1/46. Resection of a Langerhans cell histiocytosis granuloma of the hypothalamus: case report.The natural course and optimal treatment for isolated hypothalamic Langerhans cell histiocytosis (LCH) are unknown. We describe an adult female in whom total resection of a hypothalamic LCH granuloma was performed 12 years after transphenoidal resection of a pituitary adenoma. A retrospective review of the histological specimen of the first operation revealed CD1a positive cells characteristic of LCH along with a plurihormonal adenoma 12 years earlier. No other manifestations of LCH were found and MRI of the brain at the last follow-up 4 years after surgery did not show any recurrent or additional lesion. The diagnosis of isolated hypothalamic LCH is only possible by biopsy and our case demonstrates the feasability of a gross total resection in certain cases.- - - - - - - - - - ranking = 1keywords = pituitary (Clic here for more details about this article) |
2/46. Thalamic and hypothalamic tumors of childhood: endocrine late effects.Children who have received chemotherapy and radiation therapy for treatment of thalamic/hypothalamic tumors are at risk for late effects, specifically endocrine dysfunction. Evaluation of growth and pubertal development, thyroid function and integrity of the hypothalamic-pituitary-adrenal axis should be undertaken in a prospective manner. Issues of metabolic disturbances such as obesity, altered body composition/bone density as well as ultimate fertility also need to be addressed by ongoing prospective evaluations.- - - - - - - - - - ranking = 1keywords = pituitary (Clic here for more details about this article) |
3/46. Thalamic tumour presenting as frontal lobe dysfunction.A 64-year-old man presented with a change in personality and behaviour but with no neurological deficits. He exhibited impaired executive functions, apathy, unsteadiness of gait and falling; he lacked insight and exhibited purposeless behaviour, making loud grunting noises. A CT scan at 6 months revealed no abnormality, but at 9 months CT and MRI showed a bilateral thalamic tumour. The presenting symptoms had similarities to frontal lobe dysfunction progressing to dementia.- - - - - - - - - - ranking = 0.0034751049066401keywords = lobe (Clic here for more details about this article) |
4/46. Multiple system erdheim-chester disease with massive hypothalamic-sellar involvement and hypopituitarism.erdheim-chester disease (ECD) is a rare multiple system histiocytosis that is characterized pathologically by xanthogranulomatous infiltrates and radiologically by symmetrical sclerosis of long bones. The diagnosis is often confirmed by biopsy of bone or of orbital or retroperitoneal soft tissue. Intracranial involvement is rare. The authors report a case of ECD in which the diagnosis was made after biopsy of a hypothalamic mass. The mass had been discovered during a workup for panhypopituitarism in a 55-year-old man with urological and bone disease. Aside from diabetes insipidus, other features of pituitary insufficiency have seldom been reported and no patients have presented with a hypothalamic tumor. The endocrinological and neurological aspects of ECD are discussed, as is its differential diagnosis. Reported cases of the disorder associated with hypopituitarism or found during biopsy of central nervous system structures are also reviewed.- - - - - - - - - - ranking = 1keywords = pituitary (Clic here for more details about this article) |
5/46. neuroimaging findings in a suprasellar granular cell tumor.A 27-year-old woman presented with a history of amenorrhea, visual disturbance, and diabetes insipidus. magnetic resonance imaging showed a large enhancing suprasellar mass with associated edema involving the left striatum. The lesion was hypometabolic on fluorodeoxyglucose-positron emission tomography. At surgery, a subtotal resection of a vascular tumor that appeared to arise from the posterior pituitary and hypothalamus was carried out. Pathologic examination revealed a granular cell tumor. We report the preoperative neuroimaging findings in this rare posterior pituitary stalk tumor.- - - - - - - - - - ranking = 2keywords = pituitary (Clic here for more details about this article) |
6/46. 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.- - - - - - - - - - ranking = 1keywords = pituitary (Clic here for more details about this article) |
7/46. hypopituitarism and diabetes insipidus with localized hypertrophic pachymeningitis (tolosa-hunt syndrome) associated with Hashimoto thyroiditis.We report a 69-year-old woman with intracranial pachymeningitis showing hypopituitarism, diabetes insipidus, and tolosa-hunt syndrome associated with Hashimoto thyroiditis confirmed by autopsy. A large tumorous lesion of the hypothalamo-pituitary gland was revealed on magnetic resonance imaging, after the patient complained of gait and visual field disturbance. These symptoms subsided after thyroid hormone supplementation. hypopituitarism and diabetes insipidus were diagnosed after cessation of the treatment by the patient herself. Multiple cranial nerve palsies and orbito-frontalgia appeared. methylprednisolone pulse therapy improved the symptoms, but they recurred when the dose of glucocorticoid was decreased. The patient died of brain thrombosis. autopsy revealed typical findings of Hashimoto thyroiditis and intracranial pachymeningitis involving the cranial base and pituitary gland. The high titer of rheumatoid factor and Hashimoto thyroiditis in this patient suggest an immunological role in the pathogenesis of pachymeningitis.- - - - - - - - - - ranking = 2.5271075099594keywords = pituitary, pituitary gland, gland (Clic here for more details about this article) |
