Primary pituitary abscess followed by empty sella syndrome in an adolescent girl

Primary pituitary abscess is a rare pituitary pathology, particularly at a young age and is characterized by atypical clinical features making the diagnosis difficult. Correct diagnosis and therapy are mandatory due to the potentially lethal outcome of pituitary infection. We report the case of an adolescent girl presenting with headache, diabetes insipidus and central thyro-gonadic insufficiency with no history of infection, in whom the intra-operative diagnosis of primary pituitary abscess was made. Bacterial cultures indicated infection with Streptococcus spp. One year after neurosurgery and antibiotic therapy, recovery of diabetes insipidus and pituitary insufficiency was documented except for persistence of subnormal growth hormone secretion. Post-surgery, pituitary magnetic resonance imaging revealed an empty sella syndrome.     

Thyrotropin and prolactin pituitary reserve in the "empty sella syndrome".

Ten patients (8 women, 2 men) with the "empty sella syndrome" were studied to evaluate the pituitary reserve of human thyrotropin (hTSH) and prolactin (hPRL). None of the patients had signs or symptoms of hypopituitarism or primary hypothyroidism. All patients had normal baseline thyroid function tests except for 2 patients with mild elevations in total triiodothyronine as measured by competitive protein displacement assay (T3D). Eight of ten patients had normal hTSH responses to thyrotropin releasing hormone (TRH), whereas the 2 patients with elevated T3D had blunted TRH responses. In the 4 patients studied, the metabolic clearance (MCR) and production rates (PR) of hTSH were normal. In 9 of 10 patients normal baseline serum hPRL levels were detected, and each responded to TRH. In one case serum hPRL was undetectable and failed to respond to TRH. The assessment of other anterior pituitary function revealed few minor abnormalities. In summary, like other anterior pituitary hormones, the pituitary reserve in "empty sella syndrome" of hTSH and hPRL is usually normal. When abnormalities do occur, they are attributable to other co-existent endocrine pathology.     

对成年女性空泡蝶鞍患者垂体前叶激素储备功能的研究

随着影像学技术的不断进步 ,发现空泡蝶鞍患者的机率增大。 1981年Ｂｒｉｓｍａｒ报告在 48名空泡蝶鞍患者中 ,垂体功能低下或高催乳素血症占 31%〔1〕。及早发现空泡蝶鞍患者有垂体前叶分泌功能异常 ,并及时给予治疗 ,有益于改善这些患者的生活质量。一、对象和方法1.对象 :男女正常人各 4名 ,年龄 (34 .6± 3.2 )岁 (30～39岁 )。女性空泡蝶鞍患者 9人 ,年龄 (34 .8± 6 .8)岁 (2 8～43岁 ) ,既往无其他疾病 ,体格检查正常 ,血生化及三大常规检查无异常 ,其中一人因视物不清 ,另 8人因头痛进行头颅或鞍区ＭＲＩ检查时发现空泡蝶鞍 ,其在冠…     

Gonadotropin-releasing hormone-induced partial empty sella clinically mimicking pituitary apoplexy in a woman with a suspected non-secreting macroadenoma

Pituitary apoplexy has been reported as a rare complication of dynamic testing used for the study of pituitary functional reserve. In 1993, a diagnosis of non-secreting macroadenoma with moderate functional hyperprolactinaemia was made in a 43-year-old woman. Soon after the start of therapy with bromocriptine up to 5 mg/die, the patient complained of nausea and postural hypotension. As the symptoms persisted even when the dose was reduced to 2.5 mg/die, the patient was transferred to therapy with quinagolide at the dosage of 37.5 microg/die. PRL levels quickly normalized (range 1.4-5.7 ng/ml) as well as menstrual cycles, and no side-effect was reported. In 1995 a sellar magnetic resonance imaging (MRI) showed no shrinkage of the known macroadenoma. In 1996, few hours after a gonadotropin-releasing-hormone (GnRH) test, which showed normal LH and FSH response and with baseline PRL levels in the normal range, the patient started complaining of severe frontal headache, nausea and vomiting. No gross visual defects were present. An emergency computed tomography (CT) showed no evident hemorrhagic infarction in the macroadenoma. The symptoms completely resolved in few days with steroidal and antiemetic therapy. A new MRI performed in 1998 showed a partial empty sella and PRL levels were in the normal range under dopaminergic treatment. The pituitary functional reserve proved normal on dynamic testing. The temporal association between the onset of symptoms and the GnRH test strongly suggests an association between the two events. No evident signs of pituitary apoplexy (either on emergency CT or hormonal evaluation) were detected. The authors suggest that GnRH can cause severe side-effects that mimic pituitary apoplexy without related morphological evidence and that, in our particular case, it can have caused the gradual disappearance of the non-secreting macroadenoma. Moreover, a causal role of the chronic dopaminergic treatment cannot be completely ruled out.     

