SAT-244 Inflammatory Rathke Cleft Cyst Caused Hypothalamic Panhypopituitarism

Tangiku Mariko, Tatsuo Ishizuka, Kei Fujioka, Saori Inui, Hideki Tani, Takako Maruyama, Kouji Yamada
2020 Journal of the Endocrine Society  
Background: Hypothalamic panhypopituitarism was a rare disease which was caused by brain tumors such as Rathke cyst, germinoma, malignant lymphoma and craniopharyngioma, and vascular accidents such as thrombosis and hemorrhage. We have found a rare case of hypothalamic panhypopituitarism occurred by the inflammation of Rathke cyst. Case presentation: A 77-year-old woman was admitted to our hospital because of severe headache, nausea, vomiting and fever. Hyponatremia (Na 131 meq/L) and increased
more » ... CRP level (0.20 mg/dl) and no finding of gastroenterological examination and abdominal CT scanning were observed. Endocrinological examination revealed low levels of anterior pituitary hormones such as GH, LH, FSH and ACTH and increased PRL levels and low level of serum cortisol. Simultaneous CRH, TRH and LH-RH stimulations tests indicated low responses of TSH, LH, FSH and PRL and hyperesponse of ACTH (from 2.4 to 251 pg/ml). Radiological examination by enhanced MRI revealed high intensity of T2-weighted images and low and partial high intensities of T1-weighted images in swelling of pituitary gland and vanishment of high intensity of posterior lobe in T2-weighted images suggesting that the inflammatory wall of Rathke cyst. We searched previous data of ACTH and cortisol levels which showed normal level of ACTH (55.2 pg/ml) and cortisol 18.8 μ;g/dl) when headache and nausea arouse on 50 days before. Based on above results patient was diagnosed as hypothalamic hypopituitarism due to inflammatory Rathke cyst. Therefore, patient was treated with hormone replacement therapy by 20 mg of hydrocortisone. After these treatment, polyuria, urinary low osmotic pressure and hypernatremia appeared due to partial diabetes insipidus caused by the impairment of posterior lobe in pituitary gland, and diagnosed by the examination of vasopressin test. This patient was also treated by the oral desmopressin acetate hydrate (60 μ;g/day), and has been well-tolerated in the outpatient clinic. Conclusion: These results suggested that inflammatory Rathke cyst with inflammatory changes in hypothlamo-pituitary system raised a rare case of hypothalamic panhypopituitarism.Reference: Nishioka H. et al.: Clin Endocrinology (Oxf) 2006:64:184–188
doi:10.1210/jendso/bvaa046.187 fatcat:gxrqva5g7nf5towo4wkcl6tazy