| Identifier |
walsh_2016_s2_c1-1 |
| Title |
Looking for a Drop of Porcelain - Presentation PPT |
| Creator |
Shannon J. Beres; Grant T. Liu |
| Affiliation |
(SJB) (GTL) Hospital of the University of Pennsylvania, Departments of Neurology and Ophthalmology Philadelphia, PA; (GTL) The Children's Hospital of Philadelphia, Division of Ophthalmology, Neuro-ophthalmology Service Philadelphia, PA |
| Subject |
Third Cranial Nerve Palsy; Homonymous Hemianopsia; Vascular Arterial Ischemic Stroke |
| History |
A 4 year old healthy boy presented with severe headache. A right subdural hygroma was seen on brain MRI and CSF evaluation showed a leukocytosis with elevated protein. A week later he developed left leg weakness and a repeat brain MRI showed progressive leptomeningeal enhancement and new punctate infarcts with spine imaging revealing diffuse thickening and enhancement along the thecal sac. CNS vasculitis was suspected and he was started on steroids. A brain biopsy showed a subdural hemorrhage with pathology showing intraluminal fibrin thrombi, fibrinoid necrosis of some vessels and perivascular hemorrhage suspicious for CNS vasculitis, however, a thrombotic etiology was favored on second opinion. Despite steroids, rituximab, cyclophosphamide, infliximab, and IVIG he continued to have recurrent radiologic strokes with progressive weakness in his left leg, new weakness in his left arm, and a new left homonymous hemianopsia. After two months, he was transferred to a large referral center and on transfer his exam showed a 25 lb weight gain from baseline, weakness in the left leg and arm, a left homonymous hemianopsia, and normal mental status. Two months after his initial presentation, he developed right eyelid ptosis, right eye mydriasis and double vision. On exam he had normal near visual acuity, a left homonymous hemianopsia, abduction, supraduction and infraduction defects in his right eye with a fixed and dilated right pupil. His dilated fundus exam was normal with no evidence of vasculitis or papilledema. He was diagnosed with an ischemic complete 3rd nerve palsy, either peripheral or central (radiologically occult). Two weeks later, he had onset of hematuria, urinary retention, and abdominal pain; thought to be a cyclophosphamide side effect. Additional ineffective treatments included aspirin and nataluzimab and at 3 months from presentation, two raised circular lesions on his left lower abdomen and right foot developed. |
| Disease/Diagnosis |
Degos disease |
| Date |
2016-02 |
| References |
1. Moss C, Wassmer E, Debelle G, Hackett S, Goodyear H, et al, Degos disease: a new simulator of non-accidental injury, Dev. Med Child Neurol, 51,505-6, 2009. 2. Degos R, Malignant atrophic papulosis, Br J Dermatol, 100(1), 21-35, 1979. 3. Pirolla E, Fregni F, Miura IK, Misiara AC, Almeida F, Zanoni E. Degos disease - malignant atrophic papulosis or cutaneointestinal lethal syndrome: rarity of the disease, Clin Exp Gastroenterol, 8, 141-7, 2015. 4. Magro C, Poe J, Kim C, Shapiro L, Nuovo G, et al, Degos Disease: A C5b-9/Interferon-a-Mediated Endotherliopathy Syndrome, Am J Clin Pathol, 135, 599-610, 2011. |
| Language |
eng |
| Format |
application/pdf |
| Format Creation |
Microsoft PowerPoint |
| Type |
Text |
| Source |
48th Annual Frank Walsh Society Meeting |
| Relation is Part of |
NANOS Annual Meeting Frank B. Walsh Sessions; 2016 |
| 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 2016. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s6g47mxf |
| Setname |
ehsl_novel_fbw |
| ID |
179341 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6g47mxf |
