Diffuse Encephalopathy Associated with Isolated Cerebral Langerhans Cell Histiocytosis

Rompel O, Buslei R, Hammon M, Dörr HG, Chada M, Nikkhah G, Uder M, Trollmann R (2016)

Publication Type: Journal article

Publication year: 2016

Journal

Pediatric Neurology Elsevier

Book Volume: 62

Pages Range: 62-5

DOI: 10.1016/j.pediatrneurol.2016.05.006

Abstract

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.

Authors with CRIS profile

Matthias Hammon Institute of Radiology Helmuth-Günther Dörr Professur für Kinderheilkunde mit dem Schwerpunkt Kinder-Endokrinologie und Diabetologie Michael Uder Lehrstuhl für Diagnostische Radiologie Regina Trollmann Professur für Kinder- u. Jugendmedizin Schwerpunkt Neuropädiatrie

How to cite

APA:

Rompel, O., Buslei, R., Hammon, M., Dörr, H.-G., Chada, M., Nikkhah, G.,... Trollmann, R. (2016). Diffuse Encephalopathy Associated with Isolated Cerebral Langerhans Cell Histiocytosis. Pediatric Neurology, 62, 62-5. https://doi.org/10.1016/j.pediatrneurol.2016.05.006

MLA:

Rompel, Oliver, et al. "Diffuse Encephalopathy Associated with Isolated Cerebral Langerhans Cell Histiocytosis." Pediatric Neurology 62 (2016): 62-5.

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