| Identifier |
walsh_2014_s3_c5 |
| Title |
Innocent until Proven Guilty |
| Creator |
Heather E. Moss, Tiffany Stroup, Amy Lin, Oliver Graf, Jeffrey Borgeson, Aaron Halfpenny, Howard Lipton, Tibor G. Valyi-Nagy |
| Subject |
Optic Neuritis; Demyelinating Disease; Autopsy |
| History |
A 31 year-old male experienced right eye blurring and pain. He was diagnosed with optic neuritis and treated with IV steroids. Nine days later he developed headache, worsening vision, speech trouble and right-sided weakness. Two days later he developed low-grade fever and trouble walking. He had NLP vision in the right eye and saw 20/20 with a nasal field deficit with the left eye. There was bilateral disc elevation. He had receptive aphasia, right arm weakness, flaccid paraplegia, and a T4 sensory level. MRIs showed extensive enlargement and enhancement of the right optic nerve, a left temporo-parietal lesion with patchy enhancement, focal diffusion restriction, mass effect and hemorrhage, and non- enhancing, longitudinally extensive, spinal cord lesions. Extensive inflammatory (including NMO Ig), vascular and infectious evaluations were unrevealing. He was treated with IV methylprednisolone and plasma exchange followed by a steroid taper. Visual acuity improved to count fingers with the right eye. Two months after presentation he lost vision in the left eye. MRIs showed new diffuse enhancement of the left optic nerve and new enhancement of the spinal cord lesion. CSF had elevated IgG index/synthesis rate, but no oligoclonal bands. He had an IgG kappa monoclonal protein. Further testing for malignancy was unrevealing. Spinal cord biopsy showed a macrophage rich lesion. He was treated with IV methylprednisolone, plasma exchange and cyclophosphamide with recovery of left eye vision to 20/800. 7 months after presentation vision declined to light perception with the right eye and hand motion with the left eye. He received rituximab with improvement in left eye vision to 20/400. Eleven months after initial symptoms, while being treated for bacteremia he became unresponsive. MRI showed an extensive lesion of the brainstem and cerebellum with mass effect, patchy enhancement, focal diffusion restriction, and hemorrhage. Within 4 hours he lost brainstem reflexes and did not recover. |
| Disease/Diagnosis |
Neuro-myelitis optica. |
| Presenting Symptom |
A 31 year-old male experienced right eye blurring and pain. |
| Neuroimaging |
Magnetic Resonance Imaging |
| Date |
2014-03 |
| References |
1. Barnett, Prineas, Buckland, Parratt, Pollard, Massive astrocyte destruction in neuromyelitis optica despite natalizumab therapy, Mult Scler, 18, 108-112, 2012 2. Bruck, Popescu, Lucchinetti, Markovi-Plese, Gold et al, Neuromyelitis optica lesions may inform multiple sclerosis heterogeneity debate, Ann Neurol, 72, 385-394, 2012. 3. Parrat, Prineas, Neuromyelitis optica: a demyelinating disease characterized by acute destruction and regeneration of perivascular astrocytes, Mul Scler 16, 1156-1172, 2010. 4. Popescu, Lucchinetti, Pathology of demyelinating diseases, Annu Rev Pathol, 7, 185-217, 2012. 5. Sato, Lana-Peixoto, Fujihara, de Seze, Clinical spectrum and treatment of neuromyelitis optica spectrum disorders: evolution and current status, Brain Pathol, 23, 647-660, 2013. |
| Language |
eng |
| Format |
application/pdf |
| Type |
Image |
| Source |
46th Annual Frank Walsh Society Meeting |
| Relation is Part of |
Case presented at the NANOS 2014 Walsh Session, March 2 |
| Collection |
Neuro-Ophthalmology Virtual Education Library - Walsh Session Annual Meeting Archives https://novel.utah.edu/Walsh/index3.html |
| Publisher |
Spencer S. Eccles Health Sciences Library, University of Utah |
| Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah, 10 N 1900 E SLC, UT 84112-6281 |
| Rights Management |
Copyright 2014. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s66d8qm9 |
| Setname |
ehsl_novel_fbw |
| ID |
179196 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s66d8qm9 |