Serum antibodies to human pituitary membrane antigens in patients with autoimmune lymphocytic hypophysitis and infundibuloneurohypophysitis

OBJECTIVE: Serum antipituitary antibodies were investigated by the immunoblotting method using human anterior pituitary membrane preparation as the antigen. PATIENTS: Thirteen patients with autoimmune lymphocytic hypophysitis, two patients with infundibuloneurohypophysitis, four patients with isolated ACTH deficiency, 21 patients with diabetes mellitus (type 1 and type 2) and 38 healthy subjects were studied. METHODS: Human pituitary membrane antigens were electrophoresed by sodium dodecylsulphate-polyacrimide gel electrophoresis (SDS-PAGE). The antigens were transferred to polyvinylidene difluoride membrane and reacted with the sera followed by incubation with biotinylated anti-human IgG goat serum. RESULTS: Serum antibodies to 68, 49, 43 kD human pituitary membrane antigens were detected in five of 13, one of 12 patients with infundibuloneurohypophysitis and none of four patients with isolated ACTH deficiency. These antibodies were not detectable when human thyroid, liver or rat pituitary preparations were used as the antigen. CONCLUSION: These findings suggest that serum antibodies to 68, 49, 43 kD human anterior pituitary antigen are specific but not so frequently detected in autoimmune lymphocytic hypophysitis.     

A case of autoimmune hypophysitis associated with Graves' disease

In a 76-year-old woman with hyperthyroidism, hyperprolactinemia and thickening of the pituitary stalk on magnetic resonance imaging (MRI) was presented. Thyroid stimulating antibody (TSAb) was positive and anti-pituitary antibodies against 49 and 68 kD human anterior pituitary membrane antigens were detected immunologically. Secretion of pituitary hormones was almost normal except for suppressed TSH and hyperprolactinemia. As autoimmune etiologies were likely involved in the disorders, autoimmune hypophysitis associated with Graves' disease was arrived at as the plausible diagnosis.     

The spontaneous course of pituitary adenomas and occurrence of an empty sella in untreated acromegaly

Twenty-three untreated patients with acromegaly for 6-46 yr who were followed for 2-13 yr were studied. High resolution computed tomographic scans in 11 patients revealed herniation of the subarachnoidal space into the pituitary fossa (empty sella) and an intrasellar cyst in 2. Suprasellar tumor extension developed during the follow-up period in only 1 patient. Eleven patients previously (in 10 patients more than 4 yr earlier) had been treated with bromocriptine; 6 of these patients at follow-up had a solid intrasellar adenoma. Six of the patients with an empty sella or intrasellar cyst had had symptoms suggesting pituitary apoplexy. The sellar volume did not increase during follow-up in any patient with an empty sella. Plasma GH levels (basal and mean concentrations during an oral glucose load) increased (greater than 50 ng/ml) in 3 and decreased in 3 patients during follow-up. Normalization of plasma GH levels was rare, and physical well-being with complete absence of acromegalic symptoms was reported by only 4 patients. These results demonstrate that GH-secreting pituitary tumors may remain stationary for extended periods of time and that complete or partial disappearance occurs frequently, probably as a result of pituitary adenoma infarction.     

Presence of anti-pituitary hormone antibodies in patients with empty sella syndrome and pituitary tumours*

