Criteria for the non-invasive diagnosis of lymphocytic hypophysitis (LyHy) and the results of the first prospective trial of high dose methylprednisolone pulse therapy (HDMPT) in nine patients are presented. In three patients, the diagnosis was established histologically, and in the others by clinical and endocrinological assessment, MRI, CSF examination, and measurement of thyroglobulin autoantibody concentration. After HDMPT, adenopituitary function improved in four of the nine patients and diabetes insipidus ceased or improved in all four concerned patients. The MRI findings improved in seven patients. LyHy has to be considered in the diVerential diagnosis of sellar lesions. The presumptive non-invasive diagnosis of LyHy seems possible in a high proportion of patients. HDMPT may result in the improvement of clinical, endocrinological, and MRI findings. (J Neurol Neurosurg Psychiatry 1999;67:398-402) Keywords: lymphocytic hypophysitis; high dose methylprednisolone pulse therapy Lymphocytic hypophysitis (LyHy) is a rare chronic inflammatory disease with little known natural history, 1-7 usually diagnosed unexpectedly at surgery for presumptive pituitary adenoma.1 5 8 9 Experience in the treatment of LyHy is scarce. Non-invasive diagnostic criteria and results of standardised HDMPT are presented.
Patients and methodsAll patients with LyHy (mean age 41 years, seven women) diagnosed at our institution are reported on. Adenohypophyseal function was dynamically assessed as described by Thorner et al. 13 Neurohypophyseal function was assessed as described by Reeves and Andreoli.14 Evaluation of the sellar region by MRI was carried out as described by Elster. 15 In the six patients not operated on, CSF was evaluated by white cell count, cytology, global protein content, and neurotropic virus serology (herpes simplex, varicella zoster, mumps). The thyroglobulin autoantibody concentration was measured in four patients. In three patients, LyHy was diagnosed histologically. In the others, diVerential diagnosis was considered for tuberculosis, sarcoidosis, and syphilis by clinical, laboratory (tuberculin test, angiotensin I converting enzyme measurement, treponema pallidum haemaglutination test), and chest radiograhy evaluation.The high dose short lasting methylprednisolone administration aimed at minimising the side eVects of chronic corticosteroid therapy and at diVerentiating therapeutic eVects from longterm natural course of LyHy. HDMPT consisted of 120 mg methylprednisolone daily for 2 weeks, followed by a dose reduction to 80, 60, 40, and 20 mg daily for 1 week each. Each patient received one course of HDMPT.Results were assessed by endocrinology and MRI as presented above, at 3, 6, and 12 month intervals thereafter. The average follow up amounted to 29 (19-38) months.Literature research was done using the Medline program and the key word hypophysitis.
ResultsThe patient's details and courses of disease are listed in the table.Two of the three patients operated on were only biopsied because the intraoperat...