{"id":"5f9e16af-390b-4d0e-be8e-047810fd0238","slug":"enlargement-of-langerhans-cell-histiocytosis-of-the-hypothalamus-with-progression-into-the-basal-ganglia-and-white-matter","title":"Enlargement of Langerhans cell histiocytosis of the hypothalamus with progression into the basal ganglia and white matter","authors":["Sho Tamai","Megumi Ueno","Yasuhiko Hayashi","Yasuo Sasagawa","Takuya Watanabe","Ken-ichi Murakami","Mitsutoshi Nakada","Yutaka Hayashi"],"abstract":"Background: Langerhans cell histiocytosis (LCH) is a rare disease that may affect the central nervous system; it is caused by dendritic cell proliferation, and typically occurs in children. LCH frequently appears in the pituitary stalk and rarely results in multiple enhanced lesions in the brain parenchyma. Case Description: We present a case of a 40-year-old woman who deveolped panhypopituitarism and central diabetes insipidus in the postpartum period requiring hormone replacement therapy. At first, magnetic resonance imaging only revealed thickening of the pituitary stalk; while 6 months later, a single enhanced mass lesion was detected in the hypothalamus. Another 5 months later, the lesion had enlarged with appearance of multiple, enhanced satellite lesions in the basal ganglia and white matter. The patient underwent successful craniotomy to obtain a biopsy sample; LCH of the hypothalamus was definitively diagnosis by histopathological examination. Steroids were administrated and resulted in significant reduction of all lesions. Conclusions: Definitive histopathological diagnosis and subsequent appropriate therapy, such as steroid administration, are required when LCH lesions in the hypothalamus become progressively enlarged and new lesions appear in the brain parenchyma.","thumbnailUrl":"https://sni-digital-videos.s3.amazonaws.com/articles/5f9e16af-390b-4d0e-be8e-047810fd0238/featured/hero-1782420790566.png","publishDate":"2018-10-03T00:00:00.000Z","doi":"10.4103/sni.sni_229_18","categories":["Unique Case Observations","Case Report"],"fullTextUrl":"http://surgicalneurologyint.com/wp-content/uploads/2018/10/9023/SNI-9-197.pdf"}