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Langerhans cell histiocytosis presenting as a rapidly evolving frontotemporal syndrome

  • Divyani Garg
  • Radhakrishna Pedapati
  • Tripti Nakra
  • Rajesh Kumar SinghEmail author
  • Anuj Prabhakar
  • Deepa Dash
  • Rohit Bhatia
  • Manjari Tripathi
Brief Communication
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Abstract

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.

Keywords

Langerhans cell histiocytosis Hypothalamus Cognition Dementia 

Notes

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

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Copyright information

© Fondazione Società Italiana di Neurologia 2019

Authors and Affiliations

  • Divyani Garg
    • 1
  • Radhakrishna Pedapati
    • 1
  • Tripti Nakra
    • 2
  • Rajesh Kumar Singh
    • 1
    Email author
  • Anuj Prabhakar
    • 3
  • Deepa Dash
    • 1
  • Rohit Bhatia
    • 1
  • Manjari Tripathi
    • 1
  1. 1.Department of NeurologyAll India Institute of Medical SciencesNew DelhiIndia
  2. 2.Department of NeuropathologyAll India Institute of Medical SciencesNew DelhiIndia
  3. 3.Department of NeuroradiologyAll India Institute of Medical SciencesNew DelhiIndia

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