the GSGC scale in LOPD, DMD, and GSGCA scales in the natural history of LGMD R2. The development of smart care using telemedicine and eHealth technologies to share images, clinical data, reports, and video meetings of collaborative groups should be implemented. Keywords: GSGC scale, Covid-19, DMD, LGMD, Pompe.
ABSTRACT
Introduction: The airway management of patients with COVID-19 is a high risk task for anaesthesiologists. Several innovations have been born as a result of this problem, including aerosol boxes and clear plastic sheets. Aim: To compare the timing and attempt of direct laryngoscopy with and without aerosol box for intubation in patients undergoing general anaesthesia during the COVID-19 pandemic. Materials and Methods: This was a randomised controlled study was conducted in Parul Sevashram Hospital, Parul University, Vadodara, Gujrat, India from April 2021 to September 2021. A total of 50 patients were randomly divided into two equal groups as group A was intubated with an aerosol box and a macintosh laryngoscope, while group B was intubated with a clear plastic sheet and macintosh laryngoscope. With proper airway precautions and Personal Protective Equipments (PPE) comparative assessment of patients undergoing surgery in general anaesthesia was done. Time to intubate, number of attempts, ease of Endotracheal Tube (ETT) tube insertion, quality of Laryngoscopy view and Cormack Lehane scores were assessed in both the groups. Results: The mean time for intubation was high at 29.72 seconds in group A, while it was 23.16 seconds in group B;the difference was significant. Overall, 20 out of 25 (80%) patients could be intubated in 1st attempt in group B as compared to 15 out of 25 (60%) in group A. Airway visualisation using Percentage of Glottic Opening (POGO) scoring and Cormack Lehane staging were suggestive of better visualisation in group B than group A. Difficulties encountered during intubation like laryngoscopy, glottic visualisation, arm movement restriction, ETT negotiation, and stylet removal were lesser in group A as compared to group B. The incidence of complications like sore throat and airway bleeding were lower in group B as compared to group A. Conclusion: In the COVID-19 era, aerosol box and clear plastic sheets are effective barrier measures for airway management to prevent the anaesthesiologists from the aerosol transmission. But airway management with clear plastic sheet is technically easier than aerosol box.
ABSTRACT
Background: The current pandemic has reduced access to safe, monitored physical activity (PA) programs for persons with spinal cord injury (SCI). Inactivity can contribute to a multitude of secondary complications including worsening physiological and psychosocial health. The use of telerehabilitation has the potential for continuing activity engagement without the risk of virus exposure. The present study evaluates the feasibility and limited efficacy of an online group based PA program for persons with SCI. Methods: This preliminary pre-post study delivered an online group based PA program to persons with SCI (n=5). Inclusion criteria were the following: 18 years or older;sustained an SCI;living in the community;some upper limb function permitting arm movement against gravity. The program consisted of 50-minute sessions twice weekly for six weeks. After each session participants completed an adverse events diary and rate of perceived exertion. Psychosocial subscales from the NeuroQOL-SF were assessed at baseline and post-intervention. Paired sample t-tests were used to assess limited efficacy on outcomes of interest. Online PA satisfaction questionnaires were assessed at post-treatment. Results: Participants were between 3 - 32 years post-injury, females, and between the ages of 36 and 76 . 3 of the participants had quadriplegia and 4 had an incomplete injury. The program resulted in significant improvement post intervention on anxiety (p.