121/640. Spontaneous thrombosis of a giant internal carotid aneurysm in a patient who presented with hypopituitarism. This case report describes a 66-year-old woman who initially presented with features of hypopituitarism secondary to a giant intra-cavernous internal carotid aneurysm. She represented a year later with features suggestive of a subarachnoid haemorrhage, but repeat CT showed no change from the one performed previously. A repeat angiogram, however, showed complete spontaneous thrombosis of the aneurysm including the parent artery. ( info) |
122/640. Treatment of lymphocytic hypophysitis by high-dose methylprednisolone pulse therapy. A 26-year-old woman 3 months post-partum was admitted to our hospital suffering from gross visual disturbance. magnetic resonance imaging (MRI) revealed a pituitary mass, extending into the suprasellar cistern, with intense gadolinium enhancement. Lymphocytic hypophysitis (LHy) was suspected, and the patient received high dose methylprednisolone pulse therapy (HDMPT). Her visual disturbance was dramatically ameliorated on the first day following initiation of HDMPT, and MRI revealed marked mass reduction. Her pituitary function recovered 6 months after therapy. This case report suggests that HDMPT proved effective for mass reduction of severe LHy and could obviate the need for a useless surgery. ( info) |
| A 29-year-old man presented with diplopia and decreased strength in the left arm. A magnetic resonance image (MRI) showed an extensive hypothalamic and pituitary gland mass, and hormonal studies showed partial hypopituitarism and mild hyperprolactinemia without diabetes insipidus. Biopsies of the hypothalamic lesion and of a mediastinal lymph node demonstrated noncaseating granulomas, and a Kveim-Siltzbach test was positive. He was successfully treated with a regimen of high-dose intravenous methylprednisolone pulse therapy for eight weeks along with a low dose of oral corticosteroids which was maintained indefinitely. An MRI obtained immediately after pulse therapy revealed a substantial reduction in the hypothalamic-pituitary mass, which was maintained in an MRI performed 3 years later. However, hormonal deficits persisted and indefinite hormonal substitutive therapy was required. ( info) |
| Increasing survival after treatment for childhood cancer in recent years has left many patients with long-term sequelae. Following cranial irradiation, changes in hypothalamic-pituitary function evolve over several years and multiple hormone deficiencies are frequently found. In the present study we describe a boy whose initial presentation with a cerebral tumour included central precocious puberty. He was followed for more than 15 years and sequentially developed deficiencies of growth hormone, thyroid-stimulating hormone, gonadotrophins and adrenocorticotropic hormone after high-dose cranial irradiation. Long-term endocrine follow up of such children is essential for the early diagnosis and optimal management of hormone deficiencies. ( info) |
125/640. Problems in differential diagnosis of non Langerhans cell histiocytosis with pituitary involvement: case report and review of literature. Differentiation among various non Langerhans cell histiocytoses granulomatous in adults is often difficult. patients, moreover, may not have endocrinologic abnormalities. A 53-yr-old patient was admitted owing to central diabetes insipidus and partial hypopituitarism. magnetic resonance imaging revealed a space-occupying lesion near the hypophyseal stalk, along with diffuse signal uptake in the cerebellar region. Laboratory chemistry showed monoclonal gammopathy of IgGkappa, and hormone tests disclosed insufficiency in the gonadotropic and somatotropic axes. The clinical picture was marked by multiple cutaneous xanthogranulomas, ataxic gait with blurred speech, compatible with pseudobulbar pontocerebellar symptomatology. Stereotactic pituitary biopsy was histologically classified as nonspecific granulomatous disease. Supplemental biopsies taken from the cutaneous periorbital xanthogranulomas were histologically and immunohistochemically consistent with non Langerhans cell histiocytosis. Systemic cortisone treatment as well as local radiotherapy to the pituitary lesion with a total of 18.0 gy had no impact on the progression of disease-growing tumor and progressing neurologic symptoms. Systemic granulomatoses cannot always be classified according to specific defined diseases. Differential diagnosis in the current patient should include the possibility of erdheim-chester disease, necrobiotic xanthogranuloma, and adult disseminated xanthoma. ( info) |
