| Identifier |
walsh_2013_s2_c1 |
| Title |
Behind the Curtain |
| Creator |
Marc H. Levin; Pallavi Gopal; Steven L. Galetta |
| Affiliation |
(MHL) (SLG) University of Pennsylvania, Perelman School of Medicine, Department of Neurology, Philadelphia, PA; (MHL) (SLG) University of Pennsylvania, Perelman School of Medicine, Department of Ophthalmology, Philadelphia, PA; (PG) University of Pennsylvania, Perelman School of Medicine, Department of Pathology, Philadelphia, PA |
| Subject |
Diplopia; Ptosis; Myasthenia Gravis; Autoimmune Diseases |
| History |
There was no significant past medical history, no smoking history, nor any systemic symptoms. |
| Pathology |
Pathology from numerous biopsies ruled out lymphoma or other malignancies. Despite the prominent fibrotic background, the lesions did not meet current criteria to be considered the IgG4-related sclerosing form of inflammatory pseudotumor. |
| Disease/Diagnosis |
Patient had aggressive systemic inflammatory pseudotumor presenting initially as ocular myasthenia gravis. |
| Clinical |
On examination there was 2 mm of left upper eyelid ptosis with associated fatiguability and Cogans lid twitch. The remainder of his examination was normal. biopsies of paraspinal adenopathy, lung masses, and subcutaneous scalp nodules showed similar inflammatory changes. Symptoms of ocular myasthenia were initially controlled for two years on pyridostigmine but then recurred.Acetylcholine receptor (AchR) antibody positivity and abnormal single fiber electromyelography confirmed the diagnosis of myasthenia gravis. The patients symptoms were easily controlled with pyridostigmine. CT imaging of the chest excluded a thymoma, but revealed numerous lung nodules and mediastinal adenopathy that suggested metastatic disease. There were no evidence of lymphoma or other malignancy. Additional |
| Presenting Symptom |
A 55 year-old man presented with 6 weeks of intermittent left upper eyelid drooping and blurring of vision, worse later in the day. |
| Neuroimaging |
CT Scan; MRI |
| Treatment |
Pyridostigmine; Prednisone; Rituximab; Corticosteroids |
| Date |
2013-02 |
| References |
1. Blodi, Orbital Pseudotumor with Thrombocyopenia and Myasthenia, Klin Monatsbl Augenheilkd, 170, 397-400, 1977. 2. Van de Mosselaer, Van Deuren, Dewolf-Peeters, Missotten, Pseudotumor orbitae and myasthenia gravis. A case report, Arch Ophthalmol. 98, 1621-1622, 1980. 3. Rezania, Soliven, Baron, Penumalli, van Besien, Myasthenia gravis, an autoimmune manifestation of lymphoma and lymphoproliferative disorders: case reports and review of literature, Leukemia & Lymphoma, 53, 371-380, 2012. |
| Language |
eng |
| Format |
application/pdf |
| Type |
Text |
| Source |
45th Annual Frank Walsh Society Meeting |
| Relation is Part of |
Case presented at the NANOS 2013 Walsh Session, February 10 |
| 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 84112-6326 |
| Rights Management |
Copyright 2013. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s6z34w83 |
| Setname |
ehsl_novel_fbw |
| ID |
179167 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6z34w83 |
