spontaneous Remission of Diabetes Insipidus Due to CNS Sarcoidosis

Abstract

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.

Case Details

Disease Location

Cns/ pituitary gland and hypophyseal stalk

Personal Characteristics

58 -year-old female, natural birthed one son with no complications, menopause occured at 56 -year-old originally referred to treat polydipsia and polyuria which was present a months before admission, urine volume increased to 6000ml/day. She preferred cold water and lost 5kg in a months no major family histories, no abnormal findings in routine health check 149.5cm, 48.8kg, bmi 21.8kg/m^2

Clinical Characteristics

Cns sarcoidosis diagnosed by findings such as bhl, uveitis, and noncaseating epithelioid granulomas in right gluteal nodule diagnosed with central diabetes insipidus (cdi) due to cns sarcoidosis and cutaneous biopsy. Acute development of polydipsia and polyuria (original reason for treatment) swelling of pituitary gland and hypophyseal stalk observed using MRI, gadolinium enhanced both, the posterior pituitary gland's high signal on t1 had disappeared uveitis found via ophthalmologic exam, and bilateral hilar lymphadenopathy (bhl) were observed midly throughout using chest CT subthalamic/hypothalamic dysfunction serum na level increased to 150 mmol/l, serum osmolality rose to 308 mosm/kgh2o, low urinary osmolality of 101 mosm/kgh2o, urinary specific gravity was below 1.005, concentration of antiduretic hormones (adh) was subnormal 0.32 pg/ml. Three hours after ddavp admin, serum osmolality normalized to 286 mosm/kgh2o, urinary specific gravity increased to 1.018. Serum soluble IL-@ receptor level was almost normal, high value of prolactin (prl) = 42.0 ng/ml underwent x-ray, gastric intestinal fiber exam, gastric biopsy specimens, abdominal CT, electrocardiogram, ultrasonic cardiogram, and ambulatory electrocardiogram; all normal results in trh test, delay of thyearsotropin (tsh) and prl responses were observed. Peak level of prl was normal; in grh, crh and gnrh tests, dalayed responses and peak values observed suggest the subthalamic dysfunction {f-18]-2-fluoro-2-deoxy-d-glucose (fdg) tracer accumulated in bilateral hilar lymph nodes and right gluteal cutaneous nodule during PET scan, accumulation in pituitary was obscure noncaseating epithelioid granulomas found in right gluteal biopsy, epithelioid histiocytes and absence of necrosis were notable leading to sarcoidosis diagnosis, however not ruling out lymphocytic hypophysitis

Remission Characteristics

After ddavp treatment, serum sodium level was 138 mmol/l, urinary osmolality was 216 mosm/kgh2o, daily urinary volume was 2500 ml. 14 days after admission, MRI found the pituitary gland and hypophyseal stalk swelling disappeared, although high signal in posterior pituitary gland in t1 was still obscure. Uveitis was cleared, pulmonary/cutaneuous sarcoidosis was marginal 4 weeks after admission, serum ace value was elevated to 25.1 u/ml, serum prl value decreased to 33.1 ng/ml 16 weeks later, serum ace value decreased to 22.7 u/ml, serum prl value decreased to 28.4ng/ml serum ace level decreased with remission of cdi, elevated prl decreased during recovery of adh functions...suggestion that spontaneous remission of cdi was due to remission of cns sarcoidosis, although remission of the sarcoidosis is still unknown

Treatment & Mechanisms

Proposed Remission Mechanisms

Sarcoidosis elucidated uveitis

Clinical Treatment

Intranasal 2.5 µm of d-deamino vasopression (ddavp) pituitary functions were evaluationuated using 100µg of corighticotropin-releasing hormone (crh) via IV, 100 µg of growth hormones-releasing hormones (grh) via IV, 500 µg of thyearsotropin-releasing hormones (trh) via im, and 100 µg of gonadtropin-releasing hormones (gnrh) via im. Biopsy of right gluteal cutaneous nodule was performed to treat cdi, ddavp started at 5 µg and adjusted while urine volume was monitored every 8 hours to keep daily volume of 1500-3000 ml. 2 weeks after admission, without admin of steroids, required ddavp decreased to 2.5 µg/day. 16 weeks later, ddavp was sustained, but was approximately 0.3 µg/day then withdrawn. Steroid eye lotion

Non-Clinical Treatment

Systematic water intake