Abstract
Lymphocytic infundibulo-neurohypophysitis (LINH) was first reported by Saito et al. and Imura et al. as a cause of idiopathic central diabetes insipidus. Magnetic resonance (MR) imaging with a contrast medium demonstrates thickening of the pituitary stalk, enlargement of the neurohypophysis, or both with homogeneous enhancement. Histological examination reveals a posterior pituitary that is heavily infiltrated by lymphocytes with occasional plasma cells and other inflammatory cells. In early reports of the disorder, the lesion seemed to be limited to the neurohypophysis, but the present review showed cases with a combination of hypopituitarism and diabetes insipidus. Some of them showed partial hypopituitarism. The so-called lymphocytic infundibulo-panhypophysitis (LIPH) is now regarded as a lymphocytic hypophysitis variant. LINH and LIPH are essentially self-limited. In typical cases, conservative care with steroids and hormone replacement is recommended. Surgical intervention should be avoided because the natural course of the disorder may be self-limited. Pathophysiology of the disorder is still unknown. The unique clinical manifestations of the disorder are discussed and reviewed here.
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References
Goudie RB, Pinkerton PH (1962) Anterior hypophysitis and Hashimoto’s disease in a young woman. J Pathol Bacteriol 83:584–585
Beressi N, Beressi JP, Cohen R, et al (1999) Lymphocytic hypophysitis. Ann Med Interne 150:327–341
Hashimoto K, Takao T, Makino S (1997) Lymphocytic adenohypophysitis and lymphocytic infundibuloneurohypophysitis. Endocr J 44:1–10
Hashimoto K, Asaba K, Tamura K, et al (2002) A case of lymphocytic infundibuloneurohypophysitis associated with systemic lupus erythematosus. Endocr J 49:605–610
Thodou E, Asa SL, Kontogeorgos G, et al (1995) Lymphocytic hypophysitis: clinicopathological findings. J Clin Endocrinol Metab 80:2302–2311
Nishioka H, Ito H, Sano T, et al (1996) Two cases of lymphocytic hypophysitis presenting with diabetes insipidus: a variant of lymphocytic infundibulo-neurohypophysitis. Surg Neurol 46:285–291
Honegger J, Fahlbusch R, Bornemann A, et al (1997) Lymphocytic and granulomatous hypophysitis: experience with nine cases. Neurosurgery 40:713–723
Imai H, Okuyama S, Komatsuda A, et al (2000) Central diabetes insipidus due to lymphocytic infundibulo-neurohypophysitis. Am J Med 109:497–499
Imura H, Nakao K, Shimatsu A, et al (1993) Lymphocytic infundibuloneurohypophysitis as a cause of central diabetes insipidus. N Engl J Med 329:683–689
Ito K, Mukawa J, Miyagi K, et al (1992) Lymphocytic adenohypophysitis with sudden onset of diabetes insipidus in menopausal female. Neurol Med Chir (Tokyo) 32:346–350
Iwai K, Yamanaka K, Yoshioka K, et al (1998) Four cases of lymphocytic infundibuloneurohypophysitis (in Japanese). No Shinkei Geka 26:831–835
Jin-no M, Fujii T, Jin-no Y, et al (1999) Central diabetes insipidus with Behcet’s disease. Intern Med 38:995–999
Kakizawa Y, Tanaka Y, Kitazawa K, et al (1999) Three cases of lymphocytic infundibuloneurohypophysitis (in Japanese). Horumon To Rinsho (Jpn Clin Endocrinol) 47:162–166
Kamel N, Ilgin ŞD, Çorapçioğlu D, et al (1998) Lymphocytic infundibuloneurohypophysitis presenting as diabetes insipidus in a man. J Endocrinol Invest 21:537–540
Kamel N, Ilgin ŞD, Güllü S, et al (1999) Lymphocytic hypophysitis and infundibuloneurohypophysitis; clinical and pathological evaluations. Endocr J 46:505–512
Kawaguchi T, Ikeda H, Fujimura M, et al (2005) Delayed lymphocytic infundibuloneurohypophysitis following successful transsphenoidal treatment of Cushing’s disease. J Clin Neurosci 12: 320–323
Kojima H, Nojima T, Nagashima K, et al (1989) Diabetes insipidus caused by lymphocytic infundibuloneurohypophysitis. Arch Pathol Lab Med 113:1399–1401
Koshiyama H, Sato H, Yorita S, et al (1994) Lymphocytic hypophysitis presenting with diabetes insipidus: case report and literature review. Endocr J 41:93–97
Miyamoto M, Terada T, Yukawa S, et al (1997) A case of lymphocytic infundibuloneurohypophysitis (in Japanese). Progress in computed imaging 19:79–82
