A 6-year-old boy with lymphocytic infundibulo-neurohypophysitis

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  • リンパ球性漏斗下垂体後葉炎の６ 歳児例

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Abstract

Lymphocytic infundibulo-neurohypophysitis（LINH） is a rare disease that exhibits central diabetes insipidus（CDI） through autoimmune mechanisms. Importantly, LINH should be always differentiated from neoplasia such as Langerhans cell histiocytosis and germinoma. A 6-year-old boy presented with polyuria and polydipsia. Endocrinological investigations proved CDI. Hypophysis MRI showed enlargement of the stalk and diffuse Gadrinium-enhancement, suggesting the diagnosis of LINH. Hypophysis biopsy that is indispensable for the definitive diagnosis of LINH was not performed in our patient because of its invasiveness. However, the follow up MRI revealed the improvement of the stalk swelling in the time course, and this finding not only supported the diagnosis but also ruled out neoplastic diseases. He was treated with DDAVP alone, and his CDI promptly dissolved. Since he lacked headache and visual disorders, glucocorticoid treatment was not needed. Recently, serum anti-Rabphylin-3A antibody has been reported as a useful diagnostic tool for LINH. Our patient also possessed this antibody in the serum, strongly supporting the diagnosis.

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