Universität zu Köln

Sprenger-Svacina, Alina, Haensch, Johannes, Weiss, Kilian ORCID: 0000-0003-4295-4585, Hokamp, Nils Grosse, Maintz, David, Schlamann, Marc, Fink, Gereon R. ORCID: 0000-0002-8230-1856, Schloss, Natalie, Laukamp, Kai, Wunderlich, Gilbert, Lehmann, Helmar C. and Lichtenstein, Thorsten (2023). MRI correlates of motoneuron loss in SMA. J. Neurol., 270 (1). S. 503 - 511. HEIDELBERG: SPRINGER HEIDELBERG. ISSN 1432-1459

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Abstract

Background Magnetic resonance imaging (MRI) is currently explored as supplemental tool to monitor disease progression and treatment response in various neuromuscular disorders. We here assessed the utility of a multi-parametric magnetic resonance imaging (MRI) protocol including quantitative water T-2 mapping, Dixon-based proton density fat fraction (PDFF) estimation and diffusion tensor imaging (DTI) to detect loss of spinal motor neurons and subsequent muscle damage in adult SMA patients. Methods Sixteen SMA patients and 13 age-matched controls were enrolled in this prospective, longitudinal study. All participants underwent MRI imaging including measurements of Dixon-based PDFF and DTI of the sciatic nerve. SMA patients furthermore underwent measurements of muscle water T-2 (T-2w) of the biceps femoris muscle (BFM) and quadriceps femoris muscle (QFM). Ten participants returned for a second scan six months later. MRI parameter were correlated with clinical data. All patients were on nusinersen treatment. Results There were significantly higher intramuscular fat fractions in the BFM and QFM of SMA patients compared to healthy controls at baseline and after 6 months. Furthermore, T2 values significantly correlated positively with intramuscular fat fractions. The Hammersmith functional motor scale significantly correlated with the QFM's intramuscular fat fractions. DTI scans of the sciatic nerve were not significantly different between the two groups. Conclusion This study demonstrates that, water T-2 mapping and Dixon-based PDFF estimation may distinguish between adult SMA patients and controls, due to massive intramuscular fat accumulation in SMA. More extensive long-term studies are warranted to further evaluate these two modalities as surrogate markers in SMA patients during treatment.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Sprenger-Svacina, Alina UNSPECIFIED UNSPECIFIED UNSPECIFIED Haensch, Johannes UNSPECIFIED UNSPECIFIED UNSPECIFIED Weiss, Kilian UNSPECIFIED orcid.org/0000-0003-4295-4585 UNSPECIFIED Hokamp, Nils Grosse UNSPECIFIED UNSPECIFIED UNSPECIFIED Maintz, David UNSPECIFIED UNSPECIFIED UNSPECIFIED Schlamann, Marc UNSPECIFIED UNSPECIFIED UNSPECIFIED Fink, Gereon R. UNSPECIFIED orcid.org/0000-0002-8230-1856 UNSPECIFIED Schloss, Natalie UNSPECIFIED UNSPECIFIED UNSPECIFIED Laukamp, Kai UNSPECIFIED UNSPECIFIED UNSPECIFIED Wunderlich, Gilbert UNSPECIFIED UNSPECIFIED UNSPECIFIED Lehmann, Helmar C. UNSPECIFIED UNSPECIFIED UNSPECIFIED Lichtenstein, Thorsten UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-674074
DOI:	10.1007/s00415-022-11326-1
Journal or Publication Title:	J. Neurol.
Volume:	270
Number:	1
Page Range:	S. 503 - 511
Date:	2023
Publisher:	SPRINGER HEIDELBERG
Place of Publication:	HEIDELBERG
ISSN:	1432-1459
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language SPINAL MUSCULAR-ATROPHY; SKELETAL-MUSCLE; DISEASE-ACTIVITY; MEDIAN NERVE; NEUROGRAPHY; BIOMARKERS Multiple languages Clinical Neurology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/67407