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. 2023 May;44(5):505–510. doi: 10.3174/ajnr.A7832

Demographics and most frequently observed CNS imaging features of ECD, RDD, and LCH

ECD LCH RDD
Demographics M>>F, 50–70 yrs M > F, 1–3 yrs M > F, young adults
Presentation Commonly asymptomatic; CNS involvement leads to diabetes insipidus, pontocerebellar symptoms, and seizures Nonspecific, including lethargy, fever, bone pain, diabetes insipidus Lymphadenopathy, night sweats, weight loss
CNS imaging Patchy enhancement and T2 hyperintensity in the posterior fossa; enlargement and enhancement of the pituitary stalk; loss of posterior pituitary T1 signal; intra- and extra medullary spinal lesions Enlargement and enhancement of the pituitary stalk; loss of posterior pituitary T1 signal; neurodegenerative changes in the basal ganglia, pons, and dentate nuclei; parenchymal atrophy Enlarged cervical lymph nodes; enhancing dural-based masses that mimic meningiomas

Note:—M indicates male; F, female.