Skull base Lymphocytic Infundibulo- Neurohypophysitis with Hypothalamic and Optic Pathway Involvement: Report of a Case and Review of the Literature J.R. Ouma, M.D., and V.J.R. Farrell, M.D. Department of Neurological Surgery, Johannesburg Hospital, and the University of the Witwatersrand, Johannesburg, South Africa Ouma JR, Farrell VJR. Lymphocytic infundibulo-neurohypophysitis with hypothalamic and optic pathway involvement: report of a case and review of the literature. Surg Neurol 2002;57:49 –54. BACKGROUND Lymphocytic adenohypohysitis and lymphocytic infundibulo-neurohypophysitis are rare auto-immune me- diated diseases of the anterior and posterior pituitary, respectively. The former usually manifests as insuffi- ciency of anterior pituitary hormone secretion, associ- ated in many patients with disturbances of vision. The latter presents as diabetes insipidus of central origin. They present most commonly in pregnant or postpar- tum females. There have been infrequent reports in fe- males with no association with pregnancy, and in males. CASE DESCRIPTION We present a nulliparous female with central diabetes insipidus, pan-hypopituitarism, and severely impaired vi- sion. Magnetic resonance imaging demonstrated a large mass involving the hypothalamus, infundibulum, optic nerves, chiasm, and tracts. At operation, the optic path- ways were found to be grossly involved in the inflamma- tory mass. Histological examination of a biopsy demon- strated a nonspecific, mixed inflammatory infiltrate, composed predominantly of lymphocytes and plasma cells. She responded dramatically to treatment with dexa- methasone, with disappearance of the mass on serial imaging studies and improvement in vision. In addition, she received hormone replacement therapy. CONCLUSION We present a case of lymphocytic infundibulo- neurohypophysitis unique in the degree of optic pathway inflammatory involvement, with a documented response to steroids. © 2002 by Elsevier Science Inc. KEY WORDS Lymphocytic infundibulo-neurohypophysitis, optic chiasm, nerves, tract. I ncreased awareness of chronic inflammatory le- sions involving the pituitary gland and neighbor- ing structures, and the availability of magnetic res- onance imaging (MRI), have led to a growing number of reports of these conditions in the liter- ature [15]. Though lymphocytic adenohypophysitis and infundibulo-neurohypophysitis are the commonest of these conditions [15], they remain relatively un- common and poorly understood. Their importance lies in the possible association with serious compli- cations such as panhypopituitarism, central diabe- tes insipidus, and visual impairment which, if un- recognized or treated too cautiously, can lead to significant morbidity and mortality. Case Report A 23-year-old, nulliparous female clerk presented with a 4-year history of amennorhoea, a 4-month history of polyuria and polydipsia, and sudden loss of vision in the left eye 2 months before referral to our unit. She denied any history of inexplicable fatigue, heat or cold intolerance, recent unexplained changes in body weight or size of clothing, or dis- turbances in libido. A diagnosis of hypothyroidism was made by the family practitioner and levothy- roxine sodium was prescribed 2 months before re- ferral to our unit. Address reprint requests to: Dr. John Ouma, University of the Witwa- tersrand, Department of Neurosurgery, 7 York Road, Parktown, 2193, Johannesburg, South Africa. Received February 14, 2001; accepted July 25, 2001. © 2002 by Elsevier Science Inc. 0090-3019/02/$–see front matter 655 Avenue of the Americas, New York, NY 10010 PII S0090-3019(01)00647-4