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Optic nerve and spinal cord manifestations of malignant atrophic papulosis (Degos disease)
  1. F Matsuura1,
  2. K Makino1,
  3. T Fukushima1,
  4. N Matsubara1,
  5. M Shibuya2,
  6. T Higuchi3,
  7. H Hashidate4,
  8. M Yamada5,
  9. H Shibuya4,
  10. M Yamazaki1
  1. 1Department of Neurology, Niigata City General Hospital, Niigata, Japan
  2. 2Department of Dermatology, Niigata City General Hospital
  3. 3Department of Radiology, Niigata City General Hospital
  4. 4Department of Pathology, Niigata City General Hospital
  5. 5Pathological Neuroscience Branch, Brain Research Institute, Niigata University, Niigata, Japan
  1. Correspondence to:
 Dr Kunihiko Makino
 Department of Neurology, Niigata City General Hospital, 2-6-1 Shichikuyama, Niigata 950-8739, Japan; makinoncgh{at}hosp.niigata.niigata.jp

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.

  • malignant atrophic papulosis
  • pathology
  • optic nerve
  • spinal cord

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Footnotes

  • Competing interests: none declared

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