{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,29]],"date-time":"2026-01-29T22:13:18Z","timestamp":1769724798122,"version":"3.49.0"},"reference-count":30,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"24","license":[{"start":{"date-parts":[[2025,12,15]],"date-time":"2025-12-15T00:00:00Z","timestamp":1765756800000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Neurosurg Case Lessons"],"published-print":{"date-parts":[[2025,12,15]]},"abstract":"\n BACKGROUND<\/jats:title>\n Lymphocytic panhypophysitis (LPH) is a rare inflammatory disorder that primarily affects the pituitary gland. Extension into the cavernous sinus has been described, but clival involvement is exceedingly rare. To date, no cases of isolated clival extension have been documented. Anti-rabphilin-3A antibodies have been identified as diagnostic markers that may facilitate the diagnosis of this challenging condition.<\/jats:p>\n <\/jats:sec>\n \n OBSERVATIONS<\/jats:title>\n An 81-year-old man presented with headache, anorexia, and fatigue. Evaluation demonstrated panhypopituitarism followed by central diabetes insipidus. MRI revealed homogeneous pituitary enlargement with isolated clival extension. Endoscopic evaluation during transsphenoidal biopsy demonstrated thickened dura mater extending from the pituitary gland to the clivus. Histopathological analysis revealed lymphoplasmacytic infiltration with a predominance of CD20-positive B cells over CD3-positive T cells and inflammatory infiltration between the clival bone trabeculae, confirming direct invasion. The serum anti-rabphilin-3A antibody test was positive. Hormone replacement therapy resulted in clinical improvement, and follow-up MRI at 3 and 6 months showed marked resolution.<\/jats:p>\n <\/jats:sec>\n \n LESSONS<\/jats:title>\n This appears to be the first reported case of LPH with isolated clival extension. Intraoperative dural thickening and histological evidence of trabecular bone infiltration suggest a potential pathway for extrapituitary extension. Anti-rabphilin-3A antibody testing can support diagnosis, particularly in such atypical presentations.<\/jats:p>\n \n https:\/\/thejns.org\/doi\/10.3171\/CASE25737<\/jats:ext-link>\n <\/jats:p>\n <\/jats:sec>","DOI":"10.3171\/case25737","type":"journal-article","created":{"date-parts":[[2026,1,22]],"date-time":"2026-01-22T11:59:55Z","timestamp":1769083195000},"source":"Crossref","is-referenced-by-count":0,"title":["Lymphocytic panhypophysitis with clival extension and positive anti-rabphilin-3A antibodies: illustrative case"],"prefix":"10.3171","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9270-2688","authenticated-orcid":true,"given":"Takenori","family":"Kato","sequence":"first","affiliation":[{"name":"Department of Neurosurgery, Komaki City Hospital, Komaki, Aichi"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiroshi","family":"Ochiai","sequence":"additional","affiliation":[{"name":"Department of Endocrinology and Diabetes, Komaki City Hospital, Komaki, Aichi"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masayoshi","family":"Hasegawa","sequence":"additional","affiliation":[{"name":"Department of Pathology, Komaki City Hospital, Komaki, Aichi"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-2151-0966","authenticated-orcid":true,"given":"Naoko","family":"Iwata","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Diabetes and Metabolism, School of Medicine, Fujita Health University, Toyoake, Aichi"},{"name":"Department of Endocrinology and Diabetes, Daido Hospital, Nagoya, Aichi, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0009-2941-9968","authenticated-orcid":true,"given":"Haruki","family":"Fujisawa","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Diabetes and Metabolism, School of Medicine, Fujita Health University, Toyoake, Aichi"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0807-199X","authenticated-orcid":true,"given":"Atsushi","family":"Suzuki","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Diabetes and Metabolism, School of Medicine, Fujita Health University, Toyoake, Aichi"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoshihisa","family":"Sugimura","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Diabetes