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PubMedDate not supplied

[Variants of the course of early noncomplicated postoperative period following excision of hypothalamo-hypophysial region tumors in children].

A study of non-specific and specific reactions in neurosurgical patients as conducted in the early postoperative period revealed three main variations of an early postoperative clinical course, i.e. with a normal stress-reaction, with a normal stress-reaction concomitant with diabetes insi pidus, and with a lower reactivity to surgical intervention. The treatment algorithms were appropriately amended (the preventive component was added) with due respect to the above circumstances.

Adolescent↗
PubMed2003

MR imaging characteristics of pilomyxoid astrocytomas.

BACKGROUND AND PURPOSE: Pilomyxoid astrocytoma (PMA) is a recently described tumor that typically occurs in the chiasmatic-hypothalamic region in young children and has unique histopathologic and clinical characteristics. These tumors have been previously diagnosed as pilocytic astrocytoma (PA). PMA appears to have a higher rate of recurrence and CSF dissemination than typical PA. METHODS: We analyzed MR findings in four patients with PMA and compared them with those of typical chiasmatic-hypothalamic PA. RESULTS: MR findings of PMA were chiasmatic or hypothalamic enhancing solid tumor with hydrocephalus, highly homogeneous T2 signal intensity that extended into the deep white and gray matter, and CSF dissemination. CONCLUSION: Larger series are needed before the MR imaging findings of chiasmatic or hypothalamic enhancing solid tumor with hydrocephalus, highly homogeneous T2 signal intensity extending into the deep white and gray matter, and CSF dissemination can be used in the differential diagnosis of such tumors.

Astrocytoma↗
PubMed2004

[Hypopituitarism caused by hypothalamic metastasis of lung cancer].

A 40-year-old man was admitted due to thirst, general malaise, and swollen cervical lymph nodes. Cervical lymph node biopsy revealed moderately differentiated adenocarcinoma, and he was diagnosed lung cancer with hypothalamic metastasis. We gave him chemotherapy and hypothalamic irradiation. He had mild hydrodipsia at the first examination, which progressively worsened. He was given a diagnosis of diabetes insipidus caused by hypothalamic metastasis. We gave him desmopressin acetate, and his symptom improved. Predonisolone was prescribed, because of low levels of adrenocorticotropic hormone in the blood. After chemotherapy and irradiation of hypothalamus, metastatic focus diminished and desmopressin acetate was tapered. Hypopituitarism caused by hypothalamic metastasis is rare.

Adenocarcinoma↗
PubMed1991

Delay of diagnosis of growth hormone deficiency in Taiwan.

In view of the importance of early detection of growth hormone deficiency, the height and age at the first pediatric endocrinologic clinic visit of 45 patients with growth hormone (GH) deficiency between January 1988 to December 1989 were retrospectively analyzed. Twenty-three patients with idiopathic GH deficiency had a median age of 13 years (range, from 3.8 to 29.9 years) at their first visit and a median height standard deviation score (SDS) of -3.87 (-2.14 to -7.93). Nineteen children with hypothalamopituitary tumors at their first visit, at a median of 2.17 years (0.42 to 7.83 years) after diagnosis of the tumor, had a median height SDS of -3.15 (-0.58 to -4.70). Three children with GH deficiency secondary to cranial irradiation at their first visit, at 8.17 years (1.33 to 10 years) after treatment for their malignancy, had a height SDS of -3.15 (-2.13 to -3.72). It is well known that the earlier the replacement therapy is begun in such patients, the better the prognosis is for final adult height. Regular height measurement and routine screening for shortness at school entry may overcome this delay in diagnosis. In addition, patients who have received cranial irradiation or had hypothalamopituitary tumors should be regularly measured and studied if their height velocities become subnormal. Physicians dealing with children should keep the possibility of growth hormone deficiency in mind when examining a short child.

Adolescent↗
PubMed1992

[A case of hypothalamic astrocytoma with precocious puberty].

