IDIOPATHIC CAVERNOUS SINUS INFLAMMATION; THE TOLOSA-HUNT SYNDROME WITH SELLAR EXTENSION PRESENTING WITH HYPOPITUITARISM

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: SAT 164-196-Pituitary
Basic/Clinical
Saturday, June 15, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board SAT-180
Cherie Lisa Vaz*1, Jody Leonardo1, Laszlo Mechtler1, Lucia Balos1 and Ajay Chaudhuri2
1The State University of New York, University at Buffalo, 2The State Univeristy of New York, University at Buffalo
Objective: We report a case of Tolosa Hunt Syndrome causing anterior pituitary hypofunction & its successful management.

Case Presentation: A 61 y/o F presented with palpitations. She also gave a 3y h/o headache, diplopia & h/o stroke. On exam she had R 6th & 7th cranial nerve palsy & L hemiparesis. TSH 0.52mIU/L(0.4-4.5) FT4 0.7ng/dl(0.8-1.8) RAIU of thyroid 2%. With TFTs suggestive of secondary hypothyroidism pituitary panel was checked. 8am ACTH 13 pg/ml(6-50), cortisol 2.6mcg/dl, FSH 2.3 mIU/L(23-116.3) LH 0.3 mIU/ml(10-54.7) Prolactin 17ng/ml(2–20) IGF-1 71 ng/ml(41-279), GH 0.2 ng/ml. She failed an ACTH stimulation test. Pituitary MRI showed extensive abnormality at the skull base centered in the sella turcica with mild enlargement of the gland, at 12mm, heterogeneous enhancement & extensive infiltrating tissue in both cavernous sinuses & adjacent dural surfaces & encasement of ICAs. Subsequently ESR 75mm/hr(0-20), CRP 27.3(1-5) ANA 80(>=80). Autoatibody Panel + for SSA. Negative dsDNA, SSB, SMRNP, CentromereB scleroderma70, JO-1, Histone. Lyme AB<0.91(0.0-0.9) RPR 1.4ng/dl(0.8-1.8) SPEP UPEP nl, Urine immunofixation neg. Serum IGG 1344mg/dl IGG subclasses nl. CSF mild elevated protein, CSF ACE nl, cytology neg, CT torso neg. PET showed minimal FDG uptake associated with skull base abnormality. Endoscopic transphenoidal sellar biopsy showed dura with chronic inflammatory cell infiltrate of CD3 Tcells, CD138+ lymphocytes, plasma cells extending into the adenohypophysis. The patient was treated with prednisone & azathioprine. On treatment headaches resolved immediately, diplopia, ESR & CRP normalized in a few weeks, the lesion significantly improved on MRI 2 months later. Immunosuppresive therapy is gradually being tapered.

Discussion: The patient ran an extremely indolent course & was diagnosed during work up of failed hypothalamic pituitary axis. She had no persistent diabetes insipidus & preserved posterior pituitary function, unlike the few reported cases of Tolosa Hunt presenting with panhypopituitrism & diabetes insipidus(1,2,3). Laboratory work up was significant for elevated ESR & CRP which normalized on treatment, mild elevated CSF protein and +ANA. These are usually nl in Tolosa Hunt(4) but have been rarely reported to be abnormal(5).

Conclusion: This is a rare presentation of Tolosa Hunt Syndrome extending to the sella turcica & involving the adenohypophysial function. Extension of granulomatous inflammation into the cavernous sinus, to the pituitary portal system, the pituitary & hypothalamus itself causes pituitary failure. As Tolosa Hunt has a relapsing course, we are monitoring for recurrence with consideration of radiation therapy for treatment failure or recurrence.

1. Endocr J. 1996 Oct;43(5):503-10. 2. Neurol Med Chir (Tokyo). 2007 Feb;47(2):85-8. 3. Intern Med. 1995 Nov;34(11):1093-6. 4. Eur J Radiol. 2003 Feb;45(2):83-90. 5. Tolosa-Hunt syndrome Uptodate

Nothing to Disclose: CLV, JL, LM, LB, AC

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