| Identifier | walsh_2019_s3_c1 |
| Title | Every Rose Has Its Thorn |
| Creator | William Anderson; Aseem Sharma; Miguel Guzman; Sophia Chung |
| Affiliation | (WA) (MG) (SC) Saint Louis University School of Medicine, Saint Louis, Missouri; (AS) Washington University School of Medicine, Saint Louis, Missouri |
| Subject | Infection; Tumor; Nystagmus |
| Description | The MRI brain showed multiple T1 hypointense T2 hyperintense cystic lesions within the posterior fossa involving the cerebellar vermis, right cerebellar hemisphere, and brainstem. Possible scolices were identified within multiple cysts on T1 images, leading to a high suspicion for parasitic infection, specifically neurocysticercosis (NCC). MRI of the spine showed similar intramedullary lesions extending from brainstem to involve the upper cervical spinal cord. CT of the head demonstrated a single area of calcification in the right cerebellum. An extensive infectious disease, auto-immune, and paraneoplastic evaluation was pursued. Consultation with neurocysticercosis experts at the CDC was obtained. The possibility of coenurosis secondary to Taenia multiceps, T. serialis, T. brauni, or T. glomerata was considered as well. Infectious workup for Echinoccocus, Toxoplasmosis, and TB was negative. Stool ova and parasites was similarly negative. Serum testing for NCC with enzyme-linked immunoelectrotransfer blot (EITB) was sent to the CDC. Auto-immune and paraneoplastic work up were negative. Treatment for a presumed parasitic infection with anti-helminthic therapy after steroid pre-treatment was entertained. However, after further discussion with the CDC, this option was reevaluated due to the high lesion burden and potential massive inflammatory response. There was also growing skepticism for a parasitic etiology of the lesion, a biopsy was obtained for definitive diagnosis. Pathology was diagnostic for a low grade glioneuronal tumor with characteristics suggestive of a rosette-forming glioneuronal tumor (RGNT). Some cells showed oligodendroglioma-like features, however this was ruled out based on the absence of 1p 19q codeletion on chromosomal microarray. RGNT is a glioneuronal tumor that may show oligodendroglioma-like areas and is found in the fourth ventricle, and rare RGNT cases have been reported with similar multi-loculated gross and radiologic morphology. However, this potential diagnosis could not be definitively established on histopathology as the tumor failed to show characteristic rosettes with synaptophysin positive cores. |
| History | A 26-year-old woman presented with a one-year history of horizontal binocular diplopia on far-right gaze. During a routine eye examination for contact lenses, she was found to have a gaze paretic nystagmus on far-right gaze. The patient also offered a one-year history of right-sided instability and balance issues. MRI was done, and she was admitted to our institution for further evaluation and management. Further history revealed that she experienced diplopia in primary position after running a mile. She denied headache, nausea, vomiting, and other visual symptoms. She denied antecedent trauma or illness. She denied variability of diplopia or associated ptosis. Neuro-ophthalmologic examination showed 20/20 vision bilaterally, with normal pupils and color vision. External examination revealed mild orbicularis weakness. Corneal sensation was mildly reduced in both eyes. Motility showed a mild right esotropia on right gaze from a subtle sixth nerve paresis. There was a gaze paretic nystagmus in right gaze as well. Slit lamp examination was normal. Fundus examination was normal with pink optic nerves. General neurologic examination showed no other focal deficits. MRI was reviewed; CT head and MRI spine were additional tests performed. Spinal fluid analysis was not done because of concerns for herniation. Numerous laboratory studies were performed. A diagnostic procedure was performed. |
| Disease/Diagnosis | Rosette-forming glioneuronal tumor (although another low grade glioneuronal tumor could not be negated) |
| Date | 2019-03 |
| References | 1. Yang C, Fang J, Li G, Li S, Ha T, Wang J, et al. Histopathological, molecular, clinical and radiological characterization of rosette-forming glioneuronal tumor in the central nervous system. Oncotarget. 2017;8(65):109175-90. 2. Medhi G, Prasad C, Saini J, Pendharkar H, Bhat MD, Pandey P, et al. Imaging features of rosette-forming glioneuronal tumours (RGNTs): A Series of seven cases. Eur Radiol. 2016;26(1):262-70. 3. Zhang J, Babu R, McLendon RE, Friedman AH, Adamson C. A comprehensive analysis of 41 patients with rosette-forming glioneuronal tumors of the fourth ventricle. J Clin Neurosci. 2013;20(3):335-41. 4. Marhold F, Preusser M, Dietrich W, Prayer D, Czech T. Clinicoradiological features of rosette-forming glioneuronal tumor (RGNT) of the fourth ventricle: report of four cases and literature review. J Neurooncol. 2008;90(3):301-8. 5. Gao L, Han F, Jin Y, Xiong J, Lv Y, Yao Z, et al. Imaging features of rosette-forming glioneuronal tumours. Clin Radiol. 2018;73(3):275-82. 6. Kim SW, Kim MK, Oh SM, Park SH. Racemose cysticercosis in the cerebellar hemisphere. J Korean Neurosurg Soc. 2010;48(1):59-61. |
| Language | eng |
| Format | video/mp4 |
| Type | Image/MovingImage |
| Source | 2019 North American Neuro-Ophthalmology Society Annual Meeting |
| Relation is Part of | NANOS Annual Meeting 2019 |
| Collection | Neuro-Ophthalmology Virtual Education Library: Walsh Session Annual Meeting Archives: https://novel.utah.edu/Walsh/ |
| Publisher | North American Neuro-Ophthalmology Society |
| Holding Institution | Spencer S. Eccles Health Sciences Library, University of Utah |
| Rights Management | Copyright 2019. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK | ark:/87278/s6838951 |
| Setname | ehsl_novel_fbw |
| ID | 1431966 |
| Reference URL | https://collections.lib.utah.edu/ark:/87278/s6838951 |