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At least 253 records · Page 14Linked to original sources

Management of hypothalamic gliomas in children: an analysis of 33 cases.

The cases of 33 children with hypothalamic-chiasmatic gliomas are reviewed. Radiation therapy produced clinical or radiographic improvement in 11 (46%) of 24 patients. Progression was documented in 18 patients (54%). Overall, the median time to tumor progression was 60 months; it was 70 months in patients who received radiation therapy and 30 months in those who did not (P less than 0.05). Chemotherapy, either given initially or at the time of progression, caused the tumor to respond or to stabilize in 10 patients. Partial resection of the tumor led to improvement in 3 of 12 patients, obviating the need for a shunt in 2 of them; there were no deaths and postoperative morbidity was transient and minimal (diabetes insipidus, intraventricular hemorrhage, and left hemiparesis in one patient each). The 5- and 10-year survival probabilities were 93 and 74%, respectively. Patients with neurofibromatosis had a better prognosis.

Adolescent↗

Association of prolonged fever and hypernatremia: rare presentation of hypothalamic/third ventricle tumor in a toddler.

The authors describe a 2-year-old boy with a clinical presentation of prolonged fever of unknown origin and severe hypernatremia. This rare association was the result of a hypothalamic/third ventricle tumor. The lesion was removed and was found to be a low-grade neuronal tumor. After surgery, the child did generally well, but hypothalamic thermoregulatory and osmoregulatory functions were not restored. These presenting symptoms, their pathophysiology, and the implications for pediatric practice are discussed.

Child, Preschool↗

Isolated vertical diplopia as the initial manifestation of presumed pretectal and anterior hypothalamic germinomas.

A 21-year-old man with a 5-month history of diplopia caused by isolated vertical ocular misalignment had normal laboratory studies, including brain magnetic resonance imaging (MRI). Eight months after the onset of diplopia, he reported dry mouth, polydipsia, polyuria, and absent sweating. Examination now disclosed light-near dissociation of the pupillary responses, convergence-retraction nystagmus, and upgaze palsy. MRI revealed enhancing suprasellar and pretectal masses presumed to be germinomas. Two years after brain irradiation and systemic chemotherapy, no lesions are apparent on MRI and hypothalamic dysfunction has partially resolved. In a young patient with isolated vertical diplopia and normal brain imaging, one should consider an early pretectal syndrome and inquire after manifestations of hypothalamic dysfunction.

Adult↗

Recognition of the various expressions of anxiety, psychosis, and aggression in epilepsy.

Anxiety, psychosis, and aggressive behavior are among the frequent comorbid psychiatric disorders identified in patients with epilepsy. Often the clinical manifestations of these disorders vary according to their temporal relation relative to seizure occurrence. Thus, postictal symptoms of anxiety or psychosis differ in severity, duration, and response to treatment with interictal symptomatology. Psychiatric symptomatology in epilepsy can appear concurrently with the seizure disorder and improve or remit on the abolition of epileptic activity. We refer to these as paraictal psychiatric phenomena. Such is the case of aggressive disturbances associated with gelastic seizures caused by hypothalamic hamartomas. In this article, three case studies are presented to illustrate the importance of distinguishing psychiatric symptoms of anxiety, psychosis, and aggression, with respect to their temporal relation with seizure occurrence.

Adolescent↗

Inappropriate growth hormone release in the diencephalic syndrome of childhood: case report and 4 year endocrinological follow-up.

A boy with an astrocytoma in the anterior hypothalamic area and typical features of the diencephalic syndrome of childhood was followed with yearly endocrinological evaluation from 10 months of age until he was 5 1/2-years-old. Radiation therapy resulted in temporary clinical improvement. At the time of diagnosis height and bone age were advanced. Basal concentrations of growth hormone (GH) were markedly elevated and could only be partially suppressed by an oral glucose load. There was a paradoxical decline of GH levels following arginine-insulin infusion and an abnormal GH release with TRH administration. Abnormal GH response to the various stimuli persisted for 2 years after radiation therapy. Pituitary secretion of LH, FSH, TSH, prolactin and ACTH remained intact. The characteristic clinical features as well as the endocrinological findings remain unexplained.

Astrocytoma↗

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.

Adenoma↗

Hypothalamic gangliocytoma. Selective appearance of neurofibrillary changes, granulovacuolar degeneration, and argentophilic bodies.

A hamartomatous gangliocytoma was observed in the hypothalamus of a 54-year-old woman. The ganglion cells were atypical, highly pleomorphic and often multinucleated, and they possessed neurofibrillary changes, granulovacuolar degeneration, and argentophilic bodies. The neuronal changes were highly selective and were not found in other parts of the brain. The tumor was also characterized by the presence of angiomatous blood vessels having such degenerative changes as fibrosis and thrombosis. The angiomatous blood vessels were found only in the lesion. The ultrastructural features of the neurofibrillary changes were similar to those observed in Alzheimer's disease. Vascular alterations have been suggested to be a possible contributor to the morphogenesis of neurofibrillary changes. In this case the exact etiology of these neuronal changes remains unclear, however, the possibility of a regional vascular role is considered with respect to their morphogenesis.

Cytoplasmic Granules↗

Primary hypothyroidism and essential hypernatremia in a patient with histiocytosis X.

A 27 year old woman with histiocytosis X had an unusual initial presentation with features of primary hypothyroidism and a goitre. Diagnosis was made by lung and thyroid biopsies. Endocrine tests showed the presence of hypopituitarism and a discrete suprasellar mass, consistent with hypothalamic histiocytosis X, was demonstrated by computerised tomography. Radiotherapy and chemotherapy arrested the clinical progression of the disease.

Adult↗

Gelastic epilepsy and true precocious puberty due to hypothalamic hamartoma.

A new case of gelastic epilepsy and precocious puberty due to hypothalamic hamartoma is reported. After long-term medical treatment there was no observable neurological or endocrinological improvement and the clinical outcome was poor. The authors consider that early surgery for hamartoma should be reconsidered.

Diseases in Twins↗

Hypothalamic hamartoma with skeletal malformations, gelastic epilepsy and precocious puberty.

A child is described who has skeletal malformations, gelastic epilepsy, precocious puberty and a hypothalamic hamartoma. The skeletal abnormalities were detected at birth, she developed gelastic epilepsy at the age of 3 years 5 months and precocious puberty at 3 years 8 months. A hypothalamic hamartoma was found on MRI. The precocious puberty has been successfully medically managed, though her seizures are difficult to control. The combination of all four features has not been described previously.

Bone and Bones↗

Surgical treatment of intractable seizures due to hypothalamic hamartoma.

A 6.5-year-old boy developed seizures at age 2.8 years consisting of episodes of unconsciousness and laughing attacks. By age 6 years, multiple seizure types, including generalized tonic-clonic (GTC), complex partial (CPS) and akinetic seizures, and drop attacks were occurring several times daily. EEG showed multifocal epileptic discharges. Antiepileptic drugs (AEDs) did not control the seizures. With progression of the epilepsy, cognitive deterioration developed. There were no manifestations of precocious puberty. Neuroimaging disclosed a suprasellar mass in continuity with the hypothalamus, and a diagnosis of hypothalamic hamartoma was made. After surgical resection of the hamartoma, the seizures were completely alleviated, and the epileptic EEG discharges disappeared. Improvement of mental function was also noted.

Anticonvulsants↗