Oliver Rompel1, Rolf Buslei2, Matthias Hammon3, Helmuth-Günther Dörr4, Martin Chada4, Guido Nikkhah5, Michael Uder3, Regina Trollmann4. 1. Department of Radiology, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany. Electronic address: oliver.rompel@uk-erlangen.de. 2. Institute of Neuropathology, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany. 3. Department of Radiology, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany. 4. Department of Pediatrics, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany. 5. Department of Neurosurgery, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany.
Abstract
BACKGROUND: 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. PATIENT DESCRIPTION: 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. CONCLUSIONS: 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.
BACKGROUND: 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. PATIENT DESCRIPTION: 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. CONCLUSIONS: 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.