PMC8053436_01
Left: Lumbar nerve root involvement. (a) Sagittal T2 (off midline) shows multiple intradural nerve root nodular lesions approaching the exit foramina. (b) Axial T2 through L3 foramina shows enlarged L3 roots at the exit foramina with some thickening of the intradural cauda equina roots. (c) Axial T2 through L4 exit foramina shows thickened L4 roots extending outwards from foramina, while intradural cauda equina roots are thickened. (d) Axial T2 through S1 segment of sacrum shows lobulated L5 roots ventral to sacral alar, while S1 nerve roots at lateral recesses are enlarged. Right: Coronal STIR of the brachial plexus in sequential images from posterior to anterior. All the cervical roots are thickened and hyperintense. The first image shows the intradural components in the spinal canal.
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F1
This showed a similar picture with superimposed degenerative changes involving the cervical spine with evidence of midcervical cord compression due to a combination of discogenic disease and nerve root hypertrophy [Figures 1 and 2].
(a) Sagittal T2 shows hyperintense segmental lesions ventrolateral to the cervical cord at C4 and C5 vertebral levels compressing the cervical cord. (b) Axial T2 and (c) axial post-Gadolinium T1 at lower border of C5 vertebral level show bilateral C6 root lesions compressing the cervical cord which lies in the dorsal midline.
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F2
This showed a similar picture with superimposed degenerative changes involving the cervical spine with evidence of midcervical cord compression due to a combination of discogenic disease and nerve root hypertrophy [Figures 1 and 2].
Cervical decompression: Intraoperative finding of grossly thickened nerve root.
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F3
A biopsy of both sural nerve and proximal root biopsy was recommended at cervical cord decompression [Figure 3] to increase diagnostic yield and rule out the presence of dual pathology, that is, the neuropathy and radiculopathy having separate causation.
C6 nerve root biopsy: (a) H&E (hematoxylin and eosin) stained section showing endoneurial edema and a minifascicular architecture (in longitudinal and transverse profile) due to Schwann cell proliferation militating against a neoplastic or infectious process. (b) Semi-thin resin section (toluidine blue stained) showing a marked reduction in the density of large myelinated fibers and a prominent edematous stroma. The pathology is further characterized by thinly myelinated fibers surrounded by multi-layered sheaths of Schwann cells, which all favor a demyelinating process. (c) CD3 immunostaining for T lymphocytes showed prominent focal endoneurial lymphocytic activity. (d) CD68 immunostaining highlighted phagocytic activity co-localized to the cords of Schwann cells. Sural nerve biopsy: (e) Semi-thin resin section (toluidine blue stained) showing striking loss of large myelinated fibers, endoneurial edema and concentric Schwann cell proliferation. (f) CD3 immunostaining again showing a focal endoneurial inflammatory infiltrate of T-lymphocytes.
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F4
T cell inflammatory infiltrates [Figure 4]. The hypertrophy apparent in the nerve root was due to marked expansion of an edematous endoneurial matrix.