Nishiki M, Murakami Y, Ozawa Y, et al (2001) Serum antibodies to human pituitary membrane antigens in patients with autoimmune lymphocytic hypophysitis and infundibuloneurohypophysitis. Clin Endocrinol 54:327–333
Saito T, Yoshida S, Nakao K, et al (1970) Chronic hypernatremia associated with inflammation of the neurohypophysis. J Clin Endocrinol Metab 31:391–396
Shimono T, Yamaoka T, Nishimura K, et al (1999) Lymphocytic hypophysitis presenting with diabetes insipidus: MR findings. Eur Radiol 9:1397–1400
Terao T, Taya K, Sawauchi S, et al (2003) Two cases of lymphocytic infundibuloneurohypophysitis (in Japanese). No Shinkei Geka 31:1229–1235
Tsujii S, Takeuchi J, Koh M, et al (1997) A candidate case for lymphocytic infundibulo-neurohypophysitis mimicking a neurohypophysial tumor. Intern Med 36:293–297
van Havenbergh T, Robberecht W, Wilms G, et al (1996) Lymphocytic infundibulohypophysitis presenting in the post partum period: case report. Surg Neurol 46:280–283
Waki K, Yamada S, Ozawa Y, et al (1999) A case of lymphocytic infundibuloneurohypophysitis: histopathological studies. Pituitary 1:285–290
Takahashi M, Otsuka F, Miyoshi T, et al (1999) An elderly patient with transient diabetes insipidus associated with infundibuloneurohypophysitis. Endocr J 46:741–746
Ng WH, Gonzales M, Kaye AH (2003) Lymphocytic hypophysitis. J Clin Neurosci 10:409–413
Abe T, Matsumoto K, Sanno N, et al (1995) Lymphocytic hypophysitis: case report. Neurosurgery 36:1016–1019
Amagasa M, Yuda F, Kojima H, et al (2001) Natural course of lymphocytic infundibuloneurohypophysitis. Clin Neuropathol 20: 229–232
Bayram F, Keleştimur F, Öztürk F, et al (1998) Lymphocytic hypophysitis in a patient with Graves’ disease. J Endocrinol Invest 21:193–197
Bettendorf M, Fehn M, Grulich-Henn J, et al (1999) Lymphocytic hypophysitis with central diabetes insipidus and consequent panhypopituitarism preceding a multifocal, intracranial germinoma in a prepubertal girl. Eur J Pediatr 158:288–292
Cemeroglu AP, Blaivas M, Muraszko KM, et al (1997) Lymphocytic hypophysitis presenting with diabetes insipidus in a 14-year-old-girl: case report and review of the literature. Eur J Pediatr 156: 684–688
Fukuyama T, Koike K, Shiohara M, et al (2000) Lymphocytic infundibuloneurohypophysitis during the first remission in acute lymphoblastic leukemia (in Japanese). Rinsho Ketsueki 41: 1208–1213
Hikomaru T, Hashimoto N, Kikuchi H, et al (1992) Seven cases of unknown central diabetes insipidus (in Japanese). Horumon To Rinsho (Jpn Clin Endocrinol) 41:190–192
Kurino M, Seto H, Miura M, et al (1992) Three cases of lymphocytic adenohypophysitis (in Japanese). Horumon To Rinsho (Jpn Clin Endocrinol) 41:182–185
Ludmerer KM, Kissane JM (1993) Headaches, diabetes insipidus, and hyperprolactinemia in a woman with an enlarged pituitary gland. Am J Med 95:332–339
Maghnie M, Villa A, Arico M, et al (1992) Correlation between magnetic resonance imaging of posterior pituitary and neurohypophyseal function in children with diabetes insipidus. J Clin Endocrinol Metab 74:795–800
McDermott MW, Griesdale DE, Berry K, et al (1988) Lymphocytic adenohypophysitis. Can J Neurol Sci 15:38–43
Miura M, Ushio Y, Kuratsu J, et al (1989) Lymphocytic adenohypophysitis: report of two cases. Surg Neurol 32:463–470
Nakamura Y, Okada H, Wada Y, et al (2001) Lymphocytic hypophysitis: its expanding features. J Endocrinol Invest 24: 262–267
Nishioka H, Ito H, Miki T, et al (1994) A case of lymphocytic hypophysitis with massive fibrosis and the role of surgical intervention. Surg Neurol 42:74–78
Nussbaum CE, Okawara SH, Jacobs LS (1991) Lymphocytic hypophysitis with involvement of the cavernous sinus and hypothalamus. Neurosurgery 28:440–444
Omori K, Hizuka N, Sada A, et al (2002) A case of lymphocytic hypophysitis with diabetes insipidus (in Japanese). Nippon Naibunpitsu Gakkai Zasshi (Jpn Endocr J) 78:37–39
Paja M, Estrada J, Ojeda A, et al (1994) Lymphocytic hypophysitis causing hypopituitarism and diabetes insipidus, and associated with auto immune thyroiditis, in a non-pregnant woman. Postgrad Med J 70:220–224
