Optic nerve and spinal cord manifestations of malignant atrophic papulosis (Degos disease)

J Neurol Neurosurg Psychiatry. 2006 Feb;77(2):260-2. doi: 10.1136/jnnp.2005.066134.

Abstract

A fatal case of malignant atrophic papulosis (Degos disease) with optic nerve and spinal cord involvement is described. Magnetic resonance imaging (MRI) of the optic nerve showed abnormal signal enhancement on fat suppressed T1 weighted images after intravenous meglumine gadopentetate infusion. On T2 weighted sagittal images, a sawtooth pattern was observed over seven vertebral segments of the spinal cord. On necropsy, a severe loss of myelinated nerve fibres in the left optic nerve was seen, with thrombotic obstruction of the central retinal artery. Spongy degeneration was observed in all levels of the spinal cord, with patchy and motheaten patterns caused by thromboses and endothelial proliferation in subarachnoid vessels. Findings on MRI were consistent with findings on pathological examination.

Publication types

  • Case Reports

MeSH terms

  • Atrophy
  • Brain Ischemia / diagnosis
  • Brain Ischemia / pathology
  • Disease Progression
  • Fatal Outcome
  • Female
  • Humans
  • Image Enhancement
  • Intracranial Embolism / diagnosis
  • Intracranial Embolism / pathology
  • Magnetic Resonance Imaging
  • Middle Aged
  • Nerve Degeneration / diagnosis
  • Nerve Degeneration / pathology
  • Optic Nerve / pathology
  • Optic Nerve Diseases / diagnosis*
  • Optic Nerve Diseases / pathology
  • Skin / pathology*
  • Skin Diseases, Papulosquamous / diagnosis
  • Skin Diseases, Papulosquamous / pathology
  • Spinal Cord / pathology
  • Spinal Cord Diseases / diagnosis*
  • Spinal Cord Diseases / pathology