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Washington University Experience | MYELIN (IMMUNE-MEDIATED) | NMO (Neuromyelitis Optica) | NMO Spectrum Disorder | 1A0 Case 1 History
Case 1 History ---- The patient was a 36-year-old woman with ataxia, leg weakness and bilateral optic neuritis and was diagnosed with RRMS (no test for IgG-NMO was performed at that time), and treated chronically with Betaseron. A flareup in 2004 resulted in right hemiparesis with MRI contrast enhancement of a large left periventricular lesion and cord pathology. She was started on IV Cytoxan which was continued for 6 months. She was subsequently treated with Copaxone between 2005 and 2008 before starting Tysabri in 2008 but her disease continued to progress and by 2009 she was blind in both eyes. She experienced four "MS flares", the fourth of which occurred a short time before her death. Her serum JC virus antibody testing was negative. She received high dose IV methylprednisolone as well as four plasma exchanges. She developed decreased mental status and inability to swallow. Review of images provided from the outside hospital showed a longstanding left subcortical lesion involving the left posterior periventricular white matter with minimal contrast enhancement with areas of diffusion restriction. A significant portion of the lesion was T1 hypointense. There were other scattered periventricular white matter lesions in both hemispheres, all with minimal contrast enhancement. The patient was also noted to have a severely atrophic spinal cord that was present as far back as imaging in 2003. There were extensive T2 lesions throughout the cervical and thoracic cord. At our hospital JC virus PCRs were negative x 3 and her serum JC antibody testing was negative in all prior (monitored q 6 months) had also been negative. Her first LP (prior to a full course of steroids) was a traumatic tap with a protein of 70, glucose 71, Tube 4 with only 5 RBCs and 14 nucleated cells and 6% eosinophils, elevated IgG index and synthesis rate but only 2 CSF specific oligoclonal bands. The patient showed no improvement with steroids and PLEX here. She remained comatose and ventilator dependent. After post-mortem gross and microscopic examination, and the continued concern for NMO, a pre-mortem CSF sample was retrieved and sent for NMO aquaporin 4 antibody assessment. The test results returned as positive for anti-aquaporin 4 antibodies, consistent with NMO.
