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Abstract Details

A Case of Refractory Necrotizing Idiopathic Hypophysitis Presenting as Recurrent Bitemporal Hemianopsia
Autoimmune Neurology
P15 - Poster Session 15 (12:00 PM-1:00 PM)
15-011
This case report endeavors to present a rare case of necrotizing primary hypophysitis, summarizes the existing findings in the literature, and discusses medical management of this entity in refractory disease.
Hypophysitis, or inflammation of the pituitary gland and stalk, is a rare entity that can be primary or secondary in nature. Of the various histologic subtypes, one of the rarest is necrotizing hypophysitis, a type of primary hypophysitis in which the pituitary gland is inflamed due to a putative autoimmune dysfunction. This subtype has been identified and described in three known case reports.  

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A 45-year-old male with a history of pituitary adenoma and prior resections presented with acute progressive bitemporal hemianopsia. The clinical course was complicated by panhypotituitarism, and eleven years of recurrent pituitary inflammation requiring multiple endoscopic endonasal surgeries and supraorbital craniotomies. Pathology of all previously resected tissue showed necrotic fibrosis with inflamed granulation tissue; and all previous intracranial fluid drainage of abscess-like or cyst-like collections within this region also yielded negative cultures. MRI during his admission demonstrated recurrent suprasellar tumor/cystic lesion with optic chiasm edema. CSF analysis revealed elevated protein and neutrophilic pleocytosis, however negative encephalitis/meningitis panel. Given these findings the patient was started on high dose steroids and his visual symptoms resolved to baseline following six doses of intravenous methylprednisolone followed by an oral prednisone taper. Of note, he also received empiric vancomycin and meropenem given concern for possible healthcare acquired meningitis following drainage of this lesion a month prior. Ultimately, his protracted history with recurrent symptoms, histopathological findings, and response to steroids, yielded a diagnosis of necrotizing idiopathic hypophysitis.

In a patient with recurrent inflammation in the sellar region, hypophysitis should be considered in the differential and early medical intervention with steroids or immunosuppression may be indicated in refractory or recurrent disease.
Authors/Disclosures

PRESENTER 		No disclosure on file
Hera A. Kamdar, MD Dr. Kamdar has nothing to disclose.
No disclosure on file
No disclosure on file
Stephen J. Kolb, MD, PhD (The Ohio State University) Dr. Kolb has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for AveXis. Dr. Kolb has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for CureSMA. The institution of Dr. Kolb has received research support from NIH. The institution of Dr. Kolb has received research support from AveXis. The institution of Dr. Kolb has received research support from NIH.