Navegando por Palavras-chave "Myotonic dystrophy"

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    Intraocular pressure, corneal thickness, and corneal hysteresis in Steinert's myotonic dystrophy
    (Conselho Brasileiro de Oftalmologia, 2011-06-01) Garcia Filho, Carlos Alexandre de Amorim [UNIFESP]; Prata, Tiago dos Santos [UNIFESP]; Sousa, Aline Katia Siqueira [UNIFESP]; Doi, Larissa Morimoto [UNIFESP]; Melo Jr., Luiz Alberto Soares [UNIFESP]; Universidade Federal de São Paulo (UNIFESP)
    PURPOSE: Low intraocular pressure (IOP) measured by Goldmann applanation tonometry (GAT) is one of the ocular manifestations of Steinert's myotonic dystrophy. The goal of this study was to evaluate the corneal-compensated IOP as well as corneal properties (central corneal thickness and corneal hysteresis) in patients with myotonic dystrophy. METHODS: A total of 12 eyes of 6 patients with Steinert's myotonic dystrophy (dystrophy group) and 12 eyes of 6 age-, race-, and gender-matched healthy volunteers (control group) were included in the study. GAT, Dynamic Contour Tonometry (DCT-Pascal) and Ocular Response Analyzer (ORA) were used to assess the IOP. Central corneal thickness was obtained by ultrasound pachymetry, and corneal hysteresis was analyzed using the ORA device. In light of the multiplicity of tests performed, the significance level was set at 0.01 rather than 0.05. RESULTS: The mean (standard deviation [SD]) GAT, DCT, and corneal-compensated ORA IOP in the dystrophy group were 5.4 (1.4) mmHg, 9.7 (1.5) mmHg, and 10.1 (2.6) mmHg, respectively. The mean (SD) GAT, DCT, and corneal-compensated ORA IOP in the control group was 12.6 (2.9) mmHg, 15.5 (2.7) mmHg, and 15.8 (3.4) mmHg, respectively. There were significant differences in IOP values between dystrophy and control groups obtained by GAT (mean, -7.2 mmHg; 99% confidence interval [CI], -10.5 to -3.9 mmHg; P<0.001), DCT (mean, -5.9 mmHg; 99% CI, -8.9 to -2.8 mmHg; P<0.001), and corneal-compensated ORA measurements (mean, -5.7 mmHg; 99% CI, -10.4 to -1.0 mmHg; P=0.003). The mean (SD) central corneal thickness was similar in the dystrophy (542 [31] µm) and control (537 [11] µm) groups (P=0.65). The mean (SD) corneal hysteresis in the dystrophy and control groups were 11.2 (1.5) mmHg and 9.7 (1.2) mmHg, respectively (P=0.04). CONCLUSIONS: Patients with Steinert's myotonic dystrophy showed lower Goldmann and corneal-compensated IOP in comparison with healthy individuals. Since central corneal thickness and corneal hysteresis did not differ significantly between groups, the lower IOP readings documented in this dystrophy seem not to be related to changes in corneal properties.
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    Temporomandibular Joint Magnetic Resonance Imaging Analysis in Adults with Steinert's Myotonic Dystrophy
    (Soc Chilena Anatomia, 2013-03-01) Guimaraes, Antonio Sergio [UNIFESP]; Galdames, Ivan Claudio Suazo [UNIFESP]; Marie, Suely Kazue Nagahashi [UNIFESP]; Universidade Federal de São Paulo (UNIFESP); Univ Diego Portales; Universidade de São Paulo (USP)
    The aim was to analyze the characteristics of the temporomandibular joint (TMJ) in a group of adult patients suffering from Steinert's muscular dystrophy (DM1). This study included 42 adult patients aged between 21 and 69 years (mean = 38.7619; SD = 12.74) who were diagnosed for DM1. Study was conducted using the MRI of right and left TMJ sagittal images taken in maximum intercuspidation position and maximum oral opening without pain, and the following were discussed: a) the quality of the cortical bone in the mandibular fossa, tuberosity, and mandibular head; b) the relationship of mandibular head-disc-joint tuberosity in maximum intercuspidation position; c) the anatomical shape of the articular disc. All patients showed abnormalities in the shape and surface of the cortical bone in the mandibularfossa, tuberosity, and the mandibular head. With regard to the relationship of the mandibular head in the mandibular fossa, 41% was found in the region 2B, 29% in 1B, 18% in 1A, 9% in 2C, and 3% in 2A of the TMJ. About 49% of the disc in maximum intercuspidation position was found on the mandibular head, 26% were anterior displaced, 14% had anterior dislocations, 38% had alterations in the form of disc, and 30% had preserved the anatomical shape. However, 98% of the discs showed hyposignal in T1 and T2. Using MRI, the decrease in the muscle activity in patients with DM1 was found to generate degenerative changes visible in the TMJ.