OBJECTIVE We evaluated the presence of anti-pituitary hormone autoantibodies (APHA) in patients with primary empty sella syndrome and pituitary tumours and examined the correlation of positive antibodies with the hormonal deficiencies. DESIGN Case-control, retrospective study. PATIENTS Eleven patients were identified with primary empty sella syndrome or a pituitary tumour by magnetic resonance imaging or computed tomography scanning. Six healthy, normal subjects without evidence of a pituitary problem served as the control group. MEASUREMENTS Anti-pituitary hormone autoantibodies against purified pituitary hormones were measured in ail subjects utilizing an immunoblotting technique. All patients with pituitary disease had their medical records reviewed for any hormonal evaluation. RESULTS All of the normal subjects were negative for antipituitary hormone antibodies. Forty-five per cent of patients with pituitary disease (pituitary tumours or primary empty sella syndrome) had positive antipituitary hormone antibodies. Of the five patients with positive antipituitary hormone antibodies, anti-ACTH antibodies were the most common (5/5) followed by anti-TSH and anti-GH antibodies (2/5 for each). The hormonal deficiencies failed to correspond with the antipituitary hormone antibodies. Anti-ACTH antibody had a sensitivity of 50% with a specificity of 56%. The anti-TSH antibody yielded a sensitivity of 67% with a specificity of 100%. The anti-FSH/ LH antibody reported a 0% sensitivity. CONCLUSIONS Detection of antipituitary hormone antibody was unable to discriminate between empty sella syndrome and pituitary tumours. The presence of these antipituitary hormone antibodies were neither specific for, nor predictive of, the endocrine deficiencies.     

Primary empty sella: a histologic and immunocytologic study.

A primary "empty" sella turcica was found incidentally at autopsy, and the anterior lobe of the pituitary gland was studied with the immunoperoxidase technique. All five adenohypophysial cell types, ie, somatotrophs, lactotrophs, corticotrophs, thyrotrophs, and gonadotrophs (containing follicle-stimulating hormone [FSH] and luteinizing hormone [LH], were present in adequate numbers and were well granulated, indicating normal hormone storage.     

Diagnosis and treatment of autoimmune hypophysitis: a short review

Medical therapy of autoimmune hypophysitis with immunosuppressive drugs can be effective to induce remission of the disease by treating both pituitary dysfunction and compression symptoms. We describe the case of a 41-yr-old man with autoimmune hypophysitis in whom prednisone therapy induced remission of the disease but was followed by a sudden relapse after withdrawal. A second trial of corticosteroid was started and succeeded in inducing remission of the disease. Eight months after the second withdrawal pituitary function was restored, pituitary mass had disappeared, only partial diabetes insipidus remained unchanged. Review of the literature identified 30 articles, among case reports and case series, reporting a total of 44 cases of autoimmune hypophysitis treated with glucocorticoids and/or azathioprine. Combining all the cases, medical therapy resulted to be effective in reducing the pituitary mass in 84%, in improving anterior pituitary function in 45%, and in restoring posterior pituitary function in 41%. Clinical aspects of autoimmune hypophysitis are discussed and a possible algorithm for the diagnosis and treatment of the disease is proposed.     

Primary empty sella (PES): a review of 175 cases

The term primary empty sella (PES) makes reference to the herniation of the subarachnoid space within the sella turcica in patients with no history of pituitary tumor, surgery or radiotherapy. To retrospectively assess clinical features, radiological findings and the biochemical endocrine function from the records of 175 patients with a diagnosis of PES. One hundred seventy-five patients (150 females) were studied. The mean age at diagnosis was 48.2 ± 14 year. Most diagnoses were made by magnetic resonance imaging (n = 172). In most patients, the pituitary function was assessed by basal pituitary hormones measurements. Pituitary scans were ordered for different reasons: headache (33.1 %), endocrine disorders (30.6 %), neurological symptoms (12.5 %), visual disturbances (8.75 %), abnormalities on sella turcica radiograph (8.75 %) and others (6.25 %). Multiple pregnancies were observed in 58.3 % of women; headaches, obesity, and hypertension were found in 59.4, 49.5, and 27.3 % of the studied population, respectively. Mild hyperprolactinemia (<50 ng/ml) was present in 11.6 % of women and 17.3 % of men. Twenty-eight percent of our patients had some degree of hypopituitarism. In the male population, hypopituitarism represented 64 % of cases, whereas it accounted for 22 % of all females. PES seems to be more commonly found in middle-aged women, with a history of multiple pregnancies. In most patients, PES was discovered as an incidental finding on imaging studies, while in almost a quarter of patients PES was found during the diagnostic evaluation of anterior pituitary deficiency, which was more common in men.     

