Dataset Information

Pathomechanics of Gowers' sign: a video analysis of a spectrum of Gowers' maneuvers.

ABSTRACT:

Background

Gowers' sign is a screening test for muscle weakness, typically seen in Duchenne muscular dystrophy but also seen in numerous other conditions. The mildest presentations and the variations of Gowers' sign are poorly described in the literature but are important to recognize to help with early diagnosis of a neuromuscular problem.

Questions/purposes

We therefore (1) defined the characteristics of the mildest forms and the compensatory mechanism used, (2) categorized the spectrum of this sign as seen in various neuromuscular diseases, and (3) provide educational videos for clinicians.

Methods

We videotaped 33 patients with Gowers' sign and three healthy children. Weakness was categorized as: mild = prolonged or rise using single-hand action; moderate = forming prone crawl position and using one or two hands on thigh; severe = more than two thigh maneuvers, rising with additional aid, or unable to rise.

Results

The earliest changes were exaggerated torso flexion, wide base, and equinus posturing, which reduce hip extension moment, keep forces anterior to the knee, and improve balance. Patients with moderate weakness have wide hip abduction, shifts in pelvic tilt, and lordosis, which reduce knee extension moment, improve hamstrings moment arm, and aide truncal extension. The classic Gowers' sign (severe) exaggerates all mechanisms.

Conclusions

The classically described Gowers' sign is usually a late finding. However more subtle forms of Gowers' sign including mild hand pressure against the thigh and prone crawl position should be recognized by clinicians to initiate additional diagnostic tests.

SUBMITTER: Chang RF

PROVIDER: S-EPMC3369091 | biostudies-literature |

REPOSITORIES: biostudies-literature

ACCESS DATA

Similar Datasets

Project description:ObjectiveTo assess the performance of the 'separation sign' as a predictor of normal placental separation in a large cohort of women at risk for placenta accreta spectrum (PAS) and in a high-risk subgroup with placenta previa or anterior low-lying placenta and at least one previous Cesarean delivery.MethodsThis was a prospective study of women at risk for PAS referred to a specialist clinic at between 22 and 38 weeks' gestation. All women underwent ultrasound assessment for the presence of the separation sign, which detects the difference in elasticity between the myometrium and the placenta, characterized by different rates of rebound after an ultrasound probe is used to apply pressure over the uteroplacental interface. When the sign is positive, the placenta appears to move relative to the myometrium, leading to the appearance or enhancement of the clear zone. The predictive performance of the separation sign for normal spontaneous placental separation at delivery was assessed.ResultsOf the 194 included women, 163 had a positive separation sign, all of whom went on to have normal placental separation at delivery. Of the 24 women with a negative separation sign, three (12.5%) had normal placental separation and 21 (87.5%) were diagnosed with PAS. This yielded a sensitivity of 98.2% (95% CI, 94.8-99.6%) and specificity of 100% (95% CI, 83.9-100%). In the high-risk cohort (n = 35), a positive separation sign remained a reliable predictor of normal placental separation, with a positive predictive value of 100%, sensitivity of 88.9% (95% CI, 65.3-98.6%) and specificity of 100% (95% CI, 80.5-100%).ConclusionsThe separation sign could be a useful tool in women considered to be at risk for PAS, as it can facilitate the prediction of normal placental separation at delivery. This may prevent overtreatment, the associated iatrogenic morbidity and unnecessary allocation of clinical resources. © 2022 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Project description:BackgroundVagal maneuvers (VagMs) are recommended as the first-line treatment of supraventricular tachycardia (SVT). However, the optimal type of VagMs remains unproven.AimThis study aims to compare the effectiveness and adverse events amongst VagMs on SVT via network meta-analyses (NMAs).MethodsWe systematically searched randomized controlled trials (RCTs) that involved adults with SVT and compared VagMs without language restrictions. We determined the initial and final responses of conversion rate to sinus rhythm and adverse events. Risk of bias (RoB) was appraised by Cochrane revised tool, and contribution matrix was calculated. NMAs were synthesized using frequentist random-effects model and presented as relative risk (RR) with 95% CI. The order of probability was presented as surface under the cumulative ranking curve analysis (SUCRA). Sensitivity analysis was performed using both Bayesian and frequentist approach with fixed- or random-effects models. Certainty of evidence (CoE) was rated by using the Grading of Recommendations, Assessment, Development, and Evaluations methodology.ResultsFourteen RCTs with 2,180 patients were enrolled. Small portion of mixed estimates was contributed from high overall RoB studies. Compared with carotid sinus massage (CSM), the modified Valsalva maneuver (MVM) was the most effective VagM after initial performance [SUCRA: 0.9992, RR: 5.47 (1.77-16.93)] and at the end of study [SUCRA: 1.0000, RR: 3.62 (2.04-6.39), CoE: high]. The standard VM did not elicit better conversion rate to the sinus rhythm than CSM at the initial response [SUCRA: 0.4395, RR: 1.97 (0.63-6.15)] and at the end of the study [SUCRA: 0.4795, RR: 1.64 (0.94-2.87), CoE: moderate]. The SUCRA value of CSM at the initial and final responses was the least one amongst three VagMs (0.0613 and 0.0205, respectively). Adverse events amongst three VagMs were similar (CoE: low). Sensitivity analyses yielded consistent results.ConclusionWe recommended MVM as the first choice of VagM for rhythm conversion before the pharmacological management of SVT.

Project description:Dendritic cell-specific ICAM-3-grabbing non-integrin (DC-SIGN/CD209) and liver/lymph node-specific ICAM-grabbing non-integrin (L-SIGN/CD299) which are homologues of DC-SIGN are important members in C-type lectin receptors family as key molecules to recognize and eliminate pathogens in the innate immune system. DC-SIGN and L-SIGN have become hot topics in recent studies which both served as cell adhesion and phagocytic pathogen recognition receptors in mammals. However, there have been almost no studies of DC-SIGN and L-SIGN structure and characters in fish, only DC-SIGN in the zebrafish had been studied. In our study, we identified and characterized the full-length miiuy croaker (Miichthys miiuy) DC-SIGN (mmDC-SIGN) and L-SIGN (mmL-SIGN) genes. The sequence analysis results showed that mmDC-SIGN and mmL-SIGN have the same domains with other vertebrates except primates, and share some conserved motifs in CRD among all the vertebrates which play a crucial role in interacting with Ca(2+) and for recognizing mannose-containing motifs. Gene synteny of DC-SIGN and L-SIGN were analyzed for the first time and gene synteny of L-SIGN was conserved among the five fishes. Interestingly, one gene next to L-SIGN from gene synteny had high similarity with L-SIGN gene that was described as L-SIGN-like in fish species. While only one L-SIGN gene existed in other vertebrates, two L-SIGN in fish may be in consequence of the fish-specific genome duplication to adapt the specific environment. The evolutionary analysis showed that the ancestral lineages of L-SIGN gene in fishes experienced purifying selection and the current lineages of L-SIGN gene in fishes underwent positive selection, indicating that the ancestral lineages and current lineages of L-SIGN gene in fishes underwent different evolutionary patterns. Both mmDC-SIGN and mmL-SIGN were expressed in all tested tissues and ubiquitously up-regulated in infected liver, spleen and kidney at different sampling time points, indicating that the mmDC-SIGN and mmL-SIGN participated in the immune response to defense against bacteria infection.