Langerhans cell histiocytosis presenting as a rapidly evolving frontotemporal syndrome
- 143 Downloads
Langerhans cell histiocytosis (LCH) is a rare disorder in adults which usually manifests with involvement of multiple organ systems, including the central nervous system. We describe an unusual case of biopsy-proven LCH presenting with frontotemporal-dominant cognitive impairment with hypothalamic involvement, along with multisystem disease. We propose that the dementia was probably an immune-mediated process triggered by LCH which responded dramatically to high-dose steroids.
KeywordsLangerhans cell histiocytosis Hypothalamus Cognition Dementia
Compliance with ethical standards
Conflict of interest
The authors declare that they have no conflict of interest.
- 2.Allen CE, Li L, Peters TL et al (2010) Cell-specific gene expression in Langerhans cell histiocytosis lesions reveals a distinct profile compared to epidermal Langerhans cells. J Immunol Baltim Md 1950 184(8):4557–4567Google Scholar
- 5.Grois N, Pötschger U, Prosch H, Minkov M, Arico M, Braier J, Henter JI, Janka-Schaub G, Ladisch S, Ritter J, Steiner M, Unger E, Gadner H, for the DALHX- and LCH I and II Study Committee (2006) Risk factors for diabetes insipidus in langerhans cell histiocytosis. Pediatr Blood Cancer 46(2):228–233CrossRefPubMedGoogle Scholar
- 8.Allen CE, Flores R, Rauch R, Dauser R, Murray JC, Puccetti D, Hsu DA, Sondel P, Hetherington M, Goldman S, McClain KL (2010) Neurodegenerative central nervous system Langerhans cell histiocytosis and coincident hydrocephalus: treated with vincristine/cytosine Arabinoside. Pediatr Blood Cancer 54(3):416–423CrossRefPubMedPubMedCentralGoogle Scholar
- 10.Yeh EA, Greenberg J, Abla O et al (2018) Evaluation and treatment of Langerhans cell histiocytosis patients with central nervous system abnormalities: current views and new vistas. Pediatr Blood Cancer 65(1). https://doi.org/10.1002/pbc.26784