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At least 127 records · Page 7Linked to original sources
PubMed1993

Low bone mineral density in adults with previous hypothalamic-pituitary tumors: correlations with serum growth hormone responses to GH-releasing hormone, insulin-like growth factor I, and IGF binding protein 3.

The adverse consequences of growth hormone (GH) deficiency (GHD) on bone growth in children is well described. Whether adult GHD is associated with bone loss is unknown. We evaluated 14 patients with hypothalamic-pituitary tumors (HPT) acquired during adulthood (5 men, 9 women; mean age = 48.1 +/- 4.6 years; mean BMI = 28.8 +/- 1.7) and 14 age-, sex-, and weight-matched controls. Nine HPT patients were receiving gonadal steroid replacement therapy for a mean of 11 years. All subjects had basal IGF-I and IGFBP-3 levels prior to testing with GH-releasing hormone [1 microgram/kg IV bolus; responses expressed as maximum percentage increase above baseline (PERGH)]. Bone mineral density (BMD) of the spine, hip, and total body were measured by dual-energy X-ray absorptiometry. Mean BMD Z-scores of the HPT patients were significantly lower in the femoral neck, Ward's triangle, and trochanter than in controls (all P < 0.05). Mean total body BMD (g/cm2) was also lower in the patient group (1.04 +/- 0.03 versus 1.13 +/- 0.03, P < 0.05). For the subgroup of HPT patients receiving conventional gonadal steroids, mean BMD Z-scores of the lumbar spine also were significantly lower than controls (-1.84 +/- 0.43 versus -0.57 +/- 0.46, P < 0.05). PERGH and IGF-I were correlated with Z-scores of the femoral neck (r = 0.47, P < 0.01; r = 0.45, P < 0.01) and Ward's triangle (r = 0.47, P < 0.01; r = 0.41, P < 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Absorptiometry, Photon↗
PubMed1993

Germ cell tumor in the hypothalamo-neurohypophysial region: clinical features and treatment.

Twenty-one patients with germ cell tumors (17 germinomas and 4 teratomas) involving the hypothalamic-neurohypophysial (HN) region were reviewed retrospectively. Eleven patients were males and 10 females, and their ages ranged from 7 to 45 years (average 18.5 years). Diabetes insipidus was the initial and the most prominent symptom in most germinomas; in teratomas the most prominent symptom was visual disturbance. Fifteen patients with germinomas were treated by radiotherapy, and 4 with teratomas were treated by surgical resection alone. Two recent germinoma patients with extensive CSF dissemination were treated with systemic chemotherapy consisting of anticancer platinum drugs and etoposide, which resulted in a complete disappearance of the tumors. Patients with germinoma treated after the introduction of CT scanning had a greatly improved mortality rate, and their actual survival rate was 87.5% over 10 years. On the basis of this review, the authors consider that diagnosis at an early stage of the disease and chemotherapy, which can be an effective therapeutic alternative to radiation therapy, may improve not only the mortality rate but also the quality of life of patients with HN germ cell tumors.

Adolescent↗
PubMed1993

Insulin-like growth factor (IGF)-I, -II and IGF-binding proteins in the cyst fluid of a patient with astrocytoma.

Insulin-like growth factor (IGF)-I, -II and IGF-binding proteins (IGFBPs) were demonstrated in the cyst fluid of a patient with a hypothalamic astrocytoma. The astrocytoma cyst fluid was subjected to gel chromatography at low pH and the IGF-I and IGF-II levels were measured by specific radioimmunoassays. Immunoreactive IGF-I and IGF-II levels were 19 ng/ml and 78 ng/ml respectively. Several-fold higher IGF-II values were obtained when cyst fluid was not extracted or was extracted with acid ethanol before radioimmunoassay analysis. The immunoreactive IGFBP-1 concentration was 26 ng/ml. Sodium dodecyl sulphate-polyacrylamide gel electrophoresis (SDS-PAGE) and subsequent Western ligand blotting with [125I]IGF-II revealed bands at approximately 200, 34.5, 29.5, 24 and 21 kD as visualized by autoradiography. Binding studies demonstrated that these binding proteins bind specifically [125I]IGF-I and [125I]IGF-II. These observations suggest that IGFs as well as IGF-binding proteins are produced by astrocytoma cells and may act in a paracrine or autocrine fashion capable of modulating the growth of astrocytoma tumours.

