Reversible clinical and magnetic resonance imaging of central pontine myelinolysis following surgery for craniopharyngioma: serial magnetic resonance imaging studies. (33/69)

An 18-year-old girl presented with central pontine myelinolysis (CPM) following surgery for craniopharyngioma. Postoperatively, the patient developed diabetes insipidus with remarkable fluctuation of serum sodium level, suffered a seizure, and developed mental state changes and quadriparesis. Magnetic resonance (MR) imaging obtained soon after the development of the symptoms showed no significant abnormalities. MR imaging obtained 2 months later demonstrated typical trident or bat-like signal abnormalities in the center of the pons, compatible with CPM. Serial MR imaging obtained at 7 and 10 months showed the lesion had decreased in size or almost completely resolved and the patient almost completely recovered. CPM is well known, but neurosurgeons should consider the possibility following surgery for craniopharyngioma.  (+info)

Management of central diabetes insipidus with oral desmopressin in a patient with ectrodactyly and cleft lip/palate (ECP) syndrome. (34/69)

We present a female infant with facial abnormalities such as bilateral cleft lip and palate, ectrodactyly and central diabetes insipidus. She had a history of recurrent hypernatremic attacks and she was treated successfully with oral desmopressin. As an alternative to the nasal route, long-term management was achieved using oral route and she had a favorable growth and development during infancy.  (+info)

Spontaneous regression of isolated neurohypophyseal langerhans cell histiocytosis with diabetes insipidus. (35/69)

In pediatric and adolescent patients, the most common causes for a thickened pituitary stalk with central diabetes insipidus are germ cell tumors, lymphocytic infundibuloneurohypophysitis (LIN), and Langerhans cell histiocytosis (LCH). We describe here a 13-year-old girl who had an abrupt onset of polyuria and polydipsia. Magnetic resonance imaging of the brain revealed thickening of the pituitary stalk, and loss of the physiological hyperintense signal of the posterior pituitary gland. Based on a histopathology, she was diagnosed as having LCH. Another LCH lesion was not detected. The prognoses for LCH patients with single-system and single-site are generally good so we decided on only simple observation. The lesion spontaneously regressed 3 months later, resembling a typical self-limiting course of LIN. In conclusion, the present case suggests that 1) radiological differential diagnosis between LIN and LCH is so difficult that histological confirmation is crucial for correct diagnosis, 2) some past cases of histologically-unconfirmed LIN can include LCH, 3) solitary neurohypophyseal LCH can shrink spontaneously up to near remission level.  (+info)

Pituitary and systemic autoimmunity in a case of intrasellar germinoma. (36/69)

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Dominant pro-vasopressin mutants that cause diabetes insipidus form disulfide-linked fibrillar aggregates in the endoplasmic reticulum. (37/69)

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Autosomal recessive familial neurohypophyseal diabetes insipidus: onset in early infancy. (38/69)

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Short-term stimulation of the thiazide-sensitive Na+-Cl- cotransporter by vasopressin involves phosphorylation and membrane translocation. (39/69)

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Myelodysplastic syndrome complicated by central diabetes insipidus and cerebral salt wasting syndrome with peculiar change in magnetic resonance images. (40/69)

Central diabetes insipidus (CDI) could occurs in patients with myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML), because of infiltration of leukemic cells into the neurohypophysis or some other reason and it is closely associated with abnormalities of chromosome 7. We report a case of MDS with abnormalities of chromosome 7, presenting as CDI followed by deterioration of polyuria and hyponatremia with a decreased extracellular fluid volume. Magnetic resonance imaging (MRI) revealed symmetrically enhanced lesions in the hypothalamus. Fludrocortisone treatment normalized his serum sodium level and cerebral salt wasting syndrome (CSWS) was suspected.  (+info)