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 autoimmuneinflammatory 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.

Diabetes Insipidus – Diagnosis and Management

“…Autoimmune polyendocrinopathy and CDI associated with an MRI picture of a thickened PS suggest that patients with CDI and a thickened PS may share a common etiology [1,25,26]. While there is a temporal relationship between a viral infection (trigger) and the onset of CDI in about a quarter of patients with idiopathic CDI [1], anterior pituitary (AP) involvement in the course of idiopathic CDI is highly suggestive of an autoimmune neurohypophyseal basis and fits well with the bioptic demonstration of the lymphocytic infiltration of the PS [27]. This hypothesis is strengthened by the fact that the pituitary gland is susceptible to CD8 T-cell-mediated autoimmunity, triggered by a cell-specific model autoantigen [28], as well as to the development of autoimmune hypophysitis through the immunization of female SJL/J mice with mouse pituitary extracts [29].…”

Diabetes insipidus

“…The known causes of these lesions include germinoma [9, 10, 11, 12]or craniopharyngioma [13]; Langerhans cell histiocytosis [13, 14, 15]; local inflammatory, autoimmune or vascular diseases [16, 17, 18]; and trauma from surgery or accident [19]. Midline cerebral and cranial malformations [9, 20]are another possible cause of central diabetes insipidus.…”

“…The sensitivity of this diagnostic technique in normal controls is increased by integration with T2-weighted images; an age decline of 1% in the presence of this signal has been reported [27]. A thickened infundibulum or pituitary stalk (or both), although not specific, suggests the presence of infiltration in the course of various diseases [9, 10, 13, 16, 17, 28, 29]. There is currently an increased tendency to rely on MRI findings for diagnosis, which has lessened the need for any invasive procedures.…”

Lymphocytic hypophysitis: a rare or underestimated disease?

“…The affected patients show distinct clinical and radiological features including characteristic diabetes insipidus (11). In this context, we consider lymphocytic hypophysitis and lymphocytic adenohypophysitis as one and the same, but indicate lymphocytic-infundibulo-neurohypophysitis as an infiltrative and inflammatory process involving the posterior lobe of the pituitary and the infundibulum (12). Finally, we consider lymphocytic-infundibulohypophysitis to be an inflammatory process involving the anterior and posterior hypophysis and the infundibulum (13).…”