Blotting, Western↗
PubMed1983

Hamartomas of the tuber cinereum.

The hamartoma of the tuber cinereum, a midline dysraphic syndrome, corresponds to a mass of normal neuronal tissue implanted in the area of the mamillary bodies. It has no evolutionary potential, but association to other cerebral malformations has been observed. On the basis of 18 personal cases and a review of the literature, the authors evaluate the actual diagnostic criteria and the incidence of different clinical signs such as precocious isosexual puberty, seizures, and mental impairment.

Adolescent↗
PubMed1988

The MR appearance of hypothalamic hamartoma.

Hypothalamic hamartoma is the most common detectable cerebral lesion causing precocious puberty. Two histologically confirmed cases were studied by computerized tomography (CT) and magnetic resonance (MR) imaging. T2 weighted, sagittal MR images were superior to CT in delineating the tumor from surrounding grey matter. The lesion was isointense to grey matter on T1 weighted images allowing exclusion of other hypothalamic tumors. MR will undoubtedly become the imaging modality of choice in the detection of hypothalamic hamartoma.

Child, Preschool↗
PubMed1987

Limbic lymphoma.

Two cases of metastatic malignant lymphoma confined to the limbic-hypothalamic region are presented. The non-specific nature of the neurological symptoms associated with these lesions such as memory loss, impotence and confusion made neurological localization of the disease process difficult. While non-contrast computed tomography (CT) was unremarkable, contrast CT was diagnostic, showing bilateral homogenous enhancement of specific limbic-hypothalamic structures without significant mass effect. Since lymphomatous involvement of the limbic-hypothalamic area tends to be infiltrative without attendant mass effect, detection of metastasis may be elusive unless special attention is directed to this region. As lymphoma is often chemoresponsive and highly radiosensitive, early recognition of limbic-hypothalamic involvement of this disease is important.

Hodgkin Disease↗
PubMed1987

The effect of cyproterone acetate on the growth of children with central precocious puberty.

We have examined the growth and skeletal maturation of 19 children (6 male, 13 female) with central precocious puberty. The aetiology in nine patients (5 male, 4 female) was secondary to a hypothalamic hamartoma. Six children (2 male, 4 female) received no treatment whereas 13 children (4 male, 9 female) were treated with cyproterone acetate in a mean dose of 68 mg/m2 per day (range, 34-260) for a mean duration of 4.5 years (range, 0.8-7.9). There was no significant difference between height SDS for bone age at the beginning and end of observation in either treated or untreated groups. No significant relationship between the mean dose of cyproterone acetate used and change in height SDS for bone age could be determined. We conclude that cyproterone acetate has no beneficial effect on the growth prognosis of children with central precocious puberty.

Body Height↗
PubMed1986

True precocious puberty of a girl with hamartoma of the CNS successfully treated by operation.

A girl with precocious puberty due to a hypothalamic hamartoma is presented. At the age of 0.41 years vaginal bleeding was documented and signs of puberty were noted: PHIII, BII according to Tanner. The bone age was 1.3 years, and height velocity rose from the 50th to 90th percentile. Plasma concentrations of LH (5.85 mU/ml), FSH (3.29 mU/ml), growth hormone (30 ng/ml), and oestradiol (90 pg/ml) were elevated. The results of a neurological examination including an EEC recording as well as a skull roentgenogram, were unremarkable. The visual evoked potentials were grossly abnormal. A native and contrast CT scan visualized a tumour close to the suprasellar cisterna reaching the chiasma opticum. At the age of 1.2 years the tumours was removed. Histologically the tissue was identified as a hamartoma. Immediately after the operation vaginal bleeding ceased, pubertal development regressed, bone age did not advance any further, the visual evoked potentials normalized and the contrast CT did not show any tumour mass. The levels of LH, FSH, growth hormone and oestradiol 4 months post operation were decreased as follow: LH: 1.14 mU/ml, FSH: 0.70 mU/ml, GH: 15.1 ng/ml, oestradiol: 10 pg/ml. However, there was an increase of FSH (3 mU/ml) 1 year after the operation. No secondary sexual characters reappeared.

