| Identifier |
walsh_2018_s3_c1-1 |
| Title |
Coming to a Rapid Conclusion - Abstract |
| Creator |
Leanne Stunkel; Namita Sinha; Nathan Kung; Robi Maamari; Cole Ferguson; Sonika Dahiya; George Harocopos; Gregory Van Stavern |
| Affiliation |
(LS) Washington University in St Louis School of Medicine, St. Louis, Missouri; (NS) (CF) (SD) (GH) Department of Pathology & Immunology, Washington University in St. Louis, St. Louis, Missouri; (NK) Blue Sky Neurology, Denver, Colorado; (GV) Department of Ophthalmology and Visual Sciences, Washington University in St. Louis, St. Louis, Missouri |
| Subject |
vasculopathy, retinopathy, headaches, Neurologic disorders, Outer retinopathies/White dot syndromes |
| History |
A 21-year-old man presented to a retina specialist for 2.5 weeks of painless central vision loss OD. His initial examination was notable for visual acuity 20/200 OD and 20/20 OS. Fundus examination was significant for several macular areas of retinal pigment epithelial (RPE) atrophy with associated subretinal whitening. Fundus autofluorescence showed further areas of RPE atrophy centrally and temporally. OCT of the macula showed areas of atrophy of the RPE with adjacent areas of hyperreflectivity and showed choroidal thickening in the involved area. Fluoroscein angiogram showed well-circumscribed regions of early hypofluorescence and adjacent regions of transmission defects and late hyperfluorescence. The working diagnosis was acute retinal necrosis, and the patient was offered admission for IV antivirals. When we declined admission, he was started on oral valacyclovir. About 10 days later, he developed a new, frontal headache, followed a few days later by involvement of the other eye (OS).;Funduscopic examination showed a new small area of whitening just inferior to the fovea OS. He was admitted to an outside hospital for IV and intravitreal antivirals. An infectious work-up was negative. At that time, he was started on prednisone 80mg daily out of concern for inflammatory etiology. However, his vision continued to decline to count fingers OD with examination notable for a new relative afferent pupillary defect OD and a new inferonasal visual field deficit OS. He continued to have worsening headaches. Approximately 1 week later he had sudden complete loss of vision OD and began to have hallucinations. His mental status declined and he had abnormal limb movements. He was intubated for airway protection. His examination without; sedation was notable for hypertension, bradycardia, unresponsiveness, fixed 8mm pupils bilaterally, intact oculocephalic reflexes, intact corneal reflexes, intact cough and gag reflexes, overbreathing the ventilator, and extensor posturing in response to noxious stimuli. |
| Disease/Diagnosis |
Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) with secondary CNS vasculitis causing strokes in multiple vascular territories. |
| Presenting Symptom |
A 21-year-old man presented to a retina specialist for 2.5 weeks of painless central vision loss OD. His initial examination was notable for visual acuity 20/200 OD and 20/20 OS. Fundus examination was significant for several macular areas of retinal pigment epithelial (RPE) atrophy with associated subretinal whitening. Fundus autofluorescence showed further areas of RPE atrophy centrally and temporally. OCT of the macula showed areas of atrophy of the RPE with adjacent areas of hyperreflectivity and showed choroidal thickening in the involved area. Fluoroscein angiogram showed well-circumscribed regions of early hypofluorescence and adjacent regions of transmission defects and late hyperfluorescence. The working diagnosis was acute retinal necrosis, and the patient was offered admission for IV antivirals. When we declined admission, he was started on oral valacyclovir. About 10 days later, he developed a new, frontal headache, followed a few days later by involvement of the other eye (OS).;Funduscopic examination showed a new small area of whitening just inferior to the fovea OS. He was admitted to an outside hospital for IV and intravitreal antivirals. An infectious work-up was negative. At that time, he was started on prednisone 80mg daily out of concern for inflammatory etiology. However, his vision continued to decline to count fingers OD with examination notable for a new relative afferent pupillary defect OD and a new inferonasal visual field deficit OS. He continued to have worsening headaches. Approximately 1 week later he had sudden complete loss of vision OD and began to have hallucinations. His mental status declined and he had abnormal limb movements. He was intubated for airway protection. His examination without; sedation was notable for hypertension, bradycardia, unresponsiveness, fixed 8mm pupils bilaterally, intact oculocephalic reflexes, intact corneal reflexes, intact cough and gag reflexes, overbreathing the ventilator, and extensor posturing in response to noxious stimuli. |
| Date |
2018-03 |
| References |
Brownlee WJ, Anderson NE, Sims J, Pereira JA. Neurological complications of acute multifocal placoid pigment epitheliopathy. J Clin Neuroscience. 13, 76-80, 2016. Comu S, Verstraeten T, Rinkoff JS, Busis NA. Neurological Manifestations of Acute Posterior Multifocal Placoid Pigment Epitheliopathy. Stroke. 27, 996-1001, 1996. de Vries JJ, den Dunnen WFA, Timmerman EA, Kruithof, De Keyser J. Acute Posterior Multifocal Placoid Pigment Epitheliopathy With Cerebral Vasculitis: A Multisystem Granulomatous Disease. Arch Ophthalmol. 124, 910-3, 2016. Tsang BKT, Chauhan, Haward R, Whiteman I, Judith Frayne J, McLean C. Fatal Ischemic Stroke Complicating Acute Multifocal Placoid Pigment Epitheliopathy: Histopathological Findings. J Neuro-Ophthalmol. 34, 10-5, 2014. Wilson CA, Choromokos EA, Sheppard R. Acute Posterior Multifocal Placoid Pigment Epitheliopathy and Cerebral Vasculitis. Arch Ophthalmol. 106, 796-800, 1988. |
| Language |
eng |
| Format |
application/pdf |
| Type |
Text |
| Source |
2018 North American Neuro-Ophthalmology Society Annual Meeting |
| Relation is Part of |
NANOS Annual Meeting Frank B. Walsh Sessions; 2018 |
| 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 2018. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s64v0mzc |
| Setname |
ehsl_novel_fbw |
| ID |
1320249 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s64v0mzc |
