11/46. Linear accelerator stereotactic radiosurgery for the treatment of gelastic seizures due to hypothalamic hamartoma.PURPOSE: There are reports of successful gamma-knife stereotactic radiosurgery (SRS) for the treatment of gelastic seizures associated with a hypothalamic hamartoma. The authors reviewed the results of linear accelerator (LINAC) radiosurgery for patients with medically refractory gelastic seizures due to a sessile hypothalamic hamartoma. methods: Three patients with gelastic seizures received SRS between 2003 and 2004. All patients had associated partial complex and/or generalized seizures. One patient demonstrated aggressive behavior. Sessile hamartomas varying in diameter from 6 to 14 mm were identified by MRI. SRS was delivered to a single isocenter by a dedicated LINAC equipped with either a circular beam collimator or a micromultileaf collimator. patients received 1500 to 1800 cGy prescribed at the 90 to 95 % isodose line. Seizure outcome was scored according to Engel's classification. RESULTS: Two patients became free of gelastic and partial complex/generalized seizures seven and nine months after radiosurgery. These patients remain free of seizures at 17 and 15 months, respectively, after treatment (Engle Class IA). One patient experienced a decline in gelastic seizure frequency nine months after treatment (Engle Class II) without significant reduction in aggressive behavior. Follow-up MRI demonstrated no change in the size or signal characteristics of any tumor. No patient developed post-treatment cranial neuropathy or hypothalamic-pituitary suppression. CONCLUSIONS: LINAC SRS represents a safe and effective therapeutic alternative for patients with medically refractory gelastic seizures due to unresectable hypothalamic hamartomas. radiosurgery is associated with a latency of several months from treatment to reduction in seizure frequency. Further follow-up is required to establish the duration of seizure control following radiosurgery.- - - - - - - - - - ranking = 1keywords = pituitary (Clic here for more details about this article) |
12/46. Gelastic epilepsy. A clinical contribution.Gelastic (laughing) epilepsy, relatively uncommon, is usually associated with hypothalamic hamartomas, pituitary tumors, astrocytomas of the mammillary bodies, and dysraphic conditions. Cases of unknown etiology are rare. In three of the four cases reported here, the diagnoses were hamartoma of the tuber cinereum; lobar holoprosencephaly; and lissencephaly type I, grade 2. In the fourth, radiographic investigation gave a normal result; a genetic etiology was suggested because of bilateral familial idiopathic epilepsy. In all patients, EEGs showed both focal spikes and generalized spike-and-wave discharges. The primary underlying neurophysiologic disorder may be provoked by the diffuse hyperexcitability of the cortex and subsequent firing of the thalamocortical networks with which the cortical brain is reciprocally interlinked.- - - - - - - - - - ranking = 1keywords = pituitary (Clic here for more details about this article) |
13/46. intraoperative neuronavigation using diffusion tensor MR tractography for the resection of a deep tumor adjacent to the corticospinal tract.OBJECTIVE AND IMPORTANCE: Delineation of cerebral white matter tracts using MR tractography adds essential information for planning intracranial surgery. Integrating tractography with intraoperative neuronavigation may reduce the likelihood of new neurological deficits after surgery done to remove tumors adjacent to the projection fibers of eloquent cortex. We report the utility of such integration for the resection of deep (paraventricular) tumors. CLINICAL PRESENTATION: A 67-year-old male with malignant melanoma underwent stereotactic radiosurgery for a single metastasis within the paraventricular white matter of the right frontal lobe near the corticospinal tract. The lesion doubled in size within 12 months of radiotherapy. Surgical extirpation was performed aided by intraoperative neuronavigation. TECHNIQUE: MR images of the brain including MR tractography and post-contrast T1-weighted sequences were acquired and imported into a neuronavigational workstation. Asymmetric fusion of contrast-enhanced images and tractography was employed to assist in preservation of the integrity of critical white matter tracts during the surgical procedure. CONCLUSION: Inclusion of tractography in standard imaging protocols for neuronavigational systems may increase the safety of neurosurgical intervention near white matter tracts, including deep areas adjacent to the ventricles.- - - - - - - - - - ranking = 0.00069502098132802keywords = lobe (Clic here for more details about this article) |
14/46. Diencephalic syndrome due to hypothalamic tumor: a model of the relationship between weight and puberty onset.CONTEXT: Changes in body weight, statural growth rate, and puberty may be the presenting symptoms of hypothalamic-pituitary tumors. OBJECTIVE: The objective of the study was to assess the relationship between the tumor and its treatment and the weight, growth rate, and onset of puberty, using the diencephalic syndrome of emaciation as model. patients: Eleven patients seen before 1 yr of age, except one aged 9 yr, for diencephalic syndrome of emaciation due to hypothalamic pilocytic astrocytoma, were treated by surgical resection (n = 9), cranial irradiation (n = 7), and/or chemotherapy (n = 10). RESULTS: At diagnosis, growth rate was normal, despite the emaciation, and there was no hypothalamic-pituitary deficiency, except in the oldest patient. After tumor treatment, all had GH and thyroid-stimulating hormone deficiencies, but only three, who underwent major surgical resection, also had ACTH deficiency and diabetes insipidus. Eight became obese, and all but the oldest had transient precocious puberty. plasma leptin concentrations were very low at diagnosis, increased after tumor treatment, and decreased transiently in one boy when the testosterone increased. The plasma soluble leptin receptor concentrations changed in the opposite direction, leading to an increase in the free leptin index, including in the three patients whose tumor was reduced without surgery. The body mass index was correlated positively with plasma leptin (rho = 0.73, P = 0.0004) and free leptin index (rho = 0.63, P < 0.004) and negatively with ghrelin (rho = -0.49, P < 0.03) concentrations. CONCLUSIONS: The obesity that occurs after treatment of hypothalamic tumors is not due to dysregulation of leptin secretion because it and plasma soluble leptin receptor remain regulated by factors like testosterone. This study also shows the influence of weight, possibly via leptin secretion, on the transient hypothalamic-pituitary-gonadal activation that occurs during the first year of life.- - - - - - - - - - ranking = 3keywords = pituitary (Clic here for more details about this article) |
