Involvement patterns in myotilinopathy and desminopathy detected by a novel neuromuscular whole-body MRI protocol

Abstract

Whole-body MRI (WB-MRI) has been successfully applied for oncologic and cardiovascular diagnostics, whereas imaging in myopathies usually employs dedicated protocols restricted to areas of specific interest. In this study, we propose a comprehensive neuromuscular WB-MRI protocol. Eighteen patients with degenerative and inflammatory muscle diseases were included. Whole-body imaging was performed on a 1.5-T MR system using parallel imaging. Examination time was 41:26 min. Coronal and axial T1-weighted and coronal short tau inversion recovery (STIR) sequences of the whole body were acquired. Images were analysed by two radiologists. With this protocol we could detect characteristic involvement patterns in different myofibrillar myopathies (MFMs): Patients with myotilinopathy showed frequent involvement of the rhomboid muscles (4/5), the erector spinae (5/5), the biceps femoris and the semimembranosus (5/5), while the semitendinosus was relatively spared (2/5). In contrast, in desminopathy patients the ilipsoas (3/4), the sartorius, (3/4), the gracilis (3/4) and the semitendinosus (3/4) were frequently involved, while the semimembranosus was spared (1/4). As shown for MFMs, WB-MRI is an appropriate modality to detect fatty infiltration and oedema in skeletal muscles. WB-MRI could be more useful than dedicated examinations for differential diagnosis, muscle biopsy planning and noninvasive follow-up examinations.

Whole-Body MRI for Evaluation of the Entire Muscular System

“…The authors concluded that 1.5T is the preferred modality for WB MRI (Schmidt et al 2007). In accordance, two 3T muscular WB MRI studies (Kesper et al 2009;Kornblum et al 2006) showed subjectively inferior image quality and more artifacts in comparison to an own study at 1.5T (Schramm et al 2008). On the other hand, scan time at 3T can be reduced at a constant image quality and further technical innovations will improve 3T WB imaging.…”

“…In one of our WB MRI studies including 18 patients with different degenerative and inflammatory myopathies we found an involvement of the erector spinae muscles in 61.1 % (Schramm et al 2008). Affection of the erector spinae muscles seems also to be common in patients with myotonic dystrophy type 1 and 2 as described by Kornblum et al in their WB MRI study (Kornblum et al 2006).…”

“…Affection of the erector spinae muscles seems also to be common in patients with myotonic dystrophy type 1 and 2 as described by Kornblum et al in their WB MRI study (Kornblum et al 2006). It has been shown in WB MRI studies that involvement of other muscle groups like in the shoulder girdle or the upper arms seem to be a common finding in many myopathies, too (Schramm et al 2008;Shelly et al 2010). Muscle MRI and especially WB MRI can detect subclinical involvement of muscle groups, this may be helpful for early diagnosis and differential diagnosis (Sookhoo et al 2007).…”

“…For example, WB MRI protocols designed to evaluate cardiovascular or oncological diseases differ from each other considerably. In this chapter, we provide an example of a comprehensive neuromuscular WB MRI protocol as it is used at the Ludwig-Maximilians-University Hospital in Munich/Germany (Schramm et al 2008).…”

“…In comparison to oncology and cardiovascular applications, there are still only few publications about comprehensive WB MRI protocols for evaluation of the skeletal muscles (Schramm et al 2008;Kesper et al 2009;Kornblum et al 2006;Quijano-Roy et al 2012), even though this is a promising field for WB MRI in our opinion.…”