A case of hypothalamic astrocytoma with precocious puberty is presented. In July 1989, a 2-year-old girl was admitted to our hospital because of vaginal bleeding and enlargement of breasts. Breast development was Tanner's stage 3 and no pubic hair was present. Endocrinological evaluation revealed a slightly high level of LH, but the responses of LH and FSH to LH-RH test resulted in exceedingly high values similar to those in adults. Plain CT scan showed an isodense mass in the suprasellar cistern which was not enhanced following administration of contrast medium. MR imaging revealed the precise location of the mass attached to the posterior hypothalamus between the pituitary stalk and the mamillary bodies in sagittal view. The signal intensity of the mass was homogenous and isointense relative to the gray matter on T1 weighted image. But on T2 weighted image, it showed high signal intensity compared with the normal brain parenchyma. A right fronto-temporal craniotomy was performed and the tumor was partially removed. Histological examination disclosed moderate hypercellularity of glial cells but no neurons were visible. This appeared to be astrocytoma grade II. In the literature, CT and MRI behaviour of hypothalamic hamartomas are almost similar to our case. Therefore we think it is not possible at the present time to differentiate a low grade astrocytoma from hamartoma when using CT and MRI alone. In this case, the mechanism of development of precocious puberty seemed to be due to hypothalamic compression by the tumor.

Astrocytoma↗
PubMed1991

[Epilepsy with laughing seizures, hypothalamic hamartoma and precocious puberty. Contributions of MRI, computed EEG topography (CET) and ambulatory EEG (A-EEG)].

The concurrence of gelastic (laughing) seizures, hypothalamic hamartoma and precocious puberty constitutes a well defined epileptic syndrome in children; moreover mental retardation, neuropsychological deterioration and behavioral disorders have been often observed in these patients. In two cases we studied by means of MRI the appearance and the site of the hamartoma (in the posterior part of the hypothalamus with extension toward the third ventricle). The EEG study was performed by means of repeated recordings, of Computed EEG Topography (CET) and of Ambulatory EEG (A-EEG): in both patients during interictal periods paroxysmal EEG discharges prevailing in temporal or fronto-temporal regions and slight abnormalities of the background activity in the same areas were detected. Laughing seizures were recorded in each patient particularly by means of A-EEG: in case 1 bursts of high-voltage activity ("theta" waves) followed by depression of the background rhythm and by irregular spike discharges located in left temporal region were observed; in patient 2 irregular generalised spike discharges followed by slow waves or by depression of the background activity were seen. The presence of local abnormalities in both patients can support the hypothesis that the cortex, especially of the temporal anterior lobe, is involved in the origin of the laughing seizures. The significance of the mechanisms of secondary generalization as regards the seriousness of the epilepsy and of the mental impairment in these patients is also suggested.

Brain Mapping↗
PubMed1991

Diencephalic syndrome with long-term survival.

Infants and young children with the diencephalic syndrome exhibit a normal activity level despite profound marasmus. Investigation reveals an intracranial tumour and evidence of endocrine dysfunction. The condition is easily missed in Third World countries with a high prevalence of nutritional marasmus. The advent of computer tomography and magnetic resonance imaging has simplified diagnosis. The syndrome was diagnosed in 1981 in a boy of 20 months who had a large hypothalamic tumour. Initial endocrine studies revealed very high growth hormone levels. After radiotherapy, these levels reverted to normal for a time but have subsequently declined in keeping with a state of hypopituitarism. The boy's long-term survival has afforded an opportunity for study over eight years. During this time, growth has been slow but sustained. There has been some fall off in intellectual capacity. Characteristic features of the diencephalic syndrome are normal activity and a state of euphoria in a profoundly marasmic child. Investigation reveals the presence of an intracranial tumour and evidence of endocrine dysfunction. Though there are earlier references, the condition first came to medical attention at the annual meeting of the British Paediatric Association in 1951 when Russell described the typical features. In Third World countries where childhood marasmus is common, the syndrome is easily overlooked. The present report concerns a boy who has been closely followed for more than eight years.

Diencephalon↗