and Metabolism, School of Medicine, Fujita Health University, Toyoake, Aichi"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Toshinori","family":"Hasegawa","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Komaki City Hospital, Komaki, Aichi"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1754","reference":[{"issue":"1","key":"bib1","series-title":"J Clin Endocrinol Metab","first-page":"10","article-title":"Hypophysitis, the growing spectrum of a rare pituitary disease","volume":"107","author":"Langlois","year":"2022","unstructured":"Langlois FVarlamov EVFleseriu M.Hypophysitis, the growing spectrum of a rare pituitary disease. J Clin Endocrinol Metab. 2022;107(1):10-28."},{"key":"bib2","series-title":"Endocrinol Diabetes Metab Case Rep","first-page":"18-0081","article-title":"Spontaneous pregnancy after full recovery from hypopituitarism caused by lymphocytic hypophysitis","volume":"2018","author":"Funazaki","year":"2018","unstructured":"Funazaki SYamada HHara KIshikawa SE.Spontaneous pregnancy after full recovery from hypopituitarism caused by lymphocytic hypophysitis. Endocrinol Diabetes Metab Case Rep. 2018;2018:18-0081."},{"issue":"7","key":"bib3","series-title":"J Clin Endocrinol Metab","first-page":"E946","article-title":"Rabphilin-3A as a targeted autoantigen in lymphocytic infundibulo-neurohypophysitis","volume":"100","author":"Iwama","year":"2015","unstructured":"Iwama SSugimura YKiyota ARabphilin-3A as a targeted autoantigen in lymphocytic infundibulo-neurohypophysitis. J Clin Endocrinol Metab. 2015;100(7):E946-E954."},{"issue":"4","key":"bib4","series-title":"Endocr J","first-page":"373","article-title":"Diagnosis and treatment of autoimmune and IgG4-related hypophysitis: clinical guidelines of the Japan Endocrine Society","volume":"67","author":"Takagi","year":"2020","unstructured":"Takagi HIwama SSugimura YDiagnosis and treatment of autoimmune and IgG4-related hypophysitis: clinical guidelines of the Japan Endocrine Society. Endocr J. 2020;67(4):373-378."},{"issue":"5","key":"bib5","series-title":"Clin Radiol","first-page":"325","article-title":"The imaging of immunotherapy-related hypophysitis and other pituitary lesions in oncology patients","volume":"76","author":"Lasocki","year":"2021","unstructured":"Lasocki AIravani AGalligan A.The imaging of immunotherapy-related hypophysitis and other pituitary lesions in oncology patients. Clin Radiol. 2021;76(5):325-332."},{"key":"bib6","series-title":"BMJ Case Rep","first-page":"bcr2015213912","article-title":"Granulomatosis with polyangiitis affecting the skull base and manifesting as spontaneous skull base osteomyelitis","volume":"2016","author":"Harrison","year":"2016","unstructured":"Harrison LMcnally JCorbridge R.Granulomatosis with polyangiitis affecting the skull base and manifesting as spontaneous skull base osteomyelitis. BMJ Case Rep. 2016;2016:bcr2015213912."},{"issue":"5","key":"bib7","series-title":"Pituitary","first-page":"722","article-title":"A diagnostic pitfall in IgG4-related hypophysitis: infiltration of IgG4-positive cells in the pituitary of granulomatosis with polyangiitis","volume":"18","author":"Bando","year":"2015","unstructured":"Bando HIguchi GFukuoka HA diagnostic pitfall in IgG4-related hypophysitis: infiltration of IgG4-positive cells in the pituitary of granulomatosis with polyangiitis. Pituitary. 2015;18(5):722-730."},{"issue":"11","key":"bib8","series-title":"J Neurol Neurosurg Psychiatry","first-page":"1581","article-title":"Lymphocytic hypophysitis successfully treated with azathioprine: first case report","volume":"74","author":"Lecube","year":"2003","unstructured":"Lecube AFrancisco GRodr\u00edguez DLymphocytic hypophysitis successfully treated with azathioprine: first case report. J Neurol Neurosurg Psychiatry. 2003;74(11):1581-1583."},{"issue":"6","key":"bib9","series-title":"Surg Neurol","first-page":"472","article-title":"Lymphocytic hypophysitis: report of a case in a man with cavernous sinus involvement","volume":"37","author":"Supler","year":"1992","unstructured":"Supler MLMickle JP.Lymphocytic hypophysitis: report of a case in a man with cavernous sinus involvement. 