Lymphocytic infundibulo- neurohypophysitis with hypothalamic and optic pathway involvement: report of a case and review of the literature.
BACKGROUND
Lymphocytic adenohypohysitis and lymphocytic infundibulo-neurohypophysitis are rare auto-immune mediated diseases of the anterior and posterior pituitary, respectively. The former usually manifests as insufficiency of anterior pituitary hormone secretion, associated in many patients with disturbances of vision. The latter presents as diabetes insipidus of central origin. They present most commonly in pregnant or postpartum females. There have been infrequent reports in females with no association with pregnancy, and in males.


METHODS
We present a nulliparous female with central diabetes insipidus, pan-hypopituitarism, and severely impaired vision. Magnetic resonance imaging demonstrated a large mass involving the hypothalamus, infundibulum, optic nerves, chiasm, and tracts. At operation, the optic pathways were found to be grossly involved in the inflammatory mass. Histological examination of a biopsy demonstrated a nonspecific, mixed inflammatory infiltrate, composed predominantly of lymphocytes and plasma cells. She responded dramatically to treatment with dexamethasone, with disappearance of the mass on serial imaging studies and improvement in vision. In addition, she received hormone replacement therapy.


CONCLUSIONS
We present a case of lymphocytic infundibulo-neurohypophysitis unique in the degree of optic pathway inflammatory involvement, with a documented response to steroids.
Ouma
J R
JR
Department of Neurological Surgery, Johannesburg Hospital, and the University of the Witwatersrand, Johannesburg, South Africa.
Farrell
V J R
VJ
D000324:Adrenocorticotropic Hormone / Q000097:blood; D000328:Adult; D001111:Arcuate Nucleus of Hypothalamus / Q000473:pathology*; D001706:Biopsy; D005260:Female; D005640:Follicle Stimulating Hormone / Q000097:blood; D019382:Human Growth Hormone / Q000097:blood; D006801:Humans; D007031:Hypothalamus / Q000473:pathology*; D007986:Luteinizing Hormone / Q000097:blood; D008214:Lymphocytes / Q000276:immunology* / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D010900:Pituitary Diseases / Q000276:immunology* / Q000473:pathology*; D011388:Prolactin / Q000097:blood; D012016:Reference Values; D013972:Thyrotropin / Q000097:blood; D014795:Visual Pathways / Q000473:pathology*
D019382:Human Growth Hormone; D000324:Adrenocorticotropic Hormone; D011388:Prolactin; D007986:Luteinizing Hormone; D005640:Follicle Stimulating Hormone; D013972:Thyrotropin