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Clinical and Cytogenetic Study in Patients with Möbius Syndrome at the General Hospital
Tessa Buckle, PhD1; Thijs Engelen1; Berit M. Verbist, MD, PhD1; Mark A. van Buchem, MD, PhD1; Martijn J.A. Malessy, MD, PhD2; Fijs W.B. van Leeuwen, PhD1
1Radiology/Interventional Molecular Imaging Laboratory, Leiden University Medical Center, Leiden, Netherlands; 2Neurosurgery, Leiden University Medical Center, Leiden, Netherlands
Introduction: Nerve damage to the cervical or brachial plexus results in reduced sensory and/or motor function. Damage results in neuronal cell death, which in turn leads to a decrease in the volume of the dorsal root ganglion (DRG). Morphologic evaluation can potentially provide a non-invasive measure for the degree of functional recuperation after damage. The purpose of this study was to evaluate the use of D-prep MR neurography (MRN) in the evaluation of morphological changes in the DRGs and nerves of the cervical and brachial plexus. Materials and Methods: Five healthy volunteers and eight patients (Schwannoma or MPNST between C7 and T1 (n=4) or trauma (n=4) underwent an MRI of the cervical and/or brachial plexus (3T; Philips Ingenia) using standard T2 STIR and a D-prep MRN sequence. The detectability and dimensions of DRGs from C1 through T1 and nerves of the cervical and brachial plexus were assessed with both approaches. Volumetric measurements of the DRGs were performed according to the Cavalieri principle.
Results: (Volumetric) measurements were feasible in all patients and volunteers. DRGs (especially of the cervical plexus) were more clearly visualized with D-prep MRN compared to T2 STIR. In the volunteers DRG volume increased from 30-60 mm3 in C2-C4 to 180-200 mm3 in C7-C8. Spinal nerves of the cervical plexus (C2-C4; diameter 19.1 +/- 3.6 mm) and/or brachial plexus (C5-C8; diameter 39.7 +/- 4.8 mm) could be accurately visualized, and traced downward from their ganglion. In patients dimensions of unaffected nerves and DRGs were comparable to the measurements in healthy volunteers. Neuropathy after trauma (sustained > 2 months prior to scan) resulted in a decrease in DRG volume at the affected location (24.5 +/- 6%) compared to the contralateral side. Tumor invasion prohibited measurements of directly affected nerves and DRGs. Compression of nerves in close proximity to the tumor co-occurred with local edema and an increase in DRG volume (compared to the contralateral side).
Conclusions: (Volumetric) measurements, based on D-prep MRN images, could be performed on all DRGs and nerves. As differences in DRG volume were seen after tumor-related nerve-compression and neuropathy after trauma, nerve specific MRI might serve as an non-invasive in vivo measure for the degree of nerve damage.
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