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Sci Rep. 2022;12(1):4440."},{"issue":"4","key":"bib12","series-title":"Hormones (Athens)","first-page":"747","article-title":"Absence of anti\u2013rabphilin-3A antibodies in children and young adults with idiopathic central diabetes insipidus: a potential clue to elucidating a tumor etiology","volume":"22","author":"Fujisawa","year":"2023","unstructured":"Fujisawa HTakeuchi TIshii AAbsence of anti\u2013rabphilin-3A antibodies in children and young adults with idiopathic central diabetes insipidus: a potential clue to elucidating a tumor etiology. Hormones (Athens). 2023;22(4):747-758."},{"issue":"7","key":"bib13","series-title":"Endocr J","first-page":"703","article-title":"Two children with lymphocytic hypophysitis presenting with positive anti-rabphilin-3A antibody","volume":"70","author":"Murai","year":"2023","unstructured":"Murai AShinojima NIkuta GTwo children with lymphocytic hypophysitis presenting with positive anti-rabphilin-3A antibody. Endocr J. 2023;70(7):703-709."},{"issue":"4","key":"bib14","series-title":"J Pathol","first-page":"469","article-title":"Critical role of rabphilin-3A in the pathophysiology of experimental lymphocytic neurohypophysitis","volume":"244","author":"Yasuda","year":"2018","unstructured":"Yasuda YIwama SKiyota ACritical role of rabphilin-3A in the pathophysiology of experimental lymphocytic neurohypophysitis. J Pathol. 2018;244(4):469-478."},{"issue":"2","key":"bib15","series-title":"Pituitary","first-page":"321","article-title":"Lymphocytic panhypophysitis and anti-rabphilin-3A antibody with pulmonary sarcoidosis","volume":"25","author":"Takahashi","year":"2022","unstructured":"Takahashi YKameda HMiya ALymphocytic panhypophysitis and anti-rabphilin-3A antibody with pulmonary sarcoidosis. Pituitary. 2022;25(2):321-327."},{"issue":"1","key":"bib1","series-title":"J Clin Endocrinol Metab","first-page":"10","article-title":"Hypophysitis, the growing spectrum of a rare pituitary disease","volume":"107","author":"Langlois","year":"2022","unstructured":"Langlois FVarlamov EVFleseriu M.Hypophysitis, the growing spectrum of a rare pituitary disease. J Clin Endocrinol Metab. 2022;107(1):10-28."},{"key":"bib2","series-title":"Endocrinol Diabetes Metab Case Rep","first-page":"18-0081","article-title":"Spontaneous pregnancy after full recovery from hypopituitarism caused by lymphocytic hypophysitis","volume":"2018","author":"Funazaki","year":"2018","unstructured":"Funazaki SYamada HHara KIshikawa SE.Spontaneous pregnancy after full recovery from hypopituitarism caused by lymphocytic hypophysitis. Endocrinol Diabetes Metab Case Rep. 2018;2018:18-0081."},{"issue":"7","key":"bib3","series-title":"J Clin Endocrinol Metab","first-page":"E946","article-title":"Rabphilin-3A as a targeted autoantigen in lymphocytic infundibulo-neurohypophysitis","volume":"100","author":"Iwama","year":"2015","unstructured":"Iwama SSugimura YKiyota ARabphilin-3A as a targeted autoantigen in lymphocytic infundibulo-neurohypophysitis. J Clin Endocrinol Metab. 2015;100(7):E946-E954."},{"issue":"4","key":"bib4","series-title":"Endocr J","first-page":"373","article-title":"Diagnosis and treatment of autoimmune and IgG4-related hypophysitis: clinical guidelines of the Japan Endocrine Society","volume":"67","author":"Takagi","year":"2020","unstructured":"Takagi HIwama SSugimura YDiagnosis and treatment of autoimmune and IgG4-related hypophysitis: clinical guidelines of the Japan Endocrine Society. Endocr J. 2020;67(4):373-378."},{"issue":"5","key":"bib5","series-title":"Clin Radiol","first-page":"325","article-title":"The imaging of immunotherapy-related hypophysitis and other pituitary lesions in oncology patients","volume":"76","author":"Lasocki","year":"2021","unstructured":"Lasocki AIravani AGalligan A.The imaging of immunotherapy-related hypophysitis and other pituitary lesions in oncology patients. Clin Radiol. 2021;76(5):325-332."},{"key":"bib6","series-title":"BMJ Case Rep","first-page":"bcr2015213912","article-title":"Granulomatosis with polyangiitis affecting the skull base and manifesting as spontaneous skull base osteomyelitis","volume":"2016","author":"Harrison","year":"2016","unstructured":"Harrison LMcnally JCorbridge R.Granulomatosis with polyangiitis affecting the skull base and manifesting as spontaneous skull base osteomyelitis. BMJ Case Rep. 2016;2016:bcr2015213912."