Estradiol↗
PubMed1992

Metastases to the pituitary--hypothalamic axis. An MR study of 7 symptomatic patients.

Seven patients with metastatic tumour in the pituitary-hypothalamic axis were investigated by MRI. The main clinical problems were diabetes insipidus (5 cases) and general pituitary dysfunction (2 cases). No patient had visual or oculomotor symptoms. In 6 of the 7 patients the primary malignant tumour was known, but no patient had symptoms from the primary tumour; 1 had symptoms from metastases in locations other than the pituitary gland. In one patient no primary tumour was known. MR detection of a second, clinically silent, 5 mm lesion in the posterior cranial fossa initiated the search for primary tumour. MRI showed purely suprasellar tumours in 3 patients and intra- and suprasellar tumours in 4. The latter were dumbbell lesions with only a small bridge of tissue connecting the intra- und suprasellar portions. Six of the 7 suprasellar tumours seemed to be in the infundibular recess of the third ventricle; in 5 the infundibulum was visible as an enhancing linear structure at the postero-inferior border of the tumour. The pituitary fossa was normal in all cases.

Adult↗
PubMed1988

MRI of hypothalamic hamartomas in children.

Eight cases of hypothalamic hamartomas (HH) diagnosed in children by clinical, biological and CT studies have been explored by MRI and compared with a control group of twenty children without hypothalamohypophyseal signs. MRI revealed the hamartoma in all cases. Its location and position relative to the floor of the third ventricle was precisely determined. The signal intensity of the lesion was identical to that of normal grey matter on T1-weighted images (300/600.28), but an hyperintense signal was observed over T2-weighted images (1800.60/120) in seven of the eight cases. These data suggest that HH are somewhat different in structure from normal brain tissue. MRI variations in signal intensity should be taken into account in the diagnostic work-up of these lesions.

Child↗
PubMed1985

Precocious puberty and hypothalamic hamartoma. Report on a new case with ultrastructural data.

A new case of hypothalamic hamartoma associated with precocious puberty is presented. After reviewing the literature, 23 other cases appeared. The ultrastructural study of the present case revealed common features with other hamartomatous lesions of the central nervous system and with the gangliogliomas. The data suggest that these lesions are morphologically organized in a very similar manner despite their very different growing potential.

Child, Preschool↗
PubMed1982

Nystagmus.

In the last ten to fifteen years refinements in the registration techniques for ocular movements have allowed a more precise definition of nystagmus forms and have thus brought new insight into their pathophysiology. As a result we can distinguish four types of disturbances of the subsystem for the slow movements, which are fundamental to nystagmus. This and other discoveries also have consequences for the practical diagnostics of neuro-ophthalmological diseases. In this article, with the help of a simple practical scheme, extra attention will be given to the clinically relevant new information needed to make the diagnosis or differential diagnosis of conditions in which nystagmus is one of the most important signs.

Adult↗
PubMed1985

Pubertas praecox and hypothalamic hamartoma.

