MR imaging of Dejerine-Sottas disease

Abstract

We report the MR findings in two patients with clinically and histologically proved Dejerine-Sottas disease. One patient had spinal involvement with multiple thickened and clumped nerve roots of the cauda equina; the second had multiple enlarged and enhancing cranial nerves. Although these findings are not specific for Dejerine-Sottas disease, they are suggestive of the diagnosis, which is further corroborated with history and confirmed with sural nerve biopsy and laboratory studies.

fig 1.

Case 1: 7-year-old boy with spinal nerve enlargement. A–C, Sagittal T1-weighted (600/11/2 [TR/TE/excitations]) (A), axial T1-weighted (600/11/2) (B), and sagittal T2-weighted (2500/96/2) (C) images show marked enlargement of the nerves of the cauda equina (arrow, C).

fig 2.

Case 2: 23-year-old man with enlarged cranial nerves A–D, Axial noncontrast T1-weighted (600/15/1) (A) and axial spin-echo T2-weighted (2500/96/1) (B) images are not as revealing as the axial contrast-enhanced T1-weighted (600/15/1) images (C and D). There is prominent enhancement in the left porous acousticus (open arrow, C), enlargement and enhancement of the fifth cranial nerves in Meckel's cave, slightly more prominent on the right (black arrow, C) than left, and an enlarged enhancing branch of the maxillary nerve in the pterygopalatine fossa on the left (wide white arrow, C). The right geniculate ganglion (arrowhead, C) and the tympanic portion of the left seventh nerve (curved arrow, C) are enlarged and enhancing. Also evident are bilateral enlargement and enhancement of the third nerve, more prominent on the right side (arrow, D).