Rompel Oliver, Buslei Rolf, Hammon Matthias, Dörr Helmuth-Günther, Chada Martin, Nikkhah Guido, Uder Michael, Trollmann Regina
Department of Radiology, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany.
Institute of Neuropathology, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany.
Pediatr Neurol. 2016 Sep;62:62-5. doi: 10.1016/j.pediatrneurol.2016.05.006. Epub 2016 May 11.
Langerhans cell histiocytosis is a rare disease of the monocyte-macrophage system. Abnormalities of the hypothalamic-pituitary region are common in individuals with central nervous system involvement.
This six-year-old boy developed rapidly progressive aggressive behavior, central diabetes insipidus, and repeated complex partial seizures. Magnetic resonance imaging revealed a diffuse leukoencephalopathy-like pattern and numerous infratentorial and supratentorial granulomatous nodules in the brain parenchyma along with infundibular and hypothalamic mass lesions. Stereotactic serial biopsies enabled histopathologic and immunohistochemical diagnosis of Langerhans cell histiocytosis.
Similar MRI findings have rarely been described in the literature. These findings represent part of the broad neuroradiological spectrum of Langerhans cell histiocytosis of the nervous system in children.
朗格汉斯细胞组织细胞增多症是一种罕见的单核巨噬细胞系统疾病。下丘脑 - 垂体区域异常在有中枢神经系统受累的个体中很常见。
这个六岁男孩出现了快速进展的攻击性行为、中枢性尿崩症和反复的复杂部分性癫痫发作。磁共振成像显示弥漫性白质脑病样模式，脑实质内有许多幕下和幕上肉芽肿性结节，同时伴有漏斗部和下丘脑肿块病变。立体定向系列活检实现了朗格汉斯细胞组织细胞增多症的组织病理学和免疫组化诊断。
文献中很少描述类似的MRI表现。这些表现代表了儿童神经系统朗格汉斯细胞组织细胞增多症广泛神经放射学谱的一部分。
