MR imaging of Dejerine-Sottas disease.
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.
Maki
D D
DD
Department of Radiology, University of Pennsylvania Medical Center, Philadelphia, USA.
Yousem
D M
DM
Corcoran
C
C
Galetta
S L
SL
D000328:Adult; D002420:Cauda Equina / Q000473:pathology; D002648:Child; D005152:Facial Muscles / Q000473:pathology; D005154:Facial Nerve / Q000473:pathology; D015417:Hereditary Sensory and Motor Neuropathy / Q000175:diagnosis* / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D018908:Muscle Weakness / Q000175:diagnosis; D009802:Oculomotor Nerve / Q000473:pathology; D013116:Spinal Cord / Q000473:pathology; D014276:Trigeminal Nerve / Q000473:pathology