Endocrine Abstracts

Introduction: Tolosa Hunt Syndrome (THS) is a steroid-responsive idiopathic inflammatory condition affecting cavernous sinus and/or orbital apex causing painful ophthalmoplegia. We present a rare case of THS resulting in hypopituitarism.

Case: 45-year-old female presented with 10-day history of headache, periorbital pain and diplopia. Past medical history included bipolar disorder and bilateral below knee amputation from rail accident. Drug history: mirtazepine, olanzapine and epilim. On examination, left abducens nerve palsy was noted with no other neurological/ophthalmological abnormalities. Haematology, biochemistry, autoimmune screen, angiotensin converting enzyme (ACE) were normal. MR angiography showed hyperintense lesion in left cavernous sinus suggesting inflammatory tissue, but no vascular or pituitary abnormalities were noted. A diagnosis of THS was made, and prednisolone 60 mg/day was commenced. Ophthalmic pain subsided within 48 hours; ophthalmoplegia resolved over 3 months leading to steroid cessation.

Progression: Six months later patient developed polyuria, polydipsia, tiredness and weight loss. Biochemistry revealed new onset type 2 diabetes mellitus and secondary hypothyroidism. Symptoms improved with levothyroxine and diabetes treatment, but full pituitary evaluation was not undertaken. Evaluation 10 years later by an Endocrinologist revealed partial anterior hypopituitarism: secondary hypothyroidism (TSH 1.4 miu/L {0.3–5}, FT4 6.9 pmol/L {9–25}), secondary hypogonadism (LH)

Discussion: THS is a rare cause of hypopituitarism with unknown aetiological mechanism. 5 of 7 cases reported with this association are of Japanese descent; 5 of 7 had diabetes insipidus. 1 of 7 died of cerebral venous thrombosis; 6 recovered with high-dose steroids, and 2 patients showed full pituitary recovery.

Learning points:

1. THS diagnosis should be suspected with presentation of painful ophthalmoplegia and full pituitary evaluation should be undertaken.

2. Symptoms usually resolve with high dose steroids but hypopituitarism is a recognised chronic complication.