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.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Ribeiro MJ,Idbaih A,Thomas C,Remy P,Martin-Duverneuil N,Samson Y,Donadieu J,Hoang-Xuan Kdoi
10.1007/s00415-008-0751-8subject
Has Abstractpub_date
2008-04-01 00:00:00pages
575-80issue
4eissn
0340-5354issn
1432-1459journal_volume
255pub_type
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