Behind the Curtain

Update item information
Identifier walsh_2013_s2_c1-1
Title Behind the Curtain
Creator Levin, Marc H; Gopal, Pallavi; Steven L. Galetta, MD, NYU Langone
Subject Diplopia; Ptosis; Myasthenia Gravis; Autoimmune Diseases
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.
History There was no significant past medical history, no smoking history, nor any systemic symptoms.
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
Neuroimaging CT Scan; MRI
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.
Treatment Pyridostigmine; Prednisone; Rituximab; Corticosteroids
References Attached
Relation is Part of Case presented at the NANOS 2013 Walsh Session, February 10
Contributor Primary Kedar, Sachin
Contributor Secondary Rucker, Janet C
Publisher Spencer S. Eccles Health Sciences Library, University of Utah
Date 2013-02-10
Type Text
Format application/pdf
Source 45th Annual Frank Walsh Society Meeting
Holding Institution North American Neuro-Ophthalmology Association (NANOS), Copyright 2013. For further information regarding the rights to this collection, please visit
Collection Neuro-ophthalmology Virtual Education Library: NOVEL
Language eng
ARK ark:/87278/s6h15zm2
Setname ehsl_novel_fbw
Date Created 2013-12-12
Date Modified 2021-05-06
ID 179155
Reference URL
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