Hypothalamitis

A diagnostic and therapeutic challenge

Antonio Bianchi, Marilda Mormando, Francesco Doglietto, Linda Tartaglione, Serena Piacentini, Libero Lauriola, Giulio Maira, Laura De Marinis

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

To report an unusual case of biopsy-proven autoimmune hypophysitis with predominant hypothalamic involvement associated with empty sella, panhypopituitarism, visual disturbances and antipituitary antibodies positivity. We present the history, physical findings, hormonal assay results, imaging, surgical findings and pathology at presentation, together with a 2-year follow-up. A literature review on the hypothalamic involvement of autoimmune hypophysitis with empty sella was performed. A 48-year-old woman presented with polyuria, polydipsia, asthenia, diarrhea and vomiting. The magnetic resonance imaging (MRI) revealed a clear suprasellar (hypothalamic) mass, while the pituitary gland appeared atrophic. Hormonal testing showed panhypopituitarism and hyperprolactinemia; visual field examination was normal. Pituitary serum antibodies were positive. Two months later an MRI documented a mild increase of the lesion. The patient underwent biopsy of the lesion via a transsphenoidal approach. Histological diagnosis was lymphocytic "hypothalamitis". Despite 6 months of corticosteroid therapy, the patient developed bitemporal hemianopia and blurred vision, without radiological evidence of chiasm compression, suggesting autoimmune optic neuritis with uveitis. Immunosuppressive treatment with azathioprine was then instituted. Two months later, an MRI documented a striking reduction of the hypothalamic lesion and visual field examination showed a significant improvement. The lesion is stable at the 2-year follow-up. For the first time we demonstrated that "hypothalamitis" might be the possible evolution of an autoimmune hypophysitis, resulting in pituitary atrophy, secondary empty sella and panhypopituitarism. Although steroid treatment is advisable as a first line therapy, immunosuppressive therapy with azathioprine might be necessary to achieve disease control.

Original languageEnglish
Pages (from-to)197-202
Number of pages6
JournalPituitary
Volume17
Issue number3
DOIs
Publication statusPublished - 2014

Fingerprint

Magnetic Resonance Imaging
Azathioprine
Immunosuppressive Agents
Visual Fields
Polydipsia
Hemianopsia
Biopsy
Therapeutics
Asthenia
Polyuria
Optic Neuritis
Surgical Pathology
Hyperprolactinemia
Antibodies
Uveitis
Pituitary Gland
Atrophy
Vomiting
Diarrhea
Adrenal Cortex Hormones

Keywords

  • Antipituitary autoantibodies
  • Autoimmune hypophysitis
  • Empty sella
  • Hypothalamitis

ASJC Scopus subject areas

  • Endocrinology
  • Endocrinology, Diabetes and Metabolism
  • Medicine(all)

Cite this

Bianchi, A., Mormando, M., Doglietto, F., Tartaglione, L., Piacentini, S., Lauriola, L., ... De Marinis, L. (2014). Hypothalamitis: A diagnostic and therapeutic challenge. Pituitary, 17(3), 197-202. https://doi.org/10.1007/s11102-013-0487-z

Hypothalamitis : A diagnostic and therapeutic challenge. / Bianchi, Antonio; Mormando, Marilda; Doglietto, Francesco; Tartaglione, Linda; Piacentini, Serena; Lauriola, Libero; Maira, Giulio; De Marinis, Laura.

In: Pituitary, Vol. 17, No. 3, 2014, p. 197-202.

Research output: Contribution to journalArticle

Bianchi, A, Mormando, M, Doglietto, F, Tartaglione, L, Piacentini, S, Lauriola, L, Maira, G & De Marinis, L 2014, 'Hypothalamitis: A diagnostic and therapeutic challenge', Pituitary, vol. 17, no. 3, pp. 197-202. https://doi.org/10.1007/s11102-013-0487-z
Bianchi A, Mormando M, Doglietto F, Tartaglione L, Piacentini S, Lauriola L et al. Hypothalamitis: A diagnostic and therapeutic challenge. Pituitary. 2014;17(3):197-202. https://doi.org/10.1007/s11102-013-0487-z
Bianchi, Antonio ; Mormando, Marilda ; Doglietto, Francesco ; Tartaglione, Linda ; Piacentini, Serena ; Lauriola, Libero ; Maira, Giulio ; De Marinis, Laura. / Hypothalamitis : A diagnostic and therapeutic challenge. In: Pituitary. 2014 ; Vol. 17, No. 3. pp. 197-202.
@article{f65186eede184f2389aafea60c2b9440,
title = "Hypothalamitis: A diagnostic and therapeutic challenge",
abstract = "To report an unusual case of biopsy-proven autoimmune hypophysitis with predominant hypothalamic involvement associated with empty sella, panhypopituitarism, visual disturbances and antipituitary antibodies positivity. We present the history, physical findings, hormonal assay results, imaging, surgical findings and pathology at presentation, together with a 2-year follow-up. A literature review on the hypothalamic involvement of autoimmune hypophysitis with empty sella was performed. A 48-year-old woman presented with polyuria, polydipsia, asthenia, diarrhea and vomiting. The magnetic resonance imaging (MRI) revealed a clear suprasellar (hypothalamic) mass, while the pituitary gland appeared atrophic. Hormonal testing showed panhypopituitarism and hyperprolactinemia; visual field examination was normal. Pituitary serum antibodies were positive. Two months later an MRI documented a mild increase of the lesion. The patient underwent biopsy of the lesion via a transsphenoidal approach. Histological diagnosis was lymphocytic {"}hypothalamitis{"}. Despite 6 months of corticosteroid therapy, the patient developed bitemporal hemianopia and blurred vision, without radiological evidence of chiasm compression, suggesting autoimmune optic neuritis with uveitis. Immunosuppressive treatment with azathioprine was then instituted. Two months later, an MRI documented a striking reduction of the hypothalamic lesion and visual field examination showed a significant improvement. The lesion is stable at the 2-year follow-up. For the first time we demonstrated that {"}hypothalamitis{"} might be the possible evolution of an autoimmune hypophysitis, resulting in pituitary atrophy, secondary empty sella and panhypopituitarism. Although steroid treatment is advisable as a first line therapy, immunosuppressive therapy with azathioprine might be necessary to achieve disease control.",
keywords = "Antipituitary autoantibodies, Autoimmune hypophysitis, Empty sella, Hypothalamitis",
author = "Antonio Bianchi and Marilda Mormando and Francesco Doglietto and Linda Tartaglione and Serena Piacentini and Libero Lauriola and Giulio Maira and {De Marinis}, Laura",
year = "2014",
doi = "10.1007/s11102-013-0487-z",
language = "English",
volume = "17",
pages = "197--202",
journal = "Pituitary",
issn = "1386-341X",
publisher = "Kluwer Academic Publishers",
number = "3",

