| Identifier |
walsh_2017_s1_c2-slideshow |
| Title |
A 'Frosty' Altered Level of Consciousness (slideshow) |
| Creator |
Alaa Bou Ghannam, Emily McCourt, Victoria Pelak, Jeffrey Bennett, Prem S Subramanian, MD, PhD, Professor of Ophthalmology, Neurology, and Neurosurgery, University of Colorado, David Smits |
| Subject |
CNS vasculitidies, bilateral vision loss |
| History |
15-year-old previously healthy male, transferred because of 9-day history of headache, difficulty walking, diplopia, blurry vision and new onset altered level of consciousness. Neurologic examination was remarkable for delirium; with semi-coherent speech, VA of CF 2ft and 20/400, no APD, and bilateral abduction deficits. |
| Disease/Diagnosis |
The final diagnosis was primary angiitis of the central nervous system (PACNS) with frosted branch angiitis (FBA) PACNS is a rare condition (2.4 per one million), defined as vasculitis limited to the CNS with no identifiable cause. The clinical features of PACNS are highly variable. The most common symptoms are headache (50-60%) and altered cognition (50- 70%). Most patients can have hemiparesis, ataxia, cranial neuropathy, visual symptoms, extrapyramidal signs, seizures and/or myelopathy. PACNS usually arises in the fourth or fifth decade of life, with half of cases beginning between ages 37-59 years. MRI brain can be as variable as the clinical presentation and can show T2 hyperintensities involving the cortex, subcortical white matter and/or deep grey matter. Brain biopsy is needed for definitive diagnosis and typically shows transmural inflammation with injury to the vessel wall. There is typcially an angiocentric inflammatory infiltrate as seen in this case. There are different subtypes and in the granulomatous subtype, extensive beta amyloid deposition in vessel walls with associated inflammation can be present Acute frosted branch angiitis in children is a very rare presentation that has been reported mostly in Japan. The condition is bilateral and characterized by extensive perivascular exudates with a "frostlike" quality. The reported age range is between 2 and 42 years. Although the etiology of frosted branch angiitis can be unknown, other causes of retinal vasculitis should be excluded, especially viral retinitis (CMV, EBV…) sarcoidosis, multiple sclerosis, toxoplasmosis, syphilis, and Behçet's disease. Infiltrative causes such as lymphoma and leukemia may occasionally mimic FBA. These conditions were excluded in our patient, and to our knowledge, there is no other reported cases in the literature of frosted branch angiitis associated with primary CNS vasculitis |
| Date |
2017-04 |
| References |
Ito Y, Nakona M, Kyu N, Takeuchi M. Frosted branch angiitis in a child. Jpn J Clin Ophthalmol 1976;30:797-803. Kleiner RC, Kaplan HJ, Shakin JL, Yannuzzi LA, Crosswell HH, Jr, McLean WC., Jr Acute frosted retinal periphlebitis. Am J Ophthalmol. 1988;106:27-34 Birnbaum J, Hellmann DB. Primary angiitis of the central nervous system. Arch Neurol. 2009 Jun;66(6):704- 9. Salvarani C, Brown RD Jr, Calamia KT, et al. Primary central nervous system vasculitis: analysis of 101 patients. Ann Neurol. 2007;62(5):442-451. Saettele MR, Loskutov A, Sigley MJ, Lowe LH, Nielsen DB. Biopsy-proven case of childhood primary angiitis of the central nervous system presenting with bilateral panuveitis and anisocoria. Pediatr Radiol. 2015 Feb;45(2):291-5. Woolfenden AR, Wade NK, Tang P, Chalmers A, Reid G, Teal PA. Uveitis associated with Primary Angiitis of the Central Nervous System. Can J Neurol Sci. 2007 Feb;34(1):81-3. Yaari R, Anselm IA, Szer IS, Malicki DM, Nespeca MP, Gleeson JG. Childhood primary angiitis of the central nervous system: two biopsy-proven cases. J Pediatr. 2004 Nov;145(5):693-7. |
| Language |
eng |
| Format |
application/pdf |
| Type |
Text |
| Source |
49th Annual Frank Walsh Society Meeting |
| Relation is Part of |
NANOS Annual Meeting 2017: Frank B. Walsh Session 1 |
| 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 84112-6134 |
| Rights Management |
Copyright 2017. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s6z073rz |
| Setname |
ehsl_novel_fbw |
| ID |
1277675 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6z073rz |
