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Show Journal of'A] euro- Ophthalmology 21( 1): 34- 36, 2001. 1 2001 Lippincott Williams & Wilkins, Inc., Philadelphia Case Report Granulomatous Hypophysitis and Bilateral Optic Neuropathy E. M. Arsava, MD, K. Ulug, MD, T. Kansu, MD, C. F. Dogulu, MD, F. Soylemezoglu, MD, and K. Selekler, MD A 53- year- old woman with symptoms of hypopituitarism and ophthalmoplegia was diagnosed as having idiopathic granulomatous hypophysitis and later developed bilateral optic neuritis. She responded well to steroid treatment. Granulomatous hypophysitis is a rare entity, and this is the first reported case associated with optic neuritis. Key Words: Granulomatous hypophysitis- Ophthalmoplegia- Bilateral optic neuropathy. Granulomatous hypophysitis is an rarely encountered inflammatory disorder of the pituitary gland. It is generally diagnosed as pituitary adenoma with its clinical and radiologic findings. The diagnosis is made with the observation of granulomas with epitheloid histiocytes and multinucleated giant cells that can also be associated with lymphocytes. Few cases of this entity have been reported in the literature, and its association with granulomatous disorders such as tuberculosis ( 1), sarcoidosis ( 2), histiocytosis X ( 3), syphilis ( 4) and secondary granulomatous reactions resulting from ruptured Rathke cleft cyst ( 5) and mucocele ( 6) has been documented. In the group of patients with no etiologic cause to be identified, the term idiopathic granulomatous hypophysitis is used ( 7). CASE REPORT A 53- year- old female was evaluated in August 1999 in another medical center because of secondary amenorrhea. Except for the noninsulin- dependent diabetes mel-litus since 1985 in her history, she reported no previous Manuscript received November 8, 2000; accepted January 3, 2001. From the Department of Neurology ( EMA, KU, KS), Department of Neurology- Neuro- Ophthalmology Unit ( TK), and Department of Pathology ( FS), Hacettepe University Hospitals; and the Institute of Neurological Sciences and Psychiatry, Department of Neurology- Neuro- Ophthalmology Unit ( CFD), Hacettepe University, Ankara, Turkey. Address correspondence and reprint requests to Tulay Kansu, MD, Hacettepe University Hospitals, Department of Neurology, Ankara 06100 TURKEY; e- mail: tkansu@ ada. net. tr. illness. In her screening tests for pituitary hormone levels, decreased levels of thyroid- stimulating hormone, fol-licular- stimulating hormone, luteinizing hormone, and prolactin were detected. Her serum basal Cortisol level was also below the normal limits. The patient was given hormone replacement therapy, including 5 mg of oral prednisolone, daily. One month later, while she was on schedule for magnetic resonance imaging ( MRI), complaints of diplopia prominent on horizontal gaze, drooping of the eyelid, inward deviation, and blurring of vision OD emerged. Cranial MRI revealed a contrast- enhanced lesion with a 1.4- cm diameter consistent with pituitary macroadenoma. The patient was referred to our neurosurgery department where mild ptosis and limited abduction OD was noted. Transsphenoidal resection of the pituitary gland was planned for October 28, 1999, but an excisional biopsy was performed, and the operation was terminated after the visualization of the firm, fibrotic, and yellowish appearance of the pituitary gland. Microscopic examination disclosed preservation of anterior pituitary acini that were separated by inflammatory cell infiltrate exclusively composed of lymphocytes and plasma cells. Granulomas formed by epithelioid histiocytes and multinucleated giant cells were observed ( Fig. 1). Ziehl- Nielsen staining for acid- fast bacilli, me-fhenamine silver, and periodic acid Schiff results for fungi were negative. The histopathologic diagnosis was giant cell granulomatous hypophysitis. She received 5 days of intravenous dexamethasone therapy ( 4 mg every 6 hours during the perisurgical period), which was then