SHORT REPORT
Optic nerve and spinal cord manifestations of malignant atrophic papulosis (Degos disease)
1 Department of Neurology, Niigata City General Hospital, Niigata, Japan
2 Department of Dermatology, Niigata City General Hospital
3 Department of Radiology, Niigata City General Hospital
4 Department of Pathology, Niigata City General Hospital
5 Pathological Neuroscience Branch, Brain Research Institute, Niigata University, Niigata, Japan
Correspondence to:
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
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.
Keywords: malignant atrophic papulosis; pathology; optic nerve; spinal cord
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati What's this?
- Full Text
- Full Text (PDF)
- Submit a response
- Alert me when this article is cited
- Alert me when eLetters are posted
- Alert me if a correction is posted
Services
- Email this link to a friend
- Similar articles in this journal
- Similar articles in PubMed
- Add article to my folders
- Download to citation manager
- Request Permissions
Citing Articles
Google Scholar
PubMed
Topic Collections
Bookmark with
Register for free content
The full back archive is now available for all BMJ Journals. Institutional subscribers may access the entire archive as part of their subscription. Personal subscribers will also have access to all content when logged in. Non-subscribers who register have free access to all articles published before 2006 right back to volume 1 issue 1. Register here to access the free archive of all BMJ Journals.
Don't forget to sign up for content alerts so you keep up to date with all the articles as they are published.
