Presentation
Back pain and urinary incontinence. Lumbar spine MRI demonstrated abnormal signal in the conus.
Patient Data
The cord demonstrates a longitudinaly extensive spinal cord lesion, with high T2 signal involving almost the entire cord.
There is ill-defined abnormal signal within the inferior aspect of the body of the corpus callosum, predominantly just to the left of midline. A more poorly defined zone of abnormal signal surrounding the right occipital horn may also represent sub ependymal demyelination, associated with subtle ependymal enhancement. No restricted diffusion or pathological contrast enhancement detected elsewhere.
The patient was thought to have NMO and went on to have aquaporin-4 antibody assay:
Neuromyelitis Optica antibodies: POSITIVE.
Comment :
AQP4-transfected cells............POSITIVE
Rodent tissue.....................POSITIVE
NMO-IgG positive on both tissue and AQP4-transfected cells. These results are consistent with the presence of a neuromyelitis optica spectrum disorder (NMOSD). NMO-IgG has a sensitivity and specificity of 48-77% and 97-100% respectively for NMOSD (Waters et al. Neurology 2012 Feb28;78(9):665-71).
Two weeks later the signal change previously seen in the cord has all but resolved.
Case Discussion
Typical spinal appearances of neuromyelitis optica (Devic disease), with subependymal demyelination also demonstrated in the brain.
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