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At least 487 records · Page 27Linked to original sources
PubMed1981

[Acquired post-traumatic and idiopathic hypothalamic panhypopituitarism in adults. Three cases (author's transl)].

Three cases of hypothalamic panhypopituitarism in male adults are reported. Two of them, without antidiuretic hormone deficiency, succeeded to a closed head injury. The third one was associated with impaired posterior pituitary function and presumably was of idiopathic origin, since radiological and ophthalmological investigations gave normal results, although the disease seemed to have begun some 20 years before.

Adult↗
PubMed1981

Precocious puberty and hypothalamic hamartoma.

A 3-year-old boy with true precocious puberty and hamartoma of the hypothalamus is described. Preliminary diagnosis was established by the presence of unusually advanced puberty and skeletal age without other evidence of central nervous system dysfunction, elevated blood gonadotropin and testosterone concentrations, and positive computed tomography scans. Pneumoencephalography further delineated the tumor. Hypothalamic-pituitary-gonadal and adrenal functions were studied. Release of gonadotropins after the injection of synthetic luteinizing hormone-releasing hormone and the suppression of luteinizing hormone and of testosterone by oral fluoxymesterone were comparable to maximal responses of normal men. The diagnosis was confirmed by histologic and electron microscopic study of an excised portion of the tumor. Immunofluorescence studies indicated the presence of luteinizing hormone-releasing hormone in the hamartoma and radioimmunoassays detected thyroid-stimulating hormone-releasing hormone and somatostatin. The hamartoma resembles an autonomously functioning accessory hypothalamus. The ectopic neuroendocrine pathways, however, are responsive to hormonal stimulation and feedback. The clinical, endocrinologic, and pathologic features of published cases of precocious puberty and hypothalamic hamartoma are reviewed. Advanced or rapidly progressive true precocious puberty in the very young with elevated concentrations of blood gonadotropins and gonadal steroids and positive pneumoencephalography appear to be characteristic.

Child, Preschool↗
PubMed1995

[The efficacy of granisetron as a prophylactic anti-emetic agent used in conjunction with MCNU and VP16 chemotherapeutic regimens in the management of a pediatric case of hypothalamic anaplastic astrocytoma].

The management of a 12-year-old boy with hypothalamic anaplastic astrocytoma is reported herein. The pediatric patient underwent a subtotal removal of his brain tumor, and subsequently received multimodal treatment comprising radiotherapy (30.6 Gy to the whole brain and 50.4 Gy to the local lesion) and immunochemotherapy with MCNU (80 mg/m2, day 1), VP 16 (50 mg/day, days 2 to 6), and interferon-beta (3 x 10(6) units/day, initiating on day 2 for 4 weeks). The immunochemotherapy was given as of 4 weeks after radiotherapy. The administration of anti-cancer drugs caused severe nausea and vomiting, the intra-carotid infusion of MCNU being particularly emetogenic. The multi-modal treatment was then discontinued for a few weeks. A partial response was obtained, and the patient subsequently received 3 courses of maintenance therapy at six-month intervals using the same protocol as for the initial immunochemotherapy. During maintenance therapy, granisetron (40 micrograms/kg) was given intravenously 30 minutes before and one day after the injection of MCNU. Owing to the anti-emetic management of this patient, there were no complications throughout the chemotherapy. The patient has now survived for more than 3 years with a good quality of life, showing a Karnofsky performance score of 90.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1994

Autosomal dominant transmission of Pallister-Hall syndrome.

A mother and daughter with Pallister-Hall syndrome are described. This documented parent-to-child transmission supports the hypothesis of autosomal dominant inheritance of this condition. The mother survived childhood and became pregnant without exogenous endocrine supplements, demonstrating that the gene manifests variable expressivity.

Adult↗
PubMed1993

[Precocious puberty revealing Bourneville tuberous sclerosis].

BACKGROUND: Benign tumors are seen in tuberous sclerosis. They are found in many organs, and the precocious puberty due to hypothalamic hamartoma and tuberous sclerosis has been reported. However, precocious puberty exceptionally reveals the tuberous sclerosis. CASE REPORT: A 2 month-old boy was admitted because of the fortuitous discovery of polycystic renal disease. Precocious puberty developed at 13 months with enlargement of the penis and testes, appearance of pubic hair, acne and deepening of the voice. Linear growth was recently accelerated and the bone maturation was advanced. Plasma testosterone was elevated (460 ng/100 ml) and LH-RH injection induced rapid rises in plasma LH (2.6 to 28 mUl/ml) and FSH (2 to 8 mUl/ml). Brain imaging techniques (CT scan and NMR) showed a hypothalamic hamartoma and periventricular calcified lesions. Examination with the Wood lamp identified two white leaf macules in the dorsal area. Administration of an analogue of LH-RH effectively reduced the manifestations of precocious puberty. CONCLUSION: Tuberous sclerosis is exceptionally revealed by precocious puberty. The association of polycystic disease and precocious puberty has never before been reported in tuberous sclerosis.

