Castleman Disease Presenting with Pseudotumour Cerebri and Myasthenia Gravis: A Case Report and Literature Review. (4th May 2019)

• Record Type: Journal Article
• Authors: Fein, Alexander S.; Trejo Bittar, Humberto E.; Shende, Manisha R.; Scalzetti, Ernest M.; Ko, Melissa W.
• Is Part Of: Neuro-ophthalmology. Volume 43:Number 3(2019)
• Page Start: 185
• Page End: 191
• Publication Date: 2019-05-04
• Subjects: Pseudotumour cerebri -- Castleman disease -- myasthenia gravis -- POEMS syndrome -- VEGF -- interleukin-6; Neuroophthalmology -- Periodicals; 617.7
• DOI: 10.1080/01658107.2018.1484932
• Languages: English
• ISSNs: 0165-8107

Abstract

ABSTRACT: Castleman disease (CD) is a rare lymphoproliferative disorder that may present with various autoimmune, inflammatory, or neurologic syndromes. This is a case of a 21-year-old woman who presented with signs and symptoms of pseudotumour cerebri (PTC) who subsequently developed myasthenia gravis (MG), and was incidentally found to have a large mass in the posterior mediastinum. Upon resection, the mass was classified as unicentric CD involved with follicular dendritic cell sarcoma. Following treatment with IVIG in the setting of progressive weakness and dyspnea, she has had complete symptom resolution while maintained on a low dose of pyridostigmine for the last two years. There are 13 cases of MG and five cases of optic disc edema described as PTC associated with CD in the literature, but to our knowledge, this is the sole case reported of the intersection of all three conditions in one patient. Increased serum levels of interleukin-6 and vascular endothelial growth factor may provide clues as to the association of CD with these neurologic syndromes.