Primary "empty sella" in adults: endocrine findings

Increasing evidence of impaired pituitary function in many subjects with primary empty sella (PES) has been reported. We conducted a retrospective analysis of our patients with PES, in order to ascertain presenting symptoms and endocrine status on diagnosis and during follow-up. Magnetic resonance imaging (MRI) of the pituitary leading to the diagnosis of PES was performed in 8 patients (5 F and 3 M, age: 60.1 +/- 3.3 years, M +/- SE; group 1) after the diagnosis of global anterior hypopituitarism (H), and in 20 patients (F, age 56.9 +/- 2.2 years, group 2) for other clinical reasons. Baseline determinations of pituitary and target gland hormones and of IGF-I were performed. GH response to GHRH plus arginine stimulation was also evaluated. Ten age- and BMI-matched subjects (7 F, 3 M, age: 53.0 +/- 4.0 years) with normal pituitary function served as controls (C). In group 1, the presenting symptoms leading to the diagnosis of H were consciousness disturbances, hyponatremia and chronic fatigue. The GH response to stimulation was absent (peak:1.0 +/- 0.3 ng/ml) and IGF-I levels (60.1 +/- 9.3 ng/ml) were significantly lower (p<0.001) than in C and group 2 PES patients. Among group 2 PES patients, the main presenting symptoms were headache and visual alterations. Baseline hormone levels proved normal in 17 subjects, while slight hyperprolactinemia was observed in 2 and hypogonadotropic hypogonadism in one. The GH response to stimulation (12.9 +/- 3.4 ng/ml) and IGF-I levels (141.7 +/- 12.0 ng/ml) were lower (p<0.05) than in C (GH: 33.4 +/- 8.8 ng/ml, IGF-I: 193.1 +/- 20.3 ng/ml). PES is a heterogeneous condition that ranges from hypopituitarism to various degrees of isolated GH deficiency, and which needs careful endocrine assessment, treatment and follow-up.     

Corticosterone secreting adrenal carcinoma and empty sella turcica: a case report.

Pituitary adenoma was suspected in a woman of 74 with hypercorticism,hypokalaemic alkalosis and radiographically enlarged sella turcica. However, non-suppressibility of steroid excretion by high-dose dexamethasone and low plasma concentration of ACTH suggested adrenal tumour. Detailed analysis of urinary steroid excretions demonstrated unusually large amounts of corticosterone metabolites, 14.6 mg/24 h compared to a mean normal value of 0.5 mg. The basal levels of the remaining pituitary hormones were unremarkable. The patient died incidentally before a planned adrenalectomy. The autopsy disclosed an adrenal carcinoma and an empty sella turcica. The enlarged pituitary fossa was lined by a narrow rim of histological normal pituitary tissue.     

Experimental autoimmune cholangitis: a mouse model of immune-mediated cholangiopathy

BACKGROUND: Primary biliary cirrhosis (PBC) is characterised by intra-hepatic immune-mediated cholangiopathy (non-suppurative destructive cholangitis (NSDC)). Although auto-reactive immune responses against pyruvate dehydrogenase complex (PDC) have been characterised in PBC, the lack of an animal model of the disease has limited study of the mechanisms of disease induction and the development of novel approaches to therapy. AIMS: To develop and validate a mouse model of immune-mediated cholangiopathy relevant for future use in the study of the aetio-pathogenesis and therapy of PBC. METHODS: Female SJL/J, C57BL/6, NOD and BALB/c mice were sensitised with PDC, its purified E2/E3BP component, and a PDC-E2 derived peptide p163 (a dominant T-cell epitope in humans) in complete Freund's adjuvant (CFA). Morphological changes were assessed under light microscopy by a hepatic histopathologist blinded to the experimental details. Antibody responses to PDC were studied by ELISA and PDC inhibition assay. RESULTS: An initial series of experiments was performed to survey the susceptibility of female mice of a range of strains to the induction of NSDC by i.p. sensitisation with PDC, PDC-E2/E3BP or p163 in CFA. Although each animal showed a specific antibody response following sensitisation, it was found that NSDC development (assessed at 30 weeks post-sensitisation) was restricted to SJL/J mice following sensitisation with any of the mitochondrial antigen preparations. A subsequent series of experiments was performed to examine the specificity and aetiology of this disease. Significant bile duct lesions were only seen in SJL/J animals following sensitisation with CFA containing PDC, and were absent from CFA only and un-sensitised controls. Kinetic analysis revealed that this pathology developed slowly, but a high incidence of animals with severe lesions was observed after 30 weeks. CONCLUSIONS: We have described a model of experimental autoimmune cholangitis (EAC) with immunological (anti-PDC antibodies) and histological (immune-mediated cholangiopathy) features suggestive of PBC. This model may be useful in further defining the role of self-tolerance breakdown in the development of this condition.