126/640. Neuroendocrinological aspects of primary empty sella. Neuroendocrinological aspects of 42 patients (33 women, 9 men) with primary empty sella confirmed by CT, cysternography and/or MR imaging were analyzed. The prominent symptoms were headache, visual disturbances and hypertension, occurring primarily in obese women (84.5%). patients underwent dynamic endocrine testing consisting of insulin-induced hypoglycemia and anterior pituitary stimulation tests GnRH and TRH. Variable degree of pituitary dysfunction was observed in 28 (66.6%) patients. In this study 20 (47.6%) patients were presented with latent hypopituitarism, while manifest hypopituitarism, requiring replacement therapy, occurred in 8 (19%) patients. Mild hyperprolactinaemia was found in 3 patients. Even 14 (33.3%) patients had no evidence of endocrine dysfunction. Often mentioned diabetes insipidus and rhinoliquorrhea were not reported in this study. ( info) |
127/640. Sheehan syndrome: a splinter of the mind. A 40-year-old woman presented with headache and diplopia after hypotension from postpartum hemorrhage. A noncontrasted cranial magnetic resonance imaging (MRI) showed an enlarged pituitary with a rim of slight increased signal. A repeat gadolinium-enhanced cranial MRI showed peripheral enhancement of the pituitary gland surrounding an isointense central area consistent with infarction of the pituitary and the clinical diagnosis of Sheehan syndrome. The patient was treated with intravenous hydrocortisone. Immediately after treatment, her symptoms remitted and the examination normalized. One month later, a gadolinium-enhanced cranial MRI was normal. The characteristic appearance of the post-gadolinium enhanced cranial MRI helped confirm the diagnosis of Sheehan syndrome and facilitate early treatment with corticosteroids. ( info) |
| A growth-retarded, mentally deficient, young man is described with diminished secretory response of growth hormone, thyrotropin, and prolactin to the pharmacologic stimuli of insulin, arginine, chlorpromazine, and thyrotropin-releasing hormone. Gonadotropin and ACTH functions were normal both basically and upon pharmacologic stimulation. Additionally, the patient was unresponsive to exogenous thyrotropin injections. These data suggest that the hypothyroidism in this patient was due to combined thyroid dysgenesis and pituitary insufficiency, i.e., primary and secondary hypothyroidism. ( info) |
129/640. Panhypopituitarism induced by cholesterol granuloma in the sellar region--case report. A 67-year-old man with generalized fatigue and weight loss developed hyponatremia. Endocrinologic examination demonstrated panhypopituitarism. magnetic resonance imaging showed a pituitary mass extending slightly to the suprasellar region. Transsphenoidal resection of the tumor was performed. Histological examination found exclusively granulomatous tissue with cholesterol clefts, and no epithelial component. This cholesterol granuloma may be classified as xanthogranuloma of the sellar region. ( info) |
130/640. Growth without growth hormone: growth pattern and final height of five patients with idiopathic combined pituitary hormone deficiency. BACKGROUND: Growth without GH has been reported in patients with organic combined pituitary hormone deficiency (CPHD) after resection of craniopharyngiomas and hypothalamic tumours or in septo-optic dysplasia. methods: This study describes the growth pattern and final height of five children (four boys, one girl) with idiopathic CPHD (GH, TSH, ACTH, LH and FSH) who maintained normal growth despite persistent GH deficiency throughout the growth period. RESULTS: Presenting findings were borderline small penis in two children diagnosed at ages 3 and 9 years, and absence of pubertal signs in three adolescents diagnosed at age 12.8-13.7 years. The latter three patients also exhibited acromegaloid features. The height of all patients was within the 10-25th percentiles, and weight at the 25-50th percentiles. Although they were moderately overweight, accelerated weight gain was not observed. Prepubertal growth rate was 4-5 cm/year. The pubertal growth period, starting after initiation of sex hormone therapy (chronological age 15.9-16.3 years and bone age 12.5-14.5 years) continued for 4-5.5 years. Total pubertal growth was 6-11.7 cm with reduced growth spurt. Final height, which was attained at an advanced age (19-22 years), was 170-179 cm in the boys and 164 cm in the girl, equal to or exceeding the target height range. Repeated hormonal evaluations revealed undetectable GH and IGF-I levels, and no evidence of hyperprolactinaemia or hyperinsulinism. CONCLUSIONS: Final height attainment within or above target height range may occur in patients with idiopathic CPHD despite persistent GHD. As this was not mediated by GH, IGF-I, insulin or prolactin, some other growth factors probably played a growth-promoting role. ( info) |