1/38. Hypothalamic Langerhans cell histiocytosis with no eosinophils.A 26-year-old man presented with diabetes insipidus, sexual dysfunction and memory impairment. Nuclear magnetic resonance imaging and pathologic examination revealed Langerhans cell histiocytosis of the hypothalamus, which contained no eosinophils. The lesional cells were positive for S-100 protein and CD1a antigen, and negative for CD68 antigen. The possible pathogenesis, absence of eosinophils and prognosis are discussed.- - - - - - - - - - ranking = 1keywords = diabetes insipidus, insipidus, diabetes (Clic here for more details about this article) |
2/38. Langerhans cell histiocytosis limited to the pituitary-hypothalamic axis--two case reports.Langerhans cell histiocytosis rarely presents as a solitary lesion in the pituitary-hypothalamic region, and is indistinguishable from germinoma, which occurs much more frequently, especially in Japanese. A 14-year-old girl and a 9-year-old girl presented with polydipsia and polyuria as the initial symptoms. Magnetic resonance (MR) imaging demonstrated a round mass at the pituitary stalk appearing as isointense on T1-weighted imaging and hyperintense on T2-weighted imaging. Endocrinological examination revealed mild hypopituitarism with central diabetes insipidus. Both patients underwent open craniotomy. Histological examination revealed granulomatous tissue with eosinophil infiltration and frequent Langerhans histiocyte clustering, compatible with the diagnosis of Langerhans cell histiocytosis. Low-dose local irradiation of 20 Gy was administered. First patient was followed up for 8 years, and her hypopituitarism gradually improved to a minimal level with only amenorrhea as the residual symptom. Recent MR imaging showed no residual mass at the region. Second patient was followed up for 15 months, and her diabetes insipidus is stable. MR imaging performed 5 months after the treatment showed marked reduction of the mass. These cases reemphasize the importance of histological diagnosis for lesions with similar neuroimaging appearances. biopsy and low-dose irradiation are an effective treatment for this rare and essentially benign lesion, as opposed to attempting total removal of the mass.- - - - - - - - - - ranking = 2keywords = diabetes insipidus, insipidus, diabetes (Clic here for more details about this article) |
3/38. Transcallosal resection of hypothalamic hamartomas, with control of seizures, in children with gelastic epilepsy.OBJECTIVE: Hypothalamic hamartomas (HHs) are associated with precocious puberty and gelastic epilepsy; the seizures are often refractory to antiepileptic medications and associated with delayed development and disturbed behavior. The current opinion is that surgery to treat intrahypothalamic lesions is formidable and that complete excision is not technically achievable. We report our experience with a transcallosal approach to the resection of HHs. methods: Five children (age, 4-13 yr) with intractable epilepsy and HHs underwent preoperative clinical, electroencephalographic, and imaging evaluations. Two patients experienced only gelastic seizures, and three patients experienced mixed seizure disorders with drop attacks; all experienced multiple daily seizures. patients were evaluated with respect to seizures, cognition, behavior, and endocrine status 9 to 37 months (mean, 24 mo) after surgery. The HHs were approached via a transcallosal-interforniceal route to the third ventricle and were resected using a microsurgical technique and frameless stereotaxy. RESULTS: Complete or nearly complete (>95%) excision of the HHs was achieved for all patients, with no adverse neurological, psychological, or visual sequelae. Two patients experienced mild transient diabetes insipidus after surgery. Two patients developed appetite stimulation, but no other significant endocrinological sequelae were observed. Three patients are seizure-free and two patients have experienced only occasional, brief, mild gelastic seizures after surgery, all with reduced antiepileptic medications. On the basis of parental reports and our own subjective observations, the children also exhibited marked improvements in behavior, school performance, and quality of life. CONCLUSION: Complete or nearly complete resection of HHs can be safely achieved via a transcallosal approach, with the possibility of seizure freedom and neurobehavioral improvements.- - - - - - - - - - ranking = 1keywords = diabetes insipidus, insipidus, diabetes (Clic here for more details about this article) |
