Evolution of childhood central diabetes insipidus into panhypopituitarism with a large hypothalamic mass: is 'lymphocytic infundibuloneurohypophysitis' in children a different entity?

Abstract:

:We report on a 15-year-old girl who had presented with acute onset central diabetes insipidus at the age of 8 years; this was followed by growth failure due to acquired growth hormone deficiency. Initial magnetic resonance imaging showed a uniformly enlarged pituitary stalk and absence of posterior pituitary hyperintensity. Frequent patient examination and magnetic resonance imaging gave unchanged results until after 5 years a large hypothalamic mass and panhypopituitarism were found. Dynamic magnetic resonance imaging documented hypothalamic-pituitary vasculopathy. Histopathological examination revealed perivascular inflammatory lymphoplasmic infiltrates with no granulomatosis or necrosis and negative staining for S-100 protein, suggesting autoimmune inflammatory disease (lymphocytic infundibuloneurohypophysitis?). The response to glucocorticoid pulses (30 mg/kg per day for 3 days i.v.) was favorable. the hypothalamic mass being halved and partial anterior pituitary function recovery maintained for 2 years after the start of treatment. We suggest that long-term surveillance is needed for isolated and chronic thickening of the pituitary stalk and that dynamic magnetic resonance imaging can contribute to the demonstration of hypothalamic-pituitary vascular impairment associated with local vasculitis.

journal_name

Eur J Endocrinol

authors

Maghnie M,Genovese E,Sommaruga MG,Aricò M,Locatelli D,Arbustini E,Pezzotta S,Severi F

doi

10.1530/eje.0.1390635

subject

Has Abstract

pub_date

1998-12-01 00:00:00

pages

635-40

issue

6

eissn

0804-4643

issn

1479-683X

journal_volume

139

pub_type

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