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Classical Lissencephaly
Joseph Junewick, MD FACR
over 5 years ago
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ARSt Case Repository

Devic's Disease

Case Detail

Anatomy: Neck-Face
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Infectious-Inflammatory
Created: over 6 years ago
Updated: over 6 years ago
Tags: PEDS
Modality/Study Types: MR
Activities:
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History

8 year old female with acute onset of blindness.


Case Images


Diagnosis

Devic’s Disease

Clinical Notes

CSF – Pleocytosis but no oligoclonal bands.

Findings

MR – FS postgadolinium T1 images show hyperintensity in the optic chiasm and the prechiasmatic right optic nerve.

Discussion

Devic’s disease (neuromyelitis optica) is a demyelinating disease distinct from multiple sclerosis characterized by acute onset of visual loss and paresthesias. Devics’s disease is often preceded by a viral-like prodrome of headache, fatigue, myalgias, respiratory symptoms and gastrointestinal distress. There is a female prepoderance; more than half experience relapse. CSF analysis shows pleocytosis and absence of oligoclonal bands. MR demonstrates edema and inflammation in the optic pathways often with no other evidence of central demyelination. Evaluation of the spinal cord usually shows long segment (>3 vertebral levels) central edema.

Reference

VandeVyver V, Limmerling M, DePotter R, Verstaete K. Devic’s Neuromyelitis optica: Clinical and Imaging Findings. JBR-BTR (2007); 90:284-287.



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