Abstract

CT and MRI is increasingly becoming an important tool in describing the degree and pattern of muscular involvement. We have implemented the database of skeletal muscle images, IBIC-NMD. The objectives of IBIC-NMD are to support the differential diagnosis, to detect a specific pattern of muscular involvement for each muscular disease and to describe objectively the individual disease progression. It covers a range of both pediatric and adult myopathies including the muscular dystrophies, congenital myopathies, metabolic myopathies and inflammatory myopathies. The technological aspect of the project is provided by Integrative Brain Imaging Center (IBIC) of National center of Neurology and Psychiatry, Japan. Collection of clinical and molecular genetic data and muscle images is based on online support system, IBISS (Integrative Brain Imaging Support Sysytem). The database can be accessed only by authorized persons using their login and password. The database was launched in the January 2012 and up to March 2013 contains 138 records from nine Japanese neuromuscular institutes. The database include 10 patients with Duchenne muscular dystrophy, 9 patients with limb-girdle muscular dystrophy, 9 patients with GNE myopathy and 110 patients with other myopathies (BMD, MD1, FSHD, PM, IBM, Pompe disease, OPMD, FCMD, nemaline myopathy). The database is expected to foster the research of skeletal muscle image.

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