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18F-FDG PET in neurodegenerative Langerhans cell histiocytosis

Results and potential interest for an early diagnosis of the disease

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Abstract

Introduction

The so called "neurodegenerative Langerhans cell histiocytosis" (ND-LCH) is a rare and severe complication of LCH presenting as a progressive cerebellar ataxia associated with pyramidal tract signs, and cognitive impairment. MRI is the gold standard to investigate CNS lesions of ND-LCH but little is known about functional changes observed in this disease.

Objectives

To search for CNS metabolic changes in NDLCH.

Methods

Seven patients suffering from ND-LCH were investigated by 18F-FDG PET in this prospective study and compared with 21 healthy controls.

Results

ND-LCH patients demonstrated recurrent abnormalities including bilateral hypometabolism in the cerebellum, the basal ganglia (caudate nuclei), frontal cortex and, bilateral, a relatively increased metabolism in the amygdalae (p < 0.001). Functional changes in these anatomical regions may be detected in the absence of any apparent lesion on MRI.

Conclusions

ND-LCH demonstrates a recurrent 18F-FDG PET metabolic signature. Our results suggest that 18F-FDG PET might be a useful tool for an early diagnosis of ND-LCH before neuroradiologic abnormalities appear.

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Authors and Affiliations

  1. Service Hospitalier Frédéric Joliot, DRM/DSV, CEA, 4, place du Général Leclerc, 91406, Orsay, France

    M.-J. Ribeiro MD, PhD

  2. Service de Neurologie Mazarin, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, UPMC, Paris, France

    A. Idbaih MD, PhD & K. Hoang-Xuan MD, PhD

  3. Unité d'Hématologie et d'Oncologie, Pédiatrique, CHU, Nantes, France

    C. Thomas MD

  4. URA-CNRS-CEA 2210, Service Hospitalier Frédéric Joliot, Orsay, France

    P. Remy MD, PhD

  5. Département de Neurosciences, AP-HP, CHU Henri Mondor, Faculté de Médecine Paris XII, Créteil, France

    P. Remy MD, PhD

  6. Service de Neuroradiologie, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, UPM, Paris, France

    N. Martin-Duverneuil MD

  7. Urgences Cérébro-Vasculaires, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Paris, France

    Y. Samson MD

  8. Service d'Hématologie et d'Oncologie, Pédiatrique, AP-HP, Hôpital d'Enfants Armand Trousseau, Paris, France

    J. Donadieu MD, PhD

Authors
  1. M.-J. Ribeiro MD, PhD
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  2. A. Idbaih MD, PhD
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  3. C. Thomas MD
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  4. P. Remy MD, PhD
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  5. N. Martin-Duverneuil MD
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  6. Y. Samson MD
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  7. J. Donadieu MD, PhD
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  8. K. Hoang-Xuan MD, PhD
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Corresponding author

Correspondence to M.-J. Ribeiro MD, PhD.

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Ribeiro, MJ., Idbaih, A., Thomas, C. et al. 18F-FDG PET in neurodegenerative Langerhans cell histiocytosis. J Neurol 255, 575–580 (2008). https://doi.org/10.1007/s00415-008-0751-8

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  • DOI: https://doi.org/10.1007/s00415-008-0751-8

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