8/46. Hypothalamic ganglioglioma treated by temporal lobectomy. Case report and review of the literature.The authors report the case of a hypothalamic ganglioglioma with left-sided temporal lobe extension in an 8-year-old girl who presented with seizures. Other cases of ganglioglioma involving the hypothalamus have been reported in the literature; however, this site of origin is exceedingly rare and worthy of report. Treatment involved medial temporal lobectomy with the hypothalamic component of the tumor remaining untouched. The patient recovered postoperatively with no neurological deficits and was seizure free at 12 months. Neither radio- nor chemotherapy was recommended because of the tumor histology, location, and the patient's age. The authors recommend follow up and surgical treatment for possible tumor recurrence. The prognosis for hypothalamic ganglioglioma is unknown.- - - - - - - - - - ranking = 0.0041701258879681keywords = lobe (Clic here for more details about this article) |
9/46. neurologic manifestations of hypothalamic disease.The hypothalamus, in addition to regulating the anterior and posterior pituitary, controls water balance through thirst, regulates food ingestion and body temperature, influences consciousness, sleep, emotion and other behaviors. Much has been learned of these effects in human disease through the clinical manifestations that occur with hypothalamic lesions. This study reviews the clinical pathologic correlations that have been made in recent years showing that regions of the hypothalamus exert functions in humans that are similar to those identified in experimental animals. Clinical pathologic correlations have not always provided precise analysis of hypothalamic function. The hypothalamus is small and often lesions that come to clinical attention achieve considerable size before their recognition, making local anatomic dissections of the effects of the lesions difficult. Nevertheless, the use of modern non-invasive techniques including CT scans and magnetic resonance imaging (MRI) have provided new information not previously available. This paper reviews several cases of hypothalamic disorder recognized recently. (1) A 33-year-old black man with hypothalamic sarcoidosis. Manifestations of hypothalamic dysfunction included panhypopituitarism, aggressive hyperphagia, polydipsia (partially due to hyperglycemia secondary to diabetes mellitus), drowsiness, depression, and irritability. (2) A 37-year-old woman with a large intrahypothalamic tumor (biopsy showed pituitary adenoma), with drowsiness, poikilothermia, lack of satiety, confusion, and memory loss. She becomes depressed when she is transiently more alert (as after hypertonic contrast-dye infusion). (3) A 60-year-old man with hypothalamic compression by a pituitary tumor, associated with syndrome of inappropriate ADH (SIADH), severe anorexia, memory loss, but preserved thirst. After surgical decompression of the tumor his appetite acutely recovered, but he developed severe hypo(poikilo)thermia. (4) A 45-year-old woman with a suprasellar craniopharyngioma presented with severe drowsiness, hyperphagia, depression, and memory loss post-operatively, which responded to antidepressants (except for the memory loss). She had extremely labile blood pressures and serum Na for about 1 week post-operatively.- - - - - - - - - - ranking = 3keywords = pituitary (Clic here for more details about this article) |
10/46. Primary hypothalamic lymphoma with panhypopituitarism presenting as stiff-man syndrome.We report an unusual case of primary hypothalamic lymphoma with hypopituitarism presenting as Stiff-man syndrome (SMS). A 64-year-old man was hospitalized due to a 3-week history of general weakness, anorexia, vomiting, weight loss, and muscle pain and spasms precipitated by motion and tactile stimuli resulting in muscle stiffness and difficulty in mobility. physical examination revealed normal sensorimotor function and reflexes, except for bitemporal visual field defect. Routine laboratory and gastrointestinal examinations provided no remarkable clues. Endocrine assessment revealed low levels of morning cortisol, thyroxine, and anterior pituitary hormones but an increase in prolactin level. The patient's muscle pain and stiffness improved dramatically within 2 days after hydrocortisone therapy and thyroxine replacement. magnetic resonance imaging (MRI) of the brain confirmed an 18-mm enhancing hypothalamic tumor with optic chiasm involvement, which proved to be a B-cell lymphoma. The results of the extensive studies for systemic lymphoma were negative, suggesting a primary hypothalamic lymphoma. The tumor regressed completely and was invisible on MRI scan after adjuvant radiotherapy. The patient's condition was satisfactory and there was no recurrence of SMS during the 2-year follow-up period. This case demonstrated that primary hypothalamic lymphoma complicated with adrenal insufficiency may manifest as SMS. early diagnosis and prompt intervention can lead to a favorable outcome and reduce morbidity.- - - - - - - - - - ranking = 1keywords = pituitary (Clic here for more details about this article) |
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