},{"issue":"5","key":"bib7","series-title":"Pituitary","first-page":"722","article-title":"A diagnostic pitfall in IgG4-related hypophysitis: infiltration of IgG4-positive cells in the pituitary of granulomatosis with polyangiitis","volume":"18","author":"Bando","year":"2015","unstructured":"Bando HIguchi GFukuoka HA diagnostic pitfall in IgG4-related hypophysitis: infiltration of IgG4-positive cells in the pituitary of granulomatosis with polyangiitis. Pituitary. 2015;18(5):722-730."},{"issue":"11","key":"bib8","series-title":"J Neurol Neurosurg Psychiatry","first-page":"1581","article-title":"Lymphocytic hypophysitis successfully treated with azathioprine: first case report","volume":"74","author":"Lecube","year":"2003","unstructured":"Lecube AFrancisco GRodr\u00edguez DLymphocytic hypophysitis successfully treated with azathioprine: first case report. J Neurol Neurosurg Psychiatry. 2003;74(11):1581-1583."},{"issue":"6","key":"bib9","series-title":"Surg Neurol","first-page":"472","article-title":"Lymphocytic hypophysitis: report of a case in a man with cavernous sinus involvement","volume":"37","author":"Supler","year":"1992","unstructured":"Supler MLMickle JP.Lymphocytic hypophysitis: report of a case in a man with cavernous sinus involvement. Surg Neurol. 1992;37(6):472-476."},{"issue":"1","key":"bib10","series-title":"Pituitary","first-page":"75","article-title":"Lymphocytic panhypophysitis in a young man with involvement of the cavernous sinus and clivus","volume":"10","author":"Kartal","year":"2007","unstructured":"Kartal IYarman STanakol RBilgic B.Lymphocytic panhypophysitis in a young man with involvement of the cavernous sinus and clivus. Pituitary. 2007;10(1):75-80."},{"issue":"1","key":"bib11","series-title":"Sci Rep","first-page":"4440","article-title":"Studies on anti-rabphilin-3A antibodies in 15 consecutive patients presenting with central diabetes insipidus at a single referral center","volume":"12","author":"Arihara","year":"2022","unstructured":"Arihara ZSakurai KNiitsuma SStudies on anti-rabphilin-3A antibodies in 15 consecutive patients presenting with central diabetes insipidus at a single referral center. Sci Rep. 2022;12(1):4440."},{"issue":"4","key":"bib12","series-title":"Hormones (Athens)","first-page":"747","article-title":"Absence of anti\u2013rabphilin-3A antibodies in children and young adults with idiopathic central diabetes insipidus: a potential clue to elucidating a tumor etiology","volume":"22","author":"Fujisawa","year":"2023","unstructured":"Fujisawa HTakeuchi TIshii AAbsence of anti\u2013rabphilin-3A antibodies in children and young adults with idiopathic central diabetes insipidus: a potential clue to elucidating a tumor etiology. Hormones (Athens). 2023;22(4):747-758."},{"issue":"7","key":"bib13","series-title":"Endocr J","first-page":"703","article-title":"Two children with lymphocytic hypophysitis presenting with positive anti-rabphilin-3A antibody","volume":"70","author":"Murai","year":"2023","unstructured":"Murai AShinojima NIkuta GTwo children with lymphocytic hypophysitis presenting with positive anti-rabphilin-3A antibody. Endocr J. 2023;70(7):703-709."},{"issue":"4","key":"bib14","series-title":"J Pathol","first-page":"469","article-title":"Critical role of rabphilin-3A in the pathophysiology of experimental lymphocytic neurohypophysitis","volume":"244","author":"Yasuda","year":"2018","unstructured":"Yasuda YIwama SKiyota ACritical role of rabphilin-3A in the pathophysiology of experimental lymphocytic neurohypophysitis. J Pathol. 2018;244(4):469-478."},{"issue":"2","key":"bib15","series-title":"Pituitary","first-page":"321","article-title":"Lymphocytic panhypophysitis and anti-rabphilin-3A antibody with pulmonary sarcoidosis","volume":"25","author":"Takahashi","year":"2022","unstructured":"Takahashi YKameda HMiya ALymphocytic panhypophysitis and anti-rabphilin-3A antibody with pulmonary sarcoidosis. 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