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Page 1
Pathomechanics of Gowers' sign: a video analysis of a spectrum of Gowers' maneuvers.
Chang RF, Mubarak SJ. Chang RF, et al. Clin Orthop Relat Res. 2012 Jul;470(7):1987-91. doi: 10.1007/s11999-011-2210-6. Epub 2011 Dec 28. Clin Orthop Relat Res. 2012. PMID: 22203329 Free PMC article.
BACKGROUND: Gowers' sign is a screening test for muscle weakness, typically seen in Duchenne muscular dystrophy but also seen in numerous other conditions. ...Patients with moderate weakness have wide hip abduction, shifts in pelvic tilt, and lordosis, which reduce …
BACKGROUND: Gowers' sign is a screening test for muscle weakness, typically seen in Duchenne muscular dystrophy but also seen …
Juvenile dermatomyositis: a 20-year retrospective analysis of treatment and clinical outcomes.
Sun C, Lee JH, Yang YH, Yu HH, Wang LC, Lin YT, Chiang BL. Sun C, et al. Pediatr Neonatol. 2015 Feb;56(1):31-9. doi: 10.1016/j.pedneo.2014.02.006. Epub 2014 Jun 27. Pediatr Neonatol. 2015. PMID: 24985888 Free article.
After excluding one patient with an incomplete record, the remaining 31 patients who had muscle weakness were analyzed; among them, 22 (70.97%) achieved complete clinical response, but only six (19.4%) achieved complete clinical remission. Multivariate analysis showed that female …
After excluding one patient with an incomplete record, the remaining 31 patients who had muscle weakness were analyzed; among them, 22 (70.9 …
A Senile Case of Late-onset Pompe's Disease.
Takano H, Ishihara T, Kosuga M, Okuyama T. Takano H, et al. Intern Med. 2016;55(18):2723-5. doi: 10.2169/internalmedicine.55.6803. Epub 2016 Sep 15. Intern Med. 2016. PMID: 27629975 Free article.
A 72-year-old, seemingly healthy, Japanese man suddenly lost consciousness. At the emergency room, the patient's Glasgow coma scale score was 10 and a thoracic breathing pattern was observed. An arterial blood gas analysis indicated acute hypercarbic respiratory fai …
A 72-year-old, seemingly healthy, Japanese man suddenly lost consciousness. At the emergency room, the patient's Glasgow coma scale score
Gowers' sign in discitis in childhood.
Amir N, Hurvitz H, Korn-Lubetzki I, Shalev RS. Amir N, et al. Clin Pediatr (Phila). 1986 Sep;25(9):459-61. doi: 10.1177/000992288602500911. Clin Pediatr (Phila). 1986. PMID: 3742928
Three cases of discitis in early childhood presented with acute refusal to walk or change in gait pattern without neurologic deficits. In all three, a Gowers' sign was observed, although none had muscular weakness. The diagnosis of discitis was confirmed by t …
Three cases of discitis in early childhood presented with acute refusal to walk or change in gait pattern without neurologic deficits. In al …
Prednisolone in Duchenne muscular dystrophy with imminent loss of ambulation.
Pradhan S, Ghosh D, Srivastava NK, Kumar A, Mittal B, Pandey CM, Singh U. Pradhan S, et al. J Neurol. 2006 Oct;253(10):1309-16. doi: 10.1007/s00415-006-0212-1. Epub 2006 Jun 19. J Neurol. 2006. PMID: 16786214 Clinical Trial.
Till the ideal stage of the disease and the type or dosage of starting steroid therapy is defined by specially designed studies, 0.75 mg/Kg/day prednisolone therapy may be started in DMD patients at the stage of frequent falls ( > 10 / day) on walking or increased get-up time …
Till the ideal stage of the disease and the type or dosage of starting steroid therapy is defined by specially designed studies, 0.75 mg/Kg/ …
Turner Syndrome Mosaicism 45,X/46,XY with Genital Ambiguity and Duchenne Muscular Dystrophy: Translational Approach of a Rare Italian Case.
Lamanna B, Vinciguerra M, Dellino M, Cascella G, Cazzato G, Macorano E, Malvasi A, Scacco S, Cicinelli E, Loizzi V, Vimercati A, Cormio G, Paduano F, Cascardi E, Tatullo M. Lamanna B, et al. Int J Mol Sci. 2022 Nov 19;23(22):14408. doi: 10.3390/ijms232214408. Int J Mol Sci. 2022. PMID: 36430887 Free PMC article.
Duchenne muscular dystrophy (DMD) is an inherited neuromuscular disease with an X-Linked recessive pattern of inheritance that predominantly affects males, while females are usually asymptomatic. DMD has also been observed in groups of females affected by TS, not homozygou …
Duchenne muscular dystrophy (DMD) is an inherited neuromuscular disease with an X-Linked recessive pattern of inheritance that predominantly …
Spinal muscular atrophy with predominant lower extremity (SMA-LED) with no signs other than pure motor symptoms at the intersection of multiple overlap syndrome.
Tekin HG, Edem P, Özyılmaz B. Tekin HG, et al. Brain Dev. 2022 Apr;44(4):294-298. doi: 10.1016/j.braindev.2021.12.001. Epub 2021 Dec 30. Brain Dev. 2022. PMID: 34974950
CONCLUSION: It should be kept in mind that DYNC1H1 mutation, which we are accustomed to seeing with accompanying findings such as orthopedic and ocular dysmorphic findings, sensorineural EMG findings, and intellectual disability, can also observe with pure motor findings s …
CONCLUSION: It should be kept in mind that DYNC1H1 mutation, which we are accustomed to seeing with accompanying findings such as orthopedic …
Novel Homozygous Missense Mutation in CAPN3 Gene Detected in a Saudi Arabian Family With Limb-Girdle Muscular Dystrophy Type 2A.
Al-Harbi TM, Abdulmanaʼ SO, Dridi W. Al-Harbi TM, et al. J Clin Neuromuscul Dis. 2016 Dec;18(2):89-91. doi: 10.1097/CND.0000000000000129. J Clin Neuromuscul Dis. 2016. PMID: 27861222
We report a Saudi Arabian family with weakness in limb-girdle distribution: waddling gait, positive Gowers' sign, and marked muscle atrophy in the shoulder and pelvic girdle muscles. We sequenced all exonic and intronic regions of the CAPN3 gene and identified c.169 …
We report a Saudi Arabian family with weakness in limb-girdle distribution: waddling gait, positive Gowers' sign, and marked m …
Novel mutations in the gene encoding acid α-1,4-glucosidase in a patient with late-onset glycogen storage disease type II (Pompe disease) with impaired intelligence.
Muraoka T, Murao K, Imachi H, Kikuchi F, Yoshimoto T, Iwama H, Hosokawa H, Nishino I, Fukuda T, Sugie H, Adachi K, Nanba E, Ishida T. Muraoka T, et al. Intern Med. 2011;50(24):2987-91. doi: 10.2169/internalmedicine.50.5563. Epub 2011 Dec 15. Intern Med. 2011. PMID: 22185990 Free article.
On admission, the proximal muscles of the lower extremities were found to be predominantly affected, and a score of 3/5 was obtained on Medical Research Council (MRC) scale. Muscular atrophy was evident and Gowers' sign was positive. His functional vital capa …
On admission, the proximal muscles of the lower extremities were found to be predominantly affected, and a score of 3/5 was obtained …