Relationship between infrared skin radiation and functional tests in patients affected by Emery-Dreifuss muscular dystrophy: Part 2.

HYPOTHESIS: This study represents a second part of a recently published study about a new form of evaluation and development of rare genetic neurodegenerative diseases. The objective is to provide a more global vision of thermography with respect to the Emery-Dreifuss pathology, through the analysis of the data collection carried out for one year. The basic hypothesis is that thermography could become a valid tool for the diagnosis and follow-up of this pathology because is a very specific tool for registering temperature changes produced by a constant degenerative evolution of this muscular dystrophy.

Relationship between infrared skin radiation and muscular strength tests in patients affected by Emery-Dreifuss muscular dystrophy.

Considering that infrared thermography is presented as a diagnostic technique for non-invasive, non-ionizing, fast and easy to use imaging and Emery-Dreifuss muscular dystrophy is a clinical condition that seems to be related to changes in the emission of infrared radiation at the skin level due to its neurodegenerative character, we have conducted an investigation by infrared thermography and the use of functional strength tests in the lower limbs in a family of 4 affected members of Emery-Dreifuss muscular dystrophy to try to establish a relationship between the evolution of the disease and the emission of infrared radiation in this pathology at the lower limb level and provide a more general view of this disease for a better evaluation and monitoring of the disease.

Is infrared thermography (IRT) a possible tool for the evaluation and follow up of Emery-Dreifuss muscular dystrophy? A preliminary study.

HYPOTHESIS: The hypothesis of this work is that infrared thermography could become a valid tool for the diagnosis and follow-up of the Emery-Dreifuss disease due to putative temperature changes produced by a constant degenerative evolution of this muscular dystrophy. TESTING THE HYPOTHESIS: To justify this hypothesis we proposed a pilot study with 2 brothers affected of Emery-Dreifuss who present a very different age, with the principal objective to evidence a possible evolution of this pathology. Acquisition and comparison of images of computerized axial tomography (CT) and thermography (IRT) of the distal limbs in 2 affected brothers. DATA AND DISCUSSION: Important image correlations in the region of the thighs and the posterior region of the legs have been highlighted. The comparison between the CT and the thermography showed how the first results are encouraged and promising and open a possible new line of research on the evaluation and follow-up of this disease. Despite this, a larger number of studies are needed to validate the thermography as a diagnostic technique and follow-up of this pathology.

Theoretical basis for a new approach of studying Emery-Dreifuss muscular dystrophy by means of thermography.

INTRODUCTION: Emery-Dreifuss muscular dystrophy (EDMD) is a clinical condition characterized by neuro-skeletal and cardiac impairments. By means of thermography, an image acquisition technique that allows the recording of the heat emitted by objects or bodies, news insight can be obtained insights about the evaluation and follow-up of this disease. Actually, musculoskeletal disorders are a major cause of counseling and access to rehabilitation services and are some of the most important problems that affect the quality of life of many people. There are urgent both clinical and research needs for the assessment and follow-up of patients with Emery-Dreifuss muscular dystrophy and the thermography is a rapid, non-invasive, easy to use and objective technique that analyzes the temperature of the examined tissue. HYPOTHESIS: The main aim is to offer a new possible hypothesis of validating the thermography techniques that support the evaluation and clinical follow-up of the Emery-Dreifuss dystrophy. To carry out this work we rely on the evidence of the existing bibliography. To perform this work and to evaluate the current situation on this topic, a systematic review was carried and after the application of an automatic and manual filter, inclusion and exclusion criteria, a total of 0 articles was obtained. Unfortunately, there is a lack of articles that relate the use of thermography in the Emery-Dreifuss muscular dystrophy. Due to the absence of information, we have expanded the search to articles concerning the use of thermography in relation to alterations of the musculoskeletal system compatible with those of Emery-Dreifuss, genetic diseases related to the X chromosome and more generally muscular atrophy. Based on other studies and results carried out in diseases that show signs and symptoms similar to Emery-Dreifuss Muscular Dystrophy, we believe that a new line of translational research could be opened with novel findings and we think that thermography could be an optimal tool for the clinical monitoring of this pathology. We believe that it would be of a great importance to carry out an observational study, to lay the foundations for future works, that relate thermography to the Emery-Dreifuss muscular dystrophies.