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Langerhans' cell histiocytosis and the nervous system

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Summary

We report two cases of Langerhans' cell histiocytosis with unusual central nervous system (CNS) involvement. The first patient had behavioural disturbances, memory loss and diabetes insipidus. His response to a range of treatments was poor. The second patient presented with seizures and headaches suggestive of raised intracranial pressure. Etoposide (VP16) chemotherapy led to a dramatic clinical and radiological improvement. The various CNS manifestations of Langerhans' cell histiocytosis and their management are discussed.

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Authors and Affiliations

  1. Neurology Unit, Department of Medicine, Hammersmith Hospital, Du Cane Road, W12 OHS, London, UK

    David J. Burn, John D. G. Watson, Nigel J. Legg & Richard S. J. Frackowiak

  2. Department of Diagnostic Radiology, Hammersmith Hospital, Du Cane Road, W12 OHS, London, UK

    Mary Roddie

  3. Dermatology Unit, Department of Medicine, Hammersmith Hospital, Du Cane Road, W12 OHS, London, UK

    Anthony C. Chu

  4. MRC Cyclotron Unit, Hammersmith Hospital, Du Cane Road, W12 OHS, London, UK

    David J. Burn & Richard S. J. Frackowiak

  5. National Hospital for Neurology and Neurosurgery, Queen Square, London, UK

    Richard S. J. Frackowiak

Authors
  1. David J. Burn
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  2. John D. G. Watson
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  3. Mary Roddie
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  4. Anthony C. Chu
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  5. Nigel J. Legg
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  6. Richard S. J. Frackowiak
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Burn, D.J., Watson, J.D.G., Roddie, M. et al. Langerhans' cell histiocytosis and the nervous system. J Neurol 239, 345–350 (1992). https://doi.org/10.1007/BF00867593

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  • DOI: https://doi.org/10.1007/BF00867593

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