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A Case of Hypothalamic Panhypopituitarism with Empty Sella Syndrome: Case Report and Review of the Literature

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A Case of Hypothalamic Panhypopituitarism with Empty Sella Syndrome: Case Report and Review of the Literature Endocrine Journal 2009, 56 (4), 585-589 A Case of Hypothalamic Panhypopituitarism with Empty Sella Syndrome: Case Report and Review of the Literature HISAKO KOMADA*, MASAAKI YAMAMOTO*, SAKI OKUBO*, KANTO NAGAI*, KEIJI IIDA*, TAKEHIRO NAKAMURA**, YUSHI HIROTA*, KAZUHIKO SAKAGUCHI*, MASATO KASUGA* AND YUTAKA TAKAHASHI* *Division of Diabetes, Metabolism, and Endocrinology, Department of Internal Medicine, Kobe University Graduate school of Medicine, Kobe, Japan **Kobe City Medical Center West Hospital, Kobe, Japan Abstract. Empty sella syndrome is frequently accompanied with pituitary dysfunction. Most of the patients with empty sella syndrome demonstrate primary pituitary or stalk dysfunction and few cases show hypothalamic dysfunction. A 71- year-old man manifested appetite loss, nausea and vomiting with hyponatremia and adrenal insufficiency. Hormonal evaluation and cranial MRI revealed a panhypopituitarism with empty sella. Intriguingly, while the response of ACTH to CRH administration was exaggerated, the response to insulin hypoglycemia was blunted. Serum PRL levels were normal. Further, decreased level of fT4, slightly elevated basal levels of TSH, and delayed response of TSH to TRH administration were observed. These findings strongly suggest that the panhypopituitarism is caused by hypothalamic dysfunction. The presence of autoantibodies to pituitary and cerebrum in the patient’s serum implies an autoimmune mechanism as a pathogenesis. Key words: Empty sella, Hypothalamic, Panhypopituitarism, Adrenal insufficiency, Autoimmune (Endocrine Journal 56: 585-589, 2009) EMPTY sella is characterized by the herniation of the lymphocytic hypophysitis [1-2]. subarachnoid space within the sella, which is often as- It has been reported that empty sella is present in sociated with some degree of flattening of the pitu- 5.5%-23% of autopsies [3]. In most of the patients, itary gland [1-2] and first documented by Busch et al. empty sella is found incidentally. It has also been re- in 1951 [3]. These conditions are classified into two ported that 20-50% of patients have the endocrino- groups, primary or secondary empty sella. In case of logic dysfunction [4]; partial hypopituitarism has been primary empty sella, several pathogenesis has been described in 5% of patients, total hypopituitarism in proposed, such as a congenital incomplete formation 25% [5] and hyperprolactinemia in 10% [6]. The most of the sellar diaphragm and an increase in intracrani- frequent cause of the endocrinological dysfunction al pressure. The secondary empty sella is caused by is the primary pituitary dysfunction. Several studies various pathological conditions including pituitary ad- have reported primary empty sella in children with hy- enomas, injuries, infection, Sheehan’s syndrome and popituitarism due to the hypothalamic insufficiency [7, 8], however; only one case of empty sella syndrome with hypothalamic hypopituitarism in adulthood has Received Aug. 7, 2008; Accepted Mar. 12, 2009 as K08E-214 been reported as far as we have searched [9]. Here we Released online in J-STAGE as advance publication Apr. 7, 2009 present a case of panhypopituitarism with empty sella Correspondence to: Yutaka Takahashi M.D., Ph.D., Division of syndrome in which hypothalamic dysfunction was Diabetes, Metabolism, and Endocrinology, Department of Internal Medicine, Kobe University Graduate school of Medicine 7-5-1, strongly speculated with a review of the literature. Kusunoki-cho, Chuo-ku, Kobe, 650-0017, Japan. E-mail: [email protected] 586 KOMADA et al. Table 1. Laboratory data on admission Table 2. Endocrinological examinations <Hematology> <Serological examination> A) CRH/GHRH/TRH/LHRH test WBC 10800/μl CRP 0.97 mg/dl Time (min) 0 30 60 90 120 RBC 328×104/μl Anti-TPO Ab 0.3 U/ml ACTH (pg/ml) 8.4 96.8 97. 