deamino-arginine-vasopressin and Sarcoidosis

Central diabetes insipidus (CDI) is an infrequent complication of neurosarcoidosis (NS). Its presentation may be masked by adrenal insufficiency (AI) and uncovered by subsequent steroid replacement. A 45-year-old woman with a history of NS presented 2 weeks after abrupt cessation of prednisone with nausea, vomiting, decreased oral intake and confusion. She was diagnosed with secondary AI and intravenous hydrocortisone was promptly begun. Over the next few days, however, the patient developed severe thirst and polyuria exceeding 6 L of urine per day, accompanied by hypernatraemia and hypo-osmolar urine. She was presumed to have CDI due to NS, and intranasal desmopressin was administered. This eventually normalised her urine output and serum sodium. The patient was discharged improved on intranasal desmopressin and oral prednisone. AI may mask the manifestation of CDI because low serum cortisol impairs renal-free water clearance. Steroid replacement reverses this process and unmasks an underlying CDI.

Topics: Adrenal Insufficiency; Antidiuretic Agents; Central Nervous System Diseases; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Glucocorticoids; Humans; Hydrocortisone; Hypernatremia; Middle Aged; Polyuria; Prednisone; Sarcoidosis; Thirst

Topics: Adult; Anti-Inflammatory Agents; Antidiuretic Agents; Azathioprine; Biopsy; Central Nervous System Diseases; Deamino Arginine Vasopressin; Diabetes Insipidus; Diagnosis, Differential; Drug Therapy, Combination; Humans; Image Enhancement; Immunosuppressive Agents; Lung; Magnetic Resonance Imaging; Male; Pituitary Gland; Prednisolone; Receptors, Interleukin-2; Sarcoidosis; Sarcoidosis, Pulmonary; Tomography, X-Ray Computed

Central nervous system (CNS) sarcoidosis is a crucial disease and has a poor prognosis. A 58-year-old woman had acute development of polydipsia and polyuria. Her pituitary MRI demonstrated a swelling of pituitary gland and hypophyseal stalk. She was diagnosed as central diabetes insipidus (CDI) due to CNS sarcoidosis based on the examinations and pituitary MRI findings as well as a result of cutaneous biopsy. Uveitis and bilateral hilar lymphadenopathy were observed mildly throughout. However, CDI and pituitary MRI findings were getting recovered spontaneously without steroid treatment in a couple of months, suggesting an atypical clinical course of CNS sarcoidosis.

Topics: Antidiuretic Agents; Central Nervous System Diseases; Deamino Arginine Vasopressin; Diabetes Insipidus; Female; Humans; Middle Aged; Remission, Spontaneous; Sarcoidosis

A 15-year-old girl was admitted to our hospital because of polydipsia, polyuria, bilateral hilar lymphadenopathy and uveitis. A diagnosis of sarcoidosis with central diabetes insipidus was made by radiological, serological, bronchoalveolar lavage examinations, fluid restriction test and vasopression test. Prednisolone therapy improved all of her clinical findings except diabetes insipidus. So she had to continue intranasal 1-desamino-8-arginine vasopressin (DDAVP) therapy. In addition, we reviewed the clinical features of 27 patients of sarcoidosis with diabetes insipidus reported in Japan. They included 12 patients in young men and 21 patients having uveitis. These patients showed low frequency of lung complications in comparison with sarcoidosis without diabetes insipidus. Steroid therapy improved the symptoms of diabetes insipidus in only 3 patients, and all these 3 patients started steroid therapy within 1 month after the onset. Therefore we think that early diagnosis and treatment are important. Though central neurosarcoidosis was generally considered to have poor prognosis, there were only 3 patients who had recurrence by steroid tapering.

Topics: Adolescent; Antidiuretic Agents; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Female; Humans; Lymphatic Diseases; Sarcoidosis

To report a case of neurosarcoidosis with rapid progression of visual field defects.. A 28-year-old woman presented with bilateral uveitis and was diagnosed as having sarcoidosis after skin and cervical lymph node biopsy. Since bilateral excavations of the optic nerve head and visual field defects were observed, endocranial lesion was suspected. However, a computed tomography (CT) scan of the head detected nothing abnormal. It was regarded as a case of sarcoidosisaccompanied by normal-tension glaucoma and treatment was initiated with latanoprost. Four months later, the patient's visual field deteriorated rapidly. A CT scan showed a pituitary mass. Neurologicalfindings and hypopituitarism were found which improved with systemic prednisolone therapy. Diabetes insipidus developed after the start of treatment, and was treated with intranasal desmopressin therapy. After 6 weeks, head magnetic resonance imaging (MRI) showed a remarkable reduction of the enhanced regions.. Although ocular sarcoidosis is often accompanied by secondary glaucoma or optic nerve atrophy, the progression of neurosarcoidosis can lead to visual field defects. Central nervous system (CNS) sarcoidosis is rare, but a precise examination with enhanced MRI should be considered when the visual field defect progresses rapidly.