tapered gradually and stopped in the postoperative fifteenth day. The patient was followed for a 3- month symptom- free period postoperatively. In January 2000, 3 months after the surgery, she was first seen in the neuro- ophthalmology unit for evaluation of 15 days of progressive visual loss OD. Visual acuity was 20/ 400 OD and 20/ 20 OS with the Ishihara plates. There was a right relative afferent pupillary defect. Color vision was 0/ 12 OD and 12/ 12 OS. Visual field examination by Goldmann perimeter revealed a dense central scotoma extending to the temporal field OD and intact 34 GRANULOMATOUS HYPOPHYSITIS AND BILATERAL OPTIC NEUROPATHY 35 FIG. 1. Granulomatous inflammation characterized by multinucleated giant cells, epithelioid histiocytes, and lymphoplasma-cytic infiltration destructing the anterior pituitary gland ( Hema-toxylen- Eosin, x 200). field OS. The right optic disc was swollen and the left appeared normal. Eye movements were full, there was no ptosis, and the rest of the neurologic examination was within normal limits. A recurrence of granulomatous reaction was considered in diagnosis, and she was hospitalized. Cranial and orbital MRI revealed infindibular thickening, edema, and contrast- enhanced lesion in both optic nerves ( Fig. 2). Complete blood count, erythrocyte sedimentation rate, chest X- ray, and serum biochemistry revealed no abnormalities, except for the high serum glucose level. Results for serologic markers for syphilis and serum angiotensin- converting enzyme ( ACE) were found to be negative. Lumbar puncture was performed twice; cerebrospinal fluid ( CSF) pressure, protein and glucose level, cytologic examination, routine bacterio-logic cultures and fungal cultures, ACE level, and Venereal Disease Research Laboratory results were all within normal limits. Samples from CSF and biopsy specimen were also analyzed for specific culture FIG. 2. Magnetic resonance examination with T1- weighted axial image revealed a contrast- enhanced lesion involving the optic chiasm and the optic nerves. for tuberculosis and detection of tuberculous bacilli with polymerase chain reaction ( PCR). The protein purified derivative ( PPD) test result was found to be 30 mm x 30 mm, which was regarded as positive. The patient also reported abdominal tuberculosis in her father, diagnosed 5 years ago. Visual acuity regressed to the level of light perception OD and 20/ 70 OS in a period of 8 days. Because of positive PPD and family history of tuberculosis, the patient began isoniazid, rifampin, ethambutol, and pyrazinamide therapy. On the fifth day of antituberculosis therapy, the vision decreased to the level of no light perception OD and 20/ 200 OS. Ethambutol was removed from the antituberculosis regimen because of its potential risk for optic toxicity. Taking into consideration the rapid progressive character of the symptoms and the history of benefit from increased dose of steroid therapy received perioperatively, the patient was given 1 gr/ day of intravenous methylprednisolone for 5 days. Twelve hours after the onset of steroid therapy, visual acuity was 20/ 200 OD and 20/ 70 OS. On the third day of steroid therapy, visual acuity improved to 20/ 30 OD and 20/ 20 OS. Antituberculosis therapy was discontinued after PCR because the tuberculous bacilli from the CSF and biopsy specimen was found to be negative. Five days later, maintenance therapy with 1 mg/ kg/ day of oral methylprednisolone was initiated. On her control cranial MRI, mild regression of the previous lesions were detected. No uptake of gallium was detected in her whole body gallium- 67 scintigraphy. Tuberculosis culture results were reported negative 6 weeks later. The dose of methylprednisolone was tapered gradually to 8- mg daily; she was asymptomatic, and the vision was 20/ 20 OU 8 months after her discharge. DISCUSSION Inflammatory lesions of the pituitary gland, which generally present as hypopituitarism clinically and sella turcica enlargement radiologically, are rare