Hamartoma↗
PubMed1993

The therapy for hypothalamic-pituitary tumors.

Pituitary-hypothalamic tumors may profoundly affect endocrine functions. Although these are generally rare tumors of the central nervous system, they prominently figure into the differential diagnosis of children and adolescents with disorders of growth or puberty.

Adolescent↗
PubMedDate not supplied

MR in idiopathic central diabetes insipidus of childhood.

We present the cases of two children with presumed idiopathic central diabetes insipidus whose follow-up MR studies eventually revealed hypothalamic tumors. Thin-section sagittal T1-weighted MR with gadolinium administration is important in the evaluation of these children, and serial examinations are probably necessary.

Child↗
PubMed1993

Hypothalamic and pituitary pathology.

This article details the intricate anatomy and elegant physiology of the hypothalamic-pituitary axis. The discussion is meant to confer an understanding of the principles of neuroendocrinology appropriate for the radiologist interpreting imaging studies of this region. The clinical presentations of the diseases that occur in this area of the central nervous system are unique and therefore are discussed in some detail. The radiologic features of the diseases affecting the hypothalamic-pituitary axis are described and correlated with the relevant pathology.

Adenoma↗
PubMedDate not supplied

[Hypothalamic astrocytoma presenting as obesity with hypogonadism].

We report herein a case of a 30 year old male patient who consulted for a long standing obesity since childhood. Clinical examination reveals the presence of hypogonadism and gynecomastia without galactorrhea. Endocrine assessment showed an hypogonadotrophic hypogonadism with a moderate insufficiency of the adrenocortical axis. Neuro-ophthalmological examination disclosed an inferior bitemporal quadranopsia. MRI showed an extra-axial suprasellar mass, lightly hypointense in T1 and hyperintense in T2. After craniotomy and conservative biopsy, pathological examination of the tumor showed regular cells surrounded with collagen and reticulin marked with immunoperoxidase against S100 protein and GFAP (glial fibrinolytic acidic protein) strongly suggesting the diagnosis of juvenile pilocytic astrocytoma.

Adult↗
PubMed1993

[A case of small cell lung cancer associated with diabetes insipidus and Cushing's syndrome].

A 62-year-old male with small cell lung cancer (SCLC) associated with Cushing's syndrome and diabetes insipidus (DI) is reported. The patient was referred to our hospital for treatment of SCLC. A diagnosis of paraneoplastic Cushing's syndrome was made on the basis of an elevated serum ACTH (623.5 pg/ml) level, elevated excretion of urinary 17-OHCS (18.01 mg/day), obesity, hypertension, hyperglycemia, persistent hypokalemia, alkalosis, and no history of diabetes mellitus. He was also diagnosed as having DI based on polyuria and polydipsia, low specific gravity of the urine (1.007-1.010), low serum ADH (1.4 pg/ml) level, normal plasma osmolarity (29 mOsm/kg H2O), and the results of water deprivation test. DI and a left visual field defect was suggestive of metastasis to the pituitary region, but no lesion was detected by either CT scan or MRI scan. The patient failed to show a good response to intensive chemotherapy, and died of the tumor five months after commencing chemotherapy. Post-mortem examination revealed metastases to the hypothalamic-neurohypophyseal region, lungs, liver, adrenal glands, bone, bone marrow, and hilar and mediastinal lymph nodes.

Carcinoma, Small Cell↗
PubMed1993

Neurogenic diabetes insipidus: an unusual clinical presentation of small cell lung cancer.

A 61 year old man presented with chronic bronchitis, which he had been suffering from for many years, and reported the recent onset of polyuria with polydipsia. A chest X-ray showed a peripheral pulmonary nodule in the right lower lobe, that was diagnosed as small cell lung cancer following histological examination of the pulmonary tissue specimen, obtained with transbronchial biopsy. After establishing the central origin of the diabetes insipidus, its cause was revealed by magnetic resonance imaging of the brain, that demonstrated metastasis to the hypothalamic-neurohypophyseal area.

Carcinoma, Small Cell↗
PubMedDate not supplied

ACTH and TSH producing ectopic suprasellar pituitary adenoma of the hypothalamic region: case report.

A 34-year-old woman with a long-term history of amenorrhoea, headache and visual disturbances was operated for a hypothalamic tumor which could be completely removed. Postoperatively the patient developed a transient SIADH-syndrome and deep vein thrombosis; otherwise the clinical course was uneventful. There has been no sign of tumor recurrence at a follow-up period of fifteen months. Histological examination of the tumor revealed an ectopic pituitary adenoma with production of ACTH and TSH shown by immunohistochemistry.

Adenoma↗