Cases reported "Hypothalamic Neoplasms"

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1/5. Effect of interstitial stereotactic radiosurgery on behavior and subjective handicap of epilepsy in patients with gelastic epilepsy.

    patients with symptomatic epilepsy due to hypothalamic hamartomas often are compromised not only by pharmacoresistant epileptic seizures but also by behavioral disturbances and cognitive dysfunction. We report the effect of successful treatment with stereotactic interstitial radiosurgery by intrahypothalamic implantation of 125I seeds on behavior and subjective handicap. In all patients rendered seizure-free or suffering only from auras, improvement of behavior was reported by parents and colleagues or schoolteachers. parents' ratings according to the child behavior checklist showed improvements with respect to social problems and attention. Self-ratings of quality of life by adult patients showed improvements in activities, working situation, and self-perception. These improvements were not observed in patients in whom clinically manifest seizures and interictal EEG discharges persisted after radiosurgery.
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2/5. magnetic resonance imaging of hypothalamic hamartomas causing gelastic epilepsy.

    Hypothalamic hamartomas may cause a peculiar epileptic syndrome characterized by seizures of laughter and precocious puberty. Four mentally handicapped patients suffering from gelastic epilepsy were referred to our institution for investigation; three of them also presented with precocious puberty. In all four cases magnetic resonance imaging (MRI) revealed a space-occupying lesion of the hypothalamus that was considered to be a hamartoma. Biopsies were not performed. Hamartomas appear isodense in plain computed tomography scans, and they do not enhance. Such lesions display an isointense signal in T1-weighted magnetic resonance images and a hyperintense signal in proton density and T2-weighted images. MRI is the procedure of choice for detecting such lesions at the base of the brain.
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3/5. puberty due to a mixed germ cell tumour of the hypothalamus secreting BhCG and alpha-fetoprotein.

    A 14 year-old boy presented with visual impairment due to a large suprasellar tumour secreting beta-human chorionic gonadotrophin (BhCG) and alpha-fetoprotein (alpha FP). He was sexually mature with an advanced bone age of 17 years, and suffering from partial hypopituitarism. Treatment with external radiotherapy resulted in a reduction of tumour size and fall of the serum testosterone BhCG and alpha FP levels. We conclude that pubertal development had been initiated and maintained by ectopic hCG production from his intracerebral mixed germ cell tumour. patients with tumours in the pineal and suprasellar regions should be screened for elevated levels of BhCG and alpha FP. We suspect that many of these tumours cause precocious puberty by secreting BhCG.
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4/5. Neurogenic diabetes insipidus: an unusual clinical presentation of small cell lung cancer.

    A 61 year old man presented with chronic bronchitis, which he had been suffering from for many years, and reported the recent onset of polyuria with polydipsia. A chest X-ray showed a peripheral pulmonary nodule in the right lower lobe, that was diagnosed as small cell lung cancer following histological examination of the pulmonary tissue specimen, obtained with transbronchial biopsy. After establishing the central origin of the diabetes insipidus, its cause was revealed by magnetic resonance imaging of the brain, that demonstrated metastasis to the hypothalamic-neurohypophyseal area.
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5/5. Adrenal failure due to metastasis both to the hypothalamic-pituitary area and the adrenals.

    A 61-year-old white male was admitted to our hospital with a big-cell bronchogenic carcinoma whose first clinical manifestation was diabetes insipidus (DI) secondary to metastasis to the hypothalamic-pituitary area (MHP). In three months, and progressively, he developed anterior pituitary failure, as well as primary adrenal insufficiency (PAI) due to metastasis in both adrenals. Panhypopituitarism or PAI due to both MHP and adrenals has been rarely reported in the literature. A thorough examination of the oncologic patient led us to diagnose hormone insufficiency properly. The absence of reported cases might be due to the fact that the symptoms resulting from hormone insufficiency are veiled by the severe condition of the patients suffering from disseminated cancer.
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