Sarah Schlaeger1, Elisabeth Klupp1, Dominik Weidlich2, Barbara Cervantes2, Marcus Deschauer3, Benedikt Schoser4, Sarah Bublitz3, Federica Montagnese4, Christoph Katemann5, Hendrik Kooijman5, Ernst J. Rummeny2, Claus Zimmer1, Jan S. Kirschke1, and Dimitrios C. Karampinos2
1Department of Diagnostic and Interventional Neuroradiology, Technical University of Munich, Munich, Germany, 2Department of Diagnostic and Interventional Radiology, Technical University of Munich, Munich, Germany, 3Department of Neurology, Technical University of Munich, Munich, Germany, 4Friedrich-Baur-Institut, Ludwig-Maximilians-University, Munich, Germany, 5Philips Healthcare, Hamburg, Germany
Synopsis
The assessment of fatty
infiltration and edema in the whole body musculature of patients with
neuromuscular diseases typically requires the separate performance of a T1-weighted
sequence and a fat suppressed T2-weighted sequence. T2-weighted
Dixon TSE enables the generation of T2-weighted fat-separated and
water-separated images, which could be used to simultaneously assess fatty
infiltration and edema and to reduce total scan time. The present study
examines the diagnostic performance of whole body T2-weigthed Dixon
TSE imaging in 10 patients with neuromuscular diseases.
Purpose
MRI has been emerging as a
popular technique in the diagnostic work up of patients with neuromuscular
diseases in order to characterize whole body disease progression and guide muscle
biopsy sites.1 The most important skeletal muscle degenerative
alternations include fatty infiltration and edematous changes, which have been
traditionally assessed based on the grading of T1-weighted images 2
and STIR images 3, respectively. However, the acquisition of two
separate sequences in a whole body scan setting can lengthen the total
acquisition time. A T2-weighted Dixon Turbo Spin Echo (TSE) sequence enables the simultaneous
generation of T2-weighted fat-separated and water-separated images 4,5,
which could be used to simultaneously assess fatty infiltration and edematous
changes, respectively. Therefore, the purpose of the present study is to
examine the diagnostic performance of T2-weighted Dixon TSE for
accelerated simultaneous grading of whole body skeletal muscle fat infiltration
and edema in patients with neuromuscular diseases.Methods
Subjects:
10 patients (6 females and 4
males; median age 46 years; range 30 – 63 years) with varied hereditary neuromuscular
diseases were recruited: muscular dystrophies (n=8), ALS (n=1) and congenital
myopathy (n=1). Diagnosis was performed on the basis of muscle biopsies and/or
genetic testing.
MRI measurements:
Patients underwent whole-body MRI
on a 3T system (Ingenia, Philips Healthcare). Three groups of pulse sequences
were performed in 5-6 axial stacks starting from the neck region to the
talocalcanean joint: a 2-point Dixon FFE in order to generate T1-weighted
images, a STIR TSE in order to generate T2-weighted images and a
2-point Dixon TSE in order to generate T2-weighted fat- and
water-separated images (Table 1). Water-fat separation of the TSE and FFE
images was performed online using the mDixon algorithm 5.
Muscle grading:
In order to compare the diagnostic performance of the three
sequences, two different visual rating scales were used to grade the degree of
pathological transformation in the patients’ muscles. For each patient 26 thigh
muscles, 13 muscles on each leg, were scored conformingly by two evaluators.
Severity of fatty infiltration was categorized via four different grades using
the Mercuri grading scale2: score 1 for muscles with normal
appearance, score 2 for muscles with fat filling not more than 30% of the
volume, score 3 for muscles with fatty infiltration of 30 to 60% and score 4
for fatty infiltration of more than 60%. The Mercuri grading scale was applied
independently on Dixon FFE fat and the T2-weighted Dixon TSE fat
images. To rate hyperintensity indicating edema a three point scale published
by Morrow et al.3 was used: score 0 for the absence of
hyperintensity, score 1 for mild hyperintensity and score 2 for definite
hyperintensity. The Morrow grading scale was applied independently on STIR TSE
sequence and T2-weighted Dixon TSE water images. The agreement
between the grading from different sequences was assessed using the Kappa
agreement test.Results
Figure 1 shows representative
slices of the whole body scan of a severely affected LGMD2A patient. Figure 2 shows the Mercuri
grading in a patient’s thigh. Affected muscles were graded equally on both
Dixon FFE fat and T2-weighted Dixon TSE fat images: the gracilis with
score 1, the sartorius with score 2, the vastus lateralis with score 3 and the
long head of biceps femoris with score 4.
Table 2 summarizes the excellent agreement
for the Mercuri grading based on the Dixon FFE fat and the T2-weighted
Dixon TSE fat images (Kappa coefficient = 0.93). Figure 3 shows the Morrow grading
in a patient’s thigh. Affected muscles were graded equally on both the STIR TSE
and the T2-weighted Dixon TSE water images: the gracilis with score
0, the long head of biceps femoris and the semitendinosus with score 1 and the vastus
lateralis with score 2.
Tabel 3 summarizes the excellent agreement for the
Morrow grading based on the STIR TSE and the T2-weighted Dixon TSE
water images (Kappa coefficient = 0.89).
Discussion and Conclusion
The present analysis shows a
statistically equivalent rating of fatty infiltration in Dixon FFE fat and T2-weighted
Dixon TSE fat images and a statistically equivalent rating of edematous changes
in STIR TSE and T2-weighted Dixon TSE water images. The replacement
of the two sequences by only one obviously reduces the total scan time. In
addition, the employed T2-weighted Dixon TSE protocol does not
require any breath-holding. However, the generally brighter appearance of
muscle tissue in the T2-weighted Dixon TSE water requires careful
comparison of the muscles within one patient. In conclusion, T2-weighted
Dixon TSE can be considered as a good alternative in the diagnostics of
neuromuscular diseases.Acknowledgements
The present work was
supported by the German Society for Muscle Diseases, the European Research
Council (grant agreement No 637164-iBack and grant agreement
No 677661-ProFatMRI) and Philips Healthcare.References
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