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

F Matsuura1, K Makino, T Fukushima

  • 1Department of Neurology, Niigata City General Hospital, 2-6-1 Shichikuyama, Niigata 950-8739, Japan.

Journal of Neurology, Neurosurgery, and Psychiatry
|January 20, 2006
PubMed

Insights

Malignant atrophic papulosis (Degos disease) can fatally affect the optic nerve and spinal cord. MRI and necropsy revealed vascular thromboses and nerve damage, confirming the disease

Area of Science:

  • Neurology
  • Pathology
  • Radiology

Background:

  • Malignant atrophic papulosis, also known as Degos disease, is a rare and often fatal systemic vasculopathy.
  • Previous literature primarily describes cutaneous and gastrointestinal manifestations.

Observation:

  • This report details a fatal case of Degos disease with significant central nervous system involvement.
  • Magnetic resonance imaging (MRI) demonstrated characteristic findings in both the optic nerve and spinal cord.

Findings:

  • Optic nerve MRI revealed abnormal signal enhancement, consistent with inflammation or ischemia.
  • Spinal cord MRI showed a unique 'sawtooth' pattern on T2-weighted images, indicative of vascular compromise.
  • Necropsy confirmed severe demyelination in the optic nerve with central retinal artery thrombosis.
  • Spinal cord pathology exhibited spongy degeneration attributed to thromboses and endothelial proliferation in subarachnoid vessels.

Implications:

  • This case highlights the potential for extensive neurological involvement in Degos disease.
  • Advanced neuroimaging, particularly MRI, can aid in diagnosing and characterizing CNS manifestations.
  • Correlation between MRI findings and pathological examination validates the imaging techniques for Degos disease.

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