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Case Report

Langerhans Cell Histiocytosis with Initial Central Nervous System Presentation as a Mimic of Neurosarcoidosis

by
Cecilia Friedrichs-Maeder
1,*,
Christoph Friedli
1,
Stefan Kuchen
2,
Roland Wiest
3,
Ekkehard Hewer
4,
Alicia Rovó
5 and
Andrew Chan
1
1
Department of Neurology, Bern University Hospital, University of Bern, 3012 Bern, Switzerland
2
Department of Rheumatology, Immunology and Allergology, Bern University Hospital, University of Bern, 3012 Bern, Switzerland
3
Department of Neuroradiology, Bern University Hospital, University of Bern, 3012 Bern, Switzerland
4
Institute of Pathology, University of Bern, 3012 Bern, Switzerland
5
Department of Hematology, Bern University Hospital, University of Bern, 3012 Bern, Switzerland
*
Author to whom correspondence should be addressed.
Clin. Transl. Neurosci. 2019, 3(2), 15; https://doi.org/10.1177/2514183x19875064
Submission received: 12 March 2019 / Accepted: 8 July 2019 / Published: 17 September 2019

Abstract

We report the case of a 58-year-old Caucasian woman who presented with a subacute cerebellar syndrome accompanied by disturbance of the hypothalamic–pituitary axis and was diagnosed with isolated neurosarcoidosis based on radiological findings including typically located cerebral lesions (infratentorial and pituitary stalk). Due to persistent clinical and radiological disease activity during several years despite escalation of immunosuppressive treatment, the diagnosis was reevaluated, and a transsphenoidal biopsy of a lesion at the pituitary stalk was performed revealing Langerhans cell histiocytosis. In this case, we discuss the different steps leading to the diagnostic error, as well as the presence of red flags, which should have led to an earlier diagnostic reevaluation.
Keywords: Langerhans cell histiocytosis; neurosarcoidosis; MRI; central nervous system; diagnostic Langerhans cell histiocytosis; neurosarcoidosis; MRI; central nervous system; diagnostic

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MDPI and ACS Style

Friedrichs-Maeder, C.; Friedli, C.; Kuchen, S.; Wiest, R.; Hewer, E.; Rovó, A.; Chan, A. Langerhans Cell Histiocytosis with Initial Central Nervous System Presentation as a Mimic of Neurosarcoidosis. Clin. Transl. Neurosci. 2019, 3, 15. https://doi.org/10.1177/2514183x19875064

AMA Style

Friedrichs-Maeder C, Friedli C, Kuchen S, Wiest R, Hewer E, Rovó A, Chan A. Langerhans Cell Histiocytosis with Initial Central Nervous System Presentation as a Mimic of Neurosarcoidosis. Clinical and Translational Neuroscience. 2019; 3(2):15. https://doi.org/10.1177/2514183x19875064

Chicago/Turabian Style

Friedrichs-Maeder, Cecilia, Christoph Friedli, Stefan Kuchen, Roland Wiest, Ekkehard Hewer, Alicia Rovó, and Andrew Chan. 2019. "Langerhans Cell Histiocytosis with Initial Central Nervous System Presentation as a Mimic of Neurosarcoidosis" Clinical and Translational Neuroscience 3, no. 2: 15. https://doi.org/10.1177/2514183x19875064

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