Topics: Adult; Central Nervous System Diseases; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Disease Progression; Female; Humans; Magnetic Resonance Imaging; Prednisolone; Sarcoidosis; Tomography, X-Ray Computed; Treatment Outcome; Uveitis; Vision Disorders; Visual Fields

A 23-year-old man was admitted because of polydipsia and polyuria. As chest radiographs and computed tomography showed mediastinal and bilateral hilary lymph node swelling, and diffuse small nodules in bilateral lung fields, we suspected sarcoidosis. Sarcoidosis was diagnosed by biopsy of a cervical lymph node, and central diabetes insipidus was diagnosed by fluid restriction test and vasopressin load test. A pituitary mass was also revealed by magnetic resonance imaging. Prednisolone therapy improved all of his clinical findings except diabetes insipidus. He had to continue intranasal 1-desamino-8-D-arginine vasopressin (DDAVP) therapy.

Topics: Adult; Deamino Arginine Vasopressin; Diabetes Insipidus, Nephrogenic; Humans; Magnetic Resonance Imaging; Male; Prednisolone; Sarcoidosis; Tomography, X-Ray Computed

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.

Topics: Adrenal Cortex Hormones; Adult; Brain Diseases; Deamino Arginine Vasopressin; Diabetes Insipidus; Female; Hemostatics; Humans; Hyperprolactinemia; Hypogonadism; Hypothalamic Diseases; Magnetic Resonance Imaging; Male; Pituitary Gland; Prognosis; Sarcoidosis; Time Factors; Treatment Outcome

A young white man with new-onset central diabetes insipidus was discovered to have a posterior pituitary mass on magnetic resonance imaging. No other radiological abnormalities were noted in the anterior pituitary, infundibulum or hypothalamus. No other endocrinopathies were present: laboratory investigations showed normal basal concentrations of anterior pituitary hormones, including prolactin. The patient was suspected to have sarcoidosis affecting the posterior pituitary, because of the discovery of pulmonary sarcoidosis during his diagnostic evaluation. His symptoms of polydipsia and polyuria responded promptly to intranasal administration of 1-desamino-8-D-arginine vasopressin (DDAVP). The patient demonstrated complete regression of the posterior pituitary mass after a course of corticosteroid therapy. However, his diabetes insipidus persisted and he continues to need DDAVP treatment, currently at 12 months of follow-up. The resolution of the neurohypophysial mass was compatible with the diagnosis of pituitary sarcoidosis and this precluded the need for a transsphenoidal biopsy or surgery.

Topics: Adrenal Cortex Hormones; Adult; Deamino Arginine Vasopressin; Diabetes Insipidus; Humans; Lung Diseases; Magnetic Resonance Imaging; Male; Pituitary Diseases; Pituitary Gland, Posterior; Radiography, Thoracic; Sarcoidosis

We present two patients with known sarcoidosis who developed neurosarcoidosis manifested by paranoid psychosis and clinical diabetes insipidus with hypernatremia. Both had gadolinium enhanced magnetic resonance imaging which demonstrated leptomeningeal and hypothalamic enhancement. Both had elevated protein and a lymphocytosis in their cerebrospinal fluid, which improved after corticosteroid therapy. The patients improved clinically with this therapy as well. We suggest that new onset psychosis in a sarcoid patient, particularly with symptoms of hypothalamic/pituitary involvement, should be evaluated for neurosarcoidosis with an MRI and CSF examination. If the results are consistent with neurosarcoidosis, the patient should be treated promptly with corticosteroids.

Topics: Adult; Benzothiadiazines; Deamino Arginine Vasopressin; Diabetes Insipidus; Diuretics; Female; Humans; Hypernatremia; Magnetic Resonance Imaging; Male; Nervous System Diseases; Paranoid Disorders; Sarcoidosis; Sodium Chloride Symporter Inhibitors

Topics: Adult; Biopsy; Deamino Arginine Vasopressin; Diabetes Insipidus; Female; Glucocorticoids; Humans; Lymph Nodes; Methylprednisolone; Nervous System Diseases; Sarcoidosis