15/46. 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)- - - - - - - - - - ranking = 4.0006950209813keywords = pituitary, lobe (Clic here for more details about this article) |
16/46. Spontaneous regression of anterior pituitary deficits in a woman harboring a persistent hypothalamic mass.Sellar or parasellar lesions can cause anterior pituitary dysfunction either by direct damage to the anterior pituitary gland or by compression of the pituitary stalk and mediobasal hypothalamus which contain the hypophysiotropic hormones. Without treatment, the pituitary deficits in such cases are not likely to improve. We describe a case in which deficits in anterior pituitary hormones spontaneously remitted in a woman who had a persistent hypothalamic lesion that was not amenable to surgery or radiotherapy. The factors that may predict spontaneous recovery of anterior pituitary function in such cases are discussed.- - - - - - - - - - ranking = 10.26355375498keywords = pituitary, pituitary gland, gland (Clic here for more details about this article) |
17/46. Intrasellar gangliocytoma containing gastrin and growth hormone-releasing hormone associated with a growth hormone-secreting pituitary adenoma.A 74-year-old acromegalic found to have an intrasellar gangliocytocytoma and GH-secreting pituitary adenoma is described. The gangliocytoma contained immunoreactive gastrin and, to a lesser extent, GHRH, and the adenoma immunostained for GH. Gastrin has not been previously reported in hypothalamic gangliocytomas. Since this peptide has been demonstrated in normal hypothalamus and pituitary, and provokes GH release when administered intraventricularly, it may have caused GHRH release from the gangliocytoma by a local paracrine action and led to adenoma formation and acromegaly.- - - - - - - - - - ranking = 6keywords = pituitary (Clic here for more details about this article) |
18/46. CT demonstration of pituitary stalk relapse in childhood acute lymphoblastic leukaemia.central nervous system relapse in childhood acute lymphoblastic leukaemia (ALL) occurs in 8% (all risks). Leptomeningeal infiltration is the usual form of relapse and pituitary stalk involvement is rarely found. We report the CT demonstration of pituitary stalk relapse with endocrine evidence of disruption of the hypothalamo-pituitary axis.- - - - - - - - - - ranking = 7keywords = pituitary (Clic here for more details about this article) |
19/46. Case report and delineation of the congenital hypothalamic hamartoblastoma syndrome (pallister-hall syndrome).We report one new case of congenital hypothalamic hamartoblastoma syndrome (pallister-hall syndrome) and one case of a diencephalic nodule associated with craniofacial malformations. Based on a review of 11 cases of pallister-hall syndrome documented by pathological examination, two cases presumed by phenotype, three cases of hypothalamic hamartoma with craniofacial anomalies only, and several cases of related interest, we delineate the clinical, neuroradiologic, and neuropathologic manifestations which aid in differential diagnosis. Clinical manifestations in infants with pallister-hall syndrome included postaxial polydactyly with nail dysplasia, short nose with flat nasal bridge, apparently low-set, posteriorly angulated ears, kidney and lung anomalies, congenital heart defects, imperforate anus, and micropenis with undescended or hypoplastic testes in males. These manifestations were associated with varying degrees of panhypopituitarism and pituitary aplasia. In three cases of hypothalamic hamartoma associated with craniofacial anomalies only, the face resembled that of holoprosencephaly. Other cases of hypothalamic hamartoma have had associated palate or heart defects or presented with precocious puberty. Of the infants with a hypothalamic hamartoblastoma at autopsy, neuropathologic findings were consistent with a primitive neuroectodermal tumor. Surgical tissue from our sole survivor suggested such tumors might mature, and the tumor has not recurred. Neuroradiologic diagnosis may be difficult but should be attempted in infants with these clinical manifestations; due to the need for prompt initiation of appropriate therapy.- - - - - - - - - - ranking = 1keywords = pituitary (Clic here for more details about this article) |
20/46. phagocytosis by neoplastic astrocytes.Cytoplasmic accumulation of granules of hemosiderin was observed in neoplastic astrocytes related to gliomas in two patients. The first patient was a 61-year-old man who had mixed oligodendroglioma and astrocytoma of the right frontal lobe of the brain. The second patient was a 17-year-old girl who had hypothalamic astrocytoma. Granules of hemosiderin had accumulated within the cytoplasm of astrocytes in both cases. In case 2, there was also an accumulation of hemosiderin in other phagocytes. The astrocytic nature of the hemosiderin-laden cells was confirmed by immunohistochemical localization of glial fibrillary acidic protein within the cytoplasm. The term "phagocytosis" was employed because of accumulation within astrocytic perikaryon of particulate material. review of the literature discloses that various neuroepithelial derivatives such as choroidal epithelial cells, astrocytes, neurons, and ependymal cells can perform phagocytic function as revealed by accumulation of granules of hemosiderin within their cytoplasm but this finding has seldom been described.- - - - - - - - - - ranking = 0.00069502098132802keywords = lobe (Clic here for more details about this article) |
| <- Previous || Next -> |