Precocious puberty of cerebral origin is classified into pseudoprecocious puberty and true precocious puberty. Pseudoprecocious puberty is caused by HCG secreting tumours. True precocious puberty is caused by various hypothalamic diseases. Among them, hypothalamic hamartoma is the most common cause. Precocious puberty is caused by elevated blood pituitary gonadotropin concentration, secondary to the elevated hypothalamic LHRH secretion. The hypothalamic hamartoma is not infrequently associated with laughing (gelastic) seizures as well as convulsions. Diagnosis of a hypothalamic hamartoma is easily made by CT. Although the hypothalamic hamartoma is difficult to operate on, the value of surgery is stressed for treatment of precocious puberty. This is also confirmed by recent reports.

Child↗
PubMed1990

Different types of postoperative diabetes insipidus and the relation to basal and stimulated serum prolactin levels in patients with hypothalamo-hypophyseal tumorous lesions.

To evaluate the interrelationships between anterior pituitary function and the antidiuretic system in patients harbouring hypothalamo-hypophyseal tumorous lesions, combined anterior pituitary stimulation tests were performed in the pre (n = 192 patients) and postoperative (n = 151 patients) state. Basal and stimulated plasma antidiuretic hormone, serum as well as urinary osmolality and diuresis were analyzed to determine the residual functional capacity of the antidiuretic system. In 106 patients with non-prolactin (PRL) secreting tumours basal and stimulated PRL secretion of the residual anterior pituitary was studied pre- and postoperatively. It was found that in the preoperative state latent (n = 12 patients) or manifest (n = 10 patients) types of diabetes insipidus (DI) were related to a significant decrease of maximal stimulated levels of thyroid stimulating hormone as well as basal and maximal stimulated levels of follicle stimulating hormone relative to patients without DI. In the postoperative state DI lasting longer than 10 days (n = 51 patients) was associated with decreased basal and maximal stimulated concentrations of cortisol, luteinizing and follicle stimulating hormone, whereas basal and maximal stimulated levels of PRL were significantly increased compared to those patients without DI (n = 61 patients). Decompression (n = 65 procedures) via the transnasal route was related with a lower frequency of the more severe types of DI (n = 7 patients) and a significant decrease of basal and maximal PRL levels in patients with non-PRL secreting tumours. The transcranial approach (n = 86 procedures) caused a higher rate of severe DI types (n = 33 patients) and an increase of PRL secretion from the residual anterior pituitary lobe. Patients without DI or DI of mild severity (n = 50), as a group, had a significant decrease of basal and maximal PRL levels compared with preoperative values (preoperative: basal = 14.3 +/- 1.5 ng/ml, max = 31.4 +/- 1.5 ng/ml, postoperative: basal = 9.6 +/- 1.1 ng/ml, max = 24.9 +/- 2.9 ng/ml). In patients with severer degrees of DI (n = 40) PRL levels were significantly increased, respectively (preoperative: basal = 15.3 +/- 3.1 ng/ml, max = 23.9 +/- 7.6 ng/ml, postoperative: basal = 19.7 +/- 3.4 ng/ml, max = 38.6 +/- 7.9 ng/ml). It was concluded that in the surgical treatment of non-PRL secreting hypothalamo-hypophyseal lesions the results of early postoperative assessment of basal and stimulated PRL levels may predict the type of postoperative DI.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗
PubMed1992

MR imaging of the pituitary gland in central precocious puberty.

Cranial magnetic resonance imaging was performed in 17 children with central precocious puberty (CPP) and 19 aged-matched controls to compare the appearance of the pituitary gland. Gland size was measured on T1-weighted sagittal and coronal images. The gland was graded according to the concavity or convexity of the upper surface, and the signal intensity of the gland was assessed visually. The mean pituitary volume in 13 CPP children without hypothalamic tumor (292.6 mm3) was significantly greater than that in normal controls (181.35 mm3). The mean volume for the four CPP children with hypothalamic tumor was smaller (145.0 mm3). Compared to controls, the upper pituitary surface in CPP patients appeared convex in a higher proportion. The anterior pituitary was isointense to pons in all patients and controls. Although the posterior pituitary bright spot was present in 14 controls and 11 CPP patients, none with hypothalamic tumor showed it.

Astrocytoma↗