7 72.4 90 Cortisol (μg/dl) 1.2 2.3 3.2 3.3 3.2 Hb 9.8g/dl Anti-TgAb 0.9 U/ml TSH (μIU/ml) 1.937 4.587 6.56 6.886 7.007 Ht 28.4% PRL (ng/ml) 8.3 12.5 12.1 11 10.6 4 PLT 31.9×10 /μl GH (ng/ml) <0.15 1.43 2.24 1.57 1.02 LH (mIU/ml) 0.2 0.9 1.2 1.3 1.3 FSH (mIU/ml) 3.1 3.7 4.1 4.2 4.4 <Biochemistry> <Hormonal examination> CRH 100μg, GHRH 50μg, TRH 200μg, and LHRH 100μg iv. T-bil 0.5 mg/dl GH <0.15 ng/ml B) Insulin tolerance test AST 26 IU/l IGF-1 40 ng/ml Time (min) 0 30 60 90 120 ALT 21 IU/l PRL 9.0 ng/ml Glucose (mg/dl) 83 33 54 68 77 GH (ng/ml) <0.15 <0.15 0.15 <0.15 <0.15 LDH 156 IU/l TSH 1.864 μU/ml ACTH (pg/ml) 0.5 0.4 0.9 0.9 0.9 CK 103 IU/l FT4 0.71 ng/dl Cortisol (μg/dl) 9.6 9.7 13.4 10.7 10.3 TP 7.7 g/dl LH 0.3 mIU/ml insulin 0.05U/kg BW iv. Alb 4.4 g/dl FSH 3.0 mIU/ml C) GHRP-2 test Cr 0.96 mg/dl ACTH 8.1 pg/ml Time (min) 0 15 30 45 60 BUN 21 mg/dl Cortisol 2.2 μg/dl GH (ng/ml) <0.15 0.78 1.12 1.01 0.71 Na 129 mEq/l PAC 114 pg/ml GHRP-2 100μg iv. K 4.2 mEq/l PRA 0.3 ng/ml/hr D) 5% hypertonic saline test Cl 94 mEq/l ADH 1.14 pg/ml Time (min) 0 30 60 90 120 P-Osm (mOsm/kg) 266 269 272 275 277 FPG 81 mg/dl U-17-OHCS 2.8 mg/day U-Osm (nOsm/kg) 379 359 308 292 304 HbA1c 5.7% U-17-KS 3.4 mg/day Na (mEq/l) 128 132 132 135 137 P-Osm 266 mOsm/kg U-free cortisol <10.5 μg/day ADH (pg/ml) 0.5 0.65 0.6 0.88 0.87 Materials and Methods a history of herpes encephalitis when he was 60-year- old. Routine laboratory tests showed a marked hy- Detection for anti-pituitary antibody was performed ponatremia (112mEq/l (normal range, 135-149mEq/ in two methods. One was a commercially available l)). Hormonal evaluation revealed decreased lev- immunofluorescence using rat pituitary (SRL). The el of cortisol (<1.0μg/dl (normal range, 2.7-15.5μg/ other was immunoblotting analysis using mouse tis- dl)), low normal level of ACTH (11pg/ml (9-52pg/ sues. Immunoblotting analysis was performed as pre- ml)), decreased level of free T4 (0.54ng/dl (0.7-1.7ng/ viously described [10]. Briefly, 20μg protein of each dl)) and slightly elevated level of TSH (7.78μIU/ml tissue from mice was applied for SDS page. The pa- (0.05-5.00μIU/ml)). The patient was given a diagno- tient’s or control serum was used for primary antibody sis of panhypopituitarism and treated with hydrocor- in 1:500 dilutions. HRP-conjugated goat Anti-Human tisone replacement followed by levothyroxine sodium IgG (Zymed) was used for secondary antibody. replacement. After the treatment, the patient’s symp- Antibody binding was detected using an enhanced toms rapidly improved. For further evaluation, the pa- chemiluminescence kit (Amersham). tient was referred to our hospital. On the admission day, routine laboratory tests showed hyponatremia (129mEq/l), decreased serum osmolarity and mild Case report anemia. Hormonal evaluation under levothyroxine re- placement showed decreased cortisol, ACTH, fT4 and A 71-year-old man, 162.7cm in height and 63.4kg IGF-I levels. The basal levels of TSH and PRL were in weight, was admitted to a hospital because of ap- in the normal range. Urine 17-OHCS, KS and free petite loss, nausea and vomiting. The patient had lost cortisol excretion were decreased (Table 1). The bas- approximately 5kg in weight over 3 months. He had al ACTH concentrations ranged from 0.5-8.4 pg/ml, HYPOTHALAMIC DYSFUNCTION IN EMPTY SELLA 587 (A) (B) Fig. 1. MR image of the pituitary. Sagittal and (B) coronal T1-weighted MR image demonstrate a presence of empty sella. and peak concentrations after insulin-induced hypo- CRH administration was exaggerated, the response to glycemia and CRH administration were 0.9 and 97.7, insulin hypoglycemia was blunted. These results indi- respectively (Table 2). The response of TSH to TRH cate a presence of hypothalamic or pituitary stalk dys- administration was normal but delayed. The basal function. Generally, hyperprolactinemia is frequently GH concentrations were <0.15 ng/ml, and peak con- observed in empty sella syndrome with stalk dysfunc- centrations after insulin-induced hypoglycemia and tion [6]. In this case, the normal basal level of PRL GHRP-2 administration were 0.15 and 1.12, respec- and normal response of PRL to TRH administration tively. A pituitary magnetic resonance imaging (MRI) suggest that stalk function is intact. Further, low con- demonstrated empty sella (Fig 1). In T1WI image, centration of fT4 and slightly elevated levels of TSH, high signal intensity of the posterior pituitary was de- and delayed response of TSH to TRH administration tected. He showed polyuria (more than 4000ml/day) suggest hypothalamic dysfunction or TRH deficiency at the admission to our hospital. His urine and plasma [11-13]. In addition, polyuria and limited elevation osmolality were 588mOsm/kg and 266mOsm/kg, re- of urinary osmolality during hypersaline test suggest spectively. In the 5% hypertonic saline test, plasma a presence of partial central diabetes insipidus. Taken sodium concentration maintained low (128~137mEq/ together, it is strongly suggested that the panhypopitu- l). Although, his urine osmolality was higher than that itarism is caused by hypothalamic dysfunction. in plasma, the degree of urinary concentration was Although, several studies have reported hypotha- not enough and plasma ADH levels kept in low lev- lamic insufficiency with empty sella in children [7, els (Table 2).
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