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[Assessment of endocrine disorders of the hypothalamic-pituitary axis by nuclear medicine techniques].

The following article reviews nuclear medicine techniques which can be used for assessment of endocrine disorders of the hypothalamic-pituitary axis. For planar and SPECT imaging somatostatin-receptor- and dopamine-D2-receptor-scintigraphy are the most widely distributed techniques. These nuclear medicine techniques may be indicated in selected cases to answer differential diagnostic problems. They can be helpful to search for presence and localization of receptor positive tissue. Furthermore they can detect metastasis in the rare cases of a pituitary carcinoma. Scintigraphy with Gallium-67 is suitable for further diagnostic evaluation in suspected hypophysitis. Other SPECT radiopharmaca do not have relevant clinical significance. F-18-FDG as PET radiopharmacon is not ideal because obvious pituitary adenomas could not be visualized. Other PET radiopharmaca including C-11-methionine, C-11-tyrosine, F-18-fluoroethylspiperone, C-11-methylspiperone, and C-11-raclopride are available in specialized centers only. Overall indications for nuclear medicine in studies for the assessment of endocrine disorders of the hypothalamic-pituitary-axis are rare. Original studies often report only about a small number of patients. According to the authors' opinion the relevance of nuclear medicine in studies of clinically important endocrinologic fields, e.g. localization of small ACTH-producing pituitary adenomas, tumor localization in ectopic ACTH syndrome, localization of recurrent pituitary tissue, assessment of small incidentalomas, can not be definitely given yet.

Humans↗

Management of optic chiasmatic/hypothalamic astrocytomas in children.

OBJECTIVE: The management of optic chiasmatic gliomas is controversial, partly related to failure to separate out those tumors involving the optic chiasm only (chiasmatic tumors) from those also involving the hypothalamus (chiasmatic/hypothalamic tumors). The purpose of this study was: (i) to analyze the outcomes of chiasmatic and chiasmatic/hypothalamic tumors separately; and (ii) to determine the appropriateness of recommending radical surgical resection for the chiasmatic/hypothalamic tumors. METHODS: A retrospective chart review of all newly diagnosed tumors involving the optic chiasm from 1982-1996 at British Columbia's Children's Hospital was performed. RESULTS: There were 32 patients less than 16 years of age, 14 with chiasmatic and 18 with chiasmatic/hypothalamic astrocytomas, with an average duration of follow-up of 5.8 years and 6.3 years, respectively. Ten of the patients with chiasmatic tumors and none with chiasmatic/hypothalamic tumors had neurofibromatosis I. Thirteen of the 14 chiasmatic tumors were managed with observation only, and none had progression requiring active intervention. For the chiasmatic/hypothalamic tumors, eight patients had subtotal resections (>95% resection), six had partial resections (50-95%), three had limited resections (<50%), and one had no surgery. There were fewer complications associated with the limited resections, especially with respect to hypothalamic dysfunction. There was no correlation between the extent of resection (subtotal, partial, or limited) and the time to tumor progression (average 18 months). CONCLUSIONS: In conclusion, chiasmatic and chiasmatic/hypothalamic tumors are different entities, which should be separated out for the purposes of any study. For the chiasmatic/hypothalamic tumors, there was more morbidity and no prolongation of time to progression when radical resections were compared to more limited resections. Therefore, if surgery is performed, it may be appropriate to do a surgical procedure that strives only to provide a tissue diagnosis and to decompress the optic apparatus and/or ventricular system.

Adolescent↗

[Back to the high school days: an abnormal remembering characterized by concurrent as well as alternating feeling to be in the past and present].

After the successful treatment of a hypothalamic germinoma, a 31-year-old right-handed male developed a difficulty in memory and admitted to our department for detailed evaluation. Neuropsychologically he showed no personality change, confabulation, misidentification, delusion or disorientation to physical time. Neither aphasia, dementia or frontal lobe dysfunction was found. However, he showed a moderate degree of anterograde amnesia and a retrograde amnesia for the last 5 years judged by a public events test. Moreover, his temporal markings of a correctly remembered event in the post high school days shifted strongly toward the high school days. This temporal location abnormality was largely limited to the events from the days of his graduation from the high school to the period 5 years prior to the present incident. He felt personal events happened during this period as if they had occurred in the high school days, although he well knew that they actually happened much later. At the same time he felt that memories of the high school days were abnormally vivid and recent. Most interestingly, he was found to have been experiencing a funny feeling that he was living in the high school days and the present at the same time. The feeling occurred either simultaneously or alternatively. In the latter instance, he would suddenly start behaving as if he were a high school student. He would start urging his wife to go to the school together immediately. This behavior would be over in several minutes. His consciousness was clear in these instances and there was no amnesia for these periods. A brain MRI with gadolinium enhancement showed a lesion extending in the hypothalamus, anterior thalamus, basal forebrain and midbrain bilaterally. After eight months this abnormality of temporal sensation disappeared. However, tendency to make the date of public event nearer to the high school period persisted. We hypothesize that impaired temporal estimation for an event recalled from the retrograde memory store and mis-arousal of familiarity evoked at the time of spontaneous recollection are responsible for double feeling of time of an experience. These phenomenon might be attributable to the partial destruction of the Papez' and the basolateral limbic circuits.

Adult↗

Clinics in diagnostic imaging (81). Hypothalamic glioma with diencephalic syndrome.

A 15-month-old boy presented with failure to thrive. Physical examination revealed third degree malnutrition. MR imaging showed a large lobulated mass in the suprasellar region. The mass was hypointense on T1-weighted MR images, hyperintense on proton density-weighted and T2-weighted MR images, and enhanced homogeneously. The patient underwent a craniotomy for a subtotal resection of the tumour. Histopathological examination revealed a juvenile pilocytic astrocytoma that caused the diencephalic syndrome. Clinical presentation and imaging findings of the various tumours or diseases that can cause suprasellar masses in children are discussed and illustrated via further examples.

Diagnosis, Differential↗

Hypothalamic hamartoma and precocious puberty: report of a case.

Hypothalamic hamartoma is reported to be associated with precocious puberty. Here, the authors present a seven-year-old girl whose onset of puberty occurred at the age of two. Under the impression of idiopathic precocious puberty, cyproterone acetate was initially tried. Since the effect of her medication was not satisfactory, it was discontinued at the age of five years and 11 months. However, rapid advance of bone age and vaginal spotting recurred after the withdrawal of treatment. She was re-evaluated at the age of six, and a magnetic resonance image (MRI) study of the head revealed a hypothalamic hamartoma. At that time, a long-acting analog of luteinizing hormone-releasing hormone (LHRHa), leuprolide acetate, was prescribed. Her secondary sex characteristics regressed and her hypothalamic-pituitary-gonad axis was suppressed after treatment. The clinical presentation, mechanism and treatment of precocious puberty caused by hypothalamic hamartomas are fully discussed in this report.

Child↗