Sato N, Sze G, Endo K (1998) Hypophysitis: endocrinologic and dynamic MR findings. AJNR Am J Neuroradiol 19:439–444
Takao T, Asaba K, Tanaka H, et al (2000) A case of lymphocytic infundibuloneurohypophysitis showing diabetes insipidus followed by anterior hypopituitarism associated with thrombasthenia. Endocr J 47:285–291
Vanneste JAL, Kamphorst W (1987) Lymphocytic hypophysitis. Surg Neurol 28:145–149
Watanabe A, Ishii R, Hirano K, et al (1994) Central diabetes insipidus caused by nonspecific chronic inflammation of the hypothalamus: case report. Surg Neurol 42:70–73
Yamaguchi T, Abe H, Matsui T, et al (1994) Lymphocytic hypophysitis, pustulosis palmaris et plantaris and eosinophilia. Intern Med 33:150–154
Ouma JR, Farrell VJ (2002) Lymphocytic infundibuloneurohypophysitis with hypothalamic and optic pathway involvement: report of a case and review of the literature. Surg Neurol 57:49–53
Tubridy N, Saunders D, Thom M, et al (2001) Infundibulohypophysitis in a man presenting with diabetes insipidus and cavernous sinus involvement. J Neurol Neurosurg Psychiatry 71:798–801
A Ministry of Health, Labour and Welfare grants-in-aid-for-scientific-research intractable disease conquest research enterprise (2003) The surveillance-study group about a hypophysis functional disorder: a guide of a diagnosis and the treatment of the self-immune hypothalamus pituitary gland inflammation. 2002 generalization/assignment research report. Ministry of Health, Labour and Welfare, Japan, pp 141–143
Eroles Vega G, del Olmo Garcia MD, Lacambra CC, et al (2001) Adult Still’s disease and lymphocytic hypophysitis. Rev Clin Esp 201:557–558
Tamiya A, Saeki N, Mizota A (2001) Lymphocytic infundibuloneurohypophysitis associated with recurrent optic neuritis. Br J Neurosurg 15:180–183
Ahmed SR, Aiello DP, Page R, et al (1993) Necrotizing infundibulo-hypophysitis: a unique syndrome of diabetes insipidus and hypopituitarism. J Clin Endocrinol Metab 76:1499–1504
Miyagi K, Shingaki T, Ito K, et al (1997) Lymphocytic infundibulohypophysitis with diabetes insipidus as a new clinical entity: a case report and review of the literature (in Japanese). No Shinkei Geka 25:169–175
Saito J (1999) Lymphocytic infundibuloneurohypophysitis (in Japanese). Nippon Naibunpitsu Gakkai Zasshi (Jpn Endocr J) 47: 12–16
Abe T (2003) Pathophysiology of lymphocytic infundibuloneurohypophysitis (in Japanese). Shinkei Shinpo (Advances in Neurological Sciences) 250:417–424
Nishioka H, Ito H, Haraoka J, et al (1998) Idiopathic hypertrophic cranial pachymeningitis of the cavernous sinus mimicking lymphocytic hypophysitis. Neurol Med Chir (Tokyo) 38:377–382
Fujisawa I, Asato R, Okumura R, et al (1991) Magnetic resonance imaging of neurohypophyseal germinoma. Cancer (Phila) 68:1009–1014
Leger J, Velasquez A, Garel C, et al (1999) Thickened pituitary stalk on magnetic resonance imaging in children with central diabetes insipidus. J Clin Endocrinol Metab 84:1954–1960
Rivera JA (2006) Lymphocytic hypophysitis: disease spectrum and approach to diagnosis and therapy. Pituitary 9:35–45
Kristof RA, van Roost D, Klingmüller D, et al (1999) Lymphocytic hypophysitis: non-invasive diagnosis and treatment by high dose methylprednisolone pulse therapy? J Neurol Neurosurg Psychiatry 67:398–402
Lecube A, Francisco G, Rodriguez D, et al (2003) Lymphocytic hypophysitis successfully treated with azathioprine: first case report. J Neurol Neurosurg Psychiatry 74:1581–1583
Bellastella A, Bizzarro A, Coronella C, et al (2003) Lymphocytic hypophysitis: a rare or underestimated disease? Eur J Endocrinol 149:363–376
Selch MT, DeSalles AA, Kelly DF, et al (2003) Stereotactic radiotherapy for the treatment of lymphocytic hypophysitis. Report of two cases. J Neurosurg 99:591–596
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Abe, T. Lymphocytic infundibulo-neurohypophysitis and infundibulo-panhypophysitis regarded as lymphocytic hypophysitis variant. Brain Tumor Pathol 25, 59–66 (2008). https://doi.org/10.1007/s10014-008-0234-8
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DOI: https://doi.org/10.1007/s10014-008-0234-8