title: 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.
authors.affiliation: Department of Radiology, University of Pennsylvania Medical Center, Philadelphia, USA.
authors.forename: D D
authors.initials: DD
authors.lastname: Maki
authors.forename: D M
authors.initials: DM
authors.lastname: Yousem
authors.forename: C
authors.initials: C
authors.lastname: Corcoran
authors.forename: S L
authors.initials: SL
authors.lastname: Galetta
mesh_terms: 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