}

TY - JOUR

T1 - Hypothalamitis

T2 - A diagnostic and therapeutic challenge

AU - Bianchi, Antonio

AU - Mormando, Marilda

AU - Doglietto, Francesco

AU - Tartaglione, Linda

AU - Piacentini, Serena

AU - Lauriola, Libero

AU - Maira, Giulio

AU - De Marinis, Laura

PY - 2014

Y1 - 2014

N2 - To report an unusual case of biopsy-proven autoimmune hypophysitis with predominant hypothalamic involvement associated with empty sella, panhypopituitarism, visual disturbances and antipituitary antibodies positivity. We present the history, physical findings, hormonal assay results, imaging, surgical findings and pathology at presentation, together with a 2-year follow-up. A literature review on the hypothalamic involvement of autoimmune hypophysitis with empty sella was performed. A 48-year-old woman presented with polyuria, polydipsia, asthenia, diarrhea and vomiting. The magnetic resonance imaging (MRI) revealed a clear suprasellar (hypothalamic) mass, while the pituitary gland appeared atrophic. Hormonal testing showed panhypopituitarism and hyperprolactinemia; visual field examination was normal. Pituitary serum antibodies were positive. Two months later an MRI documented a mild increase of the lesion. The patient underwent biopsy of the lesion via a transsphenoidal approach. Histological diagnosis was lymphocytic "hypothalamitis". Despite 6 months of corticosteroid therapy, the patient developed bitemporal hemianopia and blurred vision, without radiological evidence of chiasm compression, suggesting autoimmune optic neuritis with uveitis. Immunosuppressive treatment with azathioprine was then instituted. Two months later, an MRI documented a striking reduction of the hypothalamic lesion and visual field examination showed a significant improvement. The lesion is stable at the 2-year follow-up. For the first time we demonstrated that "hypothalamitis" might be the possible evolution of an autoimmune hypophysitis, resulting in pituitary atrophy, secondary empty sella and panhypopituitarism. Although steroid treatment is advisable as a first line therapy, immunosuppressive therapy with azathioprine might be necessary to achieve disease control.

AB - To report an unusual case of biopsy-proven autoimmune hypophysitis with predominant hypothalamic involvement associated with empty sella, panhypopituitarism, visual disturbances and antipituitary antibodies positivity. We present the history, physical findings, hormonal assay results, imaging, surgical findings and pathology at presentation, together with a 2-year follow-up. A literature review on the hypothalamic involvement of autoimmune hypophysitis with empty sella was performed. A 48-year-old woman presented with polyuria, polydipsia, asthenia, diarrhea and vomiting. The magnetic resonance imaging (MRI) revealed a clear suprasellar (hypothalamic) mass, while the pituitary gland appeared atrophic. Hormonal testing showed panhypopituitarism and hyperprolactinemia; visual field examination was normal. Pituitary serum antibodies were positive. Two months later an MRI documented a mild increase of the lesion. The patient underwent biopsy of the lesion via a transsphenoidal approach. Histological diagnosis was lymphocytic "hypothalamitis". Despite 6 months of corticosteroid therapy, the patient developed bitemporal hemianopia and blurred vision, without radiological evidence of chiasm compression, suggesting autoimmune optic neuritis with uveitis. Immunosuppressive treatment with azathioprine was then instituted. Two months later, an MRI documented a striking reduction of the hypothalamic lesion and visual field examination showed a significant improvement. The lesion is stable at the 2-year follow-up. For the first time we demonstrated that "hypothalamitis" might be the possible evolution of an autoimmune hypophysitis, resulting in pituitary atrophy, secondary empty sella and panhypopituitarism. Although steroid treatment is advisable as a first line therapy, immunosuppressive therapy with azathioprine might be necessary to achieve disease control.

KW - Antipituitary autoantibodies

KW - Autoimmune hypophysitis

KW - Empty sella

KW - Hypothalamitis

UR - http://www.scopus.com/inward/record.url?scp=84905693825&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84905693825&partnerID=8YFLogxK

U2 - 10.1007/s11102-013-0487-z

DO - 10.1007/s11102-013-0487-z

M3 - Article

VL - 17

SP - 197

EP - 202

JO - Pituitary

JF - Pituitary

SN - 1386-341X

IS - 3

ER -