entities. As in our case, these entities are generally misdiagnosed as pituitary adenoma. Two main types of inflammatory lesions of the pituitary gland have been described. Lymphocytic hypo-physitis is pathologically characterized by the diffuse infiltration of the pituitary gland by lymphocytes with the formation of lymphoid follicles and plasma cells, commonly seen in females during pregnancy or in the puerperium ( 8). Granulomatous hypophysitis, the other form of the inflammatory lesion of the pituitary gland, which is characterized by the infiltration of the gland by multinucleated giant cells, histiocytes, plasma cells, and lymphocytes, represents approximately 1 % of sellar pathology approached via the transsphenoidal route ( 6). Granulomatous hypophysitis is seen in systemic granulomatous diseases; however, the cause is unclear in many instances ( 1- 6). The inflammation generally affects the anterior lobe of the pituitary gland. Middle- aged and elderly women are reported to be affected predominantly ( 9). Granulomatous lesions of the pituitary gland can be an incidental autopsy finding and remain silent during life ( 6). J Neuro- Ophthalmol, Vol. 21, No. 1, 2001 36 E. M. ARSAVA ET AL. In the present case, a hypofunction of the pituitary gland was noted at the onset of symptoms. The patient later presented with ptosis and abduction deficit OD. Clinical and radiological diagnosis was pituitary adenoma; however, the biopsy was consistent with granulomatous hypophysitis. Hypophysectomy was not performed, and the symptoms regressed by the increased dose of steroid therapy given during the perisurgical period. After a 3- month asymptomatic period, the patient developed signs of right optic neuropathy. Bilateral optic nerve enhancement was detected in MRI, and the second eye became symptomatic during the hospital course. After the exclusion of possible causative systemic disorders such as tuberculosis, syphilis, or sarcoidosis, the patient was diagnosed with idiopathic granulomatous hypophysitis and treated with steroid therapy with excellent response. In conclusion, this report documents a case of idiopathic granulomatous hypophysitis presenting with the symptoms of optic neuritis. To our knowledge, no other case of optic neuritis associated with this entity has been documented. In cases of optic neuritis, proceeding with the symptoms of hypopituitarism and ophthalmoplegia, the clinician should consider the possibility of parasellar pathologies, including a granulomatous lesion of that region. We recommend immunosuppressive treatment with corticosteroids after confirmatory biopsy and avoiding unnecessary surgical resection of the pituitary gland. REFERENCES 1. Esposito V, Fraioli B, Ferrante L, et al. Intrasellar tuberculoma: case report. Neurosurgery 1987; 21: 721- 3. 2. Bleish VR, Robbins SL. Sarcoid- like granulomata of the pituitary gland. A cause of pituitary insufficiency. Arch Intern Med 1952; 89: 877- 92. 3. Nishio S, Mizuno J, Barrow DL, et al. Isolated histiocytosis X of the pituitary gland: case report. Neurosurgery 1987; 21: 718- 21. 4. Fink EB. Gumma of the hypophysis and hypothalamus. Arch Pathol 1933; 15: 631- 5. 5. Albini CH, MacGillivray MH, Fischer JE, et al. Triad of hypopituitarism, granulomatous hypophysitis, and ruptured Rathke's cleft cyst. Neurosurgery 1988; 22: 133- 6. 6. Scanarini M, d'Avella D, Rotilio A, et al. Giant cell granulomatous hypophysitis: a distinct clinicopathological entity. J Neurosurg 1989; 71: 681- 6. 7. Vasile M, Marsot- Dupuch K, Kujas M, et al. Idiopathic granulomatous hypophysitis: clinical and imaging features. Neuroradiology 1997; 39: 7- 11. 8. Cosman F, Post KD, Holub DA, et al. Lymphocytic hypophysitis. Report of 3 new cases and review of the literature. Medicine ( Baltimore) 1989; 68: 240- 56. 9. Honegger J, Fahlbusch R, Bornemann A, et al. Lymphocytic and granulomatous hypophysitis: experience with nine cases. Neurosurgery 1997; 40: 713- 22. / Neuro- Ophthalmol, Vol. 21, No. 1, 2001 |
