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  • Title: 18F-FDG PET in neurodegenerative Langerhans cell histiocytosis : results and potential interest for an early diagnosis of the disease.
    Author: Ribeiro MJ, Idbaih A, Thomas C, Remy P, Martin-Duverneuil N, Samson Y, Donadieu J, Hoang-Xuan K.
    Journal: J Neurol; 2008 Apr; 255(4):575-80. PubMed ID: 18227990.
    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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