4/38. Idiopathic hypothalamic dysfunction with precocious puberty and adipsic hypernatremia first presenting in adolescence.BACKGROUND: Idiopathic hypothalamic dysfunction is a rare syndrome that has been described in young children. This syndrome is characterized by a disturbance of thirst regulation with hypernatremia, hyperosmolarity, and altered hypothalamic function. PATIENT REPORT: At age 6 years the reported patients presented with precocious puberty, by age 12 years she had hypernatremia presumed secondary to central diabetes insipidus and was treated with DDAVP, and at age 14 was identified to have hyperprolactinemia. At age 19 she presented with serum sodium of 185 mg/dl during an episode of illness associated with dehydration. After hydration, her sodium remained elevated. arginine vasopressin was measurable but inappropriate to serum sodium, while urinary cyclic amp response to vasopressin was appropriate. CONCLUSIONS: This is the first case of precocious puberty identified in association with the more classic features of idiopathic hypothalamic dysfunction, including later-onset hypernatremia, poor thermoregulatory function, obesity, and hyperprolactinemia.- - - - - - - - - - ranking = 1keywords = diabetes insipidus, insipidus, diabetes (Clic here for more details about this article) |
5/38. Transient hypothalamic hypothyroidism and diabetes insipidus after electrical injury.Transient or permanent diabetes insipidus (DI) due to damage in vasopressinergic neurons--which may be hereditary or caused by head injury, brain surgery, tumors, granulomatous disorders, infections, vascular disorders, autoimmunity, and idiopathic causes--is not rare. Hypothalamic hypothyroidism is due to decreased thyrotropin-releasing hormone secretion and is seen rarely. We report a case of transient hypothalamic hypothyroidism and transient DI due to electrical injury.- - - - - - - - - - ranking = 5keywords = diabetes insipidus, insipidus, diabetes (Clic here for more details about this article) |
6/38. triiodothyronine supplementation for hypothalamic obesity.patients with suprasellar lesions develop profound hypothalamic obesity and listlessness with no effective treatment. We added triiodothyronine (T(3)) supplementation in 3 such patients and present their response. All had previous nutritional counseling without benefit. All were treated for diabetes insipidus (DI) and hypopituitarism; serum free thyroxine (T(4)) level was normal. A 24-year-old woman (pineal tumor and astrocytoma) had weight gain (4.7 kg/yr for 3 years), cold intolerance, fatigue, dry skin, and constipation; after T(3), she lost 14 kg over 27 months and reported overall improvement. Her bone mineral density also improved. A 10.6-year-old boy (optic glioma) was gaining 6 kg/yr for 4 years; after T(3) supplement, he lost 4.3 kg over 11 months. A 12-year-old girl (mixed germ cell tumor) had weight gain (8.3 kg/yr for 3 years) and listlessness; after T(3), she lost 8.1 kg over 16 months and had improved alertness. All patients were asymptomatic despite supraphysiologic T(3) levels. We suggest that T(3) may serve as a simple and effective supplement, which can promote weight loss and improve the well being of these patients with hypothalamic obesity.- - - - - - - - - - ranking = 1keywords = diabetes insipidus, insipidus, diabetes (Clic here for more details about this article) |
7/38. A case of lymphocytic infundibuloneurohypophysitis associated with systemic lupus erythematosus.A 27-year-old man was admitted to our hospital with facial erythema and general malaise. He had previously suffered from orbital myositis, central diabetes insipidus (DI), peripheral neuritis, and hypogonadotropic hypogonadism. Physical and immunological examinations revealed that he was suffering from systemic lupus erythematosus (SLE). magnetic resonance imaging of the hypothalamic-pituitary region demonstrated a significant enlargement of the pituitary stalk and posterior pituitary. Endocrinological examinations showed that he had not only DI and hypogonadotropic hypogonadism but also hypoadrenalism and hypothyroidism, which were ascribed to the pituitary stalk lesion. Lymphocytic infundibuloneurohypophysitis associated with SLE was diagnosed. Administration of 30 mg/day of prednisolone for one month resulted in a marked reduction of the pituitary stalk thickening and posterior pituitary. It is recommended that a pharmacological dose of glucocorticoid be used in the treatment of lymphocytic hypophysitis patients who show significant thickening of the pituitary stalk and/or a large pituitary mass.- - - - - - - - - - ranking = 1keywords = diabetes insipidus, insipidus, diabetes (Clic here for more details about this article) |
8/38. Hypothalamic-pituitary sarcoidosis with hypopituitarism. Long-term remission with methylprednisolone pulse therapy.A 29-year-old man presented with diplopia and decreased strength in the left arm. A magnetic resonance image (MRI) showed an extensive hypothalamic and pituitary gland mass, and hormonal studies showed partial hypopituitarism and mild hyperprolactinemia without diabetes insipidus. Biopsies of the hypothalamic lesion and of a mediastinal lymph node demonstrated noncaseating granulomas, and a Kveim-Siltzbach test was positive. He was successfully treated with a regimen of high-dose intravenous methylprednisolone pulse therapy for eight weeks along with a low dose of oral corticosteroids which was maintained indefinitely. An MRI obtained immediately after pulse therapy revealed a substantial reduction in the hypothalamic-pituitary mass, which was maintained in an MRI performed 3 years later. However, hormonal deficits persisted and indefinite hormonal substitutive therapy was required.- - - - - - - - - - ranking = 1keywords = diabetes insipidus, insipidus, diabetes (Clic here for more details about this article) |
9/38. Hypothalamopituitary deficiency and precocious puberty following hyperhydration in diabetic ketoacidosis.We report on a 5-year-old child who survived an intracerebral crisis, following ketoacidosis-revealing diabetes (DKA), with visual impairment due to a vascular occipital lesion. Two and 4 months after the initial episode, a unique hypothalamopituitary disorder consisting in GH, ACTH, TSH deficiencies and central precocious puberty, was detected. Cranial magnetic resonance images showed no visible lesion in the hypothalamopituitary region. The most likely hypothesis is the ischemia of hypothalamopituitary and occipital regions following possible cerebral edema after hyperhydration. She survived with low visual acuteness and received a combined replacement therapy for the neuroendocrinological deficiencies. This case emphasizes that the rehydration at the initial period of DKA is critical, especially when risk factors for cerebral edema are present (young age, marked hyponatremia). The neuroendocrinological consequences of acute cerebral edema are rare, but physicians must be attentive in survivors of these accidents.- - - - - - - - - - ranking = 0.0061520929485644keywords = diabetes (Clic here for more details about this article) |
10/38. diabetes insipidus from neurosarcoidosis: long-term follow-up for more than eight years.Four patients with sarcoidosis presented as hypothalamic-hypophyseal syndrome including diabetes insipidus (DI) were followed up for more than 8 years from the onset of clinical manifestation. The mean age was 26 years, male : female ratio was 3 : 1 and the mean disease duration of 10 years. All patients had hypogonadism, hyperprolactinemia. Pituitary enlargement with thickening of the pituitary stalk were detected by magnetic resonance imaging (MRI) with gadolinium enhancement and attenuation in the intensity of the posterior lobe of the pituitary was detected without enhancement. Corticosteroid therapy resulted in the initial improvement of symptoms and gradual decrease in the tumor size but failed to cure polyuria due to DI. The use of desmopressin was necessary for a long period. None of these patients died from DI or central neurosarcoidosis.- - - - - - - - - - ranking = 1.7880863312996keywords = diabetes insipidus, insipidus